Cochlear Implantation Results in Patients With Kearns-Sayre Syndrome

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To examine the evidence for a cochlear, retrocochlear, or central site of lesion for deafness in two cochlear implant recipients with Kearns-Sayre Syndrome (KSS).


Speech perception data and electrically evoked Auditory Brainstem (eABR) and Middle Latency Responses were obtained in two patients with KSS and compared with a group of non-KSS implant recipients.


Speech perception data and electrophysiological responses for the patients with KSS were similar to those obtained in non-KSS patients.


Results are consistent with an initial cochlear site of lesion for deafness in KSS, and with relative sparing of the central auditory pathway early in the disease.

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