title: Audiological performance after cochlear implantation: a 2-year follow-up in children with inner ear malformations.
abstract: CONCLUSIONS
  Open-set speech perception in children with an inner ear malformation is equal to that of other congenitally deaf children after an average of 2 years follow-up.
  
  
  OBJECTIVE
  To analyze audiological performance after cochlear implantation in a sample of children with radiographically detectable malformations of the inner ear compared to performance in prelingually deafened children at large.
  
  
  METHODS
  Nine children with osseous inner ear malformations were compared to 22 congenitally deaf children, all of whom underwent cochlear implantation. All subjects were tested on their electrical evoked compound action potential. Speech perception tests were performed using the monosyllabic trochee polysyllabic test without visual support and the open-set monosyllabic wordlist.
  
  
  RESULTS
  In all, 20% of the congenitally deaf children in our center study have inner ear abnormalities. Inner ear malformations were limited to incomplete partition of the cochlea; none of the subjects had common cavity malformations. Electrical compound action potentials were successfully recorded in both groups intraoperatively. Speech perception tests on open-set speech yielded an average of 48.8% (SD 21.2%) in the group of children with inner ear malformations vs 54.5% (SD 21.1%) in congenitally deaf children. In four of nine cases with an inner ear malformation we encountered a minor CSF leak.
authors.affiliation: Department of Otorhinolaryngology - Head and Neck Surgery, University Medical Center Utrecht, Utrecht, The Netherlands. gijs@vanwermeskerken.com
authors.forename: Gijs K A
authors.initials: GK
authors.lastname: Van Wermeskerken
authors.forename: Erwin A
authors.initials: EA
authors.lastname: Dunnebier
authors.forename: Adriaan F
authors.initials: AF
authors.lastname: Van Olphen
authors.forename: Bert A
authors.initials: BA
authors.lastname: Van Zanten
authors.forename: Frans W J
authors.initials: FW
authors.lastname: Albers
mesh_terms: D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation; D003056:Cochlear Nerve / Q000503:physiopathology; D003638:Deafness / Q000151:congenital* / Q000000981:diagnostic imaging / Q000534:rehabilitation*; D007758:Ear, Inner / Q000002:abnormalities* / Q000000981:diagnostic imaging; D004558:Electric Stimulation; D005072:Evoked Potentials, Auditory / Q000502:physiology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications / Q000000981:diagnostic imaging / Q000503:physiopathology*; D012815:Signal Processing, Computer-Assisted; D013067:Speech Perception / Q000502:physiology; D013686:Telemetry; D014057:Tomography, X-Ray Computed