Aetiology. Ovingdean Hall School Phonics for deaf children Developing higher level thinking skills

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1 British Association of Teachers of the Deaf MAGAZINE September 2010 ISSN Aetiology Ovingdean Hall School Phonics for deaf children Developing higher level thinking skills Join BATOD to get THE Magazine for professionals working with deaf children

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3 From your editor This edition of the Magazine looks at a range of issues related to the aetiology of deafness. It contains articles about the complex range of specific conditions and syndromes which involve deafness and does so from a variety of perspectives. It provides an overview of the subject but also looks in detail at specific syndromes and conditions and includes articles written from the perspective of parents and deaf people themselves. We also look at the crucial importance of deaf children and young people being aware of the reason behind their own deafness and we tackle the topic of genetic counselling. Over recent years, partly due to improvements in medical science, many schools for the deaf have seen an increasingly complex range of children and young people who have come from a wide range of aetiological backgrounds. The Magazine contains articles illustrating this range and looking at the implications on the education provided by these schools and the training required to ensure that the children and young people are optimally supported. As usual there are also several articles on other topics which I am sure will also be of interest to readers. Some of these were unsolicited and such articles are always welcome. If there is a subject on which you are keen to write an article to share your experiences with colleagues, or if you wish to respond to an article in this issue, please do not hesitate to submit something. Although we can t guarantee to include every article received on the grounds of space alone we are very keen to be as flexible as possible, so do send us your ideas or articles, and not just on the theme of any particular edition. Forthcoming topics November Post-16 issues January 2011 TAs and support workers March 2011 Literacy and maths May 2011 Conference edition Stronger together September 2011 Assistive technology Contents Aetiology Searching for answers 4 Exploring genetics 7 Meningitis the facts 11 Congenital cytomegalovirus 12 CMV a parent s perspective 14 Who are the tests really for? 16 Living with TCS 18 Complex provision 20 The Usher child 22 At Donaldson s 24 Training needs 25 The road to Suryapur 26 General features Cochlear implants not always enough? 30 Higher level thinking 32 VI facts and figures 35 Phonics guidance 38 News from NatSIP 39 The end of an era 40 Vocab test results 41 Sound equipment management 42 Meeting social care needs 44 Testing the network 47 Regulars Reviews 52 ICT news 54 This and that 56 Abbreviations and acronyms 58 Calendar meetings and training 60 Association business Stronger together 3 Drafting ToD competencies in Europe 48 BATOD was there representing you 50 What went on at NEC on 12 June Change of address notification form 53 Subscription rates 2010/11 59 Officers of Nations and Regions inside back cover Magazine editor Need to contact BATOD about other matters? Talk to Executive Officer Paul Simpson exec@batod.org.uk answerphone/fax: Cover picture shows Natalie Burton, who has congenital cytomegalovirus. For information on advertising rates see

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5 Stronger together Association business In a period of uncertainty for the education sector, BATOD is working hard to strengthen partnerships and support everyone involved in the education of deaf children, as Gary Anderson reports In March I said that I did not have a crystal ball to predict what life would be like after 6 May. I certainly did not expect the new political landscape to look quite like it does now, and, when set against the serious financial backdrop, one thing we can guarantee is a radically different education agenda in England over the next few years. Although there is more detail to emerge, already the direction of the new Coalition Government has been set. Like you, I am anxious to find out how that detail unfolds and how the policy will be translated into practice. The Government intends to drive improvement across the whole education system and draw on the most successful practices in other countries. There is already a feeling of pace as the new broom sweeps clean! The new Secretary of State for Education Michael Gove has renamed the department with the focus on education and written to outstanding schools regarding the opportunity to become academies within the scope of the first education bill. There is a great deal of uncertainty at this time about the implications for sensory support services and the provision they make in relation to academies to ensure that pupils access needs are met. Below are the main elements of the proposed second education bill: To provide schools with the freedoms to deliver an excellent education in the way they see fit. To reform Ofsted and other accountability frameworks to ensure that headteachers are held properly accountable for the core educational goals of attainment and closing the gap between rich and poor. To introduce a slimmer curriculum giving more space for teachers to decide how to teach. To introduce a Year 6 reading test to make sure that young children are learning and to identify problems early. To give teachers and headteachers the powers to improve behaviour and tackle bullying. To ensure that standards across the education sector rise through the creation of more academies and to give more freedom to headteachers and teachers. To ensure that money follows pupils and to introduce a pupil premium so that more money follows the poorest pupils. for the next two years resonates clearly as we build on our partnership working with VIEW, NatSIP and others to maintain our central role and influence in the education of deaf children. At the Heads of Sensory Support Services meeting in Birmingham on 18 June there was a public commitment made for BATOD and VIEW to develop more formal working links to support better our common agendas. Lindsey Rousseau, Nicky Ereaut and I have a meeting planned with Hardip Begol at the Department for Education when we hope to find out more about the Government s thinking. While the new Government has been forming, BATOD has continued its work steadily. Below is a brief summary of the current activities taking place: Several NEC members are on different NatSIP workstreams, and Nicky Ereaut has led on the exciting outcomes pilot. CRIDE is moving forward. Paul Simpson, as National Executive Officer and FEAPDA President, is a key partner in the Leonardo project looking at pan-europe core competencies for ToDs (see page 48). Maureen Jefferson, Derek Heppenstall and Paul have been working with Ofqual regarding deaf pupils needs in relation to exams. Ann Underwood has been supporting the successful bid led by Dr Barry Wright for a research programme on child mental health and deafness. Ann and the team are planning the conference for 2011 in the Newcastle area, when the first Peter Preston Award will be made. Ann has been working hard with the Trustees of the BATOD Foundation whose focus is classroom acoustics. Andrea Baker is working closely with Manchester University regarding mentoring. The Steering Group met with Lorraine Petersen, Chief Executive Officer of nasen, in May to share information and discuss future joint opportunities. I have received invitations to attend regional conferences in the South West, North and Wales. I look forward to getting out and about around the regions and nations, meeting members and listening to your views so that we can respond to your needs. We wait to see the impact that the new legislation will have on all children and young people with SEN and disabilities. However, my theme of stronger together BATOD Magazine September

6 Aetiology Searching for answers Medical investigation to look for the cause of permanent childhood deafness should be offered to all families, irrespective of the degree of deafness, says Dr Breege Mac Ardle Over the past decade, there has been a significant change in the range of medical investigations that are now offered to children with permanent deafness, and their families. These changes have followed the introduction and implementation of three sets of best practice guidelines (from the British Association of Audiovestibular Physicians, BAAP, and the British Association of Paediatricians in Audiology, BAPA) which cover bilateral severe to profound, bilateral mild to moderate and unilateral permanent deafness in childhood. Advances in the field of genetics and increasing knowledge of the contribution of congenital cytomegalovirus infection to the cause of permanent deafness have had a major impact on how we practise. Parental input into the training of doctors who perform medical investigations has led to an improved understanding of informed parental consent and making joint decisions with families and, where appropriate, the child about which investigations will be performed and what their limitations are. The Newborn Hearing Screening Programme (NHSP) has also introduced a new challenge to clinicians and families as there is a need to initiate and complete medical investigation at a very young age, particularly for the small group of children who receive cochlear implants at around 12 months old. In addition, the NHSP quality assessment process has set a standard for local implementation of medical investigation of deafness. Each NHSP site should have an identified medical lead who is responsible for the implementation of medical investigation of all children with permanent childhood deafness. Why perform investigations? Most families want to know why their child is deaf, whether there is a possibility of further children being deaf and whether the deafness will progress over time and, in addition, if there are any medical or surgical treatments available. In order to give an informed response to these questions, the medical specialist will need to describe current understanding of the causes of deafness, and explain what each investigation is for and what the yield is and that in some cases interpretation of testing may create uncertainty, for example interpretation of electroretinograms in the diagnosis of possible Usher syndrome. The medical specialist must know the cause of deafness in order to detect and treat children who have other associated pathology. Causes of deafness At least 50% of all permanent childhood deafness is genetic in origin; in this group about 30% will have a syndrome and there are 400 syndromes associated with deafness. The commonest gene mutation found in deaf children in the UK occurs in the Connexin 26 del G mutation and children with this homozygous mutation tend to have severe to profound hearing loss, although the loss can be mild and the pattern of deafness can be different in deaf siblings. The commonest genetic pattern of inheritance is recessive, with a one in four chance of having a further child with deafness, followed by dominant inheritance. In rarer cases some deafness is linked to the sex chromosomes and some is caused by mitochondrial mutations where the gene mutation is passed on by females. It is important to remember that up to 40% of deaf children have an additional health or developmental problem and 20% have more than two problems. In 25% of children the deafness is caused by environmental factors; for example in the UK at present, congenital cytomegalovirus (ccmv) infection is the commonest cause of acquired hearing loss and accounts for up to 30% of childhood deafness. In ccmv, the deafness can be unilateral, bilateral, of any degree, and can progress at any age usually up to the age of six years. Only 13% of infants have symptoms of ccmv present at birth, the remaining 70% of children with ccmv look normal and are healthy. Early detection of ccmv is essential, as babies identified at less than 30 days of age can benefit from intravenous anti-viral drug treatment which modifies the damage caused by the virus, both to the brain and inner ear. It is likely that the oral anti-viral treatment for children over 30 days of age with asymptomatic ccmv deafness will be available in the future. Early detection of ccmv relies on examination of urine, oral swabs, blood tests on the baby and mother and testing of the neonatal dried blood spot (Guthrie card). The doctor investigating the child must specifically request retrieval of the Guthrie card from the local specialist metabolic laboratory. Sometimes test results may not be conclusive, but eye examination can also be helpful. Other environmental causes include congenital rubella, although this is now rare. A few babies are born each year with this and usually their mothers were born abroad or were not immunised against rubella, or were not rubella immune during the 4 B ATOD Magazine September 2010

7 Aetiology Test Genetic tests Imaging inner ear and brain ECG in profoundly deaf children with significant motor delay Ophthalmology assessment Electroretinography Parental and sibling audiograms Congenital CMV infection Urine examination Possible finding(s) Pathological gene mutations Bilateral dilated vestibular aqueducts Cochlear malformation Thin or absent hearing nerve(s), severe inner ear dysplasia Changes in the white matter of the brain Prolonged QT interval Refraction errors, cataracts, retinopathy Retinal dystrophy, retinitis pigmentosa Define type and degree of deafness Confirm diagnosis Blood and/or protein detected Possible implication(s) for management Accurate genetic counselling Advice about avoidance of ototoxic drugs in children with A1555G mutations which allows preservation of hearing Contact sport avoidance Inform syndrome diagnosis, for example Pendred or Branchio-Oto Renal, CHARGE syndrome Amplification and/or cochlear implantation may not be appropriate this may inform choice about communication mode and educational placement Confirmation of congenital CMV infection and initiation of anti-viral drugs if appropriate Medication to prevent heart arrhythmias, or pacemaker insertion Correction of refraction error, operation for squint or cataract Genetic counselling Input from visual impairment team Confirmation of Usher syndrome, metabolic syndromes, etc Input from visual impairment team Decision about cochlear implant assessment may result Decisions about communication mode and educational placement may result Accurate genetic counselling Specific counselling to parents and carers Consider Alport s disease or syndromes that affect auditory and renal systems pregnancy. Other environmental causes of deafness include prematurity and neonatal intensive care, ototoxic drugs, meningitis, head injury, middle and inner ear infections, tumours of the hearing nerve(s) and noise exposure. In approximately 25% of children no cause for the deafness is found, after aetiological investigation is completed. Medical investigation of permanent deafness Investigation has to be systematic. It involves the lead doctor who has links with medical colleagues in a variety of disciplines. It is time consuming for all involved in order to allow adequate time for parents to understand why the tests are being performed, what the tests may show, and what the results mean. Medical investigation is rarely a one-off exercise. The 25% of children for whom no cause of deafness has been found require regular medical review, particularly as new tests become available. Over time, children want to know about the cause of their deafness and the original investigations require explanation in language appropriate for their age. Deaf children may present with new symptoms which may require a review of their original medical diagnosis and in some cases with further investigation, there is a change to the original diagnosis. The process of investigation begins with a detailed history and full physical examination. It is very important for the doctor to establish if the child has clinical evidence of poor vestibular function, for example very floppy infants with delayed motor milestones, as these children will require investigation to exclude Usher syndrome and Jervell-Lange Nielsen syndrome. It is essential to have audiological certainty about the type and degree of deafness as well as whether the child has auditory neuropathy spectrum disorder. BATOD Magazine September

8 Aetiology There are two levels of investigation: level 1 are must do investigations and these include performing parental and sibling audiograms, an ophthalmology assessment, ccmv testing, blood testing for Connexin mutation and other specific gene mutation tests as indicated by the history and examination. Inner ear imaging is essential, particularly in children with severe to profound and unilateral deafness and should be considered in children with milder degrees of deafness. Electrocardiograms are only required for children with severe to profound deafness with delayed motor milestones. The child s urine should be checked for blood and protein as this may detect rarer kidney disease such as Alport s syndrome. Children with delayed milestones will require vestibular assessment. Some children may have more than one possible cause for their deafness. Level 2 investigations are required when the clinical history and examination suggest that the child may have a metabolic disease, congenital infection or a chromosomal disorder. Some examples of the tests, possible findings and implications for management are shown in the table on page 5. Input from the specialist advisory ToD The ToD is often present at medical appointments where aetiological investigation is discussed with parents. His or her ongoing contact with children and their families can help to support family knowledge, and understanding of the investigation process. The ToD can also arrange for the family to meet other parents of deaf children who have already been through the process and alert them to useful resources. Often, the ToD forms a vital communication link between the medical specialist and the family and can facilitate the process of information exchange and contribute to parental informed consent. ToDs often alert the medical specialist to particular concerns they have about a deaf child s development or academic progress which may lead to further medical investigation and a possible revision in the initial diagnosis. The ToD can also advocate for establishment and continuity of high quality medical aetiological services which adopt a family friendly philosophy. Dr Breege Mac Ardle is a consultant in audiovestibular medicine at the Nuffield Hearing and Speech Centre, Royal National Throat Nose & Ear Hospital London. Useful websites Congenital Cytomegalovirus Association British Association of Audiovestibular Physicians NDCS 6 B ATOD Magazine September 2010

9 Aetiology Exploring genetics Rachel Belk delves into the area of genetic counselling, how it fits alongside other aetiological investigations in deafness and what it can offer families Genetic counselling aims to offer unbiased information and support to people who have questions or concerns about inherited traits in their family. Usually, this means inherited illnesses like cystic fibrosis or muscular dystrophy. However, some people, both d/deaf and hearing, are interested in whether the deafness in their family is inherited. In Manchester, most families we see with a history of deafness are parents with deaf children who are having aetiological investigations. Genetic counselling may have been suggested as part of this overall picture. Genetics professionals are both doctors specialising in genetics (clinical geneticists) and genetic counsellors with a nursing, genetics, psychology or similar background. Although genetic counselling services are in a medical setting, genetics professionals centre their practice on valuing the individual s perspective and recognise that people vary greatly in their views towards deafness. Some consider deafness to be a disability, while others value being Deaf as a positive identity with linguistic and cultural differences. There are, of course, parallels between genetic counselling and education in this sensitivity to different perspectives. The most important outcome of genetic counselling is greater understanding and empowerment for the person attending. In addition, knowing more about the possible causes of deafness and the chances of further children being deaf or hearing can help with practical planning around education and language. This empowerment through understanding can, of course, extend to deaf children themselves who, not infrequently, start to ask Why am I deaf? as they get older. Genetics knowledge can be part of this development of self-identity and, perhaps, a particularly powerful component, given the importance most people attach to familial similarities and differences. An overview of the genetic causes of deafness Many aetiological investigations are offered to families by genetics professionals in partnership with other health professionals, such as paediatricians, audiological physicians and ENT doctors. Sometimes quite a number of investigations have been carried out prior to the genetic counselling referral. The genetics professional will always start by drawing a family tree, as this can give immediate clues to the inheritance of a gene causing deafness. Other tests may need to be arranged, such as a urine check, ECG (checks the electrical rhythm of the heart), eye examination, Connexin 26 gene test, MRI or CT scan of the inner ear. These are rather medical tests but they are offered because many of them check for syndromal causes of deafness where the deafness is found with other features (a syndrome is medically defined as a pattern of symptoms or signs with a common cause or link). Identifying a syndromal cause means that any related health problems could be monitored in the future. The inheritance of many genetic syndromes is well known, so making the diagnosis also gives information about the chance of other family members being or becoming deaf. It is only possible to determine the probable cause of deafness for 40 50% of children Approximately half of childhood deafness has a genetic cause. Other common causes are infections during pregnancy, for example rubella (German measles) or cytomegalovirus (CMV), or infections after birth, such as mumps or meningitis. Of those cases with a genetic cause, a third are part of a syndrome. The other two-thirds have deafness alone with no health problems. It is thought that more than 150 different genes are involved in the development and function of the ear. These many different genes, together with the other causes of deafness, make it a highly complex task to pinpoint the cause of deafness in a child. This is particularly true if both parents are also deaf as the causes of their deafness may be the same, but are more likely to be different. With current tests, it is only possible to determine the probable cause of deafness for 40 50% of children. The main different patterns of single genes being passed through the family are recessive, dominant or X-linked inheritance. Genes come in pairs and recessive genes only show their effect if an individual inherits a change in both copies of the gene, one from each parent. This pattern of deafness usually happens in just one individual in a family or sometimes in more than one brother or sister with hearing parents. Dominant genes show their effect if an individual BATOD Magazine September

10 Aetiology carries just one changed copy of a gene pair, so are often the cause if there is a family history going back several generations on one side of the family. X-linked genes are carried on the sex chromosomes so the effect of the gene is sometimes different, most commonly affecting men more severely than women. One gene accounts for between 30% and 50% of recessive deafness. This is the Connexin 26 gene and it is the only gene causing non-syndromic deafness that is routinely tested because it is a relatively common cause and is small and easy to screen for changes. We often describe this gene screening as being rather like reading through one book in the genetic library at the centre of each of our cells: this analogy describes the chromosomes as the shelves in the library, the 30,000 genes as the books on the shelves, each coding for a different chemical that the cell needs to produce, and the DNA as the paper and the ink that the genes are made from. Some genes have a role not only in the ear, but in other parts of the body as well and therefore may be a cause of deafness as part of a syndrome. Some syndromes include Usher syndrome (visual loss caused by developing pigment on the retina), Waardenburg syndrome (eyes can be different colours, hair can have a white patch or go grey early), Alport s syndrome (kidney problems), Branchio-otorenal (BOR) syndrome (unusual kidney size or shape, small skin tags near the ears or neck) and Jervell- Lange Neilson (unusual heart rhythm pattern). Individually, all these syndromes are very rare, but are well recognised as causes of deafness. It is impossible to give an exhaustive picture of all syndromes as there are literally thousands. Deafness is sometimes found as just one symptom of a condition like this Some other syndromes are caused not by a single changed gene, but by a section of chromosome being missing or duplicated, which may contain a number of genes. These syndromes can often cause significant health problems with the heart, kidneys or other major organs or in a child s learning and development. Deafness is sometimes found as just one symptom of a condition like this. When do people come for genetic counselling? It often assumed that genetic counselling is entirely focused around the time of childbirth and pregnancy. We actually see people at any time of life. As discussed above, many families are referred after deafness has been diagnosed in a child in the family. On other occasions, adults who have been deaf from birth are referred with questions about the cause of their own deafness or concerns about possible related health problems if a syndromic cause is suspected. We also see adults who have developed 8 B ATOD Magazine September 2010

11 Aetiology hearing loss later in life and, again, this has multiple causes. There are rare genetic conditions such as neurofibromatosis type 2 (NF2) which cause not only benign tumours to grow on the hearing nerves (causing profound deafness, usually in adulthood), but carry a risk of other benign tumours in the brain or spine which can be highly debilitating. Some genes have a very variable effect on hearing/deafness and a person who carries a specific changed gene may be predisposed to developing earlier or more severe deafness than the general population. Often, it is not possible to pinpoint a specific gene, but this may change as genetic testing techniques improve over the next few years. Of course, it must be remembered that deaf adults have the same chance as anyone of having a family history of a genetic illness, and individuals may be referred for genetic counselling for a reason entirely unconnected to their deafness. Is testing ever done in pregnancy? It is rare that an individual/couple comes for genetic counselling and asks about testing a future pregnancy to see if the baby would be deaf or hearing. This is the case for both deaf and hearing parents: most people want to know the information for preparation, but would not do anything actively to choose whether their child is hearing or deaf. However, occasionally someone (deaf or hearing) does ask to have a test in pregnancy and would consider having a termination if the baby were to be born deaf. The Abortion Act 1967 says that a termination can be carried out if a baby would be born with a serious disability it is possible that the obstetricians would agree that deafness would be included as a serious disability and therefore agree to a termination of pregnancy. Genetic testing in pregnancy is not something that is ever recommended by genetics professionals It is only possible to have a genetic test in a pregnancy if the gene change causing the deafness in the family is known. Genetic testing in pregnancy is not something that is ever recommended by genetics professionals the discussion would be led by the individual s or couple s questions or concerns. What about assisted reproduction? In most cases, assisted reproduction is completely separate from genetic counselling or genetic testing. However, a relatively new procedure called preimplantation genetic diagnosis (PGD) was discussed at length in the media in 2007/08. This was because of a particular clause in the Human Fertilisation and Embryology Bill 2008 which generated a great deal of controversy as the Bill went through Parliament. PGD is a procedure where genetic testing and IVF (in-vitro fertilisation, where a woman s eggs are fertilised outside the body and then one or two embryos placed back in the womb) are both involved. IVF techniques are used to fertilise several eggs and then, when the embryos have developed to an eight-cell stage, just one cell is removed from each embryo and genetic testing is carried out on that cell. This determines which embryos have which copies of one particular gene. An individual or couple might choose this procedure to avoid passing on a particular genetic condition usually a serious inherited illness such as muscular dystrophy. In families where the Connexin 26 gene is known to be the cause of deafness, it is now possible to use PGD techniques to look for the gene changes. Prior to 2007/8 there were guidelines on the use of these techniques, but they were not in primary legislation. The decision to use PGD to select for a deaf or a hearing baby was one that was made privately by an individual/couple with their doctors. It was felt that a value judgement between deaf and hearing status was being set in law The Bill focused partly on the regulation of assisted reproduction services. It was already legal for an individual/couple to use PGD to select against embryos that would be deaf, but this particular clause made it illegal for an individual/couple to select against embryos that would be hearing. It also ruled that a deaf individual/couple would not be able to request a deaf donor either for artificial insemination (donor sperm) or for donor eggs (using IVF techniques). There were already guidelines that meant that deaf people would not have been accepted as gamete (sperm or egg) donors, together with other groups of people with a wide range of conditions including, for example, high myopia (shortsightedness). The Bill made this illegal rather than only subject to guidelines. Depending on how the clause is interpreted, an additional implication may be that close hearing relatives of deaf people cannot be gamete donors for their relatives because they might carry genes that could cause deafness. One reason that this clause caused such upset and anger in the Deaf community was that it was felt that a value judgement between deaf and hearing status was being set in law, rather than continuing to leave these decisions as private ones between individuals and their doctors. This was particularly because deafness was used as the specific example when the Bill was introduced in the House of Lords and in the accompanying explanatory notes. After a great deal of BATOD Magazine September

12 Aetiology discussion, this reference to deafness was removed from the accompanying notes prior to the Bill becoming law, though the clause remains in place. How can the genetic counselling service become more accessible? The role of a genetic counsellor is a fascinating balance between science and support for individuals and families; between definite facts about genetics and specific conditions and recognition of hugely varying perspectives towards and personal experiences of the condition. Deafness, perhaps more so than the majority of conditions we see, can be viewed in very different ways. Where there is commonality in these perspectives is the recognition that communication is an issue; and that difficulties in communication can be the biggest barrier to accessing genetic services. This is not unique to genetics within medicine: the RNID and SignHealth, an organisation providing and researching health services for d/deaf people, have found that there are many barriers to communication in the health service, many of which can be simply overcome by increasing awareness and making commonsense changes to practice. Written leaflets are available to explain things like recessive inheritance Within our genetic counselling service in the North West, we have set up a specialist deafness clinic which means that expertise can be shared by the genetics professionals who work closely with this clinic. Joint clinics have also been set up with audiology health professionals around the region. Specific projects to improve information within the service take account of the different communication choices made by deaf people. Written leaflets are available to explain things like recessive inheritance and to be read alongside the summary letters we send to families after clinic discussions. But these leaflets are only helpful for people with good written English and, recognising that BSL users may have lower English skills, one of our projects worked with Deaf BSL translators and undergraduate TV production students to produce BSL translations of five commonly used leaflets on DVDs with voiceover and subtitles. These leaflets are now freely available for anyone on the website manchester.ac.uk/what-is-genetic-counselling/ Another project tackled communication difficulties for people who have NF2 and have become profoundly deaf in adulthood. Careful monitoring for benign tumours in the spine and brain is needed and, if they occur, complex decisions must be made about when and if to remove them surgically. These discussions must involve the patient fully, yet understanding can be very difficult if he or she is not yet a proficient lipreader. Even if he or she is, the complex terminology and the emotional impact can make communication very hard. This project used voice-recognition software, sold commercially for dictation of letters, so the health professionals could speak their side of the conversation onto a laptop screen for the patient to read. The project pilot was well accepted by the patients and it continues to be used in the Manchester specialist NF2 clinic. We think that this software could be useful in other medical settings and perhaps also in education. Genetic counselling is an area which brings together science, counselling, communication It was the translation of the leaflets into BSL that I found particularly inspiring. Although genetics is not a subject previously discussed widely in BSL and therefore the vocabulary is not developed in detail, this is in no way a failure of BSL rather it is a sign of the dominance of English in scientific discussion. As a result of my interest, I began working as a National Institute for Health Research Fellow in October 2007, using qualitative methods to investigate Deaf BSL users perspectives on preferred signs for particular genetic concepts and the best format for information resources. Much of my data collection has been through a website developed for the research which allows participants to respond in BSL via webcam and captures the resulting video remotely to a secure server. By analysing the responses actually in BSL, I am aiming to capture the nuances of how genetic concepts can be expressed in BSL in ways that use the benefits of a visual language. The final aim is to produce information resources in BSL about genetics that are directly useful in the genetic counselling clinic and in related areas of medicine, but might also be beneficial for biology and genetics teaching in schools. Genetic counselling is an area which brings together science, counselling, communication and the real experiences of individuals and families in a unique way, but one that has a lot of parallels with the communication of information through teaching in ways that are matched to an individual s interests and needs. There is a great deal of expertise that could usefully be shared. Rachel Belk MSc is a registered genetic counsellor specialising in deafness in the Department of Genetic Medicine at St Mary s Hospital, Manchester and an NIHR Fellow based both in the Social Research with Deaf People (SORD) group, University of Manchester, and in Nowgen. Parts of this article first appeared in an article in Signmatters magazine (now British Deaf News) in BATOD Magazine September 2010

13 Meningitis the facts Aetiology Laura Clark provides some background detail on this debilitating disease and explains how it causes hearing loss in children Most Teachers of the Deaf will at some point work with children deafened by this disease. Meningitis is the infection and swelling of the lining of the spinal cord and brain (the meninges). There were over 3,000 cases of bacterial meningitis in the UK and Republic of Ireland in Neonatal meningitis has a wide range of causes, including infection by Streptococcus agalactiae, Eschericia coli and Listeria monocytogenes. All forms of bacterial meningitis (with the exception of tuberculosis meningitis) are more common in young children, particularly in those under the age of four. Within the UK there have been several successful vaccination programmes against some of the major causes. Most of the population carry many of these organisms in their nose and throat with absolutely no harmful effects. Invasive disease is relatively uncommon and the chance of more than one case occurring in the same surroundings is small. However, in certain susceptible people invasive disease can be fatal, and for many survivors it results in a wide range of longterm after-effects. Meningitis is one of the leading causes of acquired sensorineural hearing loss up to 10% of survivors will experience some degree of permanent unilateral or bilateral hearing loss. After-effects as a result of bacterial meningitis The after-effects that can occur as a result of bacterial meningitis are wide ranging, from behavioural and emotional difficulties to severe cognitive and motor deficit, seizures and permanent profound hearing loss. Survivors of pneumococcal disease have a higher risk of long-term neurological consequences, including hearing loss, than those with other forms of the disease. Those deafened in this way may also have complex multiple disabilities. Pathophysiology of meningitic hearing loss Hearing loss caused by bacterial meningitis is thought to occur in the early stages of infection. At this point many patients, if treated quickly with appropriate antibiotics, will regain normal hearing. Post-meningitic deafness is widely believed to be due to cochlear damage caused by specific bacterial toxins and also as a consequence of the host response to infection. In pneumococcal meningitis, pneumolysin, a pore forming protein, is thought to be responsible for damage to the sensory hair cells and supporting cells within the organ of Corti. Infection of the meninges also triggers a cascade of inflammatory mediators, which travel to the perilymphatic space of the inner ear causing damage to the surrounding tissues. The host response may also lead to a decreased blood supply to cells within the cochlea (related to inflammatory destruction or constriction of the blood vessels). Prompt referral for audiology assessment As hearing loss in meningitis survivors is almost always as a result of damage to the cochlear and not the auditory nerve, cochlear implantation offers the potential for sound perception and speech recognition. Infection of the cochlea may result in the overgrowth of new bone (cochlear osteoneogenesis) and if this is widespread it may make implantation impossible. For this reason it is important that a hearing assessment is carried out as soon as possible. Direct referral by telephone prior to discharge has been shown to improve assessment within a six-week timeframe. Raising awareness of NICE guidelines within the health profession and making parents aware of the importance of prompt testing can also make a difference. The Meningitis Research Foundation (MRF) calls for formal audiological testing as soon as possible after bacterial meningitis, preferably before discharge, but within four weeks of being fit to test. Gaining a diagnosis quickly will not only make cochlear implantation possible, but is also likely to improve audiological performance after the implant takes place. Cochlear implantation, if carried out at an early age, has been shown to significantly improve the ability of postmeningitic and congenitally deaf children to recognise speech. For these groups, cause of deafness seems to have little influence on the outcome of an implant and so prompt referral to the audiologist is the key message. Cochlear implantation may be particularly important for children deafened by meningitis, as they may be more likely to live in families where other family members are not deaf. In such cases, aural and oral communication options are usually chosen by the family. This may not always be the case in congenital deafness, where other family members may be deaf as well. Prevention of disease is the most effective way of reducing death and disability and as such the MRF advocates vaccination of all children as per the UK vaccination schedule, and pneumococcal vaccination either before or as soon as possible after cochlear implantation for implant patients not previously vaccinated (this should never delay implantation). Laura Clark is an epidemiologist at the Meningitis Research Foundation. BATOD Magazine September

14 Aetiology Congenital cytomegalovirus What do we know about this cause of deafness? Jayne Ramirez Inscoe seeks to answer this question Congenital cytomegalovirus (ccmv) is the leading cause of non-hereditary deafness in children. It is also one of the main causes of unilateral, delayed onset and progressive sensorineural hearing losses and is an important cause of vestibular dysfunction (balance disorders). ccmv also causes a range of other disabilities. The virus is the second leading cause of learning difficulties and is the leading cause of cerebral palsy in children. Despite this, many health professionals, parents and Teachers of the Deaf have little awareness of the condition. What causes ccmv deafness? If a mother contracts ccmv during pregnancy, the virus can damage her unborn baby. The infection causes the most damage during the first trimester; however, there is evidence of babies being infected in the following trimesters also. Women are not screened for antibodies prior to getting pregnant. In the United States it is reported that about half of all expectant mothers have never been infected with ccmv, and this puts their unborn babies at risk. A primary infection (the first time the woman has been exposed to the virus) is known to be more damaging than a secondary or recurrent infection (when the virus which remains dormant in a person s salivary glands is reactivated). Most (90%) children born with ccmv infection are asymptomatic at birth (well babies apparently unaffected by the infection). However, around 10% of these children have delayed onset or unilateral sensorineural hearing loss (SNHL). For those born with symptoms, the incidence of SNHL is around 50%. There is currently no cure or vaccine for ccmv infection, first because ccmv is an extremely common flu-like virus from the herpes family of viruses which 70% of the population has contracted by the age of 40, mostly without symptoms. It is spread easily through contact with human bodily fluids such as urine and saliva. Second, the cost of a universal vaccination programme has so far been prohibitive given that 90% of babies infected with ccmv are deemed to be asymptomatic. The Nottingham experience Following concerns about limited outcomes for some children, I carried out a study on 16 ccmv deaf children who received a cochlear implant in Nottingham. This showed that, after three years of cochlear implant use, there was a wide range of outcomes, with average speech perception and production scores not dissimilar to the non-ccmv deaf children whose deafness had other causes. However, the children s rate of progress was generally slower and an associated cognitive difficulty produced lower scores. The high incidence of co-occurring additional difficulties appeared to affect outcomes, so further research was proposed. The Nottingham Cochlear Implant Programme monitors all children for the presence of additional difficulties and their impact on the child s language and learning potential. Some of the disabilities co-occurring with SNHL are recognised as common sequelae of ccmv, including learning difficulty, cerebral palsy and seizures. For some of the children these difficulties were known when they received a cochlear implant. However, other difficulties such as challenging behaviour, autistic tendencies and oro-motor sequencing problems appear to be common in this group and have had a notable impact on children s listening and language outcomes post-implant. To date, 34 children with known ccmv deafness have received unilateral or bilateral cochlear implants in Nottingham; 40% have three or more additional difficulties. Limited concentration span, distractibility and over-reactivity appear to be long-term difficulties for many of these children, affecting rate of progress in language and learning throughout their education. After five years of cochlear implant use, 40% attended a special school (not for deaf children), 20% went to schools for the deaf, 20% attended hearing-impaired units and 20% attended local mainstream schools. This compared with the majority of non-ccmv deaf children using implants who attended either local mainstream or units after five years cochlear implant use. The average Speech Intelligibility Rating (SIR score) for the ccmv group was between 2 and 3, five or more years after implantation, which meant that they had specific speech production difficulties and were not readily understood by unfamiliar people. We would expect an SIR score of 4 or 5 (highest score) after five years of cochlear implant use in our non-ccmv deaf population. After ten years of cochlear implant use only 28% used spoken language as their main mode of communication. The UK ccmv Association is collecting information from parents about other problems in these children, including early puberty, eating preference issues, organisational problems, mental health concerns and dyscalculia (specific maths difficulty). Other current research into ccmv Diagnosis It is now possible for the cause of deafness to be 12 BATOD Magazine September 2010

15 Aetiology confirmed retrospectively as ccmv by testing stored Guthrie card dried blood spots (health visitor s baby heel-prick test). Several recent studies now suggest that 20 30% of children with unexplained SNHL could have ccmv. Large scale screening programmes are being conducted in Europe and the US to determine the number of children who are born with or acquire SNHL as a result of ccmv infection. There is growing pressure in many countries to produce a vaccine with the hope of eradicating the disease in the same way as the rubella vaccination programme has almost completely eliminated that illness. One large scale study which monitored SNHL in 860 children found that over half of the children thought to be asymptomatic had a unilateral hearing loss. A further third of the group went on to develop SNHL, typically at around 44 months of age. Newborn hearing screening can be expected to identify only 50% of children with hearing loss with this particular cause professionals need to be aware that a late onset hearing loss is a possibility. As ccmv particularly targets the brain of an unborn baby, imaging results from MRI and CT investigations can show common features which may also be used in some cases to confirm ccmv as the aetiology of deafness. Parallel research into autism has suggested that in some cases the disorder may be due to ccmv. Treatment Trials are currently underway to ascertain the effectiveness of anti-viral drugs for those babies who are born with symptomatic ccmv to attempt to preserve their hearing abilities. However, the drugs have significant side-effects. A vaccine is being trialled which could prevent ccmv infection. It is hoped that this article will increase your awareness that: many children who have SNHL as a result of ccmv infection appear to share many additional difficulties there may be children on your caseload with unknown aetiology whose deafness may be due to ccmv. You may have children with a unilateral SNHL who are at risk of delayed onset or progressive hearing loss progress in spoken language development following cochlear implantation may be slower for these children, as they are likely to have an additional difficulty affecting listening and language development. Jayne Ramirez Inscoe has been one of the speech and language therapists on the Nottingham Cochlear Implant Programme for the past 16 years. If you would like further information about ccmv please contact Jayne.Inscoe@nuh.nhs.uk. BATOD Magazine September

16 Aetiology CMV a parent s perspective As mother of a 17-year-old with congenital cytomegalovirus and co-ordinator of the UK CMV Association Carmen Burton is well placed to shed more light on this little-known-about condition When our daughter Natalie was born in 1993, she was seriously ill and was diagnosed with congenital cytomegalovirus (ccmv). CMV is the leading cause of congenital deafness in the world and can also cause many other problems, including blindness, learning and developmental delays and health problems. Natalie was diagnosed as profoundly deaf at around four months and was fitted with hearing aids which were of little use except to stop her putting things in her ears! Ironically during my college years I had done voluntary work at a local school for the deaf and could already sign, so communication has never been an issue for us and she was a fluent signer by the time she had her first cochlear implant (CI) at the age of three. We had been told that CMV children do not do well with CIs, but at 16 Natalie is now bilingual age-appropriate in BSL and spoken English (she has level 2 BSL, passed her English GCSE at grade C and is currently taking English literature GCSE). She can use a phone and loves music and going to the theatre musicals are a particular favourite. Deafness is and has always been the least of her problems. The CMV has also left her with dyscalculia, short-term memory problems, organisational skills difficulties, behaviour, sleep and eating issues. She finds it very hard to sit still and to concentrate for any length of time. She also finds it hard to express her feelings, understand other people s feelings and mix with children her own age, and she loves routine. As long as the world is following Natalie s agenda and routines, everything is fine, if not then major problems can occur. She was also diagnosed with partial complex seizure disorder at the start of puberty. Natalie started school at a fully integrated mainstream unit where, despite her problems, she did very well until junior school, when she struggled to make friends, the work got more complex and, due to staffing issues, she began to dislike school; her grades fell and she started misbehaving. At the end of Year 5 we moved her to the Royal School for the Deaf (RSD) Derby, where with correct help and support she gained 11 GCSEs one A grade, one B and nine Cs. We are extremely proud of all her achievements as we were told at birth that if she survived she would be deaf, blind, in a wheelchair and would never be able to do anything for herself. She now attends Derby College (with support from Post-16 at RSD) and was studying childcare at level 3. She wants to work with special needs children and is also doing voluntary work at a local deaf youth club and has volunteered to work at a deaf summer school and a summer camp for deaf children. Unfortunately, due to a lot of reasons, this course was not the right one for her so, it is hoped that from September she will be taking English literature and language and theatre and drama studies at A-level. The UK CMV Association About seven years ago I was asked to run the UK CMV Association and although Natalie does have many of the side-effects of CMV, thankfully she is not as severely affected as many children, which enables me to dedicate more time to running the organisation. We currently have around 100 families as members a mixture of symptomatic and asymptomatic children. Of these children I have full details of 43 of them: 13 of these children were asymptomatic born with no symptoms of CMV; eight have profound bilateral deafness and one has unilateral deafness. Historically it has been said that symptomatic children usually only have deafness as a side-effect, but research within our group has shown this to be otherwise. Over half of these children have educational difficulties and seven struggle to sit still and concentrate. Ten of them had developmental delay, nine have eating issues and the majority are clumsy. The other 33 children for whom I have full details were all born with the symptoms of CMV these can include any or all of premature or small-for-dates, microcephaly, enlarged liver and spleen, blood that won t clot, jaundice, blueberry rash, brain calcifications, 14 BATOD Magazine September 2010

17 Aetiology seizures, deafness and visual problems. Of these children, 30 have hearing loss. For all of the children I have full details for (both symptomatic and asymptomatic) there is a wide variety in the levels and types of deafness: 34 have bilateral loss, five have unilateral. Of these 39, ten had progressive deafness and two had delayed deafness. 20 of the profoundly bilaterally deaf children have cochlear implants. Again, in the past it has been said that CMV children are not suitable candidates for cochlear implantation. While the results for these 20 children vary widely, all parents (apart from one non-user) say that even if their child has not learnt to talk the CI is worth it for improvement in quality of life that their child likes listening and gets more enjoyment from TV and listening to music and has a better awareness of what is going on around them. Within the 20 children with CIs, seven are making very good progress and can listen and speak well; most of these can use a telephone and communicate with people who are not used to talking to deaf children. All of these children were implanted at three years old or earlier, and some have other major disabilities or learning difficulties. A couple of the children are still very young and it is too soon to tell, but they are making steady progress. Three children are making slow but steady progress. The one non-user was implanted at the age of seven and is autistic which may explain the lack of use. We have three children who are making poor progress and four with good hearing but poor speech. All of these seven children were implanted at an older age and/or have other diagnosis such as autism or cerebral palsy which may account for their lack of progress. We have six children with bilateral implants one simultaneous and five sequential. It would appear that all of these children are doing well with their bilateral implants. The majority took quite a long time to get used to them, and tuning can be an issue to get the perfect map. One child refused to wear either processor for six months after her sequential switch on, but with lots of help and support she is now happily wearing both. Another child had problems with getting the correct map it would be fine for a couple of days, then she would top out and need re-tuning. After about 18 months this now seems to have settled. The longest gap between first and second implants is 13 years. It is very early days for this child only six weeks post- switch on and she reports that it sounds like beeps, but she wears it all day and slowly the beeps are beginning to turn into robotic words, so we are hopeful that with lots of practice it will be successful. When I talk to the families, it seems that once they get over the shock of their child being diagnosed as deaf, and work out an effective communication strategy and the child becomes a proficient hearing aid or CI user, for most of them deafness is the least of the problems caused by CMV. Callam, only 2lb 9oz at birth The parents seem to have and not expected to survive more difficulties with behaviour, getting their child to eat or sleep, and getting them into the correct school, with the right support than they do with their ears. The main problem with CMV is that it affects all children differently even the level of side-effects at birth is not an indication of how well or how poorly a child will develop. There is also the issue of other things developing at a later age such as delayed deafness or visual problems and the risk of epilepsy. So much with CMV is still unknown, and for many parents it is one long waiting game. Many doctors, teachers, social workers and other professionals working with our children have little or no knowledge of CMV. The major things such as ears and eyes are widely known, but the smaller things are not talked about or recognised as being side-effects of CMV. A lot of CMV children are labelled as naughty because they won t eat or sleep or are forgetful or disruptive in class. Many parents are told he s deaf, all deaf children are like that. People working with these children need to look at the whole picture and not just the obvious issues; parents say they need labels for their children to help them get the support they need but these aren t always given as the doctors just don t know enough about CMV. Many deaf children with no diagnosis for their deafness do have similar behaviours and issues to those of our CMV children, which is where the professionals get their comparisons; it makes me wonder how many of these naughty, unco-operative, struggling deaf children actually have undiagnosed ccmv. Carmen Burton is the Co-ordinator of the UK CMV Association. Carmen s daughter Natalie is pictured on the front cover of this Magazine. BATOD Magazine September

18 Aetiology Who are the tests really for? Following a small survey in London, Peter Watkin and the EaST 17 Early Support Team underline the need to provide deaf young people with greater medical understanding of their deafness Although the philosophies underpinning medicine remain pretty much a constant, the delivery of that healthcare is periodically re-invented. We are currently witnessing such a change as we emerge from paternalistic, expert-directed treatment of illness to a prevention-orientated, consumer-driven model of personalised healthcare with services tailored for each one of us. In many services for deaf children, the professionals have already moved from expert hierarchical deliveries to participatory models of care, with parents taking a central role. Within this supportive framework, aetiological investigations are vital for many parents. However, there has been little engagement of the children themselves. They need to be offered the knowledge and understanding that will enable them to be owners of their own healthcare. They need the foundations on which personal responsibility and active involvement in lifelong health decisions can be based. Our local experiences in London suggest that they haven t all been given this knowledge. A young teenager with Type 1 Usher syndrome was disturbed with the increasing loss of peripheral vision and although full clinical explanations and expert genetic counselling had been accessed by the family, the implications had not been discussed with the teenager, who perceived the loss of vision to be the downward spiral towards an early death. Another young lady who had a bilateral hearing impairment associated with dilated vestibular aqueducts (DVAs) lost all her residual hearing as she hurtled to the ground while undertaking a voluntary parachute jump. Although she had been warned of the dangers of head injury in DVA, the implications of the rapid changes in pressure that are part of the thrill of skydiving clearly hadn t been explained! To explore further whether such misunderstandings were common and whether deaf young people had become owners of the earlier aetiological discussions that had taken place with the family, we questioned a small sample living independently in our part of East London. We distributed 50 questionnaires to deaf young people living independently and with an average age of 23 years. They had been to a variety of schools in the south of England and attended various London and local audiology services. All were severely or profoundly deaf. A total of 27 (54%) responded. There were five closed questions (multiple choice or yes/no replies) about the cause of their deafness, with opportunities for additional comment. 1 Do you know the cause of your deafness? 59% of the respondents knew their aetiological diagnosis or knew that they had been investigated, but no definitive cause had been identified. However, 41% didn t know whether a cause had been found or even looked for. They attributed their profound deafness to ear infections or unnamed illness suffered in childhood. One wrote. My dad refused to be tested as he didn t want to take the blame and so I don t know the reason why I m deaf. 2 Is it important that you know the cause of your deafness? 81% of respondents considered it important to be in possession of the facts. One response was echoed by many, I don t mind that I was born deaf, but everyone should know the cause of their deafness so that they can understand their condition. Another person, who was identified with Pendred syndrome when testing became available, stated that knowing was important for my own peace of mind. I ve worried about why I was deaf since being little and had no answer until the tests. I also wanted to know because of my children I needed to know if they would be deaf. Another respondent with Usher syndrome stated that knowledge of his condition helped him plan for the future and it made him careful not to marry anyone in the family. However, not all agreed that such knowledge was useful. Of the 19% not considering it to be important, a typical response was, I m not interested I m just happy the way I am. 3 When did you start to think about the cause of your deafness? The multiple choice responses were aggregated into five-year blocks from 0 to % had wondered about the cause and of these 52% had become concerned between 10 and 15 years, with 38% becoming concerned between 5 and 10 years, and 10% between 15 and 20 years. 4 Who discussed the cause of your deafness with you? The respondents were asked to name the main person who explained the cause of their deafness to them. The results are detailed in the chart at the top of the next page. Among those where someone else had been the main source of information, one deaf young person recalled that it had been the school nurse who had almost exclusively fulfilled this role. Another recorded talks with classmates as the main information source; 44% claimed that there were no discussions with any professional about the cause for their deafness. 16 BATOD Magazine September 2010

19 Aetiology 5 Do you feel that you understand the cause of your deafness well enough or would you like to know more about it? 81% were interested in the cause of their deafness and of these all wanted to know more about it. As one respondent stated, There is a lot of information about possible treatments and advances in genetic research on the internet, but it s not possible for me to know how it affects me unless I can talk about it with someone. There was an additional closed set question to find out about their understanding of genetics, congenital infections, meningitis, and healthcare in pregnancy. This wasn t an in-depth probing of their knowledge, but simply a self-assessment of their confidence in their own background knowledge of the sort of health matters that may be involved in aetiological discussions. The answers are shown in the graph in the next column. Do you know about? Even those who confirmed that they had some knowledge commented that subjects were never properly explained so that I could understand with one noting genetics is changing all the time and while I understand something it hasn t helped me to understand my condition. Although the survey was limited, it confirmed that earlier aetiological investigations had not trickled down to many deaf young people. Counselling the parents simply isn t enough. With personalised healthcare imminent, we need to ensure that deaf young people have the personalised learning that will allow them expertise and authority in their condition. The DfE embraces personalised learning and its vision is of tailoring individual learning to what, when and how things are learnt with a learning journey that may include supporting children with multi-disciplinary partnerships that extend beyond the classroom. Personalised learning and healthcare could be joined, enabling deaf young people to develop the necessary expertise in their condition. The majority in our survey became concerned about the cause of their deafness as teenagers, and personalised learning could be part of the transition process from child to adult services. Assessing Gillick competency (whether a child under 16 is mature enough to make decisions about his or her medical treatment) may be required, but offering each child the medical details about his or her deafness should be the norm for all deaf children. In our experience this is not happening, and although personalised healthcare will eventually change the way that doctors address the ownership of medical information, it would seem that for the time being at least, joint working is required for a programme of late support as well as the now established and accepted programme of early support. The NDCS has recognised this need for later support around transition and is currently embarking on the Over to You project which aims to improve the experiences of deaf young people in accessing audiology services. The NDCS will work in partnership with the neighbouring East London primary care trusts in Tower Hamlets, Newham and Hackney to promote a better understanding of the needs of deaf young people by service providers so that they are more confident in involving them in the design and shaping of their audiology services (more information from NDCS Project Manager Valerie Copenhagen at Valerie.copenhagen@ndcs.org.uk). Over to You will very much be driven by the needs of the deaf young people themselves, but the results of our survey suggest that they will share the concerns about understanding the cause of their deafness, and ways of delivering this part of the service will be further explored. Clearly this is important because giving these young people this healthcare information is essential support for their entry to young adulthood. Peter Watkin is Consultant Audiological Physician at Whipps Cross University Hospital. The EaST 17 Early Support Team for the Families of Deaf and Hearing Impaired Children Is based at Chapel End Early Years Centre, Walthamstow, London. BATOD Magazine September

20 Aetiology Living with TCS Alex Wheeler provides an overview of Treacher Collins syndrome, reflecting on her own personal experiences of growing up with this condition Treacher Collins syndrome (TCS) is a hereditary condition resulting in abnormalities of the skull and facial bones, which ranges from a form which is so mild it may only be diagnosed in an adult following the birth of a more severely affected child, to so severe that it can result in perinatal death. With an incidence of around one in 50,000 live births it is rare and is thought to arise from abnormal development of the foetus at around four to eight weeks gestation. A faulty gene has been identified on chromosome 5 and an affected individual has a 50:50 chance of passing on the condition (autosomal dominant inheritance). However, it is estimated that 50 60% of new cases are spontaneous mutations; that is, the parents are unaffected. Even where a parent has been diagnosed with TCS, it is not possible to predict the potential severity of the condition in a child. A pregnant mother with TCS can be tested via amniocentesis but only ultrasound scanning can identify the degree of abnormality and pick up features such as cleft palate. The facial features of TCS, once seen, are so classic in the more severe cases that it is quite easy to identify children or adults with the condition. Research suggests that over three-quarters of cases have malformed facial bones, particularly the jaw. This in turn may result in dental malocclusion where the chin and upper jaw are not well aligned. The teeth may be widely spaced, malpositioned or reduced in number. About a quarter of cases involve a cleft palate and other palatal abnormalities. Ophthalmic abnormalities include downward slanting of the eyelids in a very high proportion of cases, along with problems with the lower eyelids which may be notched and lacking in eyelashes. From an audiology point of view, the most notable features are low-positioned malformed external ears which are frequently associated with atresia of the external auditory canals and anomalies of the middle ear ossicles. Scanning of affected individuals may show fused or even absent ossicles and, in some cases, no middle ear at all. Consequently, bilateral conductive hearing loss is common in TCS patients, whereas a mixed or sensorineural hearing loss is rarer. Clearly these children are candidates for bone-anchored hearing aids (Bahas). In the past, largely unsuccessful attempts were made to reconstruct outer ears; currently prosthetic ears attached with a titanium clip are the preferred option. Management The care of individuals affected by TCS requires a long-term multi-disciplinary approach from doctors, audiologists, speech therapists and psychologists. Of primary concern are breathing problems, which may arise at birth as a consequence of a small jaw, the anatomy of the airway and tongue obstruction. An emergency tracheostomy may be essential to maintain an adequate airway. This may be the first of many operations for a child with TCS, and may include early cleft palate repair where necessary. At no time was I ever asked what I wanted or how I felt about having TCS A search of the literature reveals a whole range of potential operations aimed at correcting the underdeveloped facial structures, starting around the age of five years and carrying on as the child s face continues to grow and develop. There appears to be a very strong desire on the part of doctors to intervene surgically and, while the research is full of case studies outlining procedures, there is very little detailed follow-up of cases and next to no information on psychosocial aspects of the condition or how they might be managed. These children may have a mixture of hearing loss, speech problems and facial abnormalities, which can have a significant impact on learning ability, self-esteem and social interaction/acceptance. Additionally closer inspection of the literature suggests that, in the past at least, surgical intervention has not always been successful and rarely corrects the abnormality completely. I have lived with TCS for over 40 years. Despite being born abroad to parents who had no knowledge of TCS, my condition was diagnosed promptly but no treatment was initiated until our return to the UK when I was three years old. My mother was originally told that I was so deaf I would never learn to speak, and one Medresco body-worn hearing aid was fitted when I was around three-and-a-half years old. It was many years before it occurred to anyone to give me a second hearing aid and in any case the benefit I gained from these poorly fitting aids was limited due to my ear deformities and atresia. At around the 18 BATOD Magazine September 2010

21 Aetiology same age I had a cleft palate operation which finally enabled me to eat and drink properly but was followed by many sessions of speech therapy to get my palate and tongue working. I was the classic unco-operative hearing aid user, and started lessons at our local village school wearing aids for only part of each day, my parents having refused to send me to a special school as recommended. Over the years I worked my way through a succession of hearing aids which became post-aural but were even more difficult to fit to my insubstantial ears. A succession of plastic surgeons looked me over, promising to operate when I was a teenager, and then deciding not to for reasons they did not feel it was necessary to explain. In the end, the only intervention that I ever had was orthodontic, to remove and realign the teeth in my jaw. At no time was I ever asked what I wanted or how I felt about having TCS. Eventually my parents paid for a private ITE hearing aid, but I continued to have problems, this time with a reaction to the aid in my ear. By this time I was in a small convent school and coping well, although without the support of my Teacher of the Deaf who was withdrawn when I left the state sector. I had not been operated on in the intervening years and was therefore a blank canvas After training as a Teacher of the Deaf myself, I decided to get a referral back to the specialist centre I had attended in the past and I insisted that I wanted a proper review of my hearing needs. For the first time I met a consultant audiological physician who was interested in me as a person and not just as a classic case of TCS and who asked me how I felt about my condition. Together we considered the options; I was offered (and refused) another trial with ITE aids or to go for a bone-anchored hearing aid, and was fitted instead with baby-sized post-aurals with hypoallergenic moulds. My reasons for refusing a Baha were my dislike of the design (which I still think is extraordinarily ill-conceived) and the issues at the time with post-operative infection combined with concerns that I would need more amplification than the Baha could give over time. I also liked the convenience of being able to listen to children s hearing aids through my own ear moulds and link in with their radio aids. I was treated. Now I was treated as an object of extreme interest because unlike almost every other TCS patient I had not been operated on in the intervening years and was therefore a blank canvas to try out new techniques on. I was sent for a detailed series of CT scans and my maxillofacial consultant proposed extensive reconstructive surgery, over and above what was needed to treat the symptoms I had arrived with. I also rather objected to the (medical) assumption that I was in need of fixing I went away and read the research literature and decided against surgery. My decision was greeted with some incomprehension and not a little disappointment by the surgeon. Had I been offered an operation as a teenager I would probably have taken it, but as a mature adult, my perspective was very different. I knew from my research that the operations would be painful and not without risk due to intubation difficulties and that the outcomes were not always long lasting, particularly in terms of jaw re-alignment. I also rather objected to the (medical) assumption that I was in need of fixing. I took the view that I did not need to go through surgery to change my outward appearance to make myself more acceptable to other people that who you are as a person is more important than how you look. People can accept me as I am or not at all. In the event I had the treatment needed for the problem in my jaw and have never regretted the decision to turn down the offer of reconstructive surgery. Alex Wheeler is a Teacher of the Deaf. Problems with pain in my jaw a few years ago also caused me to return for investigations to the plastic surgery unit I had attended as a child. I had ceased attending appointments as a teenager, having become very annoyed with the insensitive way that BATOD Magazine September

22 Aetiology Complex provision Wendy Eadsforth and Jill Millward describe how staff at the Royal School for Deaf Children Margate and Westgate College are committed to meeting the challenge of young people with complex needs some of the problems, the pupil becomes the centre of planning the curriculum, groups and timetables. Each pupil has an individual resource overview which highlights the resources needed. These will include educational, behavioural and care plans, risk assessments and input from medical, specialist and external agencies. Classes are grouped not only according to academic need but also communication, emotional, social and behavioural needs. Timetables can be individualised to support learning styles (for example, greater emphasis on learning through practical activities) while still meeting the National Curriculum and our new curriculum entitlement. While the advent of neonatal screening techniques has provided early and objective diagnosis of deafness, the full extent and implications of hearing loss in relation to additional needs remain less easy to establish. The Royal School for Deaf Children Margate (RSDCM) and Westgate College (part of the John Townsend Trust) currently cater for children and young people with around 20 different medical conditions and syndromes that are directly linked with deafness Down syndrome, CHARGE syndrome, CMV and Treacher Collins syndrome to name but a few. In addition, many more have conditions such as chromosomal disorders and unspecified global developmental delay, with deafness as just one part of a multiple disability. Approximately 16% of our population has been diagnosed within the autistic spectrum and a further 30% with mental health problems. Complex needs are precisely that each discreet aspect can impact in ways that compound and exacerbate others. As a consequence, learning and behaviour can be greatly affected, often in ways that may seem minor but can have a significant effect on planning. An autistic child, for example, may have certain ritualistic behavioural patterns and therefore take much longer than others to move from one setting to another or to complete a given task. To overcome Staff need to be skilled in the monitoring and maintenance of hearing aids, cochlear implants and other assistive devices As Teachers of the Deaf we all know that hearing loss can lead to difficulties in communication and cognitive development, which in turn can result in frustration and behavioural problems. For children with complex additional needs and mental health issues, the obstacles can be huge. As a school and college we need to address them with a wide and very flexible range of teaching and therapeutic approaches. At RSDCM some of our pupils and students may communicate using objects of reference or hands-on signing, others may be fluent in BSL or have recognisable speech. In the classroom, teaching and learning are supported by integrated use of visual resources such as Widgit symbols and photographs, as well as interactive ICT. We also have young people who rely heavily on their hearing, so staff need to be skilled in the monitoring and maintenance of hearing aids, cochlear implants and other assistive devices. Catering for such diversity, often within the same class group or residence, can sometimes seem like spinning plates but we are fortunate in having skilled support staff and a team of specialists on site. In terms of audiological assessment, establishing accurate and reliable hearing thresholds can be an impossible task. Children with severe/global developmental delay are often unable to 20 BATOD Magazine September 2010

23 Aetiology use modified distraction or visual reinforcement audiometry with learners who are above the normally expected age for such techniques. communicate a response to sound stimuli in any consistent way. However, we are in a position to monitor and assess auditory behaviours and use of hearing aids (including cochlear implants and other amplification/assistive devices) in the real world on an ongoing basis, when this may not be possible in the restricted timescale of a hospital appointment. Admittedly, our audiological assessment methods may be somewhat unconventional at times strict protocols have to be abandoned for many of our complex youngsters but even circumstantial evidence can provide valid information to support professionals at our students own local audiology services. This is important for appropriate and successful hearing aid programming. Many of our youngsters are fitted with hearing aids programmed according to prescriptive targets in the absence of a reliable audiogram, but what happens if over-amplification at an early stage results in rejection of hearing aids? Or if an unrecognised deteriorating loss results in too little amplification? It can take time and creativity to devise alternatives that catch genuine auditory reactions To make informed judgements we need to modify the conventional test battery to suit the developmental levels of individual students, and a comprehensive knowledge of the students by key workers can help to inform the choice of test or how it is best attempted. We might, for example, tailor response tasks or materials that engage the individual pupil s specific interests or abilities, or Even for those children who are able to grasp the concept of response tasks, issues other than hearing may affect their responses. No-sound trials can identify false positive responses in most cases, but difficulty with auditory processing, rather than simply guesswork, can be the cause of delayed or inconsistent responses. For youngsters with autism, some of the more common response tasks used for play audiometry (such as putting pegs in boards or men in boats) may become a distraction if behaviours associated with autism demand that things be immediately lined up or made into patterns. It can take time and creativity to devise alternatives that catch genuine auditory reactions. Lack of confidence can result in a set of seemingly consistent responses that are minimal rather than threshold (threshold being the level at which sounds are perceived for approximately 50% as opposed to 100% of the time). Often the problem is simply very limited concentration associated, for example, with ADHD. Whatever the cause, it requires both close observation and a good deal of time to obtain accurate information. The key to success in all areas is having well-trained and committed staff In the school and college setting, we are in a position to be flexible and to have this time. We are not bound by an appointments system made weeks in advance; if a child is having an off day we can simply reschedule several times if necessary. We have the significant advantage of being known to the students they are assessed in a familiar environment, accompanied by familiar staff, so hospital phobia is avoided. This can be particularly significant, for example, when it comes to taking ear mould impressions from some of our autistic youngsters, who may be touch averse. On one occasion last year patience paid off after nearly three months of weekly visits and we were able to reintroduce hearing aids that had been abandoned for some considerable time. Our pupils and students are diverse and as such are individuals. For us, the key to success in all areas is having well-trained and committed staff who know and understand our young people and put them at the heart of everything we do. Wendy Eadsforth is the Principal and Chief Executive Officer and Jill Millward is a teacher and educational audiologist at the Royal School for Deaf Children Margate. BATOD Magazine September

24 Aetiology The Usher child Do you know someone with Usher syndrome? Marylin Kilsby describes the symptoms and different types that have been identified Sight is precious to deaf people. However, about five per cent of those who are born with affected hearing, including some with a progressive hearing loss, have Usher syndrome. There is every chance that any professional working with deaf children and young people will come across at least one Usher child. Children and young people with Usher will have the eye condition retinitis pigmentosa (RP) and may also have poor balance. Usher is hereditary and is caused by a recessive gene. The word recessive in this context is often signed hidden, for this is what it is. Each parent of an Usher child will have an Usher gene. However, the Usher gene is hidden by its corresponding normal gene and the parent will show no symptoms of hearing or sight loss. If each parent passes on their Usher gene to the child, that child will have Usher. If you know a deaf child who develops RP, his or her deaf sibling is also at high risk of developing RP as well. RP is a term which describes a group of hereditary diseases of the retina. Even within Usher syndrome, the symptoms and progression of the RP vary considerably. The retina is the light sensitive tissue inside the eye where the first stage of seeing takes place. With RP the retina slowly degenerates and loses its ability to transmit pictures to the brain. In advanced stages, characteristic clumps of pigment appear on the retina. Spectacles do not help RP, nor is there any proven treatment which will prevent the deterioration of vision. Symptoms of RP include: night blindness and difficulty with seeing in dim light very slow or absent adjustment to varying lighting levels, for example when moving from a well-lit room to a darker corridor loss of visual fields, especially downwards at first. scotoma (areas of no vision within the visual field) disturbance of vision, for example flashing lights, afterglow of colours extreme sensitivity to bright light, glare from light surfaces and shiny objects, including glossy paper cataracts these tend to develop at a more advanced stage, although rarely during the school years. Although the symptoms of RP will be mild in the early stages, there are certain behaviours to look out for which could identify a child with Usher. The child: hangs on to you, or looks for a railing to cling on to, in poor lighting conditions often asks for the classroom lights to be switched on has difficulty in tracking a ball or a moving object complains about the bright lights hurting his or her eyes, for example outside in sunny weather may be taken by surprise by people or quickly moving objects like a tennis ball is clumsy for his or her age has frequent accidents such as bumping into people or low lying furniture often has bruises on his or her shins or forehead has poor balance especially in the gym or in the dark walks with feet turned out and legs slightly apart to maintain balance. Not all children with Usher will exhibit all these behaviours; however, a child who displays the majority of these should be monitored carefully. Often it is a member of the school staff who first suspects that a child might have Usher. So far, three types have been identified. Type 1 The child has a profound hearing loss from birth. Almost all of these children will have no vestibular function, so their balance is very poor. The symptoms are usually noticeable before the age of ten. Type 2 The child has a bilateral partial to severe hearing loss from birth, much worse in the high frequencies, with normal balance. The RP tends to start slightly later, often in early secondary school, but it can develop more quickly. There is some evidence that one of the subtypes of Type 2 may include changes of hearing in mid-life, but at school age the hearing loss remains stable. Type 3 The child seems to have normal sight and either normal hearing or a bilateral partial hearing loss at birth, again worse in the high frequencies. The deterioration of the hearing and diagnosis of RP commonly happen at roughly the same time either in adulthood or, occasionally, in children or teenagers. Balance can become affected over time. 22 BATOD Magazine September 2010

25 Aetiology There are very rare occasions where the sight deteriorates much more rapidly than usual this could indicate that an Usher Type 4 exists. Usually, the RP associated with Usher is very slow changing and most children will leave school with a good deal of useful sight. Research is currently focusing on two major elements: genetics, and treatment either to stop the RP from getting worse or to reverse its effects and improve sight. Each known type of Usher has at least two subtypes. For example, with Usher Type 2, there are three known subtypes: 2a, 2b and 2c. Usher Type 1 has six known subtypes. Only one subtype of Type 3 has been identified so far but researchers think that there is at least one other to be found. Research is continuing to find exactly where the Usher genes are for each subtype. Parents who carry exactly the same Usher gene have a one in four chance of producing an Usher child. If one parent has Type 2a and another has Type 2c, their child will not have Usher, although they will be carriers of both Usher genes. Genetics is not as simple as it sounds. Research in Sweden is looking into why the sight and hearing of Usher siblings can vary considerably. At Sense, we know pairs of siblings where one sibling will have worse hearing and the other may have poorer sight. Research into treatments is taking place in the United States, Germany, Australia and also in the UK. Interestingly, all the treatment options relate to either blocking the deterioration of the sight or reversing the effects of the RP. Possible future treatments include: gene therapy (to replace the Usher gene) stem cell treatment (to introduce stem cells which can grow into a healthy retina) drug-based therapy (capsules of drugs are implanted into the eye) retinal implants. Retinal implants are not nearly as well advanced as cochlear implants as they only allow the user to see shapes, not fine detail or colour. However, clinical trials have taken place for implants with 16 and 60 electrodes and the number of electrodes in the implant will increase in the future. So, do you know someone with Usher? Changes in the retina, which suggest that the child has RP may have been detected when the child was assessed for a cochlear implant. If the child has not been considered for an implant, the slow and subtle changes associated with RP could still be taking place. Perhaps you already work with a student who has Usher syndrome. Diagnosis is half the battle. Once you know that your student has Usher, you are in a position to help him or her to maximise his or her sight and hearing in order to manage the challenges which Usher syndrome brings. Marylin Kilsby is the Usher Projects Consultant with Sense. (With thanks to RP Fighting Blindness for the definition of RP.) Emma s story My name is Emma Boswell (née Hancock) and I work for Sense as an Usher Information and Outreach Officer. I have been doing this role for four years now and Sense, in my mind, is a wonderful charity. I really enjoy my job and it is something I am very passionate about. I am currently undergoing a part-time deafblind diploma course. I have Usher Type 1 (profoundly deaf with retinitis pigmentosa and poor balance). I have had a cochlear implant for over ten years. My best achievements to date are my wonderful family. My husband Clive is also profoundly deaf and is in the process of having a CI. Together, we have two beautiful children and two dogs. Three years ago (the day before our wedding) we completed the purchase of our house (only just!) and then we completely refurbished it, carrying out re-wiring and plastering before putting in a new shower room, boiler, windows and doors, and finally redecorating the whole house inside and out. I recently returned to work from my second maternity leave. My children are Lucybelle, aged two, and Thomas, a one year old. An age gap of 13 months! Having my guide dog Drew is another great achievement and has helped boost my confidence and my mobility. Just five years ago, I never thought I would have a house, a husband, two children and two dogs! Never in my wildest dreams! BATOD Magazine September

26 Aetiology At Donaldson s Purpose-built accommodation, a multi-disciplinary team and child-centred intervention strategies are key to the approach at Donaldson s, as Joe O Donnell and Marjorie Douglas reveal Situated in Linlithgow, Donaldson s is Scotland s national school for children who are deaf or have speech and language difficulties. The age range of the children is from two-and-a-half years up to 18 years and our work also includes significant time focusing on the transition into further education or full-time employment. Although widely recognised as a school for the deaf at the moment 61% of our children have a diagnosis of hearing loss increasingly many of our pupils have a range of additional complex needs arising from a variety of aetiologies. These aetiologies include syndrome or chromosomal abnormality (22%) pre- or post-natal infection (11%), hereditary (16%), drug or alcohol abuse during pregnancy (5%), and premature birth (5%). In total, 17% of deaf pupils have an additional diagnosis of autism spectrum disorder (ASD), with many others displaying associated traits of ASD without a formal diagnosis. The diverse range of aetiologies leads to a very high degree of variability within the school population. In addition, it is estimated by NDCS that 40% of children diagnosed with deafness have additional difficulties. These two factors result in an extremely heterogeneous group, giving rise to significant challenges for service delivery. Although there are significant educational implications for all deaf children, the needs of our population are greater and require an even more co-ordinated, intense and focused approach to education. Our service delivery in Donaldson s has developed with this in mind, beginning with purposebuilt accommodation, the establishment of an onsite multi-disciplinary team and the continual development of appropriate child-centred intervention strategies. The building meets recommended acoustic standards for deaf children. The available technology ensures that all children have the best possible access to the teacher s voice, and includes the use of Soundfield systems and personal radio aids in an integrated way in all classrooms. Consideration has been given to lighting, with emphasis on the appropriate use of natural daylight. Public areas are colour coded to indicate the designated purpose of each part of the building and to provide a visual cue to aid pupils transition and movement around the school. Visual symbols, pictures and photographs are used throughout the school to enhance understanding, communication and learning at all times. All classrooms have interactive smartboards which are connected to the Soundfield and radio aid systems to allow all children to participate in a variety of active learning situations. Donaldson s communication policy is one of inclusive communication within a sign bilingual environment. This has developed as a direct result of the changing population of the school and it allows us to adapt our teaching and support to match the optimal communication mode for each individual child. This differs from the historical view of communication policy in deaf education, where a specific mode of communication was used throughout the school, and the pupils who attended that school were encouraged to develop through that mode of communication. Our inclusive communication policy validates all modes of communication, with the focus on the successful comprehension of other people s communication and the conveying of messages to others. Communication may be through picture exchange, Sign Supported English, spoken English or full British Sign Language. We strive to achieve this through an integrated multidisciplinary team approach, centred on the child. This team can include the child, family, teachers, speech and language therapists, educational audiologist, physiotherapist, occupational therapist, school nurse, optometrist, a range of classroom support staff (including many deaf role models), deaf tutors and residential care staff. Working together seamlessly, the team produces an annual communication profile and broad targets for an individual education plan for each pupil. This document is central to the approach that is implemented by all members of the team over the coming year. Donaldson s accepts pupils from a large geographical area which brings its own challenges in terms of keeping parents involved and actively engaged in their child s educational programme. We maintain contact through a range of means, such as parent mail and a parent forum for wider sharing of information between school and parents, as well as between parents themselves. Feedback from parents on our holistic approach suggests that our flexibility to be child centred, rather than fitting children into one particular methodology, enhances the likelihood of children reaching their full potential. This gives parents confidence that we have a shared perspective of the whole child and that we can work together dynamically to address the child s often changing and complex needs as he or she moves through school. Joe O Donnell is an educational audiologist, and Marjorie Douglas is Head of Speech and Language Therapy at Donaldson s. 24 BATOD Magazine September 2010

27 Aetiology Training needs In one of our occasional articles on learning support, Anne Gough describes the levels of skill and training required by staff to deliver the high standard of support at the Seashell Royal School Manchester The Seashell Royal School Manchester is a co-educational and non-maintained school for learners aged between two and 19 years who have complex learning disabilities combined with significant communication difficulties, including little or no oral language. The learners needs arise from sensory and multi-sensory impairment combined with severe and/or profound learning difficulties, autistic spectrum disorders and physical and medical conditions. There is a strong multi-disciplinary team at the school, including speech and language therapists, physiotherapists, occupational therapists, audiologist and nursing staff who work alongside education and residential staff to devise and deliver individual learning programmes for students. The development of effective listening skills and communication is an integral and focal point of all teaching at the Royal School Manchester. Communication, listening and attention skills are taught throughout all subject areas. High staffing ratios at the school enable learners to follow individual programmes that are planned and supervised by Teachers of the Deaf and delivered by teaching assistants. A high level of skill and training is required to ensure that teaching is effective. Newly recruited staff have a two-week induction period of training which takes place in the Seashell Trust s Professional Development Centre. Training is delivered by the range of professionals on site and includes deaf awareness and disability awareness to help staff experience and understand what it might be like to have a sensory impairment. They are issued with ear plugs and blindfolds and experience being fed, washed, hoisted into wheelchairs and standing frames, pushed and walked around site and then they get to experience a lesson without sight or sound! In addition, staff complete a compulsory communication course designed and delivered by Seashell Trust s speech and language therapists that focuses on the range of communication needs across the school and college. This course helps to inform staff about the importance of building relationships with the learners and how they can help others communicate by developing their own core communication skills. It covers the areas of intensive interaction, developing the receptive language of learners to aid understanding and the different ways of helping learners develop their expressive language skills. There is a two-day behaviour management training course to help staff develop a low arousal understanding approach to dealing with challenging behaviour. Other training includes awareness of autism and the impact of autism on communication, hearing aid maintenance, manual handling, risk assessment, curriculum training and training in personal care and feeding. In addition, many of our teaching assistants have higher qualifications in profound and multiple learning difficulties, multi-sensory impairment and visual impairment. Teaching assistants all have the opportunity to gain BSL level 1 and NVQ level 3 while employed by the Trust. Signing instructors and NVQ assessors are employed by the Trust and are on site. Although general training around this complex population of learners is beneficial, it is in practice where the skills of the teaching staff are developed when working with individual students alongside the audiologist, therapists and teachers. Learners often require bespoke communication systems and this level of multi-disciplinary working ensures that programmes are delivered effectively. Most learners are very wary and cautious about assessments in the clinic and display some anxiety about undergoing a hearing test. They are often very anxious about their ears being touched and being given amplification through hearing aids and it is often not clear whether this is due to dislike of amplified noise and sounds or because they have had experience of loud sounds being over-amplified in the past. Most learners benefit from wearing hearing aids but are often touch sensitive and have difficulties processing sound. Slow introduction to amplification is essential and most learners benefit from exploring sound and vibration in a wide range of settings. Early listening skills are more likely to develop effectively in highly motivating situations such as sensory rooms, using the computer or the sound beam, in the gamelan ensemble or during sessions of live music. Prior to the use of hearing aids, amplified sound is sometimes delivered through portable amplifiers and small headphones so that learners can start to get used to the controlled amplification of sounds that they may recognise and enjoy. Anne Gough is a Senior Teacher for Sensory Support at the Seashell Royal School Manchester. BATOD Magazine September

28 Aetiology The road to Suryapur Drawing on her experiences in a school for deaf and blind girls near Kolkata, Ruth Tolland outlines some of the issues facing deaf education in India early next year with a volunteer teacher of the visually impaired. The school has a hostel for 100 deaf girls aged 6 22 years and 40 blind girls; 60 of the deaf girls attend the school and the rest work for Suryapur or have vocational training. The school also runs the new upgraded two-year ToD training course attracting trainees with entry qualifications varying from school-leaver status to Master s level. The World Health Organisation estimates in 2005 suggested that there were 278 million people worldwide with disabling hearing impairment and, of these, 63 million (6.3%) were found in India. Indian nationwide surveys have concluded that hearing loss is the second most common cause of disability after locomotor disabilities; 66.3% of conductive loss in India (often permanent) may be caused by chronic suppurative otitis media (CSOM). Ear disease, particularly discharging ears, tends to be ignored because of factors such as overcrowding, illiteracy, poor hygiene and nutrition, higher rates of nasopharyngeal conditions, lack of knowledge and insufficient healthcare. Other causes of deafness in India include newborn infections, low birth weight, poor care during pregnancy, lack of vaccination for rubella and measles, polluted water, consanguinity and poor drug administration for malaria, tuberculosis and antibiotics. One study of 1,076 children with sensorineural hearing loss in Hyderabad showed that 41.7% were born of consanguineous parents and of these 44.5% had non-syndromic deafness. Suryapur is a special residential school for deaf girls and blind girls situated in a small poor farming village outside Kolkata (formerly known as Calcutta). It is run by the Ramakrishna Vivekananda Mission (RVM), a large charity in Kolkata which helps impoverished children regardless of religion or caste. In 2008 I was invited to volunteer to set up audiology and improve teaching standards within the school. So, as a recently retired educational audiologist with a longstanding interest in India, having travelled extensively there over the years, I was delighted both by the prospect of helping and also by the opportunity to live within a community rather than to be a mere traveller. I have now worked for two periods in the school, spending nine weeks there to date, and will return All girls are from poor or destitute families; some are orphans. They arrive at any age after the age of six with no speech, sign language or education and can be troubled children as a result of maltreatment and rejection. Unfortunately, disability is regarded as a stigma within the culture, and girls are particularly vulnerable and most likely to be neglected. They are given free accommodation, food, clothes and basic education. At school they are placed first in Lower and then in Upper Kindergarten classes until they are ready to commence Class One. Currently the age range is 6 13 years in these classes. A very few will eventually achieve School Certificate level, equivalent to GCSE, by the age of 21. For my first visit, I was able to provide some donated test equipment, including an audiometer and an invaluable handheld tympanometer. I worked with a newly appointed audiologist and all girls were tested most were found to have a profound hearing loss, and a few had a severe loss or better; 66.6% of girls had mixed hearing loss, many with long-standing CSOM. A basic ENT referral system was set up and some girls received eardrops or antibiotics. A number of girls had been issued with rather old government body worn (BW) hearing aids at some point; however, most lacked sufficient amplification and few were working. The ear moulds too were old and ill-fitting and 26 BATOD Magazine September 2010

29 Aetiology Indian Sign Language. Despite a shortage of trained instructors in West Bengal, a suitable person was found during my second visit to teach one day each week. We were very lucky, as potential teachers are often deterred by the difficult journey to Suryapur. The effect on the girls morale was immediate. Lessons have continued and all girls and teachers are timetabled for a session. were often temporary ones, which meant that the BW were worn essentially as ornaments. The best of the BW aids were picked out for repair. During my second visit, work began again with another new audiologist, systems were put in place, the ENT referral was reinstated and 42 girls were scheduled to be seen. Ten girls with better speech potential were fitted with new post-aural hearing aids purchased in Kolkata with donated money, and five more received donated post-aurals. Hard acrylic earmould making was set up in the school to cater for the BW, and biopore moulds were purchased for the postaurals, although this was expensive. Auditory training and speech work was begun for all younger girls and older girls with potential, and the audiologist, who fortunately has an additional qualification in speech and language, was keen to implement a programme. A free weekly out-patient clinic was set up for adults and children living in the surrounding area and has proved successful, although only testing and advice are available at the moment. A room for audiology testing was identified and suggestions made for sound treatment, as the current room has background noise levels around 67dB. The new room is near completion and I m hoping it will be much quieter! Noise pollution is a big problem in India and the situation is not helped by concrete rooms with high ceilings and noisy fans, essential for the intense summer temperatures. On my return home this time I received a number of donated high-powered post-aurals which were taken out to the school by another visitor. Unfortunately they have not been fitted to date due to the cost of moulds and button batteries. To reduce costs in the future it will be necessary to find a suitable course in Kolkata to train up a few people so that better quality moulds can be made on site perhaps this will happen during my next visit if a suitable instructor is found. It was clear from the start that the school signs in use were insufficient to deliver an adequate education and that there was little common language between teachers and pupils. The teachers acknowledged the problem and were keen to have proper instruction in The teachers are mostly trying their best but are constrained by lack of resources, low pay and difficult daily commutes. In class there is a lack of suitable Bengali textbooks with simple language and plenty of pictures, with the result that the main teaching method tends to be talk and chalk, where the girls copy and learn by heart, too often without understanding. The audiologist and I had joint sessions with the trainee teachers three times a week and weekly workshops were held with the teachers. I particularly enjoyed working with the latest first year group of trainees. Older girls require vocational training as some may not return home and marry. Additional dowry is expected when a girl has a disability but families are often unable to provide it and become reluctant to accept the girl back. RVM has helped to arrange a few marriages and provided a dowry from donations of jewellery and saris. Currently the girls make exercise books, folders and chalk for other RVM schools, and aprons, which are sold. Working in Suryapur has proved to be an intensive but highly rewarding experience. The wonderful smiles of the girls who expect little from life is the best reward. During my first visit one 11 year old was determined to tell me how her father used to beat her but now she was in school with clothes, food and oil and a hostel mother she loved. On my last day she stood in front of me until she was satisfied I was returning to Suryapur although explaining it was not until November proved too difficult. The final assembly this time was overwhelming and I am already looking forward to seeing everyone again. Ruth Tolland is a retired educational audiologist. BATOD Magazine September

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32 Feature Cochlear implants not always enough? Bengt Danielsson and Ola Hendar consider the impact that the development of cochlear implants has had on the rehabilitation of deaf children in Sweden For nearly 20 years cochlear implants have been recommended for children who are born deaf or lose their hearing during childhood or adolescence. During this period there has been a dramatic change in the speech and hearing development of children previously profoundly deaf. The technology has developed over the years, younger children are operated on and rehabilitation is more efficient. The concept of bilingualism in special schools for the deaf and hearing-impaired has also changed during this period, from only including sign language and written Swedish to also including spoken language. The pupils increasingly ask for a combination of the two languages and for technical equipment. There are still pupils who are profoundly deaf and need to be taught in sign language, and special schools offer all pupils as good a language environment as possible. With the arrival of cochlear implants many areas of existing rehabilitation have been questioned, for example early parent support, sign language and choice of school. One reason for this seems to be that parents no longer see their children as having a disability but as normal children with the most modern hearing aid in the world. The status of sign language is questioned, both as a language and as a mode of communication, because there is uncertainty as to whether it supports speech development in children with cochlear implants. The choice of school is questioned because of uncertainty over whether the bilingual environment in special school is right for the child. These changing ideas about rehabilitation might be appropriate for one group of children with cochlear implants but should they apply to all cochlear implanted children? Longitudinal studies of children with cochlear implants mainly point in three directions: Children with cochlear implants have considerably better speech production and listening comprehension than deaf children without cochlear implants. Children with cochlear implants do not have speech production and listening comprehension on a par with normal hearing children. The distribution of results on speech production tests and tests for listening comprehension is noticeably wide for children with cochlear implants. The explanation for this can probably be found in the cause of the child s deafness. The underlying causes and the consequences of deafness are complicated areas. This explanation is used by special schools for the deaf and hearing-impaired to explain the wide distribution of results in tests and achievement of targets. We know that children with congenital difficulties or disabilities do not achieve the higher marks that others do. It is also known that they need more support during their school years. There is a lot of impressive development in technical aids and rehabilitation to support these children in the best possible way. But do we do enough? Bilingualism is positive for everyone, in all languages. This seems to be the case for everyone but children with cochlear implants. The Swedish Association of Hard of Hearing People advocates that all hearingimpaired people should be offered sign language. Some parents of children with cochlear implants want more sign language. Parents of normally hearing children use sign language to stimulate language and cognitive development. Why then do some people believe that sign language hinders children with cochlear implants in their rehabilitation? Studies in neuropsychology show that there is probably a critical period for vision, hearing, language and cognition. If children are not stimulated during this period, there are consequences for their whole development. If good quality communication does not start (irrespective of whether it is spoken or signed), this will have an effect on the child s whole cognitive development. This helps to explain why we get such a large distribution in results. Early rehabilitation is about parents finding their child s level of development and meeting the child there, both emotionally and pedagogically. The disability must not be forgotten in everyday life. It is unfortunate that parents of children with cochlear implants see them as normal children with a delay in language development. Hearing age is seen as a measure of the child s possibilities in everyday life. But there is a difference between a one-year-old child who can utter two words and an eight-year-old child 30 BATOD Magazine September 2010

33 Feature who can only utter two words. The consequences are dramatically different. Even if these two children have the same hearing age they need totally different rehabilitation. They share the need for good stimulation and a good attitude, irrespective of communication mode. Current research shows that cochlear implants do not yet result in normal hearing and communication development. What the future holds we do not know, but we expect the results to improve. Today we see that children with cochlear implants are different from children with language disorders. They develop differently and need different rehabilitation. It is not possible to control fully the factors that are crucial for the outcome of the cochlear implant, therefore it is vital not to place the child in a situation that can have longterm negative consequences. The variation among children with cochlear implants is large. Our experience in teaching indicates three different groups. The first is a group of pupils who develop their speech and hearing to the extent that they can attend mainstream classes on their own. The second group can also attend mainstream school, but need small classes, technical aids and sign language support so that they do not miss too much of the teaching. In the third group, the need for sign language is too great to attend a mainstream class, and a special school offers flexible solutions. From our experience, and from research presented so far, we recommend that rehabilitation for children born deaf or hearing-impaired, or those who become deaf in early childhood, should have a broad base, irrespective of degree of impairment or technical aids. Special schools from now on will be offering schooling for those pupils who want a bilingual environment and those whose needs will not be satisfied in any other type of school. To improve the results in school for all pupils with hearing impairment, irrespective of aids and language, we propose that all parents are offered sign language in the early rehabilitation stages. The idea is that it enhances the child s cognitive development. The first four years in a child s development are too crucial to be devoted to uncertain mono-linguistic learning. The child and parents can decide later during childhood or adolescence if they will continue with both languages, but during the first four years all children and their parents should be included in the programme. Sign language and spoken Swedish can be developed at the same time. The school management support section at the National Agency for Special Needs Education and Schools is keen to contribute actively to the evaluation of different parts of all deaf pupils development. Common parameters for education and rehabilitation are, from our point of view, important areas to develop. Our hope is that this will lead to positive developments that benefit the education for the deaf in the future. This article was first published in the Journal of the Swedish Medical Association: Läkartidningen 46/2009 ( Bengt Danielsson and Ola Hendar work for The Swedish Agency for Special Needs Education and Schools. The medical, pedagogical, communicative and psychosocial rehabilitation should not be focused on normalisation of the child regarding speech and hearing but on the importance of active communicative support during childhood and adolescence to give the child the best start possible. Special schools should be schools for all pupils who cannot attend mainstream school or who want a bilingual environment. The mandate of a special school is twofold on the one hand to offer a school for pupils who cannot attend another school; on the other hand to offer teaching to the pupils who, through their parents, actively look for a bilingual environment. This constitutes a pedagogical challenge, as special schools have the same goals as mainstream schools. The proportion of pupils in special schools who want their whole schooling in sign language is decreasing. At the same time the number of pupils who want a combination has increased. Special schools have therefore redefined the concept of bilingualism and work actively to organise their work in the best way possible. BATOD Magazine September

34 Feature Higher level thinking With young people increasingly expected to use higher level thinking skills in their learning and examinations, Wendy Booth-Boyd explains how to help deaf secondary school students to rise to the challenge Within the classroom and within every exam syllabus there is now the implicit expectation that higher level thinking skills and their terminology will be used by students and assessed in exams. Gone are the days of O-levels in which it has been estimated that in a staggering 95% of both lessons and exams, the student was required to simply regurgitate knowledge; students were only required to apply, use and manipulate information for the remaining 5%. Nowadays these percentages are almost reversed. Enabling our students to use the vocabulary associated with this change is vital. Bloom s taxonomy Once upon a time Year 11 students could just about manage the chore of ensuring that they d learnt the original BATOD list of exam vocabulary. Now the whole exam process is filled with additional terminology and requires different answers and structured responses involving analysis, evaluation and synthesis of the facts and information. Exams increasingly incorporate all levels of Bloom s taxonomy (see Figure1), often in quite linguistically challenging ways. The vocabulary involved in the basic, low level thinking skill, knowledge, is now only the foundation of both learning and examinations. Students now need to illustrate their abilities and show their understanding of the curriculum content by using and responding to more abstract terminology. Students are now asked to demonstrate the skills of comprehension and application, and perform even more challenging tasks involving analysis, synthesis and evaluation. Figure 1 Higher order thinking skills Evaluation Synthesis Analysis Application Comprehension Knowledge Lower level thinking skills It is assumed that our students (and their hearing peers) must use and have a working knowledge of words associated with each skill area (see Bloom s taxonomy verb wheel Obj_tutorial/bloomwheel.html). The vocabulary of lessons, tasks given in class, homework and examinations all now incorporate this higher level of language. Within all this, it is easy to see the extreme difficulties that some of our deaf students will face processing, applying and exhibiting these skills. It s such a lot of words. It involves a lot of quick thinking and a good grasp of both concrete and abstract concepts. Students at all Key Stages still need a thorough understanding of their subject-specific vocabularies. They also need the ability to transfer and then apply concrete information to a variety of alternative situations and processes. Awareness of this vocabulary is key, not just to jump through the examination hoops but also to promote their independent learning and their understanding of complex matters into their futures. As this is such a necessary part of the curriculum it raises several questions for us, as Teachers of the Deaf. Is it possible to tutor our students in these higher level thinking skills? Should we teach to the exam, to the (inevitable) exclusion of so many other things? How can we ensure that our students are able to take on board and use this new terminology with confidence and competence? Will practice in these higher level thinking skills positively contribute to their lifelong learning? Should this now be the basis of the ToD/LSA s work, with the students during KS3/4 or during the years before that? As well as exam vocabulary? As well as topic vocabulary? As well as everything else? Obviously, some schools and exam boards are embracing these ideas more than others. However, many GCSE, A-level exams (even some Key Stage 2 SATs questions) are incorporating Bloom s higher level thinking skills into their question types. It is worth bearing in mind that for a school to achieve a good or outstanding Ofsted inspection grading, the majority of teaching seen by the inspectors and watched as part of the school s ongoing self-evaluation needs to demonstrate the new buzz words high impact learning, assessment for learning, higher level thinking skills. They expect it. 32 BATOD Magazine September 2010

35 Feature Finding the best way of learning It can be an additional string to your bow as a ToD to elicit, via various methods, the preferred visual auditory kinaesthetic (VAK) learning style of each student. Finding out whether they are a visual, auditory or kinaesthetic learner can be useful information to have at your fingertips. When they tackle something new do they like to see demonstrations, diagrams, slides or posters (visual)? Do they prefer verbal instructions or enjoy talking it through with someone (auditory)? Do they prefer to jump right in and try it out and then modify actions or plans later (kinaesthetic)? When they spell/learn spellings do they picture the word in their mind (V)? Do they sound out the word or use a phonetic approach (A)? Do they write out the word over and over until it feels right (K)? and thrive creating videos, PowerPoints, animations, flowcharts or matching activities, such as Tarsia puzzles, during their revision, even creating their own mind maps. The six W Framework I was introduced to this innovative format for developing higher level thinking skills during an in-county training session. It can be applied to any visual stimulus or written fact (see Figure 2). Once students preferred learning styles are established, then employing relevant teaching techniques and styles and supporting them with appropriate activities can vastly improve their levels of learning and understanding. Awareness of their strengths enables teaching methods to be used which will capitalise on these strengths and play to students preferred mode of learning. It enables them to work in their most effective way. I must be honest, as a visual learner myself, having this awareness, makes it much easier to see where my students are coming from. Mulitiple intelligences Multiple Intelligence groupings are another aspect that can be used to establish students preferred mode of learning. Are they linguistic, logical, spatial, musical, kinaesthetic, naturalistic, interpersonal or intrapersonal learners? Again, knowing this will help establish how your students like to learn, what they are good at and how they learn best. For example: kinaesthetic learners like to move around, talk and touch and use body language in their learning. They would be good at physical activities (movement/acting/dance/sports) and crafts and would learn best by touching, moving, interacting with space and processing knowledge through bodily sensations. Using post-it notes with key facts or questions and their answers randomly placed around the room, to be collected in the correct sequence, or creating a dance/rhythm to remember the main points are two interesting techniques they might use. Spatial learners, however, like to draw, design, build and create things, daydream, look at pictures and slides, play with machines and watch films. They can be good at: imagining things, sensing changes, completing puzzles and reading maps, charts and tables. They would usually learn best by visualising, dreaming, using the mind's eye and working with colours and pictures. Spatial learners would enjoy Figure 2 It challenges students to think of both concrete and abstract questions and apply them to the picture/word and then create their own answers. It can be used to present a new topic and to find out what facts and aspects of the topic are already understood. If it is then revisited at the end of the topic, a comparison can be made, and the progression of the student s understanding and knowledge can be illustrated and recorded. The framework can also be used as a summary of key points and as a quick revision aid at the end of the topic. Around the stimulus are areas with the six key, W questions within them (Who, What, Why, Where, When and How). The students think of all the questions that could apply to that subject and then add them to the framework. Then they answer them. As the teacher, once you have the format of the page established (as a Word document) it can be used BATOD Magazine September

36 Feature over and over again by just inserting different pictures in the middle. Once the students are familiar with the process and format they can use it independently and readily. Having used this framework with success, especially in the humanities subjects, we modified the format to enable the students to structure and plan their essays for the describe type questions on our GCSE English syllabus (see Figure 3). The students regularly practise using the describe framework throughout KS4. Repeatedly using this framework ensures that they remember to use the points they write in each box as a distinct paragraph in their writing. Each box ideally has three significant things to describe and three key language devices associated with each of them. (Not every box has to be used for every essay some are more relevant than others.) Familiarity with the process, gained by using it repeatedly throughout their GCSE course, ensures that students in the resource base (RB) use it during their exam in the planning and writing of their answers. Implementing this in an RB setting I started on a small scale using questionnaires, available freely off the internet. During RB lessons with one Year 10 student we established the favoured learning style (it was visual). Together we researched and discussed ways of studying and revising that might work best. As part of our writing of the individual education plan we chose one subject area that needed specific vocabulary work. In the RB lessons we focused on activities to support this learning of subject-specific terminology, key concepts and the relevant higher level thinking skills. Using the subject department s own scheme of work and key word list and our own notes from lessons, I undertook to create a pack of visual resources as exemplars. The aim was to furnish the student with a wide variety of modes of visual presentation within that one curriculum area. Devising a wide variety of visual techniques that could be applied to the material that had been presented in class gave us food for thought. Discussing the process, the images, fonts and colours was a positive and valuable exercise. We then used the pack as the basis for several of our RB lessons. They were practical examples of how to use the strengths of individual learning styles to learn, consolidate and revise. It was useful to demonstrate the different visual applications and their relevance to specific subject topics and exam questions, instead of just explaining the theories of VAK and multiple intelligences and leaving the student to apply them. The student experienced a selection of strategies that he could use in the future and adopt the best, most useful techniques Figure 3 thereafter. It was a much more interesting way of presenting the material. It made the teaching and learning different and more relevant. It was more readily understandable too, as it was tailored to the favoured learning styles. This was used (and still is) to inform RB lesson planning. We use as many visual type strategies as possible. The pack itself was subsequently used by the student during GCSE revision. It was an alternative method to simply teaching/re-teaching/ re-interpreting main school lesson content in our oneto-one lesson. It also supports our school s approach to the high impact learning and increased higher level thinking/language skills that are used, expected and incorporated into the students everyday learning in lessons. From this individualised success, we ve rolled it out and now use it productively and beneficially with all our students. Higher level thinking skills continue to have a profound effect on the students during their encounters with the demands of the current curriculum. Supporting hearing-impaired students within a secondary school comes with increased additional challenges. To adapt/adopt methods to enhance students learning can only be of benefit to Teachers of the Deaf and their students. Wendy Booth-Boyd is a Teacher of the Deaf with the Somerset Hearing Support Team. 34 BATOD Magazine September 2010

37 VI facts and figures Feature Studies exploring the educational attainment of blind and partially sighted pupils and the effect of an additional SEN could have implications for deaf pupils, as Sue Keil reveals According to statistics published by the DCSF, based on the returns from the School Census, pupils with visual impairment as their primary special educational need (SEN) perform better at GCSE than all other SEN groups. These figures relate to maintained schools in England only and apply only to young people with a statement or those on School Action Plus (DCSF, 2009). Although pupils with a visual impairment do well in comparison with the other SEN groups, the published statistics indicate that their attainment is still considerably below that of their non-sen peers. The RNIB has calculated (based on statistics published by DCSF) that in 2008 half (50%) of pupils in England with a visual impairment as their primary SEN achieved five or more A* to C grades at GCSE compared to nearly three-quarters (74.6%) of pupils with no SEN. A third (34%) of pupils with a visual impairment achieved five or more A* to C grades including English and maths while just under three-fifths (57.8%) of their non-sen peers attained these grades. A serious limitation to the published DCSF statistics is that they do not apply to the whole population of blind and partially sighted pupils. They exclude pupils whose secondary SEN is a visual impairment and pupils who are on School Action. In addition, the published statistics only tell us how well blind and partially sighted pupils as a group perform in comparison to other groups of pupils. Yet blind and partially sighted children constitute a diverse population, with many having additional disabilities and/or SEN. In 2007 a quarter of pupils at School Action Plus and a third with a statement, whose primary SEN was visual impairment, had a recorded secondary SEN. The diversity of the blind and partially sighted child population has been highlighted by Miguel Perez-Pereira and Gina Conti-Ramsden (Language development and social interaction in blind children, Psychology Press Ltd, 1999), who suggest that it is often more meaningful to carry out comparisons between blind and partially sighted children than between the blind and partially sighted population and other groups. The research study In 2009, the RNIB commissioned the National Centre for Social Research (NatCen) to carry out detailed analysis of government statistics in England, Scotland, Wales and Northern Ireland. The aim of this research was to find out whether when factors such as an additional SEN, social disadvantage, ethnicity, gender, area in which pupils live and type of school attended are taken into account blind and partially sighted pupils still do less well at GCSE in comparison with pupils without special educational needs. In the remainder of this article the key findings for pupils at Key Stage 4 in England and Wales are discussed. Method NatCen analysed data from the 2007 national pupil datasets (NPDs) for England and aggregated data for for Wales relating to pupils at Key Stage 4 in maintained schools. The analysis included pupils whose primary or secondary SEN was a visual impairment. In Wales, but not in England, the NPDs included pupils on School Action as well as those on School Action Plus and with a statement. Pupils were divided into the following groups: pupils with no SEN ( No SEN ) pupils with a visual impairment as their only SEN ( VI only ) pupils with a visual impairment and an additional SEN ( VI plus SEN ) all other pupils with SEN ( Other SEN ). Key finding 1: Proportion of pupils with visual impairment and additional SEN England in 2007: at Key Stage 4 a total of 927 pupils were recorded as having a visual impairment as their primary or secondary SEN, representing 0.15% of the pupil population. Just over half (52%) of these pupils had another SEN in addition to their visual impairment (VI plus SEN). Of the 484 VI plus SEN pupils, 26% had moderate learning difficulties, 15% had a physical disability and 11% had a specific learning difficulty. Wales in : at Key Stage 4, a total of 230 pupils were recorded as having a visual impairment as their primary or secondary SEN, representing 0.2% of the pupil population. Just over two in five (41%) had additional SEN. The type of other SEN for these pupils was not reported. Key finding 2: Effect of additional SEN on educational attainment A key finding for both England and Wales was that although visual impairment did make a difference to educational attainment, the most important factor was whether or not a pupil had another SEN in addition to the visual impairment. As shown in tables 1 and 2, BATOD Magazine September

38 Feature there is a considerable gap in attainment between pupils with a visual impairment only and pupils with a visual impairment and additional SEN. In England there is a gap of 39% and in Wales it is 32%. The tables also show that the attainment of pupils with VI only is closer to that of the No SEN group while pupils with VI plus SEN attained at a similar level to all other pupils with SEN. In England, the attainment gap between pupils with No SEN and pupils with VI only achieving five or more A* to C grades at GCSE has narrowed to 10%. In comparison, there is a 49% gap between pupils with No SEN and pupils with VI plus SEN. already seen that in England pupils on School Action are not included in the schools census, and anecdotal reports from VI services suggest that some schools are recording pupils with visual impairment as being on School Action rather than School Action Plus. Another reason is likely to be that blind and partially sighted pupils with more than one additional SEN particularly pupils with the most complex needs are not recorded as having a visual impairment as their primary or even their secondary SEN. Some of these problems may relate to the vagueness around who is responsible for completing the PLASC and School Census returns, and questions have been raised about the consistency of the data. Table 1: GCSE results by SEN status pupils in England in 2007 SEN status Five or more Five or more Number of pupils GCSEs A* to C GCSEs A* to G No SEN 64% 94% 548,469 VI only 54% 87% 443 VI plus SEN 15% 52% 484 Other SEN 13% 54% 52,956 All 59% 90% 602,352 Source: Chanfreau and Cebulla (2009) from NPD England 2007 Table 2: GCSE results by SEN status pupils in Wales in SEN status Five or more Five or more Number of pupils GCSEs A* to C GCSEs A* to G No SEN 62% 93% 94,633 VI only 46% 89% 136 VI plus SEN 14% 47% 94 Other SEN 14% 54% 18,995 All 54% 86% 113,858 Source: Chanfreau and Cebulla (2009) from NPD Wales ( ) Limitations of the national pupil datasets Although this research has enabled us to unpick to some extent the national statistics on pupil attainment, because of limitations to the data recorded in the NPDs it has not been possible to consider some key factors that may affect the attainment of blind and partially sighted pupils. For example, the datasets do not record details of the type or nature of educational provision other than the type of school attended; pupils attending non-maintained special schools designated for blind and partially sighted pupils are also excluded. Most importantly, details of the nature, severity or age of onset of a pupil s visual impairment are not recorded. There are other limitations to the NPDs. National surveys of VI services carried out by the RNIB have consistently obtained higher estimates of the blind and partially sighted pupil population than the number represented in the government statistics. We have Discussion Despite the acknowledged limitations of the NPDs due to the nature of the data recorded, the RNIB felt it was important to start its programme of research into educational attainment by scrutinising the official statistics. The findings from this study have provided important insights into some of the factors that are associated with the educational attainment of blind and partially sighted young people. Importantly, they have given us a much more sophisticated understanding of the characteristics of the blind and partially sighted pupils who are represented in the official statistics. The evidence indicates that while having a visual impairment makes it less likely that a young person will attain five or more GCSEs at A* to C grade when compared with their peers with no SEN, having another special educational need in addition to a visual impairment very significantly reduces the likelihood of attaining at this level. The study has provided evidence confirming what specialist teachers and others who support the educational provision of blind and partially sighted pupils have known for a long time that pupils with a visual impairment are not a single, homogenous group. Further research is now needed to build on these findings. Topics could include comparing the attainment of blind versus partially sighted pupils, investigating the combined effect of visual impairment and specific types of additional SEN, and the effectiveness of different types of provision for different groups of pupils. Implications for deaf pupils The findings from this study may be of interest to 36 BATOD Magazine September 2010

39 Feature teachers and others providing educational support for deaf pupils. The DCSF published statistics indicate that at GCSE, pupils in maintained schools in England whose primary SEN is a hearing impairment are ranked just behind those of pupils with a visual impairment. For example, in 2008 half (50%) of pupils with a VI as their primary SEN attained five or more A* to C grades at GCSEs compared to 43% of pupils with HI as their primary SEN. However, this data tells us little about the characteristics of the deaf pupils, including the proportion with additional SEN. Yet epidemiological studies indicate that around 30% of deaf children have additional disabilities, while unpublished data from the DCSF indicates that in 2007 around a quarter of pupils whose primary SEN was a hearing impairment had a secondary SEN. Just as looking behind the published statistics for blind and partially sighted pupils has revealed a more complex picture, the same may also apply to deaf pupils. Conclusion The study found that for pupils in England and Wales having a visual impairment did make a difference to educational attainment, but the most important factor was whether or not a pupil had an additional SEN. This effect was sustained even when socio-demographic differences between pupils with and without SEN were taken into account. Further research is now needed to build on these findings. It is suggested that a similar analysis of the NPDs could provide similar insights into some of the factors associated with the educational attainment of deaf pupils that are not apparent from the published government statistics. A copy of the full research report, Educational attainment of blind and partially sighted pupils by Jenny Chanfreau and Andreas Cebulla, National Centre for Social Research for RNIB, 2009, can be downloaded from the RNIB website at Pages/edemp.aspx/ Sue Keil is a research officer with the Children and Young People s team at the RNIB. BATOD Magazine September

40 Feature Phonics guidance Trish Cope explores the NDCS document providing detailed advice on the teaching of phonics to deaf children in mainstream schools Literacy skills are critical to all future life skills. English is a core curriculum subject for all pupils, with literacy building on speaking and listening skills. For deaf pupils, writing provides a permanent visual record of fleeting speech sounds which may be a significant support to learning. Most deaf pupils (approximately 80%) are educated within mainstream education with about half individually placed in their local mainstream school. This means that they will be learning to read within the framework set out in the Primary Strategy. Phonics skills have always been an important element in successful reading for all children including deaf children and phonics was given a significant place in the original Literacy Strategy. The Rose Review (2006) increased its prominence during the early years, and synthetic phonics was recommended as the best way to ensure quick and accurate decoding, which is one of the strands in the simple model of reading. The other strand is comprehension, which may not be easily achieved by deaf pupils and requires specialist attention. The Rose Review made it clear that leading edge practice bears no resemblance to a one size fits all model of teaching and learning, nor does it promote boringly dull, rote learning of phonics. Deaf pupils may need significant additional teaching for both strands of reading, and the Rose Review makes it clear that specialised support is both available and necessary to ensure good progress in reading including phonics skills especially for those with sensory impairment: It was clear from these contributions that a wide range of expert support and guidance is available to help settings and schools match provision to need. While the Rose Review acknowledged that phonic work should be set within a broad and rich language curriculum that takes full account of developing the four interdependent strands of language: speaking, listening, reading and writing, the specific development of phonics skills now receives considerable attention during the Foundation Stage and Key Stage 1. In view of this, the NDCS decided that detailed advice should be available to mainstream teachers to enable them to meet the varied needs of deaf pupils. In 2009, it convened a group to write such guidance comprising representatives from BATOD, the Ewing Foundation and The Ear Foundation with NDCS as project manager of the group. This guidance is now available from NDCS (for a free copy helpline@ndcs.org.uk or call ). The guidance has been written for mainstream teachers but it is intended to be supported by the detailed knowledge and advice that ToDs can provide for individual pupils. The guidance is in two parts. The first contains general principles and the second has more detailed advice on the adjustments that may need to be made to the activities given in Letters and Sounds. Part 1 this section: sets out the context for including deaf pupils in mainstream settings especially in terms of literacy gives general information on childhood deafness points out the information a mainstream teacher will need to know about individual deaf pupils outlines how to make classrooms conducive to a deaf pupil s learning indicates some general techniques that may assist deaf pupils acquisition of phonics skills suggests ways of working effectively with families, teaching assistants and others. Part 2 this part describes a graduated approach to adapting any phonics programme to meet the needs of individual deaf pupils. Such a response ensures maximum progress with minimum change. For each phase of Letters and Sounds there is a description of learning objectives, general considerations for deaf pupils and comments on individual activities. Since Phase 1 has seven aspects, each one of those is covered in this way. This recognises that Phase 1 is a critical phase covering early listening skills, the development of phonological awareness and the emergence of blending and segmenting. Phase 2, which deals with the skill of linking sounds and letters, is also covered in some detail. As this section progresses through each phase, the coverage decreases. This reflects the group s feeling that once the key skills of blending, segmenting and manipulating sounds have been mastered, the following sections require less adaptation as long as consideration is given to all the general points highlighted previously. During Phases 3 to 7, the differing representations of the sounds of English are learnt and pace may be the most significant factor to consider at this stage. The adaptations that are suggested include: re-ordering aspects or activities within an aspect ensuring that sounds are audible but not uncomfortably loud practising activities individually or in small groups in a quiet room 38 BATOD Magazine September 2010

41 Feature breaking down learning objectives into smaller steps, for example additional steps to support the identification of rhyme providing additional adult support avoiding activities that aren t essential to the learning outcomes and may be more challenging than the skill to be learnt adapting activities so that children are not expected to listen and concentrate on other things as well, for example listening to a story, identifying words and making the appropriate response with a sound. Teaching assistants The roles that additional adults such as teaching assistants may undertake are also described throughout this section. They include: one-to-one additional practice, differentiated activities, working in quiet listening conditions and group work. Teachers of the Deaf Throughout the document the role of ToDs is explained and emphasised. Their role is explained in Part 1, and in Part 2 there is frequent reference to the specific advice that they can offer on individual pupils, including: the type and extent of hearing loss and its impact on listening and language skills the hearing technology worn and its effective use the level of listening and language skills that the child has developed, including his or her phonological awareness appropriate targets and suitable adaptations for individual pupils strategies for encouraging all aspects of speaking and listening, including phonics. Assessment is critical in ensuring that everyone is clear what the child knows, understands and can do and in establishing the next steps along with suitable strategies. Adaptations that may be necessary when undertaking the assessments contained in Letters and Sounds are described. It is hoped that this document will provide a basis for discussion between ToDs and mainstream teachers, enabling suitable adaptations to be made for individual deaf pupils so that they can make good progress in this essential skill. There will, of course, be much more to do to ensure that deaf pupils comprehension is such that they can make progress in all aspects of reading. Trish Cope is an education consultant for the Ewing Foundation and an honorary tutor at the University of Manchester. News from NatSIP Lindsey Rousseau has details of the latest activities and aims of this vital and busy group It is good to be able to report that, at the time of writing at least, NatSIP is alive and well! After our activities during we had a mandate from the schools, services and voluntary organisations involved with NatSIP to look at how we could continue with the work beyond March One of the working groups has the remit to find solutions to longer term NatSIP funding and it will report in September In the interim I was asked by the NatSIP reference group to seek support in order to continue with the important work we have undertaken, especially around outcomes for young people with sensory impairment. The approach to voluntary organisations and heads of services was very helpful and by May we had received resources from five voluntary organisations and 20 local authority services and schools. Most reassuring were the comments that came back from these contributors revealing that the work of NatSIP is becoming more and more valued and important as a voice for SI achievements and issues. We retain the purpose of NatSIP: improving outcomes for children and young people with sensory impairments and narrowing gaps with their peers, through joint working in services for these children. Workstreams suggested for include: Continuation of the current work on outcomes, workforce development and emotional resilience. Working with families; building on work from the Lamb Report and Implementation Plan. Commissioning services and integrated service delivery. Looking at transition with all the implications of the funding changes. Details of how we plan to take these activities forward will be on the NatSIP website and it is very much hoped that all of you who want to be involved can contribute through a regional activity. Working practices of ToDs and other professionals involved with children with hearing impairment may change as the policies of the new Coalition Government take effect. We hope that the outcomes will benefit all young people, including those we work with. NatSIP is looking forward to continuing to play a key role in ensuring that the needs of young people with sensory impairment are not overlooked. Lindsey Rousseau (NatSIP facilitator) on behalf of the NatSIP reference group. BATOD Magazine September

42 Feature The end of an era Sarah Wild laments the closure of a very special school for the deaf At the end July 2010 Ovingdean Hall School for the Deaf sadly closed its doors for the last time. The closure was announced in March 2010 and was attributed by trustees to declining student numbers over the past few years, and the impact of the inclusion agenda on special education. The closure is mourned by many in the deaf world: students, parents and carers, staff, professionals and academics all contacted the school to convey their deepest sympathy and ongoing support in the last few months. Ovingdean Hall School has a long and proud history and a central place in the development of deaf education stretching back over 150 years. The school was first established in 1841 as The Brighton and Sussex Institution for the Deaf and Dumb by two teachers, initially catering for only eight students. In the mid-19th Century the idea that deaf children should receive an education was groundbreaking. By 1945 the success of the school was reflected in the dramatic rise in the number of pupils; the Brighton premises were no longer sufficient and the school moved to the village of Ovingdean and became Ovingdean Hall School. Ovingdean Hall School has been a really special school with an exceptional ethos of caring for and supporting the whole child. The students came from all over the UK and the vast majority were weekly boarders. Many had complex additional needs and the school worked in partnership with a myriad of external agencies to support the development and progress of each young person. Central to the school s ethos was the promotion of a positive personal identity for each young person, within a supportive culture of mutual respect. The school set high personalised expectations for all students in all aspects of development, including academic, social and emotional areas, and progress was measured regularly and meticulously. The education care and therapy teams worked seamlessly with each other to provide wraparound care, support and guidance. Everyone who visited the school over the last year commented on the calm, positive atmosphere often remarking that it felt like a large, happy family. Ovingdean was a very harmonious school with genuinely warm and supportive relationships at every level. It was a really special place. Prior to the announcement of the school s closure the new leadership team had been working on developing the school to try and ensure its long-term future. The team was establishing an extended further education provision, and was also developing a separate behaviour resource for deaf students with complex social and emotional needs. Excellent work had also taken place reviewing and revising the school s communication policy. Staff at Ovingdean had worked very hard to ensure that standards were consistently good or better best illustrated by the outstanding Ofsted judgement for the Care Inspection of November The news that the school was to close after the collapse of merger talks with another school for the deaf in February 2010 was devastating for everyone involved. A great deal of work and emotional energy went into supporting the young people and parents through the process of closure. The students were incredibly brave and philosophical and tried to be as positive about their remaining time at Ovingdean as possible. Staff demonstrated exceptional levels of commitment and professionalism throughout this painful period, with an ethos of business as usual ensuring that students were kept on track and made progress. The leadership team worked tirelessly to find new placements for the students, so that no young person was left without a school place in September Despite the bleakness of the situation everyone made a conscious effort to focus on celebrating all that was great about Ovingdean to get through the troubled times and the fact that we managed to stay focused and positive is a credit to all involved. So what lessons can be learnt by our unfortunate experiences at Ovingdean? Possibly that to survive, all specialist settings need to continue to change and refine their practice, and to keep track of changing trends and philosophies in special education. We learnt too late the cost of being too closely defined by our history. The future of special education will be governed by the ability of education providers to adapt and evolve to meet the rapidly changing needs of students. Rigid adherence to tradition and dogma will only ensure a hasty demise. Sarah Wild was Head of Education at Ovingdean Hall School for the Deaf. 40 BATOD Magazine September 2010

43 Feature Vocab test results With the first stage of the British Sign Language Vocabulary Test project now complete, Wolfgang Mann and Chloë Marshall report on the results In the November 2009 issue of the BATOD Magazine, we reported on a new project that had just started, exploring deaf children s vocabulary development in BSL. For this project we developed a web-based test to measure children s strength of vocabulary knowledge the BSL Vocabulary Test (BSL VT). The test consisted of four tasks, which measured different degrees of the strength of the mapping between phonological form and meaning of BSL signs: meaning recognition (MRG), form recognition (FRG), form recall (FRC), and form/meaning association (FMA). Our aim was to find out whether there is a hierarchy of difficulty for these tasks, and therefore whether vocabulary acquisition for BSL proceeds incrementally as it does for spoken language. The mapping between phonological form and meaning is fundamental to what it means to know a sign, or word given the meaning, the language user can access the phonological form, and given the phonological form, the user can access the meaning. Many standardised vocabulary assessments draw on this mapping, although they usually focus on only one type of mapping. By measuring children s understanding of each sign in four different ways, we tried to overcome some of these limitations. We have now finished the first stage of the project during which we used the BSL VT with a group of strong BSL signers to explore whether it is suitable for assessing sign language. Working closely with colleagues from a small number of schools, we were able to test 24 deaf children aged between five and 15 years. Performance in each task correlated strongly with the others, showing that all the tasks tap children s vocabulary knowledge. The results also correlated strongly with age, with older children doing better than younger children, and they correlated with non-verbal abilities. As we had predicted though, some tasks were easier than others, meaning that there was a hierarchy of difficulty. The two recognition tasks were the easiest, and the two tasks where the children had to produce signs were the hardest. The graph shows these findings. So far then, it appears that children s acquisition of sign language vocabulary has much in common with the acquisition of spoken language. There is another respect in which this is the case. When Mean scores on BSL form-meaning mapping task MRG FRG FRC FMA children were shown a sign and asked to produce another sign with a similar meaning, to produce a semantic association, they tended to produce either a sign from the same category (eg DOG CAT) or one that was schematically related (eg DOG BONE). We know that hearing children tend to increase the proportion of categorical associations as they get older, and this is an indication of their semantic development as they learn more words, they move towards organising those words taxonomically. We found something similar for deaf children s responses on the association task of the BSL VT: children with higher overall vocabulary scores were more likely to give a categorical association than children with lower vocabulary scores. For the second stage of the project, which started in May, we are trying to replicate our findings with a UK-wide study on deaf children with different levels of signing skills. To facilitate access and administration, the test was available online from 3 May to 30 June, and Teachers of the Deaf and speech and language therapists were able to access the test and administer it to any of their pupils meeting the test criteria. We hope to have got enough data to establish age-appropriate scores/norms and standardise the test so we can make it available to schools in the future as a useful addition to the BSL assessments currently available. If you would like more details of the web-based BSL VT, please contact Dr Wolfgang Mann, wolfgang.mann.1@city.ac.uk. You can also check our project website: bsl_vocabulary_test.html/ Wolfgang Mann and Chloë Marshall are based in the Department of Language and Communication Science, City University London. Both are also associated with the Deafness Cognition and Language Research Centre. BATOD Magazine September

44 Feature Sound equipment management Doreen Mills highlights the audiology systems used to monitor and enhance the listening environment for deaf pupils at a school in Jersey At St Clement s School, Jersey, we have a Total Communication Centre which provides an island resource base and specialist support for children who are profoundly or severely deaf. The Centre also supports hearing children with language disorders who benefit from Sign Supported English. We currently provide for two hearing children with language development needs, two children who have profound to severe hearing loss and wear behind the ear digital hearing aids, and four profoundly deaf children with cochlear implants. The Centre is staffed with eight key workers and a Teacher of the Deaf and is supported by a specialist speech and language therapist who is based in school for two days a week. We also have a BSL tutor who is based in school for one day a week and an educational audiologist who visits one afternoon a week and when required to resolve technical problems. We are fortunate to be in a school that was recently built. This enabled the educational audiologist and the Teacher of the Deaf to work with the architects and engineers to ensure that the school met building regulations regarding acoustics and had the necessary environmental equipment built into the fabric of the building, for example Soundfield systems, loop systems and flashing alert fire alarms. Walls are slightly curved, most areas are carpeted and blinds are available on classroom windows, allowing the sound to travel in the best possible way. Even the electrical points were pre-agreed so that routines regarding charging of equipment could be established without practical problems. Sockets in the floor near to seating positions allow children s processors to be connected to computers without leads trailing across the rooms, for instance when mappings are checked by visiting cochlear implant teams. We have two small working classrooms where we are able to take the children to work one-to-one in a quiet environment on a regular basis; an office with a one-way mirror into one of the classrooms allowing observations that do not impact on the behaviour of the children; and a resource room in which all audiology equipment and specialist resources are kept to enable access without disrupting teaching sessions. The children all use FM radio aid systems such as the Phonak inspiro system and the Connevans Genie system. The Genie system allows the children to use a conference microphone, attached to a transmitter, when working in a group situation. This is of great benefit and means the transmitter does not need to be passed from person to person. The children are encouraged from an early age to become independent in the use of their equipment Key workers perform regular daily listening checks on hearing aids and cochlear implants to monitor the sound quality and to enable them to recognise any changes or interference that may occur. FM systems are also checked daily and an audiology log for each child is completed so that any problems with equipment are recorded along with information about when units have been charged and batteries in hearing aids or processors changed. The log is invaluable to the ToD and the educational audiologist in showing any patterns of persistent problems. Each child has an individual audiology tub, with an inventory of equipment, and the log is kept with it, so that the key worker has instant access to daily information as required. This system is followed by all the key workers, providing consistency in working practice and procedures. The children are encouraged from an early age to become independent in the use of their equipment; to remove, clean and fit their hearing aids and to report difficulties. They are expected to take ownership of their radio systems, to give and collect the transmitter from teaching staff, to remind teaching staff to mute the transmitter, to switch it on and off and to ask for a conference microphone when needed for group work. Each child has an I Can list, kept in the audiology log, which they gradually complete to develop their confidence in using equipment and to raise awareness of their hearing needs. In the hall we have a fixed Soundfield system with two handheld microphones and one collar microphone. It is important that speakers have confidence in the system so a choice is given regarding preferred use. We find the handheld microphones are more robust and are used more often. The collar microphone has the advantage of leaving hands free but speakers need reminding that it has to be placed in the correct position to avoid speech loss. 42 BATOD Magazine September 2010

45 Feature Having experienced problems with microphones not working, batteries going flat and feedback noises we have introduced working practices to keep track of them. A Soundfield fault log enables us to write down any problems when they happen and refer back to them when the educational audiologist or Connevans engineer visits us. The first child to arrive in assembly gives his or her transmitter to the key worker on the audiology rota After our last visit from a Connevans engineer we took photographs of how he had set the dials on the system so that we can reset them to his recommended levels as inevitably they get moved! We keep the photographs on the wall by the Soundfield mixer box and have found this very useful as a means of checking volume levels. Microphones, receivers and volume dials are all colour coded so that if feedback problems occur during assembly, action can be taken. Batteries present ongoing problems as they come to the end of their life and to monitor this we have three sets of four colourcoded and individually numbered batteries which we rotate weekly one working set in the microphones and a spare battery on trickle charge with the Soundfield in the hall; one set charging in our resource room, and one set resting. The numbering of the batteries enables us to identify any battery which may no longer work well. The colour coding allows for the easy rotation of all the batteries, which is done every Friday morning before whole-school assembly, ensuring that there is always a fully charged set in the microphones. We also complete a Soundfield log which enables us to keep track of where each set of batteries should be each week. The radio aids are connected to the Soundfield using an FM Genie transmitter input adapter. Genie and Phonak frequencies are different but can be matched on a conversion scale which is listed on the door of the Soundfield cupboard. Depending on who is in the assembly, the frequencies used will change. Assembly frequency notice who connects it to the system. Then the frequency for that specific assembly is displayed outside the hall on the left-hand side. As the children go into the hall they change the frequencies on their personal radio receivers, thereby promoting independence and gaining an understanding of their hearing needs. This system works very well and encourages the children to take responsibility for monitoring their hearing needs in different listening environments. If they experience any difficulties with sound quality during assembly they are encouraged to report them immediately. In the library, music room and nursery we have Classmate Infrared Soundfield systems which are used to great effect. In the nursery, the Soundfield is of particular benefit for a child with a recent bilateral implant, for whom a radio aid would not yet be appropriate. It is used at circle time and improves both the sound quality and the listening conditions for all the children, helping them to focus on the teacher. Working practices and procedures are continually evolving in school and have proved to be of great benefit to children and staff in monitoring the listening environment. Sometimes it s the small things that can make the greatest difference in everyday routines. For systems to work efficiently they have to be understood and followed by everybody in the team. Regular review is encouraged and by sharing ideas and working together, we aim to provide consistent good practice and achieve the best standards possible for the children in our care. Doreen Mills is a key worker at St Clement s School, Jersey. BATOD Magazine September

46 Feature Meeting social care needs Recent research into social care provision for deaf children and their families in England highlights that their needs may often go unrecognised. Ros Hunt has the details Many of us, both Teachers of the Deaf and social workers like me, will, in the recent past, have been part of some sort of reorganisation. Where we are then placed in the organisation and with whom we are reorganised seems to be as varied as the number of local authorities (LAs) that exist. Even if, superficially, structures of services look similar, as soon as one starts digging deeper, differences emerge. The reorganisation of LAs in England into children s and adults services can be traced back to the tragic death of Victoria Climbié and the subsequent launch of the Every Child Matters initiative. The Children Act 2004 provided the legislative framework that was hoped would encourage the development of more accessible and effective services focusing on the needs of children and young people. This included the instruction to LAs to set up children s services bringing together education and social care for children and young people. Until this point, many LAs had provided social care services for deaf children by means of a deaf services or sensory services team. This had effectively been a cradle to grave service where staff had been experts in working with deaf adults and deaf children and their families. The establishment of children s services, therefore, often meant the splitting of the old-style teams. This separation of children s and adults services has created a puzzle for LAs as to how best to meet the needs of deaf children and provide specialist services to deaf adults when the available social care resource might be both highly specialised and also quite small. What was needed was a thorough investigation as to how deaf children and their families were being served under these new arrangements and the quality of these services. A small grant from the National Deaf Children s Society enabled the Social Research with Deaf People programme at the University of Manchester to undertake this research. Phase 1 consisted of in-depth case studies undertaken in five LAs where we knew that services to deaf children and their families were being provided in different ways ( ndcs_news/social_care_radar.html). As well as enabling the research team to understand in-depth five models of service provision, the results highlighted areas of particular interest and potential concern for further investigation nationally. Phase 2 ( search_clicks.rm?id=4668&destinationtype=2&instance id=168675), which consisted of structured telephone interviews, was undertaken with the person identified in each LA as holding responsibility for the provision of social care services to deaf children and their families. Of the 147 LAs who could have been included in this phase, 52 took part, meaning that information from over one-third of LAs has been included in these findings. Service organisation effects How services were organised was extremely influential. About one-third of LAs had specialist teams or what we have called specialist team arrangements. Examples of this include children s services buying services for deaf children from their colleagues from the adult services by formal arrangement, or buying services from a local deaf specialist voluntary organisation. However, not all of these specialist teams included social care staff some consisted of education staff only and not all contained qualified social workers. In many LAs, deaf children fell under the remit of children s disability teams (CDTs), where there were rarely any staff with knowledge and experience of working with deaf children and their families. Rather, teams considered that they had expertise if someone in the team had basic communication skills. There was a distinct danger that there would be insufficient expertise to recognise the potential seriousness of a presenting problem, or understand when it was appropriate to seek advice from a professional with experience and knowledge of deaf children. From a ToD perspective, another issue of importance was that without specialist service arrangements, being deaf was rarely seen as encompassing complex developmental concerns or presenting particular safeguarding risks. This meant that a situation tended to have to escalate to a generically identifiable crisis before any social care response was possible. Preventative services Given the developmental risks for deaf children, we found specialist social workers with deaf children and their families were acutely aware of the importance of providing preventative social care services which complemented services provided by education and health colleagues. For most deaf children, only in LAs where specific social care provision for deaf children existed were social workers able to develop preventative services. The exception was in work with children under five, where joint working with children identified by newborn hearing screening had enabled 44 BATOD Magazine September 2010

47 Feature and strengthened the development of preventative services. Sadly there was little evidence that this multi-disciplinary approach had translated into routine preventative services for older deaf children. The majority of LAs without specialist social care provision, but who considered that they did provide preventative services to deaf children and their families did so overwhelmingly in the form of general services to the families of all disabled children. There was little understanding that such a broad-based approach would not meet the particular needs of deaf children and their families for whom preventative measures were closely linked with communication and associated psychosocial development. Integrated working In half of the LAs, there were no systematic arrangements for ensuring that deaf children received a joint assessment involving health, education and social care, nor a defined multi-disciplinary pathway for planning and service provision. Over 50% of LAs said that they had no formal referral arrangements between social work and education professionals where deaf children and their families may require assessment and/or service provision. Statutory child protection Only 37% of responding LAs described co-working arrangements between child protection teams and specialist social workers; 18% described a situation in which there was no co-working at all, either because specialist social workers did not exist to co-work with anyway, or because specialists working in adult services were not allowed to work on cases involving children, or because the CDT did its own child protection work and did not involve outside deaf-related specialists. The remaining LAs described various arrangements that involved getting help of different varieties from workers either within or outside the LA. In looking at the range of descriptions given to us of the external help sought, we were struck by three things: how much the help took the form of information and advice in a general sense about deafness or sign language rather than specifically in relation to any particular case how little awareness there was about the extent to which specialist knowledge might be required of cultural issues or deaf child development issues in a broad sense, rather than only of language and communication (several respondents described involving an interpreter as solving the problem of the specialist aspects of a case) how ad hoc the arrangements appeared to be. Additionally, no respondent who was not themselves a deaf specialist seemed aware that there would be issues that needed to be taken into account or that Implications for Teachers of the Deaf ToDs may experience difficulties in finding social care colleagues who have an understanding of the implications of deafness, have experience of deaf children and whose remit includes this work. It is likely to be hard to access targeted preventative social care services for deaf children and their families. In those LAs without specialist social care workers or with no deaf/sensory team or team arrangements, referrals to social care services are unlikely to be acted on unless a situation escalates into a crisis. ToDs may be asked to work outside their remit in LAs which assume that all deaf children s social care needs are met by education staff. Concerns about child protection involving deaf children may not be taken seriously enough where referrals are made to teams without experience of deaf children. would impact on the investigation if a child was deaf and not a British Sign Language user. Principal conclusions of the research study While there is evidence of some good practice that enables proactive social care involvement with deaf children and their families and thus extends the range of provision and resources for those families in such a way as to complement that provided by educational and health colleagues, such arrangements are exceptional. In only a minority of LAs would there appear to be effective, skilled and specialised social care provision for deaf children and their families. There is clear and widespread evidence of poor integrated children s services arrangements in respect to deaf children and their families which results in: a lack of specific attention to deaf children and families social care rights and needs poor recognition of need and provision of assessment severely limited ability to work preventatively within a broad understanding of safeguarding ambiguous pathways of service provision responsiveness only in situations of acute need lack of focus on the psychosocial developmental, linguistic and cultural challenges and differences of the full diversity of deaf children. In these circumstances, and with specific reference to social care, there is strong evidence to suggest that the statutory duty on LAs to co-operate within children s services to promote the well-being of children is, where deaf children are concerned, being significantly compromised. Ros Hunt is a research associate with the Social Research with Deaf People Programme at Manchester University. BATOD Magazine September

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49 Feature Testing the network A new network was given the once-over by pupils and staff at a school in Scotland, as Gill Ferris reveals Pupils and staff at Carnoustie High School have been at the forefront of new technology for deaf pupils through their participation in a trial of the new Phonak inspiro MultiTalker Network. Feedback from the trial has been most encouraging. This opportunity was supported by the education authority s Hearing Impairment (HI) team, whose members were keen to enable deaf pupils to access the latest equipment while developing their access to whole-class activities. The aim of the trial was to determine whether the inspiro network would be a worthy investment and addition to the communication options available for deaf pupils in schools in Angus. The trial was organised by the HI team in conjunction with Jeremy Hine of Connevans who set up the equipment and delivered training sessions to staff and the two deaf pupils at the centre of the trial. The pupils initial reaction was positive but also mixed with confusion as they heard sound coming from different speakers. At the end of the trial the pupils acknowledged that this had taken time to adjust to. Two deaf pupils used the network, comprising three inspiros, over a two-week period in a number of mainstream classes, including French, English, biology, religious education, PSHE and history. It was also used to facilitate access at form time and at an assembly led by a number of speakers. Not to be left out, four pupils at one of the associated primary schools also trialled the equipment for a day, concluding that the inspiro was great at cutting out background noise and that you can hear everyone better when they use the DynaMic (accompanying handheld microphone which is passed between speakers who are not wearing the transmitter pack). This is typical of the response from pupils and staff, which has been hugely positive. It was considered by all that the inspiro network was easy to use and that it created an inclusive environment, improving access in a number of situations, including class discussions, question-and-answer sessions, reading aloud in class, more than one speaker/team teaching, solo and group talks and moving between the speaker and whiteboard. The DynaMic was added towards the end of the first week and the response was such that it came to be regarded as an integral part of the network. Most hearing pupils used the DynaMic with confidence and admitted they enjoyed using it! The only concern raised related to the time the equipment held its charge. However, an inspiro is currently in use with a pupil in another school in Angus and this issue has never presented itself. In this instance the charge draining was possibly the result of only two chargers being used to charge the three inspiros. Further training in the management of the equipment was asked for by pupils and staff and it is intended that this will be followed up. This trial has proved to be a wonderful opportunity, placing pupils at the centre in deciding which communication package is right for them. Staff used the equipment with the assurance of full support from a specialist member of support staff. Pupils and staff at Carnoustie High School concluded that the ideal network consists of two inspiros, with the initiator for the classroom teacher and one inspiro for support staff, and one or two DynaMics. They also considered the tool an excellent addition to, not a replacement for, the BSL support currently used by the pupils at the centre of the trial. Thanks to the pupils and staff involved, to the education authority and the HI team, also to Jeremy Hine, to NDCS Test Drive equipment lending library for the loan of two inspiro FM systems and to Alan MacQueen (NDCS Family Officer) who continues to offer steady reassurance when staff s faith in themselves to use technology fails! Gill Ferris is a peripatetic teacher of children with a hearing impairment with Angus Council in Scotland. BATOD Magazine September

50 Association business Drafting ToD competencies in Europe As the Leonardo project moves into its second year, Paul Simpson reports on progress from the latest meetings in Europe Since the article I wrote in the May 2010 Magazine about the Leonardo project, further progress has been made as a result of two more meetings one in Leuven and one in Malta. The project to develop competencies for Teachers of the Deaf which would be applicable across the continent of Europe, with the overriding aim of reducing the exclusion of deaf children through appropriate support and intervention from Teachers of the Deaf has now reached the stage at which we are ready to make some initial moves towards consultation. In April we met in the Belgian University town of Leuven, a town where at least every other person seems to be a student. In this inspiring location we spent two days moving the project on. Since the previous meeting in Luxembourg we had all done our homework and spent time amassing key background material to support the project. In order to demonstrate evidence of need, we were building up a picture of the prevalence of deafness across the different countries of Europe and the numbers and qualifications of Teachers of the Deaf (including highlighting those areas where there was no specialist training at all). This work had shown that there were many gaps in our data. It was decided that attendance at the annual conference at Como in Italy on newborn hearing screening (attended by professionals from across Europe) might help to fill these gaps. Although Italy is not one of the partner countries (which are UK, Malta, Belgium and Luxembourg), but involved through membership of FEAPDA, it was agreed by the European agencies that attendance would qualify within the rules as the topic matter was closely related to the project s subject. Graham Groves from Telford and Wrekin and Sue Lewis from Oxford Brookes/Mary Hare were to attend and gather as much useful information as possible about prevalence in those countries from which we had had little response. Furthermore, our Belgian colleague had been accepted to deliver three presentations at the ICED meeting in Vancouver and we agreed that he too could carry out some research when attending that gathering. We decided to develop a flyer raising awareness about the project and asking for the information we needed. By this time we had already produced four different versions of the competencies and to avoid the horror of us all working on different versions we set up a Google group where the latest version could always be found and clearly marked. We left Leuven with clear tasks to perform in the few weeks before we would meet again in Malta (the University of Malta being one of the partners). Malta was very beautiful in the May sunshine (although on the first day we discovered that back home in England it was 29 degrees and only 22 degrees in Malta!) and the Maltese people were warm, hospitable and welcoming. We arrived therefore in Malta intending, by the end of the meeting, to have the definitive (but still draft of course) set of competencies ready for initial consultation. Even after so many versions there were still several aspects which needed further work and, this being a European and not an English project, we needed to ensure that the language of the competencies was as clear and unambiguous as 48 BATOD Magazine September 2010

51 Association business possible. The competencies were divided into three groups broadly following the groupings within the Training and Development Agency competencies in the UK: Knowledge and understanding (for example, Understand how children and young people process auditory and visual information and how this might affect the teaching and learning approach. ). Professional skills (for example, Provide a wide range of opportunities for the development of receptive and expressive language. ). Personal attributes (for example, Have good communication skills and a knowledge and skills base that inspires confidence from families, children and other stakeholders. ). In small groups we worked on the latest version and then swapped with each other so that each member of the group had seen and had some input into all of the groups of competencies and had an idea of how they looked in the whole document. By the end of the first long morning we had finally settled on what we felt was a set of competencies ready for presentation at the first stage of consultation. In preparation for the meeting Guido Lichtert, from the University of Leuven in Belgium, and I had prepared a document suggesting how we might undertake the consultation. Despite earlier thoughts that we might just send them out to all the Teachers of the Deaf we happened to know across Europe, it became clear that there needed to be a much more targeted approach, initially at least. We therefore prepared a discussion paper which, through the medium of the Google group, was distributed in advance of the meeting. This looked at two areas which were the subject of PowerPoint presentations and wider discussion during the afternoon. Guido is a university professor and he gave a presentation about different scientific approaches to research which he felt were essential to give the project validity. Although we needed to stress that this is definitely not a research project in the traditional sense, an understanding of the theory and underpinnings of research was nevertheless essential. He described in his presentation two possibilities: the Likert scale which allows the rating of each item in a list on a four-point scale for example, strongly agree, agree, disagree, strongly disagree (an even not an odd number so that respondents couldn t just pick the middle category, effectively not making a real decision); and the Q-sort approach in which respondents are invited to put a range of items in this case the competencies into a normal curve of distribution. This means starting with the least acceptable, important or relevant competence on the left-hand side and the most favourable, in the eyes of the respondent, on the other side of the curve. The bulk would be placed around the mid-point. This would allow us to determine what the respondents as a whole see as the most important or core and the least important competencies. Following this research methodology presentation we had a wide-ranging discussion on the purposes of the consultation and the methodology which we would adopt, including whom we should target in this initial consultation. Basically we had decided to ask first of all whether we were, in the eyes of people who had not spent a year on the project, on the right lines. We decided to approach samples from the widest possible range of people including ToDs, trainees, teacher trainers in the universities and elsewhere, parents and young deaf people themselves as well as possibly other professionals such as speech and language therapists and psychologists. We divided into two groups to discuss the target groups and the approach to be adopted and the whole of the next morning was devoted to thrashing out a plan. The forthcoming summer holiday, particularly in mainland Europe where many colleagues take two months off, would intervene before the next meeting which would be in early September in Telford and Wrekin and this concentrated our minds because we needed to make progress as the project nears the end of the first year of its two-year life. One other point which emerged from the consultation discussion was the possible use of focus groups which would allow small groups of similar, or indeed different, types of respondent physically (as opposed to electronically) to meet to discuss the document the interaction within the group allowing new ideas to emerge and be explored. It was decided that it would be good to set up some focus groups but might be difficult efficiently to do so before the next meeting, given the time of year. Therefore we decided to try to trial the competencies through a questionnaire and, if possible in our own settings, arrange focus groups in time to get significant data to consider at the next meeting. We decided that the way to do this was to take the revised competencies document now quite a significant number of pages and ask respondents to rate each one on a four-point scale (as described by Guido above). These would be categorised as follows: Competencies which were needed for every professional who was in contact with a deaf child or young person (including a mainstream teacher with such a child in his or her class). Competencies which were needed for all teachers specialising in working with deaf children in any setting. BATOD Magazine September

52 Association business Competencies which provided additional value to Teachers of the Deaf but were not essential. Competencies which were regarded as not important at all. This, it is hoped, will produce a range of competencies from those felt to be utterly essential core competencies for those who work with deaf children and young people in any way, to those which are peripheral and do not need to be part of the core skills and attributes of the ToD. We also decided to ask respondents to choose the ten or so most important competencies from each group. The next stage was for each partner to return home and seek out a range of colleagues and others with whom to trial the questionnaire as well as possibly setting up some focus groups. The rest of the time was devoted to drawing up the interim report which has to be submitted to each partner s European agency as a description of progress made so far and referring back to the previous article in the May Magazine mobilities undertaken (the essential indication of the movement between partner countries). Some partners are making good progress with their mobilities (FEAPDA has to undertake 24 over two years and has now completed 14 after one year). As always at the end of a Leonardo meeting, we finished by allocating tasks to be completed before the next meeting and revised the overall work plan. We also fixed dates for the second year s meetings Telford in September, Newbury (Mary Hare/Oxford Brookes) in November, Luxembourg in January and Malta after Easter, with possibly one more meeting in Belgium in the meantime. We left Malta pleased that the first year s work had gone to plan and we had developed a robust set of draft competencies to start work on. Paul Simpson is the Executive Officer of BATOD and President of FEAPDA. BATOD was there representing you... Between the NEC meetings, members of BATOD attend various meetings that are of particular interest to Teachers of the Deaf. This list is not exhaustive. Your representatives at the meetings listed included: Gary Anderson, David Couch, Maureen Jefferson, Elizabeth Reed-Beadle, Paul Simpson Date External participants Purpose of meeting Venue May 10 NatSIP Outcomes meeting Sense, London 10 CRIDE Survey meeting DCAL, London 11 NatSIP Reference group Sense, London Leonardo project Fourth partnership meeting University of Malta 26 Ofqual Access Consultation Forum Coventry June 7 DESF Regular meeting RNID, London 10 NDCS Regular meeting NDCS, London 18 HOSS Annual conference Birmingham 29 Ofqual Review of regulatory framework London 30 UKCoD Annual parliamentary reception House of Commons July 2 Oxford Brookes University Consultative Committee Mary Hare School 5 NatSIP Fourth working day London 7 FM UK Regular meeting London 13 Hearing and Balance UK Regular meeting London 14 GTC(E) Disabled Teachers Task Force GTC, London 14 CRIDE Survey meeting Frank Barnes School, London 20 NatSIP Reference group Sense, London Please inform the Executive Officer, Paul Simpson, if you know of any meetings where you feel representation on behalf of Teachers of the Deaf would be of benefit. Although there is no guarantee that BATOD would be able to attend every meeting, situations could be monitored and the interests of ToDs represented. 50 BATOD Magazine September 2010

53 Association business What went on at NEC on 12 June 2010 Karen Taylor files her report on the summer meeting The NEC met in London as a further attempt to cut down costs. We met from 11am to 5pm in order to reduce (in this case eliminate) overnight expenses. Unfortunately this meant some of our colleagues from more far-flung places having to leave home very early. This is clearly unacceptable so we decided to meet in London again in the future but with some overnight stays for those who can t reasonably make it and return home in one day. The morning was spent in our workstream groups productive working time which produced some key points that were fed back later in the afternoon. BATOD is hoping to forge closer links with representatives from VIEW, and we will take this forward. Both bodies are very eager to preserve their distinctive differences but to work collaboratively where we have interests in common. We always invite representatives from the different regions and nations to our June meeting but sadly we had only one additional representative attending this meeting. She was very welcome but we would like more representation from the regions and nations. We decided to invite colleagues to the March meeting (instead of the June one) in future as it coincides with the annual conference and AGM which might make it more attractive and cost effective to attend. BATOD is drafting responses to the NDCS s proposed quality standards for resource bases for deaf children and young people, and the Association is also fully involved in the national discussions about academies the NDCS has produced a very useful briefing paper on this subject. Support for Teachers of the Deaf whose jobs are in danger of being cut in the current economic climate is also being developed for use by members. Putting articles in the BATOD website members area on a regular basis was suggested, as well as investigating the highlighting of activities for continuing professional development (CPD) through a DVD or videojug. Developments in the mentoring project for new ToDs are to be discussed at the September meeting of the NEC. The BATOD publication, The Language of Examinations, is to be adapted further, making it more generic and available electronically. News and information about CPD activities in the regions could be made available to other regions. Four audiological articles have been identified for inclusion in forthcoming editions of the Magazine, including one on Soundfield amplification. Information on the use of FM systems is being collated, and a working party will be set up to look at guidelines for ToDs working with cochlear implant centres. Following this workstream feedback the NEC was asked to evaluate the purpose of the BATOD stand which is taken to various exhibitions and meetings across the country. The suggestions made will be considered at the next Steering Group meeting. This was followed by reports from the President, Executive Officer and Treasurer which covered areas such as the demise of various organisations under the new Government (Becta and the GTC(E)). Investigations are under way to ensure that the HOSS Forum and the ToD list, currently hosted by Becta, continue. Paul Simpson, our Executive Officer, gave a presentation about the Leonardo da Vinci European Project, which is developing pan-european competencies for ToDs. An article about this project appears on page 48 of this Magazine. BATOD s budget is expected to break even this year. There were 1,595 members on 9 June! There were reports from all the regions. The Wales region has a number of ToDs on its committee who are newly qualified. They highlighted the fact that there are several authorities who only have one ToD working within them. The South region has a new chair and is trying to encourage more members to join the committee. The Northern Ireland region has a new chair and chair elect. There is a stable committee in the North, with good membership. The Midland region meets regularly and has new membership. Paul Simpson pointed out that nomination forms for election to the NEC would appear in the September Magazine and he urged members to stand again and to encourage as many colleagues as possible to put themselves up for election. Regional representatives should consider standing for election which would in turn release more opportunities for new people to replace them on the NEC as regional representatives. A caption competition towards the end of the day caused much frivolity life on the NEC isn t all early starts and hard work!! Karen Taylor is the President Elect of BATOD. BATOD Magazine September

54 Regulars Reviews Title Publisher Price Reviewer Connectors Scholastic 4.75 each Sue Watters, peripatetic Teacher of the Deaf, Northamptonshire I had three books to review that are new to the Connectors series: Animal Heroes by Kathy White (ISBN ) Music Magic and Imagination by Jill Eggleton (ISBN ) The Right To Survive by Jill Eggleton (ISBN ) These books are designed for independent group reading, where pupils, once trained to predict, clarify, ask questions and summarise a text, can be left to discuss a book without teacher support. At the beginning of the book there is a choice of coloured faces. Each pupil chooses a colour and when his or her coloured face appears on a page he or she is the group leader and has to get the group to talk about the book. There is a good teacher s manual which explains explicitly how to model the comprehension strategies. There is also a video on the Scholastic website which gives a good idea about how these books work: resources/77520/ In the manual there is an observation sheet for the teacher to complete and a self-evaluation prompt for pupils. There are also worksheets in the teacher s book. They tend to be open-ended, rather than comprehension exercises, for example Music Magic and Imagination asks pupils to list what they knew before, what they learnt from reading the book and what they would like to learn after reading the book. My pupils find this type of worksheet difficult to complete independently, but with discussion they were able to do it. Unfortunately, as a peripatetic teacher I was unable to assess these books as they are intended to be used. My pupils were attracted to Animal Heroes, mainly due to the lovely picture of a dog on the cover. However, no one volunteered to read either of the others. When one pupil read Music Magic and Imagination, she liked reading about the Didgeridoo and looking at the photographs of strange instruments. I used The Right to Survive with a Year 8 special school pupil who was studying the environment. We read each two-page spread together with me asking the questions and her making the prediction, clarifying (checking unknown words in a dictionary), asking questions and giving a summary. I also had a look at Mysterious Places by Sue Adasiewicz (ISBN ), also published by Scholastic (price 4.75).This is from a series designed for Year 5 and 6 pupils called Shockwave. This book was the least popular of those I reviewed. One of my more able Year 8 pupils said, I hate boring factual books. To gain her interest I got her to use the Fact File to test me with questions such as Who was Maria Reiche? We then read the book and completed the summary chart on the Fact File card. The book would be suitable for a support assistant working with a reading group, as the teacher s notes give detailed instructions of what to read and which questions to ask. Overall I found that for my statemented deaf pupils these books were more successful with Year 7 8 pupils, rather than the Year 4 6 pupils that they were intended for. In a class situation the books may work well for group reading. They do not directly connect with National Curriculum subjects, but offer an opportunity for discussion of general knowledge topics. However, my pupils did not find the topics of great interest. Title Understanding and Managing DSP Hearing Aids Publisher DVD issued by the Ewing Foundation Price 20 Reviewer Corinda M Carnelley, peripatetic Teacher of the Deaf, London Borough of Croydon This short DVD tells you everything you ever wanted to know about testing DSP hearing aids. As with all Ewing Foundation materials, it is measured and balanced. A wide selection of test boxes is shown, and step-by-step instructions are given for testing the aids. There are even instructions for determining whether an aid is operating in linear or non-linear mode. The second part of the film explains how to balance a hearing aid with a radio aid, again showing a wide selection of radio aids and talking through the process in small steps. This DVD would be extremely useful as a reminder about gold star standards for people who regularly use a test box. It could also be used as part of a wider training package about hearing aids in general and DSP aids in particular. 52 BATOD Magazine September 2010

55 Regulars Title Authors Feelings & Emotions DVD Consultant: Vanessa Harrison, Researcher: William Mathieson Loggerhead Films Publisher Publisher 2010 Price 75 ISBN Reviewer Sue Watters, peripatetic Teacher of the Deaf, Northamptonshire This DVD features a collection of 30 film clips designed to teach the understanding of feelings and emotions. The clips cover a wide range of emotions and include both comfortable and uncomfortable feelings. The DVD, intentionally, does not include speech, as this allows different interpretations of the thoughts, feelings and behaviours of the characters in the clips. Alongside the DVD there is a booklet, which gives suggestions of ways to use the DVD, and for each clip, a vocabulary list, questions and talking points. On screen each clip can be watched slowly or in stills and then repeated. There are worksheets on the DVD in PDF format, which can be printed out. Disappointingly, they are just the same as the questions and talking points in the booklet. This is a very useful resource, providing lots of emotions to discuss, with each emotion shown in at least two other clips. Deaf pupils often find it hard to explain how they are feeling, so the vocabulary list that goes with each clip should help them to develop a greater understanding of how to express their feelings and emotions. The clips are suitable for a wide age range: from aged 8 to adult. The fact that teenagers are shown in some clips makes this resource particularly useful for secondary school pupils. My colleague and I used the DVD with secondary pupils in Years 7, 8 and 9. Our pupils commented that they would have preferred to have heard dialogue or seen subtitles. However, we felt that not having dialogue meant that the pupils concentrated more on the emotional body language. The clips elicited some useful discussion among our students. We found the worksheets useful and liked the vocabulary lists, which helped with lesson preparation. The main downside with the DVD is its casing. I struggled to get the DVD out of the cover and would have preferred it in a proper DVD case. The box was the size of an old video case rather too bulky for a peripatetic teacher to carry around. The case was not very attractive and detracted from the value of this resource. Also I felt that at 75 it was quite expensive, so more a resource for a team or school than for an individual teacher. Overall this is a well-produced resource, which will be useful when teaching deaf pupils emotional vocabulary. It will be particularly useful for PSHE lessons and deaf peer groups. BATOD Magazine September

56 Regulars ICT news Persuading children to practise maths is a lot easier if you tap into their love of modern technology, as Sharon Pointeer and Andrea Louie Smith discovered at Knightsfield School We are always looking for innovative ways to engage pupils and enhance learning. Some time ago I attended the Handheld Learning Conference in London where I was interested to hear about the work being done at Clunbury School in Shropshire using Nintendo DS gaming consoles in the teaching of maths. Further research revealed other projects using games-based learning, such as the one described on the Learning and Teaching Scotland website (see opposite). Would these devices and games appeal to the secondary-aged pupils in my school? Naturally I decided to find out and bought a class set of Nintendo DS consoles to use in maths lessons. These have now been in use for just over two terms and the following description and evaluation has been written by Knightsfield s Maths Co-ordinator, Andrea Louie Smith. Use of the Nintendo DS in maths teaching A new class set of Nintendo DS games consoles has raised enthusiasm and generated huge interest in mathematics. The small handheld computer games console, which is a popular seller in games shops, offers pupils the ability to practise their maths skills in an innovative way. The Nintendos are used in a number of ways in the classroom: as an individual activity within the lesson structure, as the main focus for the lesson and as a reward for good work or behaviour. Their role within the lesson is also varied, aided by the range of games available. Products The maths games available for the DS aim to improve mental agility and/or ability with specific processes. These include educational games such as Make 10, which allows pupils to focus on number bonds, helping them to develop and practise recall of number facts. The puzzles gradually increase in difficulty and the storybook style presentation makes this game accessible for a variety of ages. Maths Training is part of the popular Brain Training range. This game, which is in a compendium style, focuses on the four operations: addition, subtraction, multiplication and division. Each task is presented in a similar way. Questions can be set to focus on a specific operator or for a mixture of all the operations. This game has the added advantage of different styles of task such as quick daily tests or more complex questions. The final game purchased by the school is Big Brain Academy. This game has been the most popular with the pupils as it covers a wide range of mathematical and logical thinking skills. Activities in this game include spatial activities such as identifying missing lines in drawings or accurately copying geometrical patterns and numerical puzzles. Engagement The Nintendos offer a versatility previously only available in dedicated ICT suites with a wide range of educational maths software. They provide individual access to specific activities within the structure of the lesson; for example, as practice for long multiplication or division instead of, or in addition to, exercises from the book. The Nintendos are frequently used to help practise particular elements of maths, such as times tables. Pupils answer random questions against the clock in order to achieve a medal of bronze, silver or gold. The variations offered enable all pupils to engage fully and they promote a friendly and competitive atmosphere. The level of ability can be set either by the pupil or the teacher. This ensures that all pupils are sufficiently challenged regardless of ability. Pupils always use the same console and can keep their own profile and build on their achievement, further increasing their motivation to participate in the lesson and the task. The Nintendos are also wireless enabled. This allows the games to be played interactively in groups, no longer limiting the challenge to beating the timer, but also to beating each other. The competition encourages pupils to work faster, resulting in them completing more questions. It is surprising how many questions can be answered in a ten-minute session on the DS compared to ten minutes working from an exercise book! Communication is key for deaf pupils. The interactive nature of the DS has enabled pupils to practise discussing processes and methods with each other and has encouraged collaborative learning. 54 BATOD Magazine September 2010

57 Regulars Resources Maths Games for Nintendo DS Professor Kageyama s Maths Training Make 10 Challenge Me: Maths Workout Maths Play Clever Kids: Maths Maths Made Simple Maths Blaster General puzzles, including maths and logic Big Brain Academy Dr Kawashima s Brain Training More Brain Training Professor Layton and the Curious Village Professor Layton and Pandora s Box Junior Brain Trainer Brain Challenge Brain Buster Challenge Me: Brain Puzzles Improvement The Nintendo DS have helped pupils to improve in many different areas of the maths curriculum. The wide range of games available, each focusing on selected strands and/or specific levels, provides flexibility. The Nintendos have played a significant part in our intervention programme designed for pupils with numeracy difficulties and dyscalculia. They have raised levels of numeracy as well as confidence, allowing these pupils to take a more active part in lessons. Effectively communicating the processes and methods surrounding maths and logical thinking is a challenge for any maths teacher, let alone Teachers of the Deaf. The interactive nature of the Nintendos has enabled pupils to practise discussing processes and methods with each other and has encouraged collaborative learning. Peer support has added another level of complexity and further encouraged communication. Additionally, the Nintendos can be used as a carrot. They can be offered as a reward for meeting targets, for example for quality or quantity of independent work. No matter what year group, there is a game or activity which can be used to tempt the pupils. It can be very difficult to engage, let alone enthuse, those pupils who find maths challenging. The use of the Nintendos removes the barrier to learning for these pupils. They find themselves within a familiar tech-rich environment and the maths questions become just another challenge similar to Capture the Flag in Resistance: Fall of Man, or Stage Completion in MotorStorm, both of which are popular games for current games consoles. Creating this familiarisation has led to genuine enjoyment of both the use of the DS and of maths in general. The enthusiasm and enjoyment is clearly seen and heard in the classroom. There are frequent requests to use the games and howls of protest when it is time to put them away! The increasing number of pupils who now own a Nintendo DS also means that there is the opportunity for pupils to buy and practise maths-based games at home. At Knightsfield this has happened with several pupils, the consequences of which have been rewarding. Pupils who are normally quiet and introverted have found themselves the expert on a game, and are able to offer support to their peers, resulting in improved confidence and communication. Using a Nintendo DS at home is not homework, but pupils are often keen to practise their tables or other maths skills in this way. Other members of the family can get involved, with pupils playing competitively against their brothers and sisters. All said and done Modern children have modern expectations. They have grown up in the Digital Age. The technology which appears new to us, is just the norm to them. Transferring data using a USB memory stick is second nature to them, just as going to the library was 20 years ago. Today s pupils know intuitively how to work anything from a new digital video recorder to a mobile phone. It seems logical when thinking of lesson resources that bringing games technology to the classroom is a way to ensure that pupils enjoy, understand and are able to make progress in a subject which is vital to adult life. Andrea Louie Smith, Mathematics Co-ordinator, Knightsfield School. Websites worth a visit Bringing Computer Games into the Classroom this section of the Learning and Teaching Scotland website contains lots of information and links related to research projects in the use of gaming technology. While a number of these projects started some time ago, the site is kept up to date with current developments, including lots of video clips of the technology in use ictineducation/gamesbasedlearning/index.asp/ Clunbury School there is only brief reference to use of the Nintendo DS consoles on the school s own website; however, Teachers TV has a video about ICT use at the school which won an ICT Excellence award in The school is also featured on the Next Generation Learning website in the section about gaming technology AtSchool/Technology/Gaming/ If you would like to contribute anything to these pages, please contact Sharon Pointeer at ICTNewspage@BATOD.org.uk. BATOD Magazine September

58 Regulars This and that news to Film tips Sue Frazer, a BATOD member from the North region, has drawn our attention to a website which contains a deaf film project which she recommends. Called Biomation, it can be found at The website contains a film entitled Survival tips for deaf teenagers. Another film from the website, Top 5 tips for communicating with Deaf young people, was shown at the highly regarded film festival Deaffest 2010 in May this year. You can find out more about the festival at For your student and you They could win a great prize Which may transform their lives We ll tell you how So please join us now The future is bright Just check out our Site! (go to news) Accessible news BATOD member Elizabeth Mackinder would like to share the following snippet of news: Some of you may be aware of the series of reading books that come with a CD which contains a BSL translation of the text. There is now a new addition to this aid to literacy which revolves around the day's news. It is accessible on the internet at It can also be used by those who are learning BSL to increase their receptive skills. The dangers of loud music Reported cases of tinnitus among the young are on the increase, raising the fears of national charities such as Deafness Research UK that with constant misuse of music players at high volume, the so-called 'MP3 generation' are putting their hearing at serious risk and storing up major trouble for the future. These concerns follow in the wake of latest statistics released by the Scottish Executive, showing that over the last 12 months, approximately 6,000 people under 55 consulted their GP reporting tinnitus symptoms. These are much greater numbers and a much younger age range than would be expected, prompting fears that this trend is set to continue unless remedial action is taken. Around one in five people between 55 and 65 years old report tinnitus symptoms, but hearing naturally deteriorates past the age of 55. Some 2,798 people in the age range consulted their GP, which is nearly as many as those reported consulting over 55. The World Health Organisation reports that some four million people in the UK risk damage to their ears by exposure to loud music, and its findings, suggesting that as many of 75% of under-55s are experiencing difficulty with their hearing and tinnitus in particular, are supported by these new figures. This growth in reported tinnitus is taking place amid a troubling lack of awareness among the young of the dangers of loud music. Deafness Research UK continues to fund cutting-edge research, while offering support and advice to people suffering now. The charity has produced a free tip sheet on how to avoid ear damage due to MP3 players and a free fact sheet, Managing Tinnitus, is also available. Both of these publications, together with a wealth of additional free information and guidance, are available from the charity's freephone number on BATOD Magazine September 2010

59 Regulars New programmes from BSLBT Children are curious creatures, with millions of questions floating around in their heads. This is why the British Sign Language Broadcast Trust (BSLBT) commissioned Drip Media Ltd and Remark! to produce a series of education programmes that give those answers that children demand (while also meeting the Key Stage 3 requirement). The two programmes target deaf school children, their peers, their teachers, and other members of their families. The Secret Lab An eccentric scientist, Professor Knowitall (with his two laboratory assistants as his secret weapons) creates a suitable mess in his lab in an attempt to uncover the mysteries that science throws up. The explanations, tailored especially for deaf children, are supported with visual elements, such as actual objects and graphic images. Galloway Wildlife Galloway Wildlife Conservation Park is a wild animal conservation centre set in 27 acres of mixed woodland in the south of Scotland, owned by a deaf director, John Denerley. In the programme, two keen reporters visit the park to meet some rare breeds and discover the habits and effects of endangered species. The two programmes cover content relevant to the Key Stage 3 curriculum. They were aired in August but will be on the BSLBT website for a year BATOD Magazine September

60 Regulars Abbreviations and acronyms used in this Magazine A-level A2-level ADHD AGM AS-level ASD AVUK BAAP Baha BAPA BATOD BOR BSL BSLBT BSL VT BW ccmv CD CDT CHARGE CI CMV CPD CRIDE CSOM CT db DCAL DCSF DESF DfE DS DSP DVA DVD DVR ECG ENT ESRC FEAPDA FM FMA FRC FRG GCSE GP GTC(E) HA HI HOSS ICED ICT IEP ISBN ITE IVF KS LA Advanced Level Advanced Level (Second part) Attention Deficit Hyperactivity Disorder Annual General Meeting Advanced Subsidiary Level (First part) Autistic Spectrum Disorder Auditory Verbal UK British Association of Audiovestibular Physicians Bone-Anchored Hearing Aid British Association of Paediatricians in Audiology British Association of Teachers of the Deaf Branchio-Oto-Renal British Sign Language British Sign Language Broadcasting Trust British Sign Language Vocabulary Test Body Worn Congenital Cytomegalovirus Compact Disk Children s Disability Team Syndrome including Coloboma and/or cranial nerves, Heart defects, Atresia choanae, Retarded growth and development, Genital anomalies and Ear anomalies Cochlear Implant Cytomegalovirus Continuing Professional Development Consortium for Research in Deaf Education Chronic Suppurative Otitis Media Computerised Tomography Decibel Deafness Cognition and Language Research Centre Department for Children, Schools and Families Deaf Educational Support Forum Department for Education Double Screen (Nintendo DS) Digital Signal Processing Dilated Vestibular Aqueduct Digital Versatile Disk Digital Video Recorder Electrocardiogram Ear, Nose and Throat Economic and Social Research Council Fédération Européenne d Associations de Professeurs de Déficients Auditifs (European Federation of Associations of Teachers of the Deaf) Frequency Modulation (radio) Form/Meaning Association Form Recall Form Recognition General Certificate of Secondary Education General Practitioner General Teaching Council (for England) Hearing Aid Hearing-Impaired or Hearing Impairment Heads of Sensory Services International Congress on the Education of the Deaf Information and Communication Technology Individual Education Plan International Standard Book Number In The Ear In-Vitro Fertilisation Key Stage Local Authority LSA Learning Support Assistant MP3 Media Player MRF Meningitis Research Foundation MRG Meaning Recognition MRI Magnetic Resonance Imaging MSc Master of Science NatCen National Centre for Social Research NatSIP National Sensory Impairment Partnership NDCS National Deaf Children s Society NEC National Executive Council NF2 Neurofibromatosis Type 2 NHSP Newborn Hearing Screening Programme NICE National Institute for Clinical Excellence NIHR National Institute for Health Research NPD National Pupil Dataset NVQ National Vocational Qualifications O-levels Ordinary Levels Ofqual Office of the Qualifications and Examinations Regulator Ofsted Inspectorate PDF Portable Digital Format PGD Pre-implantation Genetic Diagnosis PLASC Pupil Level Annual School Census PSHE Personal, Social and Health Education QA Quality Assessment QT interval A measure of the time between the start of the Q wave and the end of the T wave in the heart's electrical cycle RB Resource Base RNIB Royal National Institute of Blind People RNID Royal National Institute for Deaf People RP Retinitis Pigmentosa RSD Royal School for the Deaf Derby RSDCM Royal School for Deaf Children Margate RVM Ramakrishna Vivekananda Mission SAT Standard Assessment Test/Task SEF Self-Evaluation Form SEN Special Educational Needs Sense National charity for people with deafblindness SI Sensory Impairment SIR Speech Intelligibility Rating SNHL Sensorineural Hearing Loss SORD Social Research with Deaf People TA Teaching Assistant TBA To Be Arranged TCS Treacher Collins Syndrome TDA Training and Development Agency (for schools) ToD Teacher of the Deaf TV Television UK United Kingdom UKCoD United Kingdom Council on Deafness US United States USB Universal Serial Bus VAK Visual Auditory Kinesthetic VI Visually Impaired or Visual Impairment VIEW Visual Impairment: Education and Welfare: professional association for teachers of visually impaired children VRA Visual Reinforcement Audiometry If you have found an acronym in the Magazine that isn t explained in this list, then use to help you to work it out. 58 BATOD Magazine September 2010

61 Association business BATOD Magazine September

62 Regulars Meetings and training Calendar This page is an extract from the Calendar to be found on the BATOD website. Please note that it is not exhaustive. Items noted on this Calendar may have been advertised within the Magazine or the information reported by telephone. BATOD is not necessarily the organising body. Please contact the organising body (column 2) for details of conferences, not the Editor of this Magazine. Date Organisation Meeting topic Venue September 22 The Ear Foundation Introduction to Non Professional Counselling The Ear Foundation, all professionals working with deaf children Nottingham NG7 2FB 23 Mary Hare Training Services Earmoulds and Impression Taking Refresher Course Mary Hare School, Newbury RG14 3BQ 24 Mary Hare Training Services Supporting Early Listening Skills Mary Hare School, Newbury RG14 3BQ 24 The Ear Foundation Introduction to Deaf Children for LSAs The Ear Foundation, learning support assistants Nottingham NG7 2FB 25 BATOD NEC Association business TBA 28 The Ear Foundation Cochlear Implants and Children who are Slow to Progress The Ear Foundation, all professionals working with deaf children Nottingham NG7 2FB 29 The Ear Foundation Bilateral Implantation all professionals working The Ear Foundation, with deaf children Nottingham NG7 2FB 30 Mary Hare Training Services Open Day for Professionals (Mary Hare Secondary) Mary Hare School, Newbury RG14 3BQ October 1 The Ear Foundation Working with Children with Cochlear Implants in Primary Exeter Royal Academy for Deaf School Teachers of the Deaf, learning support assistants, Education speech and language therapists 5 The Ear Foundation Behaviour Management: Under 11s all professionals The Ear Foundation, working with deaf children Nottingham NG7 2FB 5 Cochlear Baha Information Focus Holiday Inn Brighouse, near Leeds 5 Cochlear Cochlear Implants Information Focus Holiday Inn Brighouse, near Leeds 6 The Ear Foundation Cochlear Implants and Children with Complex Needs The Howardian Centre, Cardiff all professionals working with complex children 7 9 Downsed Down Syndrome Education Conferences London 7 Auditory Verbal UK Supporting Hearing-Impaired Children in Mainstream AVUK, Bignell Park Barns, Education Chesterton, Bicester, OX26 1TD 11 Mary Hare Training Services Critical Self Review (two-day course: Day Two is Mary Hare School, Newbury 3 December) RG14 3BQ 12 Mary Hare Training Services Transition to HE: facts and experiences Mary Hare School, Newbury RG14 3BQ 13 BATOD North Audiological Competencies: Making a difference Woodlands Conference Centre Southport Road Chorley PR7 1QR 13 Mary Hare Training Services BSA Certificate in Otoscopy & Impression Taking Mary Hare School, Newbury RG14 3BQ 14 Mary Hare Training Services BSA Certificate in Otoscopy & Impression Taking (Paediatric) Mary Hare School, Newbury RG14 3BQ 15 Mary Hare Training Services Working with Cochlear Implants Children in Early Mary Hare School, Newbury Years (babies and toddlers) RG14 3BQ 15 The Ear Foundation Literacy in the Primary Years Teachers of the Deaf, The Ear Foundation, learning support assistants, speech and language therapists Nottingham NG7 2FB 15 Cued Speech Association UK Introductory one-day workshop London The Calendar on the BATOD website is edited as soon as we know about meetings. Additional information about courses and registration forms may also be linked to the calendar entries. 60 BATOD Magazine September 2010

63 Association business Officers of Nations and Regions BATOD contacts and Magazine Distribution Northern Ireland Chairperson: Janice McKillop, 6 Kingsfort Lodge, Old Kilmore Road. Moira, Craigavon BT67 0QG Secretary: Heather Lammey, 45 The Oaks, Newtownards, County Down BT23 8GZ Treasurer: Antonette Burns, 39 Wynchurch Avenue, Rosetta, Belfast BT6 0JP Scotland Chairperson: Jean McAllister, 26 Willowdale Crescent, Glasgow G69 7NL Secretary: Eleanor Hutchinson, Flat 6, Cow Wynd, Falkirk FK1 5EA Treasurer: Anne Pack, 63 High Beveridgewell, Dunfermline, Fife KY12 9ER Wales Chairperson: Revolving post Secretary: Election imminent Treasurer: Rhian Gibbins, Rhianfa, 24c Forest Hill, Aberdulais, Neath SA10 8HD East Chairperson: Kathryn Cutmore, 29 Chapel Road, Tolleshunt D Arcy, Maldon, Essex CM9 9TL Secretary: Sara Brierton, 16 College Road, Impington, Cambridge CB24 9TD Treasurer: Karen Taylor, CSSS, Woodside Road, Norwich NR7 9QL Midland Chairperson: Cate Latchford, Buxton House,The Row, All Stretton, Shropshire SY6 6JS Secretary: Ursula Walker, 207 Bentley s Lane, Walsall WS2 8SP Treasurer: Robert Miller, 13 Derby Close, Broughton Astley, Leicestershire LE9 6BE North Chairperson: Elaine Rayner, 25 Frosterley Drive, Great Lumley, Chester-le-Street, County Durham DH3 4SJ Secretary: Trish Cope, 23 North Drive, High Legh, Knutsford, Cheshire WA16 6LX Treasurer: Mary Fortune, 30 Alexandra Road, Stockton Heath, Warrington WA4 2UT South Chairperson: Mary Ensor, 9 Landrock Road, Crouch End, London N8 9H Secretary: post vacant Treasurer: Donald Allan, Freewaters Cottage, Freewaters Close, Ickleford, Hitchin, Hertfordshire SG5 3TQ South West Chairperson: Wanda Garner, 10 Ashleigh Road, Exmouth EX8 2JY Joint Secretary: Hazel Sutherland, 8 Osney Crescent, Paignton, Devon TQ4 5EY; Denise Tudor, Cliff Court, Cliff Road,Torquay TQ2 6RE Treasurer: Beverley George, 8 Forder Heights, Plymouth PL6 5PZ Articles, information and contributions for the Association Magazine should be sent to: BATOD Executive Officer Mr Paul Simpson tel/fax magazine@batod.org.uk...as should Association information and general queries. Advertisements for the Association Magazine should be sent to: Mr Arnold Underwood BATOD Publishing and Advertising 41 The Orchard Leven, Beverley East Yorkshire HU17 5QA tel/fax advertising@batod.org.uk Full guidelines for submissions and abstracts of papers published in the Journal Deafness and Education International are to be found at Enquiries related to the Journal to: Dr Linda Watson l.m.watson@bham.ac.uk Manuscripts should be submitted online at DISCLAIMER The Editors and the Association do not necessarily endorse items or the contents of advertisements published in the Magazine and cannot accept responsibility for any inaccuracies. Please note that items from this Magazine may not be reproduced without the consent of BATOD and the source must be acknowledged. Photocopying items may breach copyright. BATOD Magazine distribution from: The Seashell Trust, Stanley Road, Cheadle Hulme, Cheshire SK8 6RQ Association Magazine ISSN Published by The British Association of Teachers of the Deaf, 41 The Orchard, Leven, Beverley HU17 5QA Printed by The Nuffield Press Ltd, 21 Nuffield Way, Ashville Trading Estate, Abingdon, Oxon OX14 1RL Magazine Project Manager: Kath Mackrill

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