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Not a “sound” decision: Is cochlear implantation always the best choice?
International Journal of Pediatric Otorhinolaryngology 74 (2010) 1144–1148
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International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Not a ‘‘sound’’ decision: Is cochlear implantation always the best choice? Lynne C. Graham O’Brien a, Margaret Kenna a,b, Marilyn Neault a,b, Terrell A. Clark a,c, Betsy Kammerer a,c, Jennifer Johnston a, Erik Waldman a, Sarah Pierce Thomas a, Peter Forbes d, Greg R. Licameli a,b,* a
Department of Otolaryngology and Communication Enhancement, Children’s Hospital Boston, United States Dept. of Otology and Laryngology, Harvard Medical School, United States c Dept. of Psychiatry, Harvard Medical School, United States d Clinical Research Program, Children’s Hospital Boston, United States b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 1 November 2009 Received in revised form 25 June 2010 Accepted 3 July 2010 Available online 7 August 2010
Objective: To review the candidacy criteria used to counsel parents of profoundly deaf children, to determine if these criteria have changed over time, and to evaluate eventual communication outcomes for these patients. Design: Retrospective review of 483 pediatric cochlear implant candidates from September 1995 to December 2006 seen at a tertiary care pediatric hospital. Results: Out of 483 implant candidates, 191 patients were initially felt not to be favorable candidates based on CI team evaluation. Of this group, 3 had insufficient records to review and were excluded. The remaining 188 patients underwent a detailed analysis of specific possible contraindications to implantation. This included audiologic, medical and psychosocial parameters. The data was divided into two time periods: Group 1 included 44 patients from 1995 to 2000, and Group 2 included 144 patients from 2001 to 2006. In Group 1, there was a higher percentage of children with language deprivation and developmental concerns and patients not ready, compared to Group 2 which had a higher percentage of families not ready and inadequate support systems. Group 1 had a higher percentage of patients who ultimately underwent cochlear implant, but otherwise the two groups were largely similar. Conclusion: Analysis of our data showed that the degree of concern that the cochlear implant team has in relationship to specific candidacy criteria has changed over time. Recommendations against a cochlear implant were often revisited after initial concerns were addressed. The use of a team approach, in conjunction with a validation tool, is important for establishing criteria for successful cochlear implantation in children to support appropriate counseling of patients and families and to plan postimplant management. ß 2010 Published by Elsevier Ireland Ltd.
Keywords: Pediatric cochlear implantation Cochlear implant outcomes
1. Introduction The approval of cochlear implantation (CI) for children by the Food and Drug Administration (FDA) in June of 1990 greatly expanded the treatment options for children with bilateral severeto-profound sensorineural hearing loss. Cochlear implants, along with appropriate aural (re)habilitation, have proven to be highly effective in terms of speech and language acquisition as well as a significant cost–benefit to society [1,5,13,26,31]. More recently, the potential outcomes for CI have expanded to include, in some patients with complex medical and neurological issues, improved auditory and environmental awareness without necessarily the expectation for spoken language development.
* Corresponding author at: Dept. of Otolaryngology and Communication Enhancement, Children’s Hospital Boston, 300 Longwood Ave. LO-367, Boston, MA 02115, United States. Tel.: +1 617 355 8852; fax: +1 617 730 0726. E-mail address: [email protected] (G.R. Licameli). 0165-5876/$ – see front matter ß 2010 Published by Elsevier Ireland Ltd. doi:10.1016/j.ijporl.2010.07.002
Criteria used to determine if patients will obtain sufficient benefit from a CI to proceed with implantation are constantly evolving as experience, outcome data, and technology improve. Initial FDA regulations in 1990 approved cochlear implant surgery for children between 2 and 18 years with bilateral profound sensorineural hearing loss who were not benefiting from appropriately fitted hearing aids. In 2000, the FDA modified the regulations with approval to implant children as young as 12 months of age [22]. This change was a direct result of data showing that earlier implantation correlated, in general, with higher levels of performance [25,30]. Additional changes in FDA criteria, which are specific to each approved device, have included the candidacy of individuals with severe-to-profound hearing loss in addition to those with profound hearing loss [23]. Some examples of technological advances affecting outcome include the development of the modiolar-hugging electrode array [4,8,9] and creation of improved speech processing strategies [3,6,15,16,29]. Many factors other than age and degree of hearing loss clearly correlate with the potential amount of benefit a child will obtain
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from a CI. Papsin and Gordon summarized that ‘‘duration of deafness, age at receipt of cochlear implantation, educational setting, form of communication, cognitive, motor, and social development, speech-language development, access to and participation in therapy and education providing support for oral speech-language development, family structure and support, IQ, and socioeconomic status, all play a critical role in the outcome for each child’’ [27]. Residual hearing, auditory neuropathy spectrum disorder, anatomical inner ear abnormalities, and other specific medical and developmental issues further affect CI candidacy due to their impact on outcome. As candidacy criteria for implantation have expanded, some potential candidates for CI present with increasingly complex medical or social profiles. Some studies have noted that up to 40% of children with severe-to-profound hearing loss have multiple medical issues, complicating the decision-making process regarding a patient’s potential benefit from CI [10–12,18,28]. Additionally, although CI has become a standard treatment option for children who are deaf, it will never be the only option; there will always be individuals and families for whom cultural factors, comorbid conditions and/or psychosocial issues will be absolute or relative contraindications to CI. Children’s Hospital Boston (CHB) is a tertiary care pediatric medical facility. Before 1995, patients felt to be reasonable CI candidates were evaluated at CHB and then referred to other implant centers for surgery. In 1995, when CI surgery became available at CHB, candidates were evaluated by a 5-member team; by 2002, the team had grown to 16 individuals representing five disciplines (audiology, psychology, speech-language pathology, otolaryngology, and deaf education), and also included a program coordinator and an educational outreach coordinator, who is also a teacher of the deaf. Most CI centers form multidisciplinary teams to evaluate potential candidates. In addition, a standardized pre-operative instrument or tool would be useful, as it could: (1) allow comparison of outcomes between centers and populations; (2) reduce confusion for patients and their families when choosing a center; (3) potentially remove some of the subjectivity from the evaluation; (4) provide more accurate prognostic information to patients; and (5) help in the post-CI planning of service provision. However, no such single pre-operative tool or instrument exists, despite numerous efforts to devise one [14,24]. Recently, our team modified a previously existing pre-operative screening instrument, the Children’s Implant Profile (ChIP), and incorporated the four outcome prediction levels (A–D) of another pre-implant candidacy tool, the AuSpLan, to help provide more objective and accurate information about potential post-CI outcome to the CI team and the CI candidates and their families [14,20]. The development of the Children’s Hospital Boston modified ChIP (mChIP-CHB) raised questions about patients evaluated in the past by the CI team and who were initially not felt to be favorable candidates. Specifically, we wanted to address reasons for the initial recommendation against CI, whether these reasons have changed over the time of existence of the CI team, whether these patients eventually did or did not receive a CI, and what their communication outcomes were. 2. Materials and methods Institutional Review Board (IRB): The Children’s Hospital Boston IRB approved this project. A retrospective record review was undertaken of 483 children who underwent an evaluation for CI at CHB between September 1995 and December 2006. We focused on children for whom the initial recommendation was against a CI. Contraindications were categorized according to common themes, including not meeting audiologic criteria or being too old for a pediatric facility (‘‘adult’’).
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Others included: children who were past the critical age of language learning and whose primary mode of communication was manual (‘‘prolonged period without auditory access’’); children who were younger than the FDA-approved guideline age of 24 months (before November 2000) or 12 months (after November 2000) (‘‘below FDA age guidelines’’); children who had too much hearing and performed well with hearing aids and therefore did not meet FDA criteria (‘‘audiological contraindication’’); children who went too long without any form of communication and for whom signed language was felt to be the most appropriate option (‘‘severe language deprivation’’); children who had significant developmental delay, were not capable of acquiring symbolic language and/or had a significant psychiatric disorder or cognitive limitations (‘‘developmental contraindication’’); and children who had medical contraindications that were not developmental (e.g. ventilator dependence, severe cardiac disease) in nature. Psychosocial factors included: families who did not followthrough with multiple required appointments (‘‘inconsistent with appointments’’); families who investigated CI as an option but then decided to continue with existing hearing aid usage and/or visual communication, such as American Sign Language (‘‘family choice’’); families who had no insurance or payment options (‘‘insurance barriers’’); families who initiated the candidacy process but then discontinued contact with the team for a prolonged period, which lasted for periods up to nearly 4 years (‘‘initial consult only’’); children who were not psychologically ready for and/or resistant to cochlear implantation (‘‘child not ready’’); families who were still interested but not ready at the present time (‘‘guardians not ready’’); a combination of a child and family not being ready (‘‘family not ready’’); when a family’s residence elsewhere (out of state or out of country) resulted in the inability of the family to commit to maintaining their care at CHB for the required 1 year post-operative care (‘‘geographical contraindication’’); and when the family and/or the educational placement would be unable to support the CI commitment (‘‘inadequate support system’’) (Table 1). Primary reasons were then tabulated. In order to compare results longitudinally, the groups were subdivided based upon date of initiation of candidacy process, with the dividing factor between the two groups being the change in FDA regulations in November 2000 to allow implantation at the age of 12 months rather than the previous 24 months. Group 1 consisted of those children evaluated between September 1, 1995 and December 31, 2000, and Group 2 consisted of those evaluated between January 1, 2001 and December 31, 2006 (Table 1). Candidates for bilateral CI and those requiring re-implantation are not included in this report. 3. Results Between September 1995 and December 2006, 483 children underwent an evaluation for CI at CHB. Of these 483, CI was not initially recommended for 191. From this group of 191 patients, 3 were excluded due to insufficient data. The remaining 189 patients were divided into two time periods: Group 1 (1995–2000, n = 44) and Group 2 (2001–2006, n = 144). For Group 1, the ages ranged from 0.2 to 15.5 years, with a mean age of 5.32 years, median of 3.8 years and a standard deviation of 3.8 years. For Group 2, the ages ranged from 0.1 to 21.0 years, with a mean age of 5.7 years, median of 3.8 years and a standard deviation of 5.0 years. Gender was evenly split for each group (Group 1: 49% male 51% female, Group 2: 52% male, 48% female), with race and socioeconomic status equally represented in both groups. The rate of initial recommendation against cochlear implantation was 30% in Group 1 and 40% in Group 2. Primary reasons for not recommending a CI are listed in
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Table 1 Primary contraindicator to cochlear implantation by group (n = 189). Description
Primary reason Adult Audiologic contraindication Below FDA age guidelines Child not ready Developmental contraindication Family choice Family not ready Geographical contraindication Guardians not ready Inadequate support system Inconsistent with appointments Initial consult only Insurance barriers Medical contraindicationa Prolonged period without auditory access Severe language deprivation Unknown Total a
Over 18 years old; referred to an adult implant center Too much hearing; does not meet FDA guidelines Under age 12 months Not psychologically ready and/or resistant Significant developmental delay, not capable of symbolic language, psychiatric/cognitive issues Investigated then decided against CI, continued with hearing aids and/or the Deaf Culture [American Sign Language] Combination of child and guardians not ready Residency elsewhere, could not stay in Boston for required 1 year post-op care Still interested, but not ‘‘ready’’ right now Family and/or educational placement a significant concern No follow-through on multiple appointments Initiated process, but no further contact from family No insurance or payment options Medical contraindication, other than developmental Older child (over age 5 years) whose primary mode of communication is manual Too long without spoken language
Group 1 (1995–2000)
Group 2 (2001–2006)
n (%)
n (%)
0 6 1 5
2 22 1 5
(0) (13.25) (2.25) (11.0)
(1.25) (15.25) (0.75) (3.5)
8 (18.0)
6 (4.25)
1 (2.25) 0 (0)
4 (2.75) 1 (0.75)
0 1 0 4 1 1 6
(0) (2.25) (0) (9.0) (2.25) (2.25) (13.25)
2 11 9 14 21 3 23
(1.25) (7.75) (6.25) (9.75) (14.5) (2.0) (16.0)
5 (11.0)
11 (7.75)
5 (11.0) 1 (2.25) 45 (100%)
6 (4.25) 3 (2.0) 144 (100%)
Examples of medical exclusions include: no auditory nerve, severe cochlear ossification, recurrence of extracochlear malignancy, significant cardiac disease, etc.
Table 1. Audiologically, many patients had hearing that was ‘‘too good’’ by FDA guidelines at the time of their evaluation; in others, medical conditions existed which were thought to preclude the development of useful spoken language or at least the ability to use the device to derive significant environmental and audiologic information. Thirty-four children had more than one contraindication. Subgroup analysis of the 188 patients was performed (Tables 1–4). The reasons for not recommending CI varied
Table 2 Outcomes post-CI candidacy evaluation.
Cochlear implant Cued speech Hearing aid(s) Sign language Spoken language without CI Unknown Total
Group 1 (1995–2000) n (%)
Group 1 (2001–2006) n (%)
12 1 7 10 0 15 45
28 0 29 29 1 57 129
(26.75) (2.25) (15.5) (22.25) (0) (33.25) (100%)
(19.5) (0) (20) (20.25) (0.75) (39.5) (100%)
Table 3 Primary contraindicator to cochlear implantation for patients subsequently implanted at another facility. Reason
n (%)
Adult Audiologic contraindication Below FDA age guidelines Developmental contraindication Inadequate support system Inconsistent with appointments Initial consult only Medical contraindication Severe language deprivation Total
1 3 2 4 1 2 1 3 2 19
(5.25) (15.75) (10.5) (21.25) (5.25) (10.5) (5.25) (15.75) (10.5) (100)
significantly between the two groups (Table 1). In Group 1 there was a greater percentage of children who were ‘‘not ready’’ for implantation as compared to Group 2 (11.25% versus 3.5%), a greater percentage of children with severe language deprivation (11.25% versus 4.0%) and a greater percentage of children with developmental contraindications (18.25% versus 4.25%). By contrast, there were a greater percentage of children in Group 2 who had only an initial consult (2.25% versus 14.5%), whose guardians were not ready (2.25% versus 8.25%), or who had inadequate support systems (0% versus 6.25%) (Table 1). Of the 117/188 (62%) patients whose long-term outcome was known, 40 (27.75%) were subsequently implanted at a later date. Rate of later implantation did not vary by group (12/44 [27%] from Group 1 and 28/144 [19%] from Group 2 (Table 2)). Twenty-one were implanted at CHB and 19 at other institutions. For the 19 who were implanted elsewhere, 10 were determined not to be CI candidates at CHB due to either developmental (n = 4; 21%), audiologic (n = 3; 16%) or medical (n = 3; 16%) reasons (Table 3). For the 21 subsequently implanted at CHB, nine (43%) were initially either ‘‘guardians not ready’’ or ‘‘initial consult only’’ (Table 4). In reviewing the data, these two categories might be collapsed into one, as most of the time the families did not follow up because they were not yet ready. Table 4 Primary contraindicator to cochlear implantation for patients subsequently implanted at CHB. Reason
n (%)
Audiologic contraindication Child not ready Family choice Family not ready Guardians not ready Inadequate support system Inconsistent with appointments Initial consult only Medical contraindication Severe language deprivation Total
2 2 1 1 5 2 2 4 1 1 21
(9.5) (9.5) (4.75) (4.75) (24) (9.5) (9.5) (19) (4.75) (4.75) (100)
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The primary communication modality post-candidacy evaluation was examined when the data were available. The communication outcomes between Group 1 and Group 2 were remarkably similar and nearly equally distributed among three outcomes (CI, ASL and hearing aids) (Table 2). 4. Discussion Pediatric CI candidacy criteria are in evolution. For many patients, initial recommendations from our team against a CI were later revisited and CI subsequently recommended. These changes in the recommendations with regard to CI were often due to improved medical stability, improved educational/rehabilitative programming options or development of more realistic expectations on the part of the child and/or the family (the latter often achieved by intense education and counseling by the CI team). A recommendation to wait must always be balanced against the possibility of a poorer outcome if the implant is performed after the early period of optimal brain plasticity related to language acquisition has passed. Additionally, outcome expectations by the parents or the implant team may change over time, with fluency in spoken communication no longer anticipated for some. Depending on a patient’s medical and communication history, the goal of a CI now may be primarily to provide improved environmental awareness and to give a greater sense of ‘‘connection’’ to the world, rather than an expectation of development of normal, or even any, spoken language. A related study by our Team [7] reviewed 40 medically complex pediatric patients, ages 4–14 years, with cochlear implants who had co-morbid conditions such as microcephaly, extreme prematurity with motor impairment, encephalopathy, seizure disorder, autism spectrum disorder, neuromuscular disorder, and genetic syndromes with global developmental disorder, who were at minimum 2 years post-activation of the cochlear implant speech processor. It was noted that 25 of the 40 patients consistently wore the speech processor, with 48% of the total achieving ‘‘minimal or no benefit from access to auditory stimuli to support language development or communication’’ from the CI. Of the remaining 15, 8 used the speech processor inconsistently and 7 were no longer users of the CI. For these 7 non-users, reasons discussed in the Team presentation included, aversive reactions to auditory stimuli, generalized system dysregulation, and equipment struggles in conjunction with neuromuscular disorders and decreased head control. 29/40 demonstrated benefit from CI use, such as ‘‘steady gains in speech production’’ with spoken communication as the primary communication modality (11/40), limited spoken English skills (8/40) and obvious auditory benefit from CI use despite minimal or no gains in speech production 10/40. For these medically complex children, it was determined that counseling to address the full range of outcomes is necessary to ensure appropriate post-implant expectations as well as plan for appropriate services. In addition, additional consultations were often indicated both pre- and post-operatively, (e.g. neurological, physical and adaptive therapy), and surgical planning was often more complicated due to the child’s complex and/or multiple medical needs. The conclusion, however, was that complex medical history and/or neurological disorders definitely did not automatically ‘‘rule out’’ a deaf child from consideration for cochlear implantation. While auditory progress may be slower, spoken language as a primary mode of communication may be attainable in some patients, and even if spoken language does not develop, speech production is not the only indication of benefit from cochlear implantation. Positive changes in general responsiveness, receptive language, and social engagement are often seen when access to sound is provided by CI. Similar results were found by Meinzen-Derr et al. in their pediatric CI population at Cincinnati Children’s Hospital [21].
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For our CI Team, the mandatory element for a favorable recommendation for cochlear implant is appropriate expectations regarding outcome. The family must demonstrate a true ability to understand the limitations of CI and the range of potential outcomes. Informed consent is paramount for any optional surgical intervention. If it is believed that a family does not have appropriate expectations they are guided to improve understanding of their child’s communication and the limitations of CI technology until they are able to demonstrate an appropriate understanding of the information and provide truly informed consent. It is critical to ensure that there are feasible alternatives for those patients for whom CI is ultimately contraindicated, and that supports are in place to ensure communicative success for all the children who initially present for CI candidacy consideration. Otherwise, if the family is unclear about available options and how to best support their child’s communicative needs, there is the risk that a family will continue to ‘‘shop’’ for an implant center to provide them with a surgery and not necessarily focus on other communicative options or strategies. A recent study by Children’s Hospital of Philadelphia (CHOP) noted that their modification of the ChIP ‘‘fails to capture the complexity of the decision-making process’’ [17]. In order to assist pediatric CI teams in objectifying their recommendations about CI candidacy, the Children’s Hospital Boston modified ChIP or mChIPCHB is undergoing validation at our center. (Addendum) This tool has already been found to assist in counseling families at CHB for whom the CI team has concerns and/or the evaluation process is complicated. It demonstrates to the families and caregivers the critical factors necessary for a successful outcome following implantation, and often helps in the planning process for post-CI services. Meanwhile, the data collection is helping the CI team to better track those risk factors that are relative contraindications to CI versus those that have less impact on the ultimate benefit a patient and their family receives from the CI. Analysis of our data showed that patients presented with different concerns for the CI implant team over time. Many factors, which often meant an initial recommendation against CI in the past, are not weighed as heavily now, such as significant developmental delays (e.g. cerebral palsy). Other factors are still concerning and predictive of limited benefit, such as children who have not yet demonstrated the potential for acquiring symbolic language in any modality (ASL, cued speech, spoken language or total communication). With the changes in FDA criteria and the success of implantation in children worldwide, more families are considering CI as a potential option for their children, including those with medically complex backgrounds. In addition, a recent study by Bradham and Jones [2] of potential CI candidates in the US demonstrates that the population who could benefit from CI technology is being underserved and underrecognized. Many reasons for this include continued shortage of qualified personnel and programs to evaluate and care for these children, reimbursement barriers, and disparities in implantation rates based on socioeconomic and ethnicity factors. Another study, by Mathews et al. [19] showed that pediatricians demonstrated deficits in current and accurate knowledge about CIs and Early Hearing Detection and Intervention (EHDI) programs. Therefore, CI programs should be on the forefront of candidacy information and implementation, and may well need to take an educational role with regard to primary care and other providers. 5. Conclusions Careful consideration of the medical, audiologic and psychosocial issues for each patient by a multidisciplinary CI team is crucial in ensuring that all relevant factors are taken into account when counseling parents and patients about CI candidacy. Overall, there
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is an increase in the number of children presenting for potential CI candidacy. Reasons for this include increased patient and family access to CI teams and centers, CI becoming a standard of care in the management of children with severe-to-profound hearing loss, easing of FDA guidelines for pediatric CI, widening of outcome expectations and anticipated benefit, and the early identification of infants with severe-to-profound hearing loss through EHDI programs. The use of a team approach, in conjunction with a validation tool, is important for establishing criteria for successful cochlear implantation counseling as well as planning for management after CI. References [1] E.A. Beadle, D.J. McKinley, T.P. Nikolopoulos, J. Brough, G.M. O’Donoghue, S.M. Archbold, Long-term functional outcomes and academic-occupational status in implanted children after 10 to 14 years of cochlear implant use, Otol. Neurotol. 26 (2005) 1152–1160. [2] T. Bradham, J. Jones, Cochlear implant candidacy in the United States: prevalance in children 12 months to 6 years of age, Int. J. Pediatr. Otorhinolaryngol. 72 (2008) 1023–1028. [3] M. Brendel, A. Buechner, B. Krueger, C. Frohne-Buechner, T. Lenarz, Evaluation of the Harmony sound processor in combination with the speech coding strategy HiRes 120, Otol. Neurotol. 29 (2) (2008) 199–202. [4] R.J. Briggs, M. Tykocinski, E. Saunders, W. Hellier, M. Dahm, B. Pyman, et al., Surgical implication fo perimodiolar cochlear implant electrode design: avoiding intracochlear damage and scala vestibule insertion, Cochlear Implants Int. 2 (2) (2001) 135–149. [5] A.K. Cheng, H.R. Rubin, N.R. Powe, N.K. Mellon, H.W. Francis, J.K. Niparko, Costutility analysis of the cochlear implant in children, JAMA 284 (2000) 850–856. [6] G. Clark, The multi-channel cochlear implant: past, present and future perspectives, Cochlear Implants Int. 10 (Suppl. 1) (2009) 2–13. [7] T.C. Clark, J. Johnston, A. Nussbaum, L. O’Brien, Kammerer, Functional outcomes in medically complex children who receive cochlear implants, Paper presented at CI2007, April 2007. [8] L.T. Cohen, E. Saunders, G.M. Clark, Psychophysics of a prototype peri-modiolar cochlear implant electrode array, Hear. Res. 155 (1–2) (2001) 63–81. [9] L.T. Cohen, E. Saunders, M.R. Knight, R.S. Cowan, Psychophysical measures in patients fitted with contour and straight nucleus electrode arrays, Hear. Res. 212 (1–2) (2006) 160–175. [10] N.L. Cohen, Cochlear implant candidacy and surgical considerations, Audiol. Neurootol. 9 (2004) 197–202. [11] L.C. Edwards, R. Frost, F. Witham, Developmental delay and outcomes in paediatric cochlear implantation: implications for candidacy, Int. J. Pediatr. Otorhinolaryngol. 70 (2006) 1593–1600. [12] R. Filipo, E. Bosco, P. Mancini, D. Ballantyne, Cochlear implants in special cases: deafness in the presence of disabilities and/or associated problems, Acta Otolaryngol. Suppl. 552 (2004) 74–80.
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Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Not a ‘‘sound’’ decision: Is cochlear implantation always the best choice? Lynne C. Graham O’Brien a, Margaret Kenna a,b, Marilyn Neault a,b, Terrell A. Clark a,c, Betsy Kammerer a,c, Jennifer Johnston a, Erik Waldman a, Sarah Pierce Thomas a, Peter Forbes d, Greg R. Licameli a,b,* a
Department of Otolaryngology and Communication Enhancement, Children’s Hospital Boston, United States Dept. of Otology and Laryngology, Harvard Medical School, United States c Dept. of Psychiatry, Harvard Medical School, United States d Clinical Research Program, Children’s Hospital Boston, United States b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 1 November 2009 Received in revised form 25 June 2010 Accepted 3 July 2010 Available online 7 August 2010
Objective: To review the candidacy criteria used to counsel parents of profoundly deaf children, to determine if these criteria have changed over time, and to evaluate eventual communication outcomes for these patients. Design: Retrospective review of 483 pediatric cochlear implant candidates from September 1995 to December 2006 seen at a tertiary care pediatric hospital. Results: Out of 483 implant candidates, 191 patients were initially felt not to be favorable candidates based on CI team evaluation. Of this group, 3 had insufficient records to review and were excluded. The remaining 188 patients underwent a detailed analysis of specific possible contraindications to implantation. This included audiologic, medical and psychosocial parameters. The data was divided into two time periods: Group 1 included 44 patients from 1995 to 2000, and Group 2 included 144 patients from 2001 to 2006. In Group 1, there was a higher percentage of children with language deprivation and developmental concerns and patients not ready, compared to Group 2 which had a higher percentage of families not ready and inadequate support systems. Group 1 had a higher percentage of patients who ultimately underwent cochlear implant, but otherwise the two groups were largely similar. Conclusion: Analysis of our data showed that the degree of concern that the cochlear implant team has in relationship to specific candidacy criteria has changed over time. Recommendations against a cochlear implant were often revisited after initial concerns were addressed. The use of a team approach, in conjunction with a validation tool, is important for establishing criteria for successful cochlear implantation in children to support appropriate counseling of patients and families and to plan postimplant management. ß 2010 Published by Elsevier Ireland Ltd.
Keywords: Pediatric cochlear implantation Cochlear implant outcomes
1. Introduction The approval of cochlear implantation (CI) for children by the Food and Drug Administration (FDA) in June of 1990 greatly expanded the treatment options for children with bilateral severeto-profound sensorineural hearing loss. Cochlear implants, along with appropriate aural (re)habilitation, have proven to be highly effective in terms of speech and language acquisition as well as a significant cost–benefit to society [1,5,13,26,31]. More recently, the potential outcomes for CI have expanded to include, in some patients with complex medical and neurological issues, improved auditory and environmental awareness without necessarily the expectation for spoken language development.
* Corresponding author at: Dept. of Otolaryngology and Communication Enhancement, Children’s Hospital Boston, 300 Longwood Ave. LO-367, Boston, MA 02115, United States. Tel.: +1 617 355 8852; fax: +1 617 730 0726. E-mail address: [email protected] (G.R. Licameli). 0165-5876/$ – see front matter ß 2010 Published by Elsevier Ireland Ltd. doi:10.1016/j.ijporl.2010.07.002
Criteria used to determine if patients will obtain sufficient benefit from a CI to proceed with implantation are constantly evolving as experience, outcome data, and technology improve. Initial FDA regulations in 1990 approved cochlear implant surgery for children between 2 and 18 years with bilateral profound sensorineural hearing loss who were not benefiting from appropriately fitted hearing aids. In 2000, the FDA modified the regulations with approval to implant children as young as 12 months of age [22]. This change was a direct result of data showing that earlier implantation correlated, in general, with higher levels of performance [25,30]. Additional changes in FDA criteria, which are specific to each approved device, have included the candidacy of individuals with severe-to-profound hearing loss in addition to those with profound hearing loss [23]. Some examples of technological advances affecting outcome include the development of the modiolar-hugging electrode array [4,8,9] and creation of improved speech processing strategies [3,6,15,16,29]. Many factors other than age and degree of hearing loss clearly correlate with the potential amount of benefit a child will obtain
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from a CI. Papsin and Gordon summarized that ‘‘duration of deafness, age at receipt of cochlear implantation, educational setting, form of communication, cognitive, motor, and social development, speech-language development, access to and participation in therapy and education providing support for oral speech-language development, family structure and support, IQ, and socioeconomic status, all play a critical role in the outcome for each child’’ [27]. Residual hearing, auditory neuropathy spectrum disorder, anatomical inner ear abnormalities, and other specific medical and developmental issues further affect CI candidacy due to their impact on outcome. As candidacy criteria for implantation have expanded, some potential candidates for CI present with increasingly complex medical or social profiles. Some studies have noted that up to 40% of children with severe-to-profound hearing loss have multiple medical issues, complicating the decision-making process regarding a patient’s potential benefit from CI [10–12,18,28]. Additionally, although CI has become a standard treatment option for children who are deaf, it will never be the only option; there will always be individuals and families for whom cultural factors, comorbid conditions and/or psychosocial issues will be absolute or relative contraindications to CI. Children’s Hospital Boston (CHB) is a tertiary care pediatric medical facility. Before 1995, patients felt to be reasonable CI candidates were evaluated at CHB and then referred to other implant centers for surgery. In 1995, when CI surgery became available at CHB, candidates were evaluated by a 5-member team; by 2002, the team had grown to 16 individuals representing five disciplines (audiology, psychology, speech-language pathology, otolaryngology, and deaf education), and also included a program coordinator and an educational outreach coordinator, who is also a teacher of the deaf. Most CI centers form multidisciplinary teams to evaluate potential candidates. In addition, a standardized pre-operative instrument or tool would be useful, as it could: (1) allow comparison of outcomes between centers and populations; (2) reduce confusion for patients and their families when choosing a center; (3) potentially remove some of the subjectivity from the evaluation; (4) provide more accurate prognostic information to patients; and (5) help in the post-CI planning of service provision. However, no such single pre-operative tool or instrument exists, despite numerous efforts to devise one [14,24]. Recently, our team modified a previously existing pre-operative screening instrument, the Children’s Implant Profile (ChIP), and incorporated the four outcome prediction levels (A–D) of another pre-implant candidacy tool, the AuSpLan, to help provide more objective and accurate information about potential post-CI outcome to the CI team and the CI candidates and their families [14,20]. The development of the Children’s Hospital Boston modified ChIP (mChIP-CHB) raised questions about patients evaluated in the past by the CI team and who were initially not felt to be favorable candidates. Specifically, we wanted to address reasons for the initial recommendation against CI, whether these reasons have changed over the time of existence of the CI team, whether these patients eventually did or did not receive a CI, and what their communication outcomes were. 2. Materials and methods Institutional Review Board (IRB): The Children’s Hospital Boston IRB approved this project. A retrospective record review was undertaken of 483 children who underwent an evaluation for CI at CHB between September 1995 and December 2006. We focused on children for whom the initial recommendation was against a CI. Contraindications were categorized according to common themes, including not meeting audiologic criteria or being too old for a pediatric facility (‘‘adult’’).
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Others included: children who were past the critical age of language learning and whose primary mode of communication was manual (‘‘prolonged period without auditory access’’); children who were younger than the FDA-approved guideline age of 24 months (before November 2000) or 12 months (after November 2000) (‘‘below FDA age guidelines’’); children who had too much hearing and performed well with hearing aids and therefore did not meet FDA criteria (‘‘audiological contraindication’’); children who went too long without any form of communication and for whom signed language was felt to be the most appropriate option (‘‘severe language deprivation’’); children who had significant developmental delay, were not capable of acquiring symbolic language and/or had a significant psychiatric disorder or cognitive limitations (‘‘developmental contraindication’’); and children who had medical contraindications that were not developmental (e.g. ventilator dependence, severe cardiac disease) in nature. Psychosocial factors included: families who did not followthrough with multiple required appointments (‘‘inconsistent with appointments’’); families who investigated CI as an option but then decided to continue with existing hearing aid usage and/or visual communication, such as American Sign Language (‘‘family choice’’); families who had no insurance or payment options (‘‘insurance barriers’’); families who initiated the candidacy process but then discontinued contact with the team for a prolonged period, which lasted for periods up to nearly 4 years (‘‘initial consult only’’); children who were not psychologically ready for and/or resistant to cochlear implantation (‘‘child not ready’’); families who were still interested but not ready at the present time (‘‘guardians not ready’’); a combination of a child and family not being ready (‘‘family not ready’’); when a family’s residence elsewhere (out of state or out of country) resulted in the inability of the family to commit to maintaining their care at CHB for the required 1 year post-operative care (‘‘geographical contraindication’’); and when the family and/or the educational placement would be unable to support the CI commitment (‘‘inadequate support system’’) (Table 1). Primary reasons were then tabulated. In order to compare results longitudinally, the groups were subdivided based upon date of initiation of candidacy process, with the dividing factor between the two groups being the change in FDA regulations in November 2000 to allow implantation at the age of 12 months rather than the previous 24 months. Group 1 consisted of those children evaluated between September 1, 1995 and December 31, 2000, and Group 2 consisted of those evaluated between January 1, 2001 and December 31, 2006 (Table 1). Candidates for bilateral CI and those requiring re-implantation are not included in this report. 3. Results Between September 1995 and December 2006, 483 children underwent an evaluation for CI at CHB. Of these 483, CI was not initially recommended for 191. From this group of 191 patients, 3 were excluded due to insufficient data. The remaining 189 patients were divided into two time periods: Group 1 (1995–2000, n = 44) and Group 2 (2001–2006, n = 144). For Group 1, the ages ranged from 0.2 to 15.5 years, with a mean age of 5.32 years, median of 3.8 years and a standard deviation of 3.8 years. For Group 2, the ages ranged from 0.1 to 21.0 years, with a mean age of 5.7 years, median of 3.8 years and a standard deviation of 5.0 years. Gender was evenly split for each group (Group 1: 49% male 51% female, Group 2: 52% male, 48% female), with race and socioeconomic status equally represented in both groups. The rate of initial recommendation against cochlear implantation was 30% in Group 1 and 40% in Group 2. Primary reasons for not recommending a CI are listed in
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Table 1 Primary contraindicator to cochlear implantation by group (n = 189). Description
Primary reason Adult Audiologic contraindication Below FDA age guidelines Child not ready Developmental contraindication Family choice Family not ready Geographical contraindication Guardians not ready Inadequate support system Inconsistent with appointments Initial consult only Insurance barriers Medical contraindicationa Prolonged period without auditory access Severe language deprivation Unknown Total a
Over 18 years old; referred to an adult implant center Too much hearing; does not meet FDA guidelines Under age 12 months Not psychologically ready and/or resistant Significant developmental delay, not capable of symbolic language, psychiatric/cognitive issues Investigated then decided against CI, continued with hearing aids and/or the Deaf Culture [American Sign Language] Combination of child and guardians not ready Residency elsewhere, could not stay in Boston for required 1 year post-op care Still interested, but not ‘‘ready’’ right now Family and/or educational placement a significant concern No follow-through on multiple appointments Initiated process, but no further contact from family No insurance or payment options Medical contraindication, other than developmental Older child (over age 5 years) whose primary mode of communication is manual Too long without spoken language
Group 1 (1995–2000)
Group 2 (2001–2006)
n (%)
n (%)
0 6 1 5
2 22 1 5
(0) (13.25) (2.25) (11.0)
(1.25) (15.25) (0.75) (3.5)
8 (18.0)
6 (4.25)
1 (2.25) 0 (0)
4 (2.75) 1 (0.75)
0 1 0 4 1 1 6
(0) (2.25) (0) (9.0) (2.25) (2.25) (13.25)
2 11 9 14 21 3 23
(1.25) (7.75) (6.25) (9.75) (14.5) (2.0) (16.0)
5 (11.0)
11 (7.75)
5 (11.0) 1 (2.25) 45 (100%)
6 (4.25) 3 (2.0) 144 (100%)
Examples of medical exclusions include: no auditory nerve, severe cochlear ossification, recurrence of extracochlear malignancy, significant cardiac disease, etc.
Table 1. Audiologically, many patients had hearing that was ‘‘too good’’ by FDA guidelines at the time of their evaluation; in others, medical conditions existed which were thought to preclude the development of useful spoken language or at least the ability to use the device to derive significant environmental and audiologic information. Thirty-four children had more than one contraindication. Subgroup analysis of the 188 patients was performed (Tables 1–4). The reasons for not recommending CI varied
Table 2 Outcomes post-CI candidacy evaluation.
Cochlear implant Cued speech Hearing aid(s) Sign language Spoken language without CI Unknown Total
Group 1 (1995–2000) n (%)
Group 1 (2001–2006) n (%)
12 1 7 10 0 15 45
28 0 29 29 1 57 129
(26.75) (2.25) (15.5) (22.25) (0) (33.25) (100%)
(19.5) (0) (20) (20.25) (0.75) (39.5) (100%)
Table 3 Primary contraindicator to cochlear implantation for patients subsequently implanted at another facility. Reason
n (%)
Adult Audiologic contraindication Below FDA age guidelines Developmental contraindication Inadequate support system Inconsistent with appointments Initial consult only Medical contraindication Severe language deprivation Total
1 3 2 4 1 2 1 3 2 19
(5.25) (15.75) (10.5) (21.25) (5.25) (10.5) (5.25) (15.75) (10.5) (100)
significantly between the two groups (Table 1). In Group 1 there was a greater percentage of children who were ‘‘not ready’’ for implantation as compared to Group 2 (11.25% versus 3.5%), a greater percentage of children with severe language deprivation (11.25% versus 4.0%) and a greater percentage of children with developmental contraindications (18.25% versus 4.25%). By contrast, there were a greater percentage of children in Group 2 who had only an initial consult (2.25% versus 14.5%), whose guardians were not ready (2.25% versus 8.25%), or who had inadequate support systems (0% versus 6.25%) (Table 1). Of the 117/188 (62%) patients whose long-term outcome was known, 40 (27.75%) were subsequently implanted at a later date. Rate of later implantation did not vary by group (12/44 [27%] from Group 1 and 28/144 [19%] from Group 2 (Table 2)). Twenty-one were implanted at CHB and 19 at other institutions. For the 19 who were implanted elsewhere, 10 were determined not to be CI candidates at CHB due to either developmental (n = 4; 21%), audiologic (n = 3; 16%) or medical (n = 3; 16%) reasons (Table 3). For the 21 subsequently implanted at CHB, nine (43%) were initially either ‘‘guardians not ready’’ or ‘‘initial consult only’’ (Table 4). In reviewing the data, these two categories might be collapsed into one, as most of the time the families did not follow up because they were not yet ready. Table 4 Primary contraindicator to cochlear implantation for patients subsequently implanted at CHB. Reason
n (%)
Audiologic contraindication Child not ready Family choice Family not ready Guardians not ready Inadequate support system Inconsistent with appointments Initial consult only Medical contraindication Severe language deprivation Total
2 2 1 1 5 2 2 4 1 1 21
(9.5) (9.5) (4.75) (4.75) (24) (9.5) (9.5) (19) (4.75) (4.75) (100)
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The primary communication modality post-candidacy evaluation was examined when the data were available. The communication outcomes between Group 1 and Group 2 were remarkably similar and nearly equally distributed among three outcomes (CI, ASL and hearing aids) (Table 2). 4. Discussion Pediatric CI candidacy criteria are in evolution. For many patients, initial recommendations from our team against a CI were later revisited and CI subsequently recommended. These changes in the recommendations with regard to CI were often due to improved medical stability, improved educational/rehabilitative programming options or development of more realistic expectations on the part of the child and/or the family (the latter often achieved by intense education and counseling by the CI team). A recommendation to wait must always be balanced against the possibility of a poorer outcome if the implant is performed after the early period of optimal brain plasticity related to language acquisition has passed. Additionally, outcome expectations by the parents or the implant team may change over time, with fluency in spoken communication no longer anticipated for some. Depending on a patient’s medical and communication history, the goal of a CI now may be primarily to provide improved environmental awareness and to give a greater sense of ‘‘connection’’ to the world, rather than an expectation of development of normal, or even any, spoken language. A related study by our Team [7] reviewed 40 medically complex pediatric patients, ages 4–14 years, with cochlear implants who had co-morbid conditions such as microcephaly, extreme prematurity with motor impairment, encephalopathy, seizure disorder, autism spectrum disorder, neuromuscular disorder, and genetic syndromes with global developmental disorder, who were at minimum 2 years post-activation of the cochlear implant speech processor. It was noted that 25 of the 40 patients consistently wore the speech processor, with 48% of the total achieving ‘‘minimal or no benefit from access to auditory stimuli to support language development or communication’’ from the CI. Of the remaining 15, 8 used the speech processor inconsistently and 7 were no longer users of the CI. For these 7 non-users, reasons discussed in the Team presentation included, aversive reactions to auditory stimuli, generalized system dysregulation, and equipment struggles in conjunction with neuromuscular disorders and decreased head control. 29/40 demonstrated benefit from CI use, such as ‘‘steady gains in speech production’’ with spoken communication as the primary communication modality (11/40), limited spoken English skills (8/40) and obvious auditory benefit from CI use despite minimal or no gains in speech production 10/40. For these medically complex children, it was determined that counseling to address the full range of outcomes is necessary to ensure appropriate post-implant expectations as well as plan for appropriate services. In addition, additional consultations were often indicated both pre- and post-operatively, (e.g. neurological, physical and adaptive therapy), and surgical planning was often more complicated due to the child’s complex and/or multiple medical needs. The conclusion, however, was that complex medical history and/or neurological disorders definitely did not automatically ‘‘rule out’’ a deaf child from consideration for cochlear implantation. While auditory progress may be slower, spoken language as a primary mode of communication may be attainable in some patients, and even if spoken language does not develop, speech production is not the only indication of benefit from cochlear implantation. Positive changes in general responsiveness, receptive language, and social engagement are often seen when access to sound is provided by CI. Similar results were found by Meinzen-Derr et al. in their pediatric CI population at Cincinnati Children’s Hospital [21].
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For our CI Team, the mandatory element for a favorable recommendation for cochlear implant is appropriate expectations regarding outcome. The family must demonstrate a true ability to understand the limitations of CI and the range of potential outcomes. Informed consent is paramount for any optional surgical intervention. If it is believed that a family does not have appropriate expectations they are guided to improve understanding of their child’s communication and the limitations of CI technology until they are able to demonstrate an appropriate understanding of the information and provide truly informed consent. It is critical to ensure that there are feasible alternatives for those patients for whom CI is ultimately contraindicated, and that supports are in place to ensure communicative success for all the children who initially present for CI candidacy consideration. Otherwise, if the family is unclear about available options and how to best support their child’s communicative needs, there is the risk that a family will continue to ‘‘shop’’ for an implant center to provide them with a surgery and not necessarily focus on other communicative options or strategies. A recent study by Children’s Hospital of Philadelphia (CHOP) noted that their modification of the ChIP ‘‘fails to capture the complexity of the decision-making process’’ [17]. In order to assist pediatric CI teams in objectifying their recommendations about CI candidacy, the Children’s Hospital Boston modified ChIP or mChIPCHB is undergoing validation at our center. (Addendum) This tool has already been found to assist in counseling families at CHB for whom the CI team has concerns and/or the evaluation process is complicated. It demonstrates to the families and caregivers the critical factors necessary for a successful outcome following implantation, and often helps in the planning process for post-CI services. Meanwhile, the data collection is helping the CI team to better track those risk factors that are relative contraindications to CI versus those that have less impact on the ultimate benefit a patient and their family receives from the CI. Analysis of our data showed that patients presented with different concerns for the CI implant team over time. Many factors, which often meant an initial recommendation against CI in the past, are not weighed as heavily now, such as significant developmental delays (e.g. cerebral palsy). Other factors are still concerning and predictive of limited benefit, such as children who have not yet demonstrated the potential for acquiring symbolic language in any modality (ASL, cued speech, spoken language or total communication). With the changes in FDA criteria and the success of implantation in children worldwide, more families are considering CI as a potential option for their children, including those with medically complex backgrounds. In addition, a recent study by Bradham and Jones [2] of potential CI candidates in the US demonstrates that the population who could benefit from CI technology is being underserved and underrecognized. Many reasons for this include continued shortage of qualified personnel and programs to evaluate and care for these children, reimbursement barriers, and disparities in implantation rates based on socioeconomic and ethnicity factors. Another study, by Mathews et al. [19] showed that pediatricians demonstrated deficits in current and accurate knowledge about CIs and Early Hearing Detection and Intervention (EHDI) programs. Therefore, CI programs should be on the forefront of candidacy information and implementation, and may well need to take an educational role with regard to primary care and other providers. 5. Conclusions Careful consideration of the medical, audiologic and psychosocial issues for each patient by a multidisciplinary CI team is crucial in ensuring that all relevant factors are taken into account when counseling parents and patients about CI candidacy. Overall, there
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is an increase in the number of children presenting for potential CI candidacy. Reasons for this include increased patient and family access to CI teams and centers, CI becoming a standard of care in the management of children with severe-to-profound hearing loss, easing of FDA guidelines for pediatric CI, widening of outcome expectations and anticipated benefit, and the early identification of infants with severe-to-profound hearing loss through EHDI programs. The use of a team approach, in conjunction with a validation tool, is important for establishing criteria for successful cochlear implantation counseling as well as planning for management after CI. References [1] E.A. Beadle, D.J. McKinley, T.P. Nikolopoulos, J. Brough, G.M. O’Donoghue, S.M. Archbold, Long-term functional outcomes and academic-occupational status in implanted children after 10 to 14 years of cochlear implant use, Otol. Neurotol. 26 (2005) 1152–1160. [2] T. Bradham, J. Jones, Cochlear implant candidacy in the United States: prevalance in children 12 months to 6 years of age, Int. J. Pediatr. Otorhinolaryngol. 72 (2008) 1023–1028. [3] M. Brendel, A. Buechner, B. Krueger, C. Frohne-Buechner, T. Lenarz, Evaluation of the Harmony sound processor in combination with the speech coding strategy HiRes 120, Otol. Neurotol. 29 (2) (2008) 199–202. [4] R.J. Briggs, M. Tykocinski, E. Saunders, W. Hellier, M. Dahm, B. Pyman, et al., Surgical implication fo perimodiolar cochlear implant electrode design: avoiding intracochlear damage and scala vestibule insertion, Cochlear Implants Int. 2 (2) (2001) 135–149. [5] A.K. Cheng, H.R. Rubin, N.R. Powe, N.K. Mellon, H.W. Francis, J.K. Niparko, Costutility analysis of the cochlear implant in children, JAMA 284 (2000) 850–856. [6] G. Clark, The multi-channel cochlear implant: past, present and future perspectives, Cochlear Implants Int. 10 (Suppl. 1) (2009) 2–13. [7] T.C. Clark, J. Johnston, A. Nussbaum, L. O’Brien, Kammerer, Functional outcomes in medically complex children who receive cochlear implants, Paper presented at CI2007, April 2007. [8] L.T. Cohen, E. Saunders, G.M. Clark, Psychophysics of a prototype peri-modiolar cochlear implant electrode array, Hear. Res. 155 (1–2) (2001) 63–81. [9] L.T. Cohen, E. Saunders, M.R. Knight, R.S. Cowan, Psychophysical measures in patients fitted with contour and straight nucleus electrode arrays, Hear. Res. 212 (1–2) (2006) 160–175. [10] N.L. Cohen, Cochlear implant candidacy and surgical considerations, Audiol. Neurootol. 9 (2004) 197–202. [11] L.C. Edwards, R. Frost, F. Witham, Developmental delay and outcomes in paediatric cochlear implantation: implications for candidacy, Int. J. Pediatr. Otorhinolaryngol. 70 (2006) 1593–1600. [12] R. Filipo, E. Bosco, P. Mancini, D. Ballantyne, Cochlear implants in special cases: deafness in the presence of disabilities and/or associated problems, Acta Otolaryngol. Suppl. 552 (2004) 74–80.
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