title: A review of cochlear implantation in mitochondrial sensorineural hearing loss.
abstract: OBJECTIVE
  Mitochondrial sensorineural hearing loss (SNHL) may be nonsyndromic (occurring in isolation), associated with the A1555G mutation in the MTRNR1 gene. Mitochondrial SNHL may also be syndromic, associated with the A3243G point mutation in the MTTL1 gene. In syndromic cases-mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS), maternally inherited diabetes and deafness, Kearns-Sayre syndrome, and chronic progressive external ophthalmoplegia-the SNHL compounds already existing disabilities. The genetic basis for mitochondrial SNHL and postulated sites of pathologic changes are discussed.
  
  
  METHODS
  Sources used were relevant clinical and basic science publications.
  
  
  METHODS
  A search of the entire databases of Medline and Web of Science, using various subject headings and free-text terms, was used to identify patients with mitochondrial disease having cochlear implants.
  
  
  METHODS
  The data from publications were critically reviewed and tabulated to assess implantation outcomes.
  
  
  RESULTS
  The data were not amenable to formal meta-analysis or valid data summarization, other than descriptive statistics.
  
  
  CONCLUSIONS
  There is an increasing awareness of the prevalence of mitochondrial SNHL and its progressive nature. High-risk candidates warrant genetic testing and family screening. Correlating the data for mitochondrial SNHL as a treatable entity is important, and the authors present an overview of these patients successfully rehabilitated by cochlear implantation.
authors.affiliation: Department of Otolaryngology, Belfast City Hospital, Queen's University, Belfast at the Belfast City Hospital, UK. rajsinn@aol.com
authors.forename: A R
authors.initials: AR
authors.lastname: Sinnathuray
authors.forename: V
authors.initials: V
authors.lastname: Raut
authors.forename: A
authors.initials: A
authors.lastname: Awa
authors.forename: A
authors.initials: A
authors.lastname: Magee
authors.forename: J G
authors.initials: JG
authors.lastname: Toner
mesh_terms: D000328:Adult; D000368:Aged; D001301:Audiometry, Pure-Tone; D001921:Brain / Q000000981:diagnostic imaging*; D019929:Cochlear Implantation; D004272:DNA, Mitochondrial / Q000235:genetics; D005260:Female; D015870:Gene Expression / Q000235:genetics; D006319:Hearing Loss, Sensorineural / Q000150:complications / Q000175:diagnosis* / Q000601:surgery*; D006801:Humans; D007625:Kearns-Sayre Syndrome / Q000150:complications / Q000235:genetics; D017241:MELAS Syndrome / Q000150:complications / Q000000981:diagnostic imaging* / Q000235:genetics; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D017354:Point Mutation / Q000235:genetics; D016133:Polymerase Chain Reaction; D012313:RNA / Q000235:genetics; D000077278:RNA, Mitochondrial; D012343:RNA, Transfer / Q000235:genetics; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D015837:Vestibular Diseases / Q000150:complications / Q000503:physiopathology
chemical_list: D004272:DNA, Mitochondrial; D000077278:RNA, Mitochondrial; D012313:RNA; D012343:RNA, Transfer