COCHLEAR IMPLANTS Chapter 18

1. COCHLEAR IMPLANTSChapter 18 THE FACTS

2. CI Manufacturers • Medel • Bionics • Cochlear

3. Who Makes the Decision for CIandWhen Should CI for Children with HI? • Educator of the Deaf? Parents? Physician? Audiologist? Child? Team? Others? • Before 1 year, 18 months, 2 years, before 5? • When person is old enough to make decisions? • Never?

4. CI Factoids • About 70,000 people worldwide have cochlear implants • About 25,000 people in the United States have cochlear implants • About half of all CI recipients are children • CIs can help an estimated 200,000 children in the United States who do not benefit from hearing aids. • The demand for CIs is increasing annually by 20%. • About 250 hospitals across the country perform cochlear implant procedures. • A recent study on cochlear implants demonstrated that special education in elementary school is less necessary when children have had "greater than two years of implant experience" before starting school.  These children are mainstreamed at twice the rate or more of age-matched children with profound hearing loss who do not have implants. • The benefits of a CI to society amount to a lifetime savings of $53,198 per child. • By the time a child with hearing loss graduates from high school, as much as $420,000 can be saved in special education costs if the child is identified and given appropriate early intervention.

5. CI Study of 181 Children • Most parents had normal hearing, majority (white) and had more education and higher incomes than the general population. • Families tended to be intact with both a mother and a father who involved their child with CI in family activities on a regular basis. • Children enrolled in the full range of educational placements available across the United States and Canada. • Fairly even distributions of children from public and private schools, special education and mainstream classes and oral and total communication methodologies were represented. • Educational placement changed as children gained increased experience with a cochlear implant. • They received an increased emphasis on speech and auditory skills in their classroom settings and tended to move from private school and special education settings to public school and mainstream programs. • Data support the position that early cochlear implantation is a cost effective procedure that allows deaf children to participate in a normal school environment with hearing age mates. Geers A, Brenner C. (2003) Background and educational characteristics of prelingually deaf children implanted by five years of age. Ear Hear. 24(1 Suppl):2S-14S.

6. Study of Children in US and CA • Use of a cochlear implant has had a dramatic impact on the linguistic competence of profoundly hearing-impaired children. • More than half of the children in this sample with average learning ability produced and understood English language at a level comparable with that of their hearing age mates. Such mature language outcomes were not typical of children with profound hearing loss who used hearing aids. • Use of a visual (i.e., sign) language system did not provide the linguistic advantage that had been anticipated. • Children educated without use of sign exhibited a significant advantage in their use of narratives, the breadth of their vocabulary, in their use of bound morphemes, in the length of their utterances and in the complexity of the syntax used in their spontaneous language. • An oral educational focus provided a significant advantage for both spoken and total language skills. Geers AE, Nicholas JG, Sedey AL. (2003).Language skills of children with early cochlear implantation. Ear Hear. 24(1 Suppl):46S-58S.

7. Canadian Study Children with cochlear implants have increased educational opportunities, with those children in mainstream and those who have moved toward mainstream demonstrating improved progress in speech perception ability. Daya H, Ashley A, Gysin C, Papsin BC. (2000). Changes in educational placement and speech perception ability after cochlear implantation in children. J Otolaryngol. 29(4):224-8.

8. British Study • Age at implantation and duration of deafness were found to be significant predictors of placement two years after implantation. • The duration of deafness of children in schools for the deaf or units was twice that of children in mainstream education. • Fifty-three per cent of children who were in pre-school at the time of implantation were in mainstream schools two years after implantation, whereas only 6% of those who were already in educational placements at the time of implantation were in mainstream education. • The results indicate that children who are given implants early, before an educational decision has been made, are more likely to go to mainstream schools than those given implants when already in an educational setting. Archbold S, Nikolopoulos TP, O'Donoghue GM, Lutman ME. (1998) Educational placement of deaf children following cochlear implantation. Br J Audiol. 32(5):295-300.

9. Cost Effective • Children with greater than 2 years of implant experience were mainstreamed at twice the rate or more of age-matched children with profound hearing loss who did not have implants. • Also placed less frequently in self-contained classrooms and used fewer hours of special education support. • A cost-benefit analysis based on conservative estimates of educational expenses from kindergarten to 12th grade shows a cost savings of cochlear implantation and appropriate auditory (re)habilitation that ranges from $30000 to $200000. • CI accompanied by aural (re)habilitation increases access to acoustic information of spoken language, leading to higher rates of mainstream placement in schools and lower dependence on special education support services. • The cost savings that results from a decrease in the use of support services indicates an educational cost benefit of cochlear implant (re)habilitation for many children. Francis HW, Koch ME, Wyatt JR, Niparko JK. (1999). Trends in educational placement and cost-benefit considerations in children with cochlear implants. Arch Otolaryngol Head Neck Surg. 1999 125(5):499-505.

10. Profound vs. Severe Loss & CI • Highly significant difference between the educational placement of implanted children and hearing-aided profoundly deaf children (p<0.00001) • No statistically significant difference between implanted children and hearing-aided severely deaf children. • Implanted profoundly deaf children who have received their implants before beginning school have the same profile of educational placement as aided severely deaf children rather than aided profoundly deaf children of the same age in the UK. • This is likely to have significant implications for the future management of profoundly deaf children and to influence future planning of educational support services. Archbold SM, Nikolopoulos TP, Lutman ME, O'Donoghue GM. (2002). The educational settings of profoundly deaf children with cochlear implants compared with age-matched peers with hearing aids: implications for management. Int J Audiol. 41(3):157-61.

11. CI by 6 Months • By the age of 2 years the subject implanted in infancy achieved scores on the GAEL-P which were nearly equivalent to those achieved at the age of 5 1/2 years by children implanted at later ages. • Age-equivalent scores on the Reynell Developmental Language Scales were achieved by the subject implanted in infancy and the ability to discriminate speech patterns was demonstrated using the Visual Habituation Procedure. • CONCLUSION: This report demonstrates enhanced language development in an infant who received a cochlear implant at the age of 6 months. Miyamoto RT, Houston DM, Kirk KI, Perdew AE, Svirsky MA. (2003). Language development in deaf infants following cochlear implantation. Acta Otolaryngol. 123(2):241-4.

12. Advocating Oral with CI? • Data from Clarion cochlear implant pediatric clinical trials were examined retrospectively to uncover trends in candidacy and postimplant benefit over time. • In particular, age at implantation, educational setting, and communication mode were examined with respect to speech perception performance after implantation. • The results showed: 1) age at implantation is decreasing, 2) children in oral education programs obtain more benefit from a cochlear implant than children in total communication programs, 3) children who undergo implantation before 2 years of age show greater benefit than children who undergo implantation between 2 and 3 years of age, 4) more younger children are using oral communication than older children, and 5) more children with good auditory skills before implantation and more residual hearing are undergoing implantation. Osberger MJ, Zimmerman-Phillips S, Koch DB. (2002). Cochlear implant candidacy and performance trends in children. Ann Otol Rhinol Laryngol Suppl. 189:62-5.

13. TC vs. Oral • Spoken word recognition improved at a faster rate in the oral children with early implantation. • Children who underwent implantation before 3 years of age had significantly faster rates of language development than did the children with later implantation. • The oral children demonstrated more rapid gains in communication abilities than did the children who used total communication. Kirk KI, Miyamoto RT, Lento CL, Ying E, O'Neill T, Fears B. (2002). Effects of age at implantation in young children. Ann Otol Rhinol Laryngol Suppl. 189:69-73.

14. Families of Children with CI • Children with hearing loss and their families who sought CIs are not significantly different from children with hearing impairments whose parents were not seeking a CI. • Results provided no support for the notion that children with hearing loss from families seeking a CIs for their child evidence more behavioral deviance than children with hearing impairments whose parents have not sought an implant. Knutson JF, Boyd RC, Goldman M, Sullivan PM. (1997). Psychological characteristics of child cochlear implant candidates and children with hearing impairments. Ear Hear. 18(5):355-63.

15. Sign vs. Oral & CI (Sweden) • The aim of the study was to explore patterns of communication between 22 children with cochlear implants (CI) and their parents, teachers and peers in natural interactions over a 2-year period. • The children, between 2 and 5 years old when implanted, had used the implant between 1 and 3.5 years at the end of the study. • Analyses of videorecorded interactions showed that meaningful oral communication was more easily obtained in the home setting than in the preschool setting. • Patterns of communication between parent-child, content and complexity of dialogues, quality of peer interactions, communicative styles of adults, and the use of sign language in communication turned out to be important factors when explaining the result of the CI on the individual child's development. • The children with the best oral skills were also good signers. Preisler G, Tvingstedt AL, Ahlstrom M. (2002). A psychosocial follow-up study of deaf preschool children using cochlear implants. Child Care Health Dev. 2002 Sep;28(5):403-18.

16. CI and Auditory Nerve Three pictures of auditory nerve tissue (from top to bottom), showing normal synapses in a hearing cat, long and flat synapses in a deaf cat, and like-normal synapses -- short and curved -- in a deaf cat treated with a cochlear implant. (Image courtesy of Johns Hopkins Medical Institutions) Research has clearly demonstrated the ability of cochlear implants in very young animals to forge normal nerve fibers that transmit sound and to restore hearing by reversing or preventing damage to the brain’s auditory system.

17. Teacher Role • The study examined factors associated with teachers' ratings of functional communication skills of students with cochlear implants. • Deaf students living in and around a metropolitan area were surveyed to locate 51 with cochlear implants. • Teachers rated each student's functional use of the implant, given three defined ratings. • Additional information regarding sex, communication option, placement, home language, rural or nonrural address, etiology, and presence or absence of an additional disability was gathered. • The data indicated that students with a known etiology and a rural address, and who used sign language at home or school, were less likely than others to use the implant as a primary channel for receptive communication. • The authors suggest that the teacher's role in implant use warrants more attention. Easterbrooks SR, Mordica JA. (2000) Teachers' ratings of functional communication in students with cochlear implants. Am Ann Deaf. 145(1):54-9.

18. Show Video(s) • 60 Minutes • Oral Deaf • Celia’s Story • Sound & Fury

19. NAD CI Statement • The NAD recognizes the rights of parents to make informed choices for their deaf and hard of hearing children, respects their choice to use cochlear implants and all other assistive devices, and strongly supports the development of the whole child and of language and literacy. Parents have the right to know about and understand the various options available, including all factors that might impact development.

20. NAD CI Statement • The NAD has always and continues to support and endorse innovative educational programming for deaf children, implanted or not. Such programming should actively support the auditory and speech skills of children in a dynamic and interactive visual environment that utilizes sign language and English.

21. Seven Steps to CI • Initial contact • Pre-CI counseling • Formal evaluation • Surgery • Fitting/mapping • Follow-up • AR

22. Roles • Audiologist • SLP • Educator of the Deaf • ENT

23. Child CI Candidate Criteria 12 – 24 months Pediatric Profound bilateral SNHL Limited benefit from appropriate binaural Has Lack of progress in auditory skill development High motivation and appropriate expectations from family • Severe to profound bilateral SNHL • MLNT – 30% or less in best aided condition (25 mo to 4 yrs 11 mo) • LNT – 30% or less in best aided condition (5 yrs to 17 yrs 11 mo) • Lack of progress in auditory skill development • No medical contraindications • High motivation and appropriate expectations (including children when appropriate age

24. Adult CI Candidate Criteria Pediatric Moderate to profound SNHL 50% or less – sentence recognition – in ear to be implanted 60% or less – sentence recognition – in opposite ear or binaurally Pre-linguistic or post-linguistic onset of moderate to profound SNHL No medical contraindications A desire to be a part of the hearing world

25. A cochlear implant is an assistive technology: • That bypasses the damaged part of the cochlea and sends electrical signals to the auditory nerve which relays these signals to the brain • Designed to provide sound detection to a full range of sounds to children with severe to profound hearing loss who obtain minimal benefit from hearing aids. • With unknown outcomes related to providing a child full access to spoken language for education and life success

26. Increasing numbers • Clerc Center- 1999-2000- no students with implants • Kendall,2002- 10 students (8 in candidacy process) • MSSD, 2002- 5 students (varying degrees of usage) • Nucleus Implants- worldwide • 1994-10,000 • 1999-26,000 • 2002-36,450 (Do not have statistics for Advanced Bionics and Med-El)

27. Why Are the Numbers Increasing • Early Identification • Improved technology • Changing candidacy requirements • Lower surgical risk • Changing attitudes

28. Implant Components • Three manufacturers of implants commonly used in the United States: • Cochlear Corporation (Nucleus) • Advanced Bionics- (Clarion) • Med-El • Surgically implanted components • Receiver coil • Electrode array • External components • Microphone • Speech Processor • Transmitter with a magnet

29. How does an implant work? • Sound picked up by microphone • Electrical pulses of sound signals sent to speech processor • Speech processor codes sound signals • Code is sent to transmitter • Transmitter sends coded sound across skin to internal receiver (via FM transmission) • Receiver converts code to electrical signals • Electrical signals sent to electrode array • Signals recognized as sound by the brain

30. LEVELS OF PERFORMANCE • Sound Awareness • Basic discrimination of sounds • Voice monitoring • Understanding environmental sounds • Understanding single words and/or phrases • Understands details in sentences • Understanding connected speech

31. Why are the outcomes different for each child? • Age at time of implant • Pre-implant duration of deafness • Etiology of hearing loss • Residual hearing prior to implant • Family support • Implant technology/channels • Consistency of usage • Appropriate programming of device • Additional special needs • Quality of educational and habilitative environment

32. Candidacy Requirements Who is a candidate: • Age- FDA says 18months, but doing as young as one year (some earlier) • Intact auditory nerve • Degree of loss- was profound, now increasingly more in severe range • Hearing aid trial was 3 months in many places, now not as strict in many Centers Issue of who "is not" a candidate Centers do not seem to be denying children access to this surgery, however many centers strongly suggest participation in a full mainstream environment in coordination with the surgery. Some implant centers may not consider "signing" students and families as candidates.

33. Process John's Hopkins Hospital- The Listening Center     * Initial consult * ABR   * Audiology evaluation (with and without hearing aids, may take many visits    * CT scan- looks at anatomy of cochlea     * Promontory test- looks at which ear stimulates best to an electrical signal       * ENT consult       * Rehab consult      * Outreach with educational programs * Surgery * Mapping * Habilitation/Rehabilitation

34. Deciding which ear to implant-some issues to consider • Anatomy (lack of calcification, is there an auditory nerve, malformed/no cochlea) • Perhaps one ear accepts electrical stimulation better than other • Leave ear with better hearing, implant worse ear, then if not successful can revert back to aiding that ear • Implant better ear (opposite argument)- It has already benefited from hearing aid, will more readily acclimate to implant • Facial nerve too close to cochlea-may pick other ear • If no difference may want it on right- as speech and hearing centers of brain on left • Want on right- later for later when driving. Can hear people in the car

35. Surgical Considerations After ·Usually up and around in 1-2 days ·May be some swelling externally ·Warned of some possible nausea from anesthesia ·  Wait 4-5 weeks for all swelling/healing to take place before activation General ·Usually outpatient, 1-2 hours ·Two parts of implant are inserted during surgery- electrode array in cochlea and the implant body placed in mastoid bone. Body holds a magnet that attaches to external components of the implant. During ·Shave area ·Mastoid bone uncovered (skin flap) ·Carve space for body of implant ·Drill hole to cochlea ·Small opening in cochlea to insert electrode array ·Incision closed

36. What about insurance • Cost of cochlear implant is $40,000-50,000 • Most private insurance companies are paying for surgery • Most of the time Medicaid pays 90-100% • May be problems with insurance related to child being too young based on FDA guidelines • Implant manufacturers have special departments to handle insurance related problems and secure payment. • Some insurance companies pay for post implant training/mapping • Only a few insurance companies pay for upgrades (ear level) ( may get coupon for upgrade from the manufacturer)

37. Mapping/Programming Issues • Each person has an individual program called a • Map based on determining threshold levels, and maximum comfort levels for each electrode. ·Determine how many electrodes can be stimulated comfortably ·Various strategies- one not best for all (SPEAK, CIS, ACE). Not one program suited for all people. ·Takes multiple sessions to program ·May initially be uncomfortable ·Neural response telemetry-Objective measure of response to electrode stimulation ·   Speech perception errors may be used to monitor the mapping (may be difficult due to impact of articulation errors, not good for young children)

38. Research issues • Change of research playing field now that candidacy requirements have changed • Much of the research focuses on speech perception and speech production • Age of implantation under 5 indicates advantage and ease in spoken language learning, yet still variability • There does not appear to be an age cut off when an implant does not appear useful in some way

39. Research issues • Oral children may have faster gains than TC kids, but what about ultimate outcomes? • CI increases speech and language regardless of the modality of language programming provided. (U. of Michigan, 2000) • Children with better communicative interaction skills at preverbal level were also most likely to have good speech perception and production skills three years after implantation. (Tait and Lutman, Robinson, 2000)

40. Parents’ Perceptions and Experiences: Gallaudet Research Institute findings (439 families) • 52% of families chose an implant to increase ease in development of spoken language • 43% of families perceived a significant increase in their child’s ability to understand words • 62% of families continued to use sign language support in the home although use of speech increased

41. The bottom line • Outcomes related to development of spoken language will be unique for each child • Educational programming must be designed to address the individual needs of students with implants

42. CI Guidelines In 2002, the FDA lowered the recommended age requirement to 12 months of age. While this is the FDA-recommended age, this age is not legally binding and some hospital centers in clinical trials are completing the procedure earlier based on expectations of improved outcomes for early implantation. In addition, specific circumstances may allow for earlier implantation. For example, if meningitis is the cause of hearing loss, it may be important for the child to be implanted as early as possible as this condition causes ossification (bone build up) in the cochlea, making it increasingly difficult to surgically insert the electrode array as time passes. Note: There may be questions related to insurance payment for the procedure if it is completed prior to 12 months of age.

43. CI Guidelines • The FDA states that a child should have a bilateral (both ears) profound sensorineural hearing loss; however, increasing numbers of children with hearing loss in the severe range are being considered for cochlear implants.

44. CI Guidelines • Negligible functional benefit (limited open-set speech recognition) from appropriate amplification is often mentioned as a criterion. • When such measures cannot be obtained on young children, hospital centers make individual decisions regarding whether or not a child would be able to do well on such tests given documented hearing levels and traditional hearing aids. • There are varied implant center requirements regarding the use of traditional hearing aids prior to implantation. • Some centers waive an extended hearing aid trial requirement in the interest of time when it is clear that the child would perform better with a cochlear implant.

45. CI Guidelines • A child who is failing to progress in speech, language, and listening development with traditional hearing aids based on parent report and educational information may be considered as a candidate. • Family willingness to follow recommendations; enroll in speech, language, and listening therapy; and return for follow-up appointments is a factor in candidacy. • Having no medical contraindications to electrode insertion or receiver placement is a factor in candidacy. • Educational and home environments that are supportive of cochlear implants are factors in candidacy.

46. Not CI Candidates • a child that does not have the eighth nerve (auditory nerve) which carries sound from the cochlea to the brain as determined by a CAT scan (x-ray) and/or Magnetic Resonance Imaging (MRI) during the candidacy process. • a child who has significant residual hearing levels and receives good benefit from traditional hearing aid devices.

47. Other Factors – Non-candidacy • Some centers may not implant children with severe emotional, behavioral, or cognitive delays when it is perceived that these characteristics may prevent participation in the educational/training programs necessary to actualize benefit from the cochlear implant. • Some children obtain substantial access to sound from the technology of state-of-the-art digital hearing aids or other hearing devices. Without surgical intervention, these devices may be an equally effective choice for some children.

48. CI Student Placement Options • a neighborhood or private school with no additional supports • inclusion in a neighborhood or private school with supports integrated within the school (itinerant teachers, resource teachers, speech and language specialists, etc.) • a self-contained classroom for children with hearing loss using: • an oral approach, • cued speech, • total communication, or • American Sign Language, • a day school for deaf children that uses: • an oral only approach, • total communication, or • a bilingual approach (American Sign Language and English).

49. Communication Mode • Manual • ASL • Bilingual/Bicultural • Manually Coded English • Simultaneous Communication • Total • Multisensory • Aural/Oral • Multisensory • Unisensory • Acoupedic • Auditory/verbal

50. Sign Language & CI • "Continued use of a total communication approach might be the most effective means for facilitating language growth in a child with a cochlear implant. • Nonetheless, it is essential that the child be exposed to an enriched auditory environment for as many hours a day as possible. • There is a great need for a strong commitment to maximize the auditory component with a TC approach. • In addition, it might be necessary for the school staff to adjust their expectations and teaching priorities, especially if manual communication is the focus of the child's educational placement." • McKinley, A., & Warren, S. (2000). The effectiveness of cochlear implants for children with prelingual deafness. Journal of Early Intervention, 23.