Ear and hearing · 2008
Cochlear implantation results in patients with Kearns-Sayre syndrome.
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Pijl Sipke, Westerberg Brian D
Аннотация
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.
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.