title: Osseointegrated hearing device placement in congenital lamellar ichthyosis.
abstract: OBJECTIVE
  To present a case of osseointegrated hearing device placement in a child with conductive hearing loss related to manifestations of congenital lamellar ichthyosis.
  
  
  METHODS
  A 5-year-old female patient with congenital lamellar ichthyosis resulting in conductive hearing loss because of bilateral external auditory canal stenosis and tympanic membrane blunting.
  
  
  METHODS
  Unilateral osseointegrated hearing device placement using a traditional skin flap technique.
  
  
  METHODS
  Frequency and severity of adverse skin reactions, device usage, and audiometric testing.
  
  
  RESULTS
  After 51 months of follow-up postoperatively, the patient has only required 2 treatments for minor skin reactions (Holgers Grade I). Aided speech reception threshold was 15 dB hearing level (HL) compared to 35 dB HL unaided. The subject has used the device continuously with parental report of improvement in school performance.
  
  
  CONCLUSIONS
  Osseointegrated hearing device placement may be a viable option in patients with congenital lamellar ichthyosis despite the skin-related comorbidities known to be associated with this disease condition.
authors: Department of Otolaryngology-Head and Neck Surgery, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock, Arkansas 72205, USA.
authors: Abby R
authors: AR
authors: Nolder
authors: Michael B
authors: MB
authors: Gluth
mesh_terms: D001309:Auditory Threshold / Q000502:physiology; D001844:Bone Conduction / Q000502:physiology*; D002675:Child, Preschool; D005260:Female; D006314:Hearing Loss, Conductive / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D006320:Hearing Tests; D006801:Humans; D017490:Ichthyosis, Lamellar / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D016348:Osseointegration / Q000502:physiology*; D013067:Speech Perception / Q000502:physiology*; D016896:Treatment Outcome