Pediatric Cochlear Implantation in Auditory Neuropathy

Abstract

Auditory neuropathy (AN) is a hearing disorder caused by desynchronized neural discharges of the auditory nerve. Some reports have shown that cochlear implantation has been successful for rehabilitation of children with AN, and that their altered neural synchrony can be restored by electrical stimulation introduced by cochlear implants. Most recipients achieved open-set speech perception abilities and showed progressive improvements in their communication skills. One case-control study showed that there were no signifi cant differences in speech perception abilities between a group with AN and a group without AN after cochlear implantation. Consistent with previous reports, nine children with AN who received cochlear implants at Dong-A University Hospital benefi ted considerably from cochlear implantation. Although most recipients have demonstrated positive outcomes, some children in published reports have shown very poor performance of minimal improvements in close-set speech perception and no improvements in open-set speech perception. Cochlear implantation is considered a useful tool for the rehabilitation of children with AN, but the procedure needs to be done after the parents are informed fully about the uncertainty of results until the prognosis for this procedure can be established fi rmly. Additional long-term follow-up studies including a large number of recipients and more basic research to reveal the underlying pathophysiology of AN is necessary to establish cochlear implantation as a standard treatment option for this disorder.