title: Outcomes and special considerations of cochlear implantation in waardenburg syndrome.
abstract: OBJECTIVE
  The objective of this study was a state-of-the-art analysis of cochlear implantation in patients with Waardenburg syndrome (WS).
  
  
  METHODS
  Twenty-five patients with WS treated with cochlear implants in our department from 1990 to 2010.
  
  
  METHODS
  The 25 patients with WS underwent 35 cochlear implantations.
  
  
  METHODS
  Hearing outcome was evaluated using HSM sentence test in 65 dB in quiet, Freiburg Monosyllabic Test, and categories of auditory performance for children and compared with that of a control group. Anatomic abnormalities of the inner ear were examined using magnetic resonance imaging and computed tomography of the temporal bones.
  
  
  RESULTS
  The mean follow-up time was 8.3 years (range, 0.3-18.3 yr). The majority achieved favorable postimplantation performance with mean HSM scores of 75.3% (range, 22.6%-99%) and Freiburg Monosyllabic Test scores of 67.8% (range, 14%-95%). However, in 4 cases, the results were less satisfactory. The comparison with the control group did not reveal any statistical significance (p = 0.56). In 6 patients (24%), behavioral disorders caused temporary difficulties during the rehabilitation procedure. Except of isolated large vestibule in 1 patient, the radiological assessment of the 50 temporal bones did not reveal any temporal bone abnormalities.
  
  
  CONCLUSIONS
  Most patients with WS performed well with cochlear implants. However, WS is related to behavioral disorders that may cause temporary rehabilitation difficulties. Finally, temporal bone malformations that could affect cochlear implantation are notcharacteristic of WS.
authors.affiliation: Otorhinolaryngology Department, Hanover Medical University, Hanover, Germany. Kontorinis.Georgios@mh-hannover.de
authors.forename: Georgios
authors.initials: G
authors.lastname: Kontorinis
authors.forename: Thomas
authors.initials: T
authors.lastname: Lenarz
authors.forename: Alexandros
authors.initials: A
authors.lastname: Giourgas
authors.forename: Martin
authors.initials: M
authors.lastname: Durisin
authors.forename: Anke
authors.initials: A
authors.lastname: Lesinski-Schiedat
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation; D003054:Cochlear Implants; D005260:Female; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D017060:Patient Satisfaction; D016896:Treatment Outcome; D014849:Waardenburg Syndrome / Q000601:surgery*