1. NECHEAR The Effect of UNHS on Early Cochlear Implantation Antonia Brancia Maxon, Ph.D. Diane Brackett, Ph.D. Jennifer Cox, M.A. Alicia Ayles, M.A. New England Center for Hearing Rehabilitation

2. NECHEAR Why is Early Identification of Hearing Loss so Important? Congenital hearing loss interferes with the most basic human need to communicate with others Hearing loss is the most frequent birth defect Undetected hearing loss has serious negative consequences There are dramatic benefits associated with early identification of hearing loss

3. NECHEAR UNHS lowers age of intervention Infants with hearing loss should be fit with hearing aids by four months old Infants with hearing loss should be enrolled in early intervention by four months old

4. NECHEAR UNHS lowers age of intervention When a six month hearing aid trial is used –Benefit from traditional amplification can be determined by 10 months old Infants who do no benefit should not have to wait for the implant.

5. NECHEAR UNHS affects the age of cochlear implant candidacy identification Until recently the recommended age of implantation was 18 months old. Now 12 months is recommended lowest age. Surgeons are implanting even younger infants.

6. NECHEAR Early Identification Ensures Some Critical Criteria Are Met Objective measures are available to make a diagnosis within the first month of life. –will know degree of hearing loss –will know the configuration of hearing loss –will know if there are middle ear problems Can begin process of candidacy evaluation

7. NECHEAR Early Identification Ensures Some Critical Criteria Are Met Parents have accepted the hearing loss and are ready to begin appropriate intervention. Parents are aware of communication modality options. Parents know the role of and need for sensory devices.

8. NECHEAR Candidacy Bilateral severe to profound sensorineural hearing loss Infant/toddler cannot benefit from traditional amplification

9. NECHEAR Diagnosis to Decision - Parent Need to understand: – no device is a cure – therapy is long-term – language learning is an ongoing process – access to acoustic speech cues how access relates to child’s progress

10. NECHEAR Diagnosis to Decision - Parent Need to: –understand the difference between reacting to cues received through hearing and other senses –identify auditory responses –learn how to use the home, family, environment in a positive way for auditory and language learning

11. NECHEAR Diagnosis to Decision - Parent Need to be comfortable with equipment –know how to use it properly –know how to modify clothing to accommodate it –accept that people will look, question, comment on it –able to troubleshoot equipment and child’s auditory behavior

12. NECHEAR Diagnosis to Decision - Parent Need to understand the audiogram –know the degree and configuration –know the basics of acoustic cues of speech –know how speech perception is affected by their child’s hearing loss –know what their child actually receives

13. NECHEAR Diagnosis to Decision - Child Child needs to be fit with best amplification –must get the most auditory stimulation possible body-worn FM system snap-on transducers power transducers earmolds - good fit at all times

14. NECHEAR Diagnosis to Decision - Child Child needs to be enrolled in an EI program that stress auditory function –learning to use hearing –learning to respond to sound –learning to respond to speech –learning the need for communication

15. NECHEAR Assessing Auditory Function From initial enrollment in EI always assessing auditory function Should not make decision about hearing aid or cochlear implant based on pure tone audiogram Need to determine if child is able to make maximal use of residual hearing

16. NECHEAR Assessing Auditory Function Child needs to receive speech at an appropriate level with amplification –detection is not enough –have to look across frequencies for access to all speech sounds –speech needs to be received at a useable level

17. NECHEAR Assessing Auditory Function Child must have good access to speech of others and own speech – at different distances from sound source Child should alert to a variety of speech and non-speech sounds –at different distances from sound source

18. NECHEAR Assessing Auditory Function Child’s output –vocalization precursor to speech production –intentional vocalization precursor to language –vocalization + gesture intention to communicate

19. NECHEAR Indications the Child is a Candidate Child shows limited or no detection of amplified speech Comparisons of audiological data and functional use of hearing Knowledge of the child’s auditory limitations

20. NECHEAR Factors that Facilitate CI Success Parents know about hearing loss and accept long-term problems Parents understand the implant is not a cure Parents are committed to implant use Parents are committed to therapy

21. NECHEAR Factors that Facilitate CI Success Family has access to therapy and mapping facilities Family is motivated One parent at home - minimal day care The household is organized Child is vocalizing Child has normal cognitive level

22. NECHEAR Absence of one or more of those facilitating factors does not rule out the child’s candidacy.

23. NECHEAR Why Implant Early Critical period of communication development Children implanted below 10 years old –children implanted before age 3 showed a greater period of auditory plasticity –open-set speech recognition could not be achieved after 6 years old (Manrique, et al, 1999)

24. NECHEAR Why implant early? Early is better for children with hearing aids (Apuzzo & Yoshinaga-Itano, 1995) Early implantation –allows for extended listening time before social and school demands start –decreases chance of a large gap between chronological and communication age –promotes acquisition of speaking and language

25. NECHEAR Why implant early? Children implanted early (2-3 years) make greater growth than later implanted (3-5 years) children –expressive vocabulary –receptive vocabulary –dramatic improvements first year post implant (Brackett and Zara, 1998)

26. NECHEAR Why implant early? Surgical risks are minimal Complications are infrequent Objective testing can be used to predict appropriate programming Can use audiological behavioral measures for programming (Cohen, Drous, Shapiro & Waltzman, 2003)

27. NECHEAR Information Needed by Parents Parents wanted most information prior to surgery, but wanted continued informational support post-implant Parents felt emotional support was most lacking Majority of parents felt there needed to be a professional liason between CI center and educational program (Most and Zaidman-Zait, 2003)

28. NECHEAR Cochlear Implant Surgeon Selection Competent and experienced with infants Familiar with surgical placement of receiver/stimulator package that reduces potential damage from head impact

29. NECHEAR Cochlear Implant Surgeon Selection Familiar with techniques that reduce intracranial complications Willing to make accommodations for child and family regarding hospitalization, pre- and post-surgery conditions

30. NECHEAR Mapping Audiologist Selection Competent and experienced with infants Familiar with VRA Availability of multiple testers Availability of appropriate equipment and reinforcement

31. NECHEAR Mapping Audiologist Selection Knows about speech acoustics Uses functional measures to fine tune the map Accepts input from parents and rehabilitation professional Maps the implant “on demand” Knows about aural rehabilitation

32. NECHEAR Surgery to Stimulation Child –becomes familiar with and gets used to equipment –uses hearing aid on non-implanted ear Aural rehabilitation –communication focus continues –conditioning sessions to facilitate mapping

33. NECHEAR Initial Stimulation Short term goal is good access to speech across frequencies Weekly sessions until have a good map Use input from parent(s) and rehabilitation professional Use functional responses to modify maps

34. NECHEAR Rehabilitation Professional Selection Knowledgeable about: –infant behavior –infant development –infant communication –auditory development –activities that facilitate auditory-vocal behavior

35. NECHEAR Rehabilitation Professional Selection Knowledgeable about: –infant adaptation to equipment –home therapy suggestions –troubleshooting the equipment –troubleshooting the child’s behaviors

36. NECHEAR Rehabilitation Programming Keeping external components on head Keeping body processor comfortable Continued conditioning for mapping when necessary Mapping as required –parent request –rehabilitation professional request

37. NECHEAR Case Study - Stevie UNHS - failed bilaterally (before mandate in CT) Bilateral profound SNHL diagnosed - 5 mos Hearing aids fit - 5 mos Enrolled EI (2x/week) - 6 mos FM fit - 10 months

38. NECHEAR Case Study - Stevie Aided response levels - 50-60 dB HL Aided SAT - 45 dB HL CI evaluation - 10 mos CI surgery - 13 mos Initial stimulation - 14 months

39. NECHEAR Case Study - Stevie (evaluation at 32 months) Speech perception: 100% spondees, 83% monosyllables Speech production: 75% consonants, 97% vowels Vocabulary: Receptive - not basal, Expressive - <1.9 years Utterances: 4-5 words, not many verbs, rising inflection for questions

40. NECHEAR Case Study - Stevie (evaluation at 46 months) Speech perception: 100% consonants, 100% vowels Speech production: 90% consonants, 100% vowels Vocabulary: Receptive - 10 month delay, Expressive - 8 month delay Sentences: 5-7 words, nouns and verbs some adjectives, possessive, plural markers emerging, some pronouns.

41. NECHEAR Case Study - Anna UNHS - failed bilaterally (post mandate) Bilateral profound SNHL diagnosed - 3.5 weeks Hearing aids fit - 4 weeks Enrolled EI (2x/week) - 4 weeks Hearing aids and FM fit - 4 weeks

42. NECHEAR Case Study - Anna Aided response levels - 40-50 dB HL Aided SAT - 35 dB HL CI evaluation - 10 mos CI surgery - 11 mos Initial stimulation - 12 months

43. NECHEAR Case Study - Anna (evaluation 28 months) Speech perception: excellent Speech production: errors - m/n, g/d, omits ch, t, m, z Vocabulary: Receptive - 21 months, Expressive - 21 months PLS: Auditory Comp - 31 months, Expressive Comp - 28 months, Total Language - 30 months

44. NECHEAR Universal Newborn Hearing Screening Lowers the age of hearing loss diagnosis Lowers the age amplification is provided Lowers the age of enrollment in early intervention POTENTIALLY lowers the age of cochlear implantation

45. NECHEAR Benefits of Early Cochlear Implantation Minimizes the effects of auditory deprivation Electrical stimulation provides necessary information to auditory system at critical period for speech and language acquisition. Provides child with a good chance to develop normal speech and language