Deaf Children and Young People

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1 Deaf Children and Young People Professor Barry Wright Clinical Lead - National Deaf Children Young People and Family Service, York National Deaf Child and Adolescent Mental Health Service (NDCAMHS)

2 Definitions World Health Organisation (2008) British Society of Audiology (2004) Mild: db Mild: db Moderate: db Moderate: db Severe: db Severe: db Profound: >80 db Profound: >95 db

3 Definitions World Health Organisation (2008) British Society of Audiology (2004) Mild: Able to hear words spoken at a normal voice at 1m Moderate: Able to hear words spoken in a raised voice at 1m Severe: Able to hear some words shouted in the better ear Profound: Unable to hear and understand a shouted voice Mild: Difficulty following speech Moderate: Likely to need hearing aid to hear speech Severe: Likely to rely on lip reading and hearing aid Profound: Unable to hear and understand a shouted voice

4 Prevalence of Deafness Profound deafness in the UK: 9 per 10,000 3 year olds. 16 per 10,000 9 to 16 year olds

5 Causes of Deafness - 1 Sensorineural deafness Problems either in hair cell function, in the nerve transmission of sound or in the auditory cortex.

6 Causes of deafness - 2 Problems with the transmission of sound from the outer ear, through the middle ear to cochlear. Commonest cause is Otitis Media with effusion (OME) glue ear. 50% of children aged 2 4 year old will have at least one episode of OME

7 Causes of deafness - 2 In utero infections e.g. Rubella Cytomeglovirus Toxoplasmosis In utero damage from drugs Chemotherapy agents, some antibiotics In utero anoxia Jaundice the commonest cause of neonatal acquired deafness in the UK in the 1950s and 60s. This has now dramatically reduced.

8 Causes of deafness - 3 Syndromes e.g. CHARGE, USHER etc Non-syndromic genetic hearing loss Well over 30 single gene mutations have been identified to cause predominantly deafness Almost 50% are gene problems codes for the intracellular channel protein Connexin 26 in the inner ear Autosomal recessive Other more common genes include GJB6 and MYO6

9 Diagnosis: Screening and Testing In the UK all babies are screened within a few days of birth. Transient evoked otoacoustic emissions (TEOAE) followed by automated auditory brainstem response testing (AABR). TEOAE Computerized listening to the cochlear response when an electronic click is made down the ear canal. The median age for fitting hearing aids in a profoundly deaf infant is 16 weeks in the UK. AABR

10 Neuroimaging Imaging research shows that language centres work as well with a signed language as a spoken language. [Brocas area (inferior pre-frontal region) and Wernickes (superior temporal region)]

11 Outcomes It is not possible to define one specific outcome common to deaf children. Deaf children are all unique. There is a complex overlap of multiple factors: Cause of deafness Severity Age of onset Co-morbidities Communication factors Educational opportunities Family and community support Each child is uniquely individual Robust public health, communication and education policies are essential

12 Deaf Culture and Sign Language The use of sign language as first language is usually central to the definition of Deaf Culture. Many Deaf people do not consider the absence of one sensory modality to be a disability, but rather a difference. Some deaf parents do not seek hearing aids, cochlear implants and genetic testing for their children.

13 Deaf Culture and Sign Language - 2 The term Deaf (with a capital D) is used to describe the cultural identity of the Deaf community. Most deaf people find the terms hearing impairment, hearing disability, hearing problems, deaf and dumb or deaf mute offensive percent of deaf children will be born to a deaf parent. Deaf children born to hearing parents often face difficult choices

14 Deaf Culture and Sign Language - 3 Sign Language Each country has their own language (i.e. BSL, ASL) Presented in sign space in front of the body and directed to the vision of the person / persons receiving. Facial expressions essential part of language

15 Education Choices Sign language schooling Schools with sign-supported oral language Deaf schools focusing on an oral language Mainstream education with hearing impaired unit Mainstream school Learning Disability School (Special School) Local Schools Doncaster School for the Deaf St John s Wetherby Hanson Bradford

16 Education In the 1990s about 50% of deaf children were in specialist deaf schools. Today only 4% are in specialist schools. The remaining are in mainstream schools with deaf inclusion units with varying degrees of quality and varying policies about communication methods. In school deaf children tend to gravitate towards one another.

17 Cognition Intelligence Deaf children with no other neurological deficits and with healthy cultural and linguistic experiences have the same IQ as hearing children. Many studies report lower mean IQs for deaf children compared to hearing children but care should be taken in interpreting the results. Co morbidities and differing family and educational backgrounds confound the results. Assessment of intelligence in deaf children is not straightforward. Most instruments were designed for testing hearing children. Effects of co morbidities on Cognition Each syndrome or condition associated with deafness may have idiosyncratic effects on cognition. e.g. CHARGE syndrome is associated with global developmental delay and 70% of children with CHARGE syndrome will have an IQ below 70.

18 Language Deaf children born to hearing parents may be significantly delayed in spoken language compared to hearing children 2/3 are still at only one-word level at three years old. Deaf children with deaf parents using BSL have no language delays Some parents are told not to sign with their children to improve their development of oral language, but there is little evidence for this advice

19 Language continued Children with Otitis Media with Effusion (OME) About a third have problems with expressive language Long-lasting OME can cause articulation and sound discrimination problems at age 9 years. Most children with OME have no long-term language and literacy problems.

20 Visual learning Visual periphery and motion processing skills are often enhanced in deafness. A greater amount of the visual cortex is dedicated to peripheral than central processing in deaf compared to hearing individuals.

21 Academic Many deaf children underachieve in numeracy and literacy. Deaf children taught spoken language often struggle with written language, reading and comprehension. Typical reading delay on leaving school is 5years Children with OME have higher rates of inattention and over-activity % of deaf children have additional educational needs (over and above communication needs) Nearly half of all deaf children in the UK leave school with no qualifications. Only 7% left school with any A 'Levels.

22 Parental stress Many hearing parents experience stress and adjustment when they learn they have a deaf child. This parental stress occurs at the same time as the child s crucial development Most parents are caring and loving. Some parents may be overprotective. Some may be rejecting

23 Environmental learning Deaf children frequently miss out on incidental (background) comments. All their learning is needs based and face-to-face. Deaf children are often not included in everyday conversation. They may be less exposed to emotional and social language Some deaf children have less accrued knowledge than hearing children ( fund of knowledge )

24 Theory of Mind Theory of Mind = Being able to see things from another s perspective and make accurate guesses about other s feelings, attitudes and beliefs. Deaf children from hearing families show significant delays in ToM. (But not those from deaf families). ToM delays in deaf children improve with age. This is different from ASD.

25 Social Deaf children are more likely to have social and emotional delay than hearing children. Factors influencing degree of social immaturity: degree of hearing loss, age of onset of deafness, theory of mind delay and the presence of co-morbidities. Deaf children in mainstream school with hearing children have high degrees of anxiety and low self-esteem especially related to communication issues. This is less prevalent in specialist deaf schools.

26 Mental Health problems 26% of deaf children with BSL in the community (who are not being seen by child mental health services) are above the threshold for mental health problems that impact upon their day to day lives Factors that increase the likelihood of problems: Low IQ, poor parent-child communication, co-morbid physical problems and language delays.

27 Autism Spectrum Disorder Autism occurs in about 1.6% of deaf children in US (Compared to 1% in hearing children) Some disorders (such as rubella, CMV and CHARGE) may specifically increase rates of autism. However some deaf children are misdiagnosed with ASD WHY?

28 Interventions Hearing aids/cochlear Implant Language development Communication Visual/environment Cultural Educational Social Communication technologies Mental Health Early Intervention and Parent support/teachers of the Deaf Genetic

29 Interventions IMPORTANT NOTE: Developmental Delay in deaf children is caused mainly by co-morbidities such as neurological problems or syndromes. The other cause of delays is mediated by the environment. THIS IS THE AREA WHERE PROFESSIOANLS AND FAMILIES CAN INTERVENE.

30 Cochlear Implantation

31 Cochlear Implants In the past some proponents of CI have advised families not to learn to sign to avoid early recruitment of the auditory cortex. Bilingualism appears to be the most effective method of ensuring children have access to (one or the other or both) language from as early as possible.

32 Cochlear Implants May improve reading and writing skills Improved oral language acquisition Understanding a conversation without lipreading and speaking on the telephone

33 Communication Technologies Texting Internet Minicom Vibration and light alarms Hearing dogs for the deaf

34 Cultural Deaf parents usually bring their children up in the Deaf culture. Hearing parents vary in their approach and face a range of challenges. There are practical and social challenges to provide a deaf child from a hearing family with access to the Deaf culture. Children often want to choose the older they get

35 Communication In order for children to develop emotionally, socially, culturally and educationally they need language and communication as soon as possible after birth. Visual or oral or both

36 Early Intervention Lack of early intervention increases risk of developmental delay. Helping parents make language visual for children is a useful intervention. Parents may require specific support in issues such as boundary setting, learning social rules and communicating.

37 SUMMARY Deaf children are at greater risk of developmental delay The main key areas that affect the likelihood of developmental delay are: Degree of hearing loss Co-morbid difficulties Responses of the family and community

38 For animated films made by deaf children See Biomation/org to access these free. Thanks to Simon Collins and Dan Axon Thanks also to Ben Sessa for helping with locating pictures and supporting material for this presentation, which has been taken from a book chapter by Prof Barry Wright For National Deaf CAMHS see: and

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