title: Audiological performance after cochlear implantation in children with inner ear malformations.
abstract: OBJECTIVE
  To prove that cochlear implantation is a beneficial method of rehabilitation in deaf children with malformations of the inner ear.
  
  
  METHODS
  The evaluation of auditory responses to speech (EARS) test battery was performed on the children in this study after an average implant use of 3 years.
  
  
  RESULTS
  Individual results of six children with inner ear anomalies receiving cochlear implants are presented in this study. Three of the patients showed an incomplete partition (Mondini dysplasia), one had a cochlear hypoplasia and two suffered from an intraoperative cerebrospinal fluid leak. The majority of the children in this study are successful implant users. Wherever possible, test scores are included and subjective case reports given.
  
  
  CONCLUSIONS
  Results are similar to those in children with normal cochleas, therefore inner ear malformations found in as many as 20% of patients with congenital sensorineural hearing loss are no contraindication for cochlear implantation. Nevertheless, factors influencing the success of implantation are multiple, including a thorough preoperative radiological examination, a well-performed surgery and an individually tailored postoperative rehabilitation programme.
authors.affiliation: Department of Otorhinolaryngology, Medical School, University of Vienna, Waehringer Guertel 18-20, A-1097 Vienna, Austria.
authors.forename: Christoph
authors.initials: C
authors.lastname: Arnoldner
authors.forename: Wolf Dieter
authors.initials: WD
authors.lastname: Baumgartner
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Gstoettner
authors.forename: Brigitte
authors.initials: B
authors.lastname: Egelierler
authors.forename: Christian
authors.initials: C
authors.lastname: Czerny
authors.forename: Erich
authors.initials: E
authors.lastname: Steiner
authors.forename: Jafar
authors.initials: J
authors.lastname: Hamzavi
mesh_terms: D001302:Audiometry, Speech; D002558:Cerebrospinal Fluid Otorrhea / Q000150:complications; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation / Q000379:methods; D003054:Cochlear Implants; D007758:Ear, Inner / Q000002:abnormalities* / Q000000981:diagnostic imaging; D005260:Female; D005500:Follow-Up Studies; D034381:Hearing Loss / Q000534:rehabilitation*; D006801:Humans; D007759:Labyrinth Diseases / Q000150:complications* / Q000151:congenital; D008297:Male; D012189:Retrospective Studies; D013067:Speech Perception; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome