title: Cochlear implantation in mitochondrial deafness due to A7445G mutation.
abstract: OBJECTIVE
  This study aimed to determine if cochlear implantation (CI) improved speech discrimination in the New Zealand pedigree with the A7445G mitochondrial DNA (mtDNA) mutation and sensorineural hearing loss (SNHL).
  
  
  METHODS
  Nine patients had their speech discrimination and pure tone audiograms evaluated. Pre- and post-implant results were compared. Type of CI and patient age at implantation was recorded.
  
  
  RESULTS
  Speech discrimination testing shows greatly improved functional hearing after CI in this group of patients.
  
  
  CONCLUSIONS
  Individuals with mtDNA-related SNHL should be identified by screening and offered assessment for CI suitability because functional hearing improves after CI.
authors.affiliation: Department of Otolaryngology, Christchurch Public Hospital, Christchurch, New Zealand. rsalter@clear.net.nz
authors.forename: Rachelle L
authors.initials: RL
authors.lastname: Love
authors.forename: Philip
authors.initials: P
authors.lastname: Bird
mesh_terms: D000293:Adolescent; D000328:Adult; D001301:Audiometry, Pure-Tone; D019929:Cochlear Implantation; D004252:DNA Mutational Analysis; D004272:DNA, Mitochondrial / Q000235:genetics*; D003638:Deafness / Q000175:diagnosis / Q000235:genetics* / Q000534:rehabilitation*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D028361:Mitochondrial Diseases / Q000175:diagnosis / Q000235:genetics* / Q000534:rehabilitation*; D009520:New Zealand; D010375:Pedigree; D012313:RNA / Q000235:genetics; D000077278:RNA, Mitochondrial; D013063:Speech Discrimination Tests; D013577:Syndrome; D055815:Young Adult
chemical_list: D004272:DNA, Mitochondrial; D000077278:RNA, Mitochondrial; D012313:RNA