Cochlear implantation results in patients with Kearns-Sayre syndrome

Ear Hear. 2008 Jun;29(3):472-5. doi: 10.1097/01.aud.0000310791.83193.62.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Auditory Cortex / physiopathology
  • Auditory Pathways / physiopathology
  • Cochlear Implantation*
  • Cochlear Nerve / physiopathology
  • Deafness / rehabilitation*
  • Evoked Potentials, Auditory / physiology
  • Evoked Potentials, Auditory, Brain Stem / physiology
  • Female
  • Humans
  • Kearns-Sayre Syndrome / physiopathology
  • Kearns-Sayre Syndrome / rehabilitation*
  • Male
  • Middle Aged
  • Reaction Time / physiology
  • Speech Discrimination Tests