title: Clinical outcomes following cochlear implantation in children with inner ear anomalies.
abstract: OBJECTIVE
  A significant proportion of children with congenital hearing loss who are candidates for cochlear implants (CIs) may have inner ear malformations (IEMs). Surgical and speech outcomes following CI in these children have not been widely reported.
  
  
  METHODS
  The charts of children who were evaluated for a CI between 1/1/1986 and 12/31/2014 at a university-based tertiary level pediatric cochlear implant center were reviewed. Principal inclusion criteria included (i) age 1-18 years, (ii) history of bilateral severe to profound sensorineural hearing loss, and (iii) limited benefit from binaural amplification. Exclusion criteria included (i) underlying diagnosis of neurodevelopmental disorder and (ii) lack of follow up for speech assessment if a CI was performed. The following outcome measures were reviewed: (i) imaging findings with magnetic resonance imaging or high resolution computed tomography, (ii) intraoperative complications, and (iii) speech perception categorized as the ability to perceive closed set, open set, or none.
  
  
  RESULTS
  The prevalence of IEMs was 27% (102 of 381), of which 79% were bilateral. Cochlear dysplasia accounted for 30% (40 of 136) of the anomalies. Seventy-eight of the 102 patients received a CI (78%). Surgery was noted to be challenging in 24% (19 of 78), with a perilymphatic gusher being the most common intraoperative finding. Cochlear dysplasia, vestibular dysplasia and cochlear nerve hypoplasia were associated with poor speech perception (open OR closed set speech recognition scores, 0-23%), although the outcomes in children with enlarged vestibular aqueduct were similar to those of children with normal inner ear anatomy (65%).
  
  
  CONCLUSIONS
  Cochlear implantation is safe in children with IEMs. However, the speech perception outcomes are notably below those of patients with normal anatomy, with the exception of when an enlarged vestibular aqueduct is present.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA.
authors.forename: Amal
authors.initials: A
authors.lastname: Isaiah
authors.affiliation: Albany Medical College, Albany, NY, USA.
authors.forename: Daniel
authors.initials: D
authors.lastname: Lee
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA.
authors.forename: Felicity
authors.initials: F
authors.lastname: Lenes-Voit
authors.affiliation: UT Dallas Callier Center for Communication Disorders, Dallas, TX, USA.
authors.forename: Melissa
authors.initials: M
authors.lastname: Sweeney
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA.
authors.forename: Walter
authors.initials: W
authors.lastname: Kutz
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA.
authors.forename: Brandon
authors.initials: B
authors.lastname: Isaacson
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA.
authors.forename: Peter
authors.initials: P
authors.lastname: Roland
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA. Electronic address: kenneth.lee@utsouthwestern.edu.
authors.forename: Kenneth H
authors.initials: KH
authors.lastname: Lee
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation / Q000009:adverse effects / Q000379:methods*; D003054:Cochlear Implants; D007758:Ear, Inner / Q000002:abnormalities*; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000151:congenital / Q000601:surgery*; D006801:Humans; D007223:Infant; D007431:Intraoperative Complications; D007759:Labyrinth Diseases / Q000151:congenital / Q000601:surgery*; D008279:Magnetic Resonance Imaging; D008297:Male; D015995:Prevalence; D013067:Speech Perception / Q000502:physiology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
keywords: Cochlear implants; Hearing restoration; Inner ear malformations; Speech recognition