Cochlear implantation results in patients with Kearns-Sayre syndrome.

OBJECTIVES

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).

DESIGN

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.

RESULTS

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

CONCLUSIONS

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.