Cochlear implantation results in patients with Kearns-Sayre syndrome.
OBJECTIVE
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).


METHODS
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.
Pijl
Sipke
S
School of Audiology and Speech Sciences, University of British Columbia, Vancouver, BC, Canada. SPijl@providencehealth.bc.ca
Westerberg
Brian D
BD
D000328:Adult; D001303:Auditory Cortex / Q000503:physiopathology; D001306:Auditory Pathways / Q000503:physiopathology; D019929:Cochlear Implantation; D003056:Cochlear Nerve / Q000503:physiopathology; D003638:Deafness / Q000534:rehabilitation*; D005072:Evoked Potentials, Auditory / Q000502:physiology; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005260:Female; D006801:Humans; D007625:Kearns-Sayre Syndrome / Q000503:physiopathology / Q000534:rehabilitation*; D008297:Male; D008875:Middle Aged; D011930:Reaction Time / Q000502:physiology; D013063:Speech Discrimination Tests