title: Cochlear implantation results in patients with Kearns-Sayre syndrome.
abstract: 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.
authors.affiliation: School of Audiology and Speech Sciences, University of British Columbia, Vancouver, BC, Canada. SPijl@providencehealth.bc.ca
authors.forename: Sipke
authors.initials: S
authors.lastname: Pijl
authors.forename: Brian D
authors.initials: BD
authors.lastname: Westerberg
mesh_terms: 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