SCIC. Australia s largest cochlear implant program. Presenter: Sharan Westcott. Audiometry Nurses Association of Australia 2013

Transcription

1 SCIC Audiometry Nurses Association of Australia 2013 Australia s largest cochlear implant program Presenter: Sharan Westcott

2 SCIC Topics for today Overview of SCIC services including remote services Bilateral vs Unilateral CI Cochlear Implant Outcomes for children with CMV cytomegalovirus)?

3 Cochlear Implants today 270,000 cochlear implant recipients worldwide 8,000 cochlear implant recipients in Australia 30+ years of research, knowledge & experience Internal: Cochlear Implant External Speech Processor

4 SCIC Cochlear Implant What is a Cochlear Implant?

5 SCIC: Australia s largest cochlear implant program SYDNEY: Gladesville & Penrith, REGIONAL: Newcastle, Canberra, Gosford, Lismore, Port Macquarie, Northern Territory

6 SCIC Founded in 1984 Australia s largest cochlear implant program Whole of life program Provide services to children and adults of all ages, with significant hearing loss Services at no cost to the client Metropolitan, rural and outreach sites across Australia Over 3,000 surgeries Highly specialised clinical teams

7 SCIC Main Office, Old Gladesville Hospital, Sydney SCIC: a not for profit Cochlear Implant Service

8 SCIC permanent & visiting sites Services are provided at: 5 centres in Sydney & regional NSW 20 visiting sites throughout NSW & NT

9 SCIC Clients STYLE Logos, headings, sub headings and text boxes ARE NOT TO BE ALTERED This means, no moving around, no deleting, etc This will ensure that when flicking through slides, the heading transition and text position is seamless

10 SCIC Clients Active client base is over 2, clients have bilateral implants Youngest child implanted = 3 months Oldest adult implanted = 94 years = 355 cochlear implant surgeries

11 SCIC CI multi-disciplinary team Professional team: EAR Surgeons Audiologists Habilitationists/Therapists Family Counsellors (Social worker/psychologist) Electrophysiology team: Biomedical engineer, medical Scientist & audiologists. Supported by: Management & Administrative team SCIC Board

12 SCIC TEAM

13 SCIC surgical sites 10 SCIC surgeons operating at: 8 hospitals in Sydney metro, 2 in Newcastle 2 in Canberra 1 in Darwin 1 in Port Macquarie (near future) SCIC Clinics are not co-located in hospitals

14 Cochlear Implant Surgery Surgery 2 hours 30 mins. 1 night stay in hospital or day stay for young adults Comprehensive testing at all SCIC surgeries to ensure device is working (EABR)

15 No of Patients Age at First Implant Age at First Implant (Years)

16 Is Age at Surgery a Factor in Outcomes? No statistical difference in speech perception outcomes between age groups Elderly: >70 at surgery Controls: at surgery N= N= Word Sentence Word Sentence Pre-Op Post Op

17 Who would benefit from a cochlear implant : Australian Criteria Unaided hearing Moderate-severe sensorineural hearing loss in the low-mid frequencies (+ poor aided speech scores) Severe-profound sensorineural hearing loss in the mid-high frequencies

18 Hearing Threshold (db HL) (ANSI ) Traditional CI vs. Hybrid Candidates Current -10 Indications for CI Hearing Threshold (db HL) (ANSI ) Frequency (Hz) Shaded area indicates hearing loss suitable for Combined Hearing Aid & Cochlear Implant Frequency (Hz) acoustic amplification Hearing Threshold (db HL) (ANSI ) Hybrid Candidacy Criteria Frequency (Hz) electrical stimulation with cochlear implant > 10% but 60% Aided word score in ear to be implanted 80% word score in the contralateral ear

19 New candidates Single sided deafness (SSD) Normal hearing in one ear and a profound or total loss of hearing in the other ear Example Left = normal Right = total hearing loss

20 Soft Sounds CI CI CI IMPROVED ACCESS to Hearing CI CI CI= Cochlear Implant thresholds Speech Area Severe h/loss AIDED Profound h/loss AIDED

21 Research shows children with a moderate to severe hearing loss greater than average 65dB would benefit from a cochlear implant Leigh et al, 2008 Sentence recognition comparing children using hearing aids and cochlear implants

22 No. children Impact of Universal Newborn Hearing Screening on age of first cochlear implant in SCIC children <10 years old Universal Newborn Hearing Screening

23 MAPping a toddler s hearing

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25 2 Ears are better than one 586 clients have bilateral implants 69 had simultaneous surgeries

26 Cost of CI Device and surgery: PUBLIC Fully covered for under a state health allocation. NSW and Victoria have best funding allocation for adults. Funding for 1 device per adult Currently public waiting times in NSW for adults about 6 months. No waiting times for children, public bilateral funding available

27 Cost of Device and surgery: PRIVATE Currently device is fully funded by Private Health insurance including bilateral device SCIC surgeons charge no gap so surgery is covered by Private Health. This is a question that needs to be asked if client does not want out of pocket expenses. Gap can be as large as $5,000 Anaesthetist: may charge a gap

28 SCIC Outcomes for bilateral Bilateral cochlear implantation in children identified in newborn hearing screening: Why the rush? Research: Maree McTaggart and Kylie Chisholm

29 Background: European Consensus Statement European Bilateral Pediatric Cochlear Implant Forum Consensus Statement Currently we feel that the infant or child with unambiguous cochlear implant candidacy should receive bilateral cochlear implants simultaneously as soon as possible after definitive diagnosis of deafness to permit optimal auditory development; an atraumatic surgical technique designed to preserve cochlear function, minimize cochlear damage, and allow easy, possibly repeated reimplantation is recommended. JD Ramsden, K Gordon, A Aschendorff, L Borucki, M Bunne, S Burdo, N Garabedian, W Grolman, R Irving, A Lesinski-Schiedat, N Loundon, M Manrique, J Martin, C Raine, J Wouters, & BC Papsin Otology & Neurotology (2012), 33 (4)

30 Background: European Consensus Statement cont d Benefits of bilateral CI in children Improved speech understanding in background noise Improved listening in the real world even when audiological test results suggest minimal improvement Speech & language can develop more rapidly Risks of delaying bilateral CI In the early stages of bilateral CI use: Asymmetries have been noted in bilateral brainstem and cortical function Poorer speech perception and detection of speech in noise in the 2 nd implanted ear compared to the 1 st

31 Question still to be answered: 1. With sequential cochlear implantation, do patients who have a short delay between the 1 st and 2 nd CI do better than those with a long delay (is there a critical window)?

32 AIM: This study aims to investigate: 1. The impact of receiving one or two cochlear implants on speech & language development, and functional auditory behaviour. 2. If bilateral implantation has a positive impact on speech & language development, is there any difference between children who receive their 2 nd implant sequentially vs. simultaneously?

33 Methods: Participants Group 1 Unilateral Cochlear Implant N = 9 (bimodal in 2 subjects, 2 intermittent HA users) Group 2 Bilateral sequential Cochlear Implant N = 10 Group 3 Bilateral simultaneous Cochlear Implant N = 9 < 18 months Mean age: 9m Mean age CI1: 8m Mean age CI2: 15m Mean age: 9m

34 Methods: - Moeller Family Rating Moeller Rating (Rated retrospectively by early interventionists) 1 = limited participation 2 = below average participation 3 = average participation 4 = good participation 5 = ideal participation

35 Methods: - Assessments IT-MAIS (Questionnaire) 1. Vocal behavior affected while wearing h/aids or CI? 2. Production of syllables & syllable-sequences recognized as speech? 3. Spontaneous alerting to name in quiet -auditory cues only? 4. Spontaneous alerting to name in noise -auditory cues only? 5. Spontaneous alerting to environmental sounds -auditory cues only? 6. Spontaneous alerting to sounds in new environments -auditory cues only? 7. Spontaneous recognition of sounds auditory cues only? 8. Discriminates between 2 speakers voices auditory cues only? 9. Discriminates between speech & non-speech stimuli auditory cues only? 10. Responses to intonation (motherese) auditory cues only? 0 = Never 1 = Rarely 2 = Occasionally 3 = Frequently 4 = Always

36 Results: Moeller family rating No significant difference between groups Unilateral Sequential bilateral Simultaneous bilateral 1y: m= 4.2 SD=.83 1y: m= 4.3 SD=.82 1y: m= 3.8 SD= y: m= 3.4 SD= y: m= 3.7 SD= 1.7 2y: m= 3.4 SD= 1.51 While parental engagement may be a contributor to outcomes, in this study the family ratings were not significantly different across groups. 1y: F= 0.73, p= y: F= 0.11, p= 0.90

37 Results: - PLS-4 (Year 1 & 2) PLS (Preschool Language Scale) Pre CI 1 yr post 2 yrs post Receptive Expressive +1 SD Mean -1 SD Repeated measure ANOVA: Significant improvement over time for the 3 groups (F=15.92, p<.001) No significant difference between groups (F=.94, p=.41) Repeated measure ANOVA: Significant improvement over time for the 3 groups (F=8.1, p=.02) No significant difference between groups (F=.85, p=.44)

38 Results: - Expressive Vocabulary Growth (6-12mths post-so) mths 12 mths Unilateral unilateral Sequential sequential simultaneous Simultaneous N=9 N=10 N=9

39 Results: - Functional benefit IT-MAIS [Infant Toddler- Meaningful Auditory Integration Scale] Pre-CI 6 mths 12 mths unilateral sequential simultaneous Unilateral Sequential Simultaneous Pre: F=.27, p=.77 6m: F= 1.53, p=.24 1y: F= 3.68, p=.04

40 Conclusion & Discussion: Question 1: What is the impact on speech & language development, and functional auditory behaviour of bilateral cochlear implantation (compared to unilateral CI)? Children implanted unilaterally did not appear to be disadvantaged in their speech, language & functional outcomes when assessed at 1 and 2 years post-ci (NB all received their 1 st CI <12 months of age) Ongoing monitoring of performance is needed to investigate whether children implanted bilaterally show greater long-term gains than those implanted unilaterally.

41 Conclusion & Discussion: Question 2: Is there any difference in those who receive their 2 nd implant sequentially or simultaneously? Children who were implanted bilaterally did not appear to be disadvantaged in their speech, language & functional outcomes if their second cochlear implant was delayed. Findings are consistent with the European Consensus Statement : There is no evidence to date to suggest that children undergoing sequential procedures with short inter-implant delays of less than 1 year perform any differently to children receiving simultaneous implants

42 Conclusion & Discussion: It is important to note that: 1. All sequential subjects in this study received their 2nd CI at <18 months of age, which is still relatively early 2. Higher level binaural processing skills were not evaluated in this study, which is an area for future research.

43 Practical clinical considerations: Benefits of simultaneous bilateral CI: 1. One anaesthetic & surgical session: reduces parental stress & financial cost of a 2 nd hospital stay) 2. One acute period for switch-on and familiarization with the signals from both ears : easier for families and reduces cost associated with a second series of switchon & mapping appointments 3. One series of acute habilitation sessions : easier for families and reduces costs associated with habilitation All of the above are particularly relevant for families who have to travel long distances to access services.

44 Further practical clinical considerations: Potential challenges with sequential bilateral: Encouraging the child to practice wearing their new implant alone when they are already very comfortable and reliant on the first.

45 SCIC Helping all ages to hear CMV and cochlear implants Longitudinal outcomes Maree McTaggart 1, Catherine Birman 2, Carmel Ramsey 1 and Colleen Psarros 2 1 SCIC Newcastle 2 SCIC Sydney

46 CMV and CI aims of paper What is CMV and how does it present? How is CMV diagnosed? What is the long term outcome of CI recipients with CMV at SCIC?

47 What is CMV and how does it present? Human Cytomegalovirus (HCMV) is an extremely common viral infection affecting between 10-25% of the entire population at any one time. Most healthy people who are infected by HCMV after birth have no symptoms. Adults with reduced immunity may develop a fever, headaches, sore throat, overwhelming tiredness, muscle and joint pains, enlarged lymph nodes and a tender liver. In patients with severely reduced immunity, pneumonia and hepatitis may develop.

48 Congenital CMV Congenital CMV infection occurs when a mother suffers a primary infection or a reactivation of a previous infection during pregnancy. CMV is one of the TORCH infections that lead to congenital abnormalities. If a pregnant woman with reduced immunity acquires a significant CMV infection, her baby may be affected in the womb. Up to 5/1000 live births are affected.

49 Congenital CMV Congenital CMV is under diagnosed as it is frequently asymptomatic at birth. Congenital CMV causes 20-30% of all sensorineural hearing loss (SNHL) in children the most common single cause (Fowler et al, J Clin Virol, 2006).

50 Congenital CMV Hg loss can occur in up to15% of babies who are asymptomatic at birth, and 66% who are symptomatic. Variability in the severity of hearing loss Unilateral or bilateral Late onset SNHL occurs

51 CMV can harm the foetus at any point during pregnancy. Prenatal Cytomegalovirus

52 How is CMV diagnosed? Infectious CMV may be shed in the bodily fluids of any infected person. Amniocentesis at > 20 weeks gestation can be done to detect fetal transmission from the mother. The window of opportunity for determining the presence of CMV in a baby in their blood and urine is the first three (3) weeks of life. Laboratory Diagnosis of CMV infection: Serology (CMV IgM and IgG, in blood) Viral culture (urine/saliva) CMV PCR (blood, urine, dried blood spot).

53 Saliva swab- Identified 59 of the 60 (98%) of the children with confirmed congenital CMV Dried Blood spot PCR- Identified 17 of 60 (28%) of children with confirmed congenital CMV JAMA April 14, 2010

54 What are the long term implications? Once infected CMV remains latent in the body for the rest of the person s life. Early predictors of poor cognitive outcome are: Microcephaly (most specific predictor), and an abnormal CT (most sensitive predictor). All other presentations including SNHL are not predictive. If no neurological abnormality is present by one year, then the child is unlikely to be at an increased risk of neurodevelopmental impairment (Ivarsson et al, Pediatr, 1997). However, late onset SNHL may occur.

55 Confirmed vs unconfirmed CMV cases 19 N= Confirmed Unconfirmed

56 CMV as cause of deafness in children implanted at SCIC, over the past 3 decades. (% incidence of population implanted at SCIC) 6 / 272 / % 2.2% Newborn screening introduced 3.4% 15 /

57 Additional disabilities in CMV cases at SCIC Developmental Delay None 13 none Learning difficulties Cerebral palsy Developmental delay CP+DD CP+DD+Vision DD+Vision Other 1 3

58 CATEGORY OF AUDITORY PERFORMANCE (CAP) (1) 0 No awareness of environmental sound 1 Awareness of environmental sound 2 Responds to speech sounds 3 Recognises environmental sounds 4 Discriminates at least two speech sounds 5 Understands common phrases without lipreading 6 Understands conversations without lipreading (familiar) 7 Uses telephone with familiar talker

59 CMV CAP scores Category of Auditory Performance (CAP) (1) No awareness of environmental sound 1 Awareness of environmental sound 2 Responds to speech sounds 3 Recognises environmental sounds Discriminates at least two speech sounds 5 Understands common phrases without lip reading 6 Understands conversations without lip reading (familiar) 7 Uses telephone with familiar talker CAP 0 CAP 1 CAP 4 CAP 5 CAP 6 CAP 7

60 CMV: CAP and Additional disabilities 7 Scatterplot of cap score against Additional disabilities Spreadsheet1 in CMV stats 10v*50c cap score = *x cap score Additional disabilities

61 Psychophysics stability over time Recipient with 20yrs use yr T 20yr T 10yr C 20yr C Channels mapped BP+2

62 Long term CMV cases CI22M Recipient 17 years yr T 17yr T 10yr C 17yr C Recipient with cerebral palsy, developmental delay Electrodes Map CG Recipient of 17yrs (BP+2) 10yrs (CG) 10yrT 17yrT Recipient with no additional needs yr C Channels

63 Impedances kohms Recipient with no additional needs Impedance (kohms) Recipient with cerebral palsy and developmental delay Current Long term CMV cases CI 24RST Impedances and Psychophysics Recipient 13yrs Impedances Recipient 13 yrs map T 2yrs T 13yrs C2yrs Channels MP 2yrs MP 13yrs CG 2yrs CG 13yrs channels Recipient 13yrs Impedances Recipient 13 yrs map T 2yrs T 13yrs C 2yrs Channels Imp MP 2yrs Imp MP 13yrs Imp CG 2yrs Imp CG 13yrs Channels

64 Occupations of CMV adults Truck driver Veterinary nurse Butcher Hairdresser Trainee nurse Disability pension (2) Remainder are school aged (26)

65 Communication mode augmentative communication Auslan Total Communication Oral 15% 6% 61% 18%

66 In Conclusion It is the additional disabilities that impact on outcomes and management of CI recipients, not the CMV itself. There is no evidence of the latent CMV reactivating at this stage (20 years of data on one case). There has been only one case of a deteriorating SNHL. It was not identified that a reactivation of CMV was the cause of the progressive loss, however it was also not ruled out.

67 SCIC Helping all ages to hear SCIC AUSTRALIA S LARGEST COCHLEAR IMPLANT PROGRAMME SYDNEY NEWCASTLE CANBERRA GOSFORD LISMORE PORT MACQUARIE PENRITH DARWIN ALICE SPRINGS First.last@scic.org.au