title: Cochlear implantation in children with congenital inner ear malformations.
abstract: OBJECTIVE
  To assess the audiologic and surgical outcomes for pediatric cochlear implant patients with inner ear malformations.
  
  
  METHODS
  Retrospective review of 315 pediatric cochlear implant cases from 1994 to 2002.
  
  
  METHODS
  Twenty-eight pediatric cochlear implant patients with known inner ear malformations determined on high-resolution computed tomography (HRCT) of the temporal bone were the subjects of review. Results of HRCT findings, intraoperative findings, postoperative complications, and objective measures of both closed- and open-set testing of speech perception were analyzed.
  
  
  RESULTS
  Patients with the constellation of an incompletely partitioned (IP) cochlea, enlarged vestibular aqueduct (EVA), and a dilated vestibule (i.e., Mondini's malformation) as well as those with an isolated EVA or partial semicircular canal aplasia have relatively good levels of speech perception. Patients with total semicircular canal aplasia, isolated IP, cochlear hypoplasia, or common cavity demonstrated lower levels of performance. Poor performance may be related to associated developmental delays rather than labyrinthine anatomy alone. Complications of surgery were relatively limited.
  
  
  CONCLUSIONS
  Cochlear implantation can be successfully performed in children with inner ear malformations. These children and their parents can expect significant auditory benefits from this intervention. The various types of inner ear malformations may have quite different prognoses for good auditory performance.
authors.affiliation: W. Paul Biggers, MD, Carolina Children's Communication Disorders Program, Department of Otolaryngology-Head and Neck Surgery, University of North Carolina, Chapel Hill, North Carolina, 27599-7600, USA. buchman@med.unc.edu
authors.forename: Craig A
authors.initials: CA
authors.lastname: Buchman
authors.forename: Benjamin J
authors.initials: BJ
authors.lastname: Copeland
authors.forename: Kathy K
authors.initials: KK
authors.lastname: Yu
authors.forename: Carolyn J
authors.initials: CJ
authors.lastname: Brown
authors.forename: Vincent N
authors.initials: VN
authors.lastname: Carrasco
authors.forename: Harold C
authors.initials: HC
authors.lastname: Pillsbury
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003051:Cochlea / Q000002:abnormalities; D019929:Cochlear Implantation; D007758:Ear, Inner / Q000002:abnormalities* / Q000601:surgery; D006801:Humans; D007223:Infant; D011183:Postoperative Complications; D012189:Retrospective Studies; D013067:Speech Perception / Q000502:physiology; D013701:Temporal Bone / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014723:Vestibular Aqueduct / Q000002:abnormalities; D014722:Vestibule, Labyrinth / Q000002:abnormalities