Sipke Pijl1, Brian D Westerberg. 1. School of Audiology and Speech Sciences, University of British Columbia, Vancouver, BC, Canada. SPijl@providencehealth.bc.ca
Abstract
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.
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.