Implants in Children with Multiple Disabilities andor Auditory Neuropathy

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Our ability to implant children with a cochlear device has no more serious potential for damage and financial waste than in instances in which the child is deaf but has additional disabilities. It must be admitted, if not generally acknowledged, that children with multiple disabilities, including mental retardation or severe language-learning difficulties, have been implanted already. In some children who are deaf and blind but otherwise intact, the decision to implant may be a good one. However, the parents of children with multiple disabilities, including deafness, are among the most vulnerable as they seek help for the child whose future may seem bleak. Everyone must understand the implant's role as only one factor in the habilitation and long-term development of any child with deafness. Unless this is clear to all parents, some well-meaning surgeons will continue to be persuaded that an implant is critical for a particular child. Professional implant teams that include audiologists, speech/language pathologists, educators, psychologists, and social workers can be of help in protecting the surgeon from undue family pressure in this regard. Unfortunately, because the implant is no longer an experimental device for children, parents who are refused by one team will usually be able to find another team that will provide the implant.

Interest in, and ability to, identify infants and children with auditory neuropathy has added to the complexity of the decision about implantation. I am referring to a condition in which both conventional and electophysiologic measures of auditory function show abnormal auditory nerve function with relatively intact cochlear function; that is, the infant, toddler, or child with auditory neuropathy will likely show robust pre-neural evidence of cochlear hair cell function (intact evoked otoacoustic emissions) and absent or grossly abnormal 8 th cranial nerve auditory transmissions (measured through evoked electrophysiologic measures, such as auditory brainstem response testing). Conventional behavioral audiometry in these cases generally suggests profound bilateral hearing impairment. The precise site of lesion in many cases of auditory neuropathy can only be inferred, particularly in the absense of a syndrome. Implantation in a cochlea with evidence of normal hair cell function means sacrifice of a normal organ, which presents a serious ethical dilemma. Several of these children have been implanted, with mixed results.15 If a decision is made to implant a child with auditory neuropathy, the quality of family and educational support must be strong and the expectations of family, educators, and the child carefully tempered.

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