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AN/AD & Cochlear Implantation

Early Cochlear Implantation

Given the fluid nature of AN/AD and the possibility of spontaneous recovery early and almost immediate cochlear implantation is only strongly recommended in the few cases where the cause of the AN/AD is clearly hereditary and when the history of other family members would indicate that this is the best route.  It is however always the parents’ choice and implantation can be very successful especially when preceded by and later supported by CS.  Cued Speech is always a useful tool for teaching language and syntax and clarifying the new sounds that the child will hear through the implant.

Dr Berlin writes:

Management and Outcomes

Auditory Neuropathy/Dys-synchrony as a diagnosis does NOT necessarily mean the nerve of hearing is irreparably damaged or that nothing can be done for it.  In about 2/3rds of the cases there is no demonstrable peripheral nerve damage and in those who have such damage, palliation is still possible. (Starr et al.)

 ‘In virtually all well-programmed cases we have seen so far, cochlear implants have helped synchronize the auditory nervous system and assist in speech and language development or clarity. (Shallop et al.  House group etc.)

‘Hearing aids have been recommended and we tried them for 20 years. We have yet to see patients learn language and show normal speech development with hearing aids and Auditory Verbal Therapy (with no visual cues) at the same rate as a child with simple outer hair cell loss.  This conflicts in part with other people’s reports in the literature (eg Rance et al.) but, had we published even our first 10 years of experience, we would have also thought that hearing aids were of value because they seemed to enhance sound field sensitivity and “improve the audiogram”.  Here we must repeat an important principle of patient management which I first heard from Dr. Nancy Young, a Chicago-based paediatric otolaryngologist:  Treat the CHILD not the TEST RESULTS.

‘As we said earlier, a few AN/AD children we have followed learn language with no intervention although they persist in having trouble hearing in noise. They are often mis-diagnosed later in life as having “Central Auditory Processing Disorders”. The absence of middle ear muscle reflexes and an abnormal ABR test quickly identifies them as having a mild form of Auditory Neuropathy.

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