title: Usher syndrome: characteristics and outcomes of pediatric cochlear implant recipients.
abstract: OBJECTIVE
  To evaluate the characteristics and outcomes of pediatric cochlear implant recipients diagnosed with Usher syndrome (US).
  
  
  METHODS
  Retrospective study of consecutive pediatric cochlear implant recipients (1991-2010).
  
  
  METHODS
  Tertiary care children's hospital.
  
  
  METHODS
  Children who received a cochlear implant who were diagnosed with US either before or after implantation.
  
  
  METHODS
  Electroretinography and ophthalmologic findings, cochlear anatomy based on preoperative imaging, age of independent ambulation, age at implantation, speech perception level, and communication method.
  
  
  RESULTS
  Approximately 26 (3.7%) of 712 cochlear implant recipients were diagnosed with US based on the results of electroretinography and/or genetic testing. Preoperative imaging revealed no evidence of cochlear malformations. Average age of independent ambulation was 21.9 months (range, 12-30). Average age at implantation was 3.3 years (range, 6 mo to 11.6 yr). Mean follow-up after implantation was 7.8 years (range, 10 mo to 15.6 yr). Open-set speech perception was present in 92% of children, with use of a primarily oral communication mode in 69.2%.
  
  
  CONCLUSIONS
  In this large series of patients with the diagnosis of US who have undergone cochlear implantation, CT and MRI imaging were normal. Significant delay in independent ambulation was present in this population secondary to abnormal vestibular function associated with US Type I. A majority of children developed significant open-set speech perception and oral communication skills. Implantation of US children provides them with the opportunity to develop useful hearing and oral communication.
authors: Department of Otolaryngology-Head and Neck Surgery, Nationwide Children's Hospital and The Ohio State University, Columbus, Ohio, USA.
authors: Kris R
authors: KR
authors: Jatana
authors: Denise
authors: D
authors: Thomas
authors: Lisa
authors: L
authors: Weber
authors: Marilyn B
authors: MB
authors: Mets
authors: Josh B
authors: JB
authors: Silverman
authors: Nancy M
authors: NM
authors: Young
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003051:Cochlea / Q000601:surgery; D019929:Cochlear Implantation; D003054:Cochlear Implants; D004596:Electroretinography; D005260:Female; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000503:physiopathology / Q000601:surgery*; D006320:Hearing Tests; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D013060:Speech / Q000502:physiology*; D013067:Speech Perception / Q000502:physiology*; D016896:Treatment Outcome; D052245:Usher Syndromes / Q000503:physiopathology / Q000601:surgery*