Cochlear implantation results in patients with Kearns-Sayre syndrome.

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.

• Authors: Sipke Pijl, Brian D Westerberg
• Journal: Ear and hearing (Ear Hear) Vol. 29 Issue 3 Pg. 472-5 (Jun 2008) ISSN: 0196-0202 [Print] United States
• PMID: 18382375 (Publication Type: Case Reports, Journal Article)

Topics

• 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, rehabilitation)
• Male
• Middle Aged
• Reaction Time (physiology)
• Speech Discrimination Tests