title: The Outcome of Cochlear Implantation for Mitochondrial Disease Patients With Syndromic Hearing Loss.
abstract: OBJECTIVE
  To evaluate the outcome and to confirm the validity of cochlear implantation for syndromic deafness in patients with mitochondrial disease.
  
  
  METHODS
  Retrospective case review.
  
  
  METHODS
  Tertiary referral center.
  
  
  METHODS
  We reviewed medical charts of 367 cochlear implantation cases at Kyoto University Hospital between 1987 and 2012. We identified five patients with syndromic mitochondrial disease who underwent cochlear implantation surgery. The mean age of the patients (four women and one man) when they underwent surgeries was 44.4 years (range, 30-64 yr; median, 41 yr).
  
  
  METHODS
  Therapeutic and rehabilitative.
  
  
  METHODS
  In four of five patients, speech perception performance was measured using Japanese vowels, consonant-vowel syllables, and short sentences.
  
  
  RESULTS
  Only 1.4% (5 of 367) of cochlear implantation cases at Kyoto University Hospital underwent cochlear implantation surgery because of syndromic mitochondrial diseases. Four of those patients showed significantly improved speech perception outcomes, and the beneficial effects of the intervention continued long after surgery. One patient could not perform speech perception test presumably because of poor cognitive function.
  
  
  CONCLUSIONS
  Mitochondrial disease patients who underwent cochlear implantation surgery sustained gains in hearing performance even long after surgery. A single patient showed poor postoperative speech perception associated with cognitive problems. Cochlear implantation for mitochondrial disease patients seems to be a viable treatment option in the absence of significant cognitive impairment.
authors.affiliation: *Department of Otolaryngology Head and Neck Surgery, Graduate School of Medicine, Kyoto University, Kyoto City, Kyoto; and †Department of Otolaryngology, Tenri Hospital, Tenri City, Nara, Japan.
authors.forename: Norio
authors.initials: N
authors.lastname: Yamamoto
authors.forename: Hideaki
authors.initials: H
authors.lastname: Okuyama
authors.forename: Harukazu
authors.initials: H
authors.lastname: Hiraumi
authors.forename: Tatsunori
authors.initials: T
authors.lastname: Sakamoto
authors.forename: Hitomi
authors.initials: H
authors.lastname: Matsuura
authors.forename: Juichi
authors.initials: J
authors.lastname: Ito
mesh_terms: D000328:Adult; D019929:Cochlear Implantation; D003638:Deafness / Q000209:etiology* / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D028361:Mitochondrial Diseases / Q000150:complications*; D012189:Retrospective Studies; D013067:Speech Perception