title: Bilateral cochlear implantation in bilateral Langerhans cell histiocytosis.
abstract: BACKGROUND
  Langerhans' cell histiocytosis (LCH) is a rare proliferative disorder that can have otologic manifestations in up to 30% of patients. Treatment of local and systemic disease may include medical, surgical, and radiation therapies. Involvement of the temporal bone can lead to conductive and, rarely, sensorineural hearing loss. Post-labyrinthectomy cochlear implantation can be an effective treatment option for sensorineural hearing loss in the setting of persistent LCH.
  
  
  METHODS
  A retrospective case review at a tertiary academic medical center. Hearing in Noise Test (HINT) performed before and after bilateral cochlear implantation was examined.
  
  
  RESULTS
  Following bilateral partial labyrinthectomy, post-operative testing showed a HINT in quiet of 17%. Left-sided cochlear implant followed by immunosuppressive therapy for persistent disease showed marked improvement with post-operative HINT in a quiet room of 80% and 63% at 1 and 2 years. Fifty-five months after left implantation, and 10 months after right cochlear implantation, binaural post-operative HINT in quiet was 81%.
  
  
  CONCLUSIONS
  Surgical excision of LCH lesion remains a mainstay of treatment for temporal bone involvement. Bilateral cochlear implant with adjuvant immunosuppression in our patient demonstrated both immediate and delayed improvement in auditory function after staged cochlear implantation in the setting of persistent disease.
authors: Jamie M
authors: JM
authors: Segel
authors: Brian J
authors: BJ
authors: McKinnon
mesh_terms: D019929:Cochlear Implantation; D003054:Cochlear Implants; D007758:Ear, Inner / Q000601:surgery; D004432:Ear, Middle / Q000601:surgery; D005260:Female; D006312:Hearing Loss, Bilateral / Q000150:complications / Q000601:surgery*; D006319:Hearing Loss, Sensorineural / Q000601:surgery; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications*; D006801:Humans; D008875:Middle Aged; D012189:Retrospective Studies; D013701:Temporal Bone / Q000503:physiopathology; D016896:Treatment Outcome
keywords: Cochlear implant; Langerhans' histiocytosis