title: Cochlear implantation in a patient with mitochondrial encephalopathy, lactic acidosis and stroke-like episodes syndrome.
abstract: OBJECTIVE
  The aim of this study was to investigate the usefulness of a Nucleus C124M cochlear implant in a patient with mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome.
  
  
  METHODS
  A 29-year-old woman with MELAS syndrome presented with sensorineural hearing loss and diabetes mellitus and underwent multichannel cochlear implantation. The follow-up period was 10 months. The case history, assessment of mitochondrial disease, indications for the cochlear implant and the benefits of cochlear implantation were evaluated.
  
  
  RESULTS
  Nine months after the surgery the patient could use the telephone and was quite satisfied with the improvement in communication due to the cochlear implant.
  
  
  CONCLUSIONS
  Cochlear implantation can be recommended for patients with MELAS syndrome if they have residual retrocochlear function. Single photon emission computerized tomography was found to be very useful for evaluating retrocochlear function.
authors.affiliation: Department of Otolaryngology, Toyama Medical and Pharmaceutical University, Sugitani, Toyama, Japan. satsuki@ms.toyama-mpu.ac.jp
authors.forename: Satsuki
authors.initials: S
authors.lastname: Yasumura
authors.forename: Shin
authors.initials: S
authors.lastname: Aso
authors.forename: Michiro
authors.initials: M
authors.lastname: Fujisaka
authors.forename: Yukio
authors.initials: Y
authors.lastname: Watanabe
mesh_terms: D000328:Adult; D001301:Audiometry, Pure-Tone; D001309:Auditory Threshold / Q000502:physiology; D001933:Brain Stem / Q000000981:diagnostic imaging; D003054:Cochlear Implants; D003638:Deafness / Q000000981:diagnostic imaging / Q000534:rehabilitation*; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005260:Female; D006801:Humans; D017241:MELAS Syndrome / Q000000981:diagnostic imaging / Q000534:rehabilitation*; D009778:Occipital Lobe / Q000000981:diagnostic imaging; D011474:Prosthesis Design; D013069:Speech Reception Threshold Test; D015899:Tomography, Emission-Computed, Single-Photon; D016896:Treatment Outcome