title: Hearing rehabilitation with a binaural cochlear implant in a patient with Erdheim-Chester disease.
abstract: OBJECTIVE
  Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis. This paper reports an exceptional case of bilateral neural involvement, responsible for profound hearing loss. Bilateral cochlear implantation was performed.
  
  
  METHODS
  We present a 57-year-old man affected by ECD with profound bilateral hearing loss.
  
  
  METHODS
  The patient underwent cochlear implantation with a binaural Digisonic(®) cochlear implant, 7 years after the initial diagnosis.
  
  
  RESULTS
  Speech intelligibility rose to a plateau after about 6 months of cochlear implant use. The average outcome of speech intelligibility over time was 55% for dissyllabic words without lip reading, and 70% for sentences. Perception score decreased before the patient died from ECD.
  
  
  CONCLUSIONS
  A description of the ECD and its otological manifestations is presented. This paper reports the effective hearing rehabilitation of profound bilateral hearing loss by the means of a binaural Digisonic(®) cochlear implant.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Querat
authors.forename: Hung
authors.initials: H
authors.lastname: Thai-Van
authors.forename: Denis Vital
authors.initials: DV
authors.lastname: Durand
authors.forename: François
authors.initials: F
authors.lastname: Cotton
authors.forename: Stéphane
authors.initials: S
authors.lastname: Gallego
authors.forename: Eric
authors.initials: E
authors.lastname: Truy
mesh_terms: D019929:Cochlear Implantation / Q000295:instrumentation*; D003054:Cochlear Implants; D031249:Erdheim-Chester Disease / Q000150:complications*; D017809:Fatal Outcome; D006312:Hearing Loss, Bilateral / Q000209:etiology / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D013065:Speech Intelligibility; D013067:Speech Perception
keywords: Auditory pathways; Binaural cochlear implant; Erdheim–Chester disease