title: Cochlear implantation in Pendred syndrome and non-syndromic enlarged vestibular aqueduct - clinical challenges, surgical results, and complications.
abstract: OBJECTIVE
  To explore specific clinical issues, surgical results, and complications of 80 cochlear implantations (CI) in 55 patients with Pendred syndrome (PS) or non-syndromic enlarged vestibular aqueduct (NSEVA).
  
  
  BACKGROUND
  Previous studies have focused either on unselected case series or on populations with mixed cochlear malformations. PS/NSEVA accounts for up to 10% of congenital SNHL, rendering this a large group of cochlear implant candidates. The abnormal inner ear anatomy of these patients may be associated with a lower surgical success rate and a higher rate of complications.
  
  
  METHODS
  Retrospective review of patients' medical records and CT/MRI.
  
  
  METHODS
  Tertiary referral center.
  
  
  METHODS
  The medical records and CT/MRI images of 55 PS/NSEVA patients receiving 80 cochlear implantations from 1982-2014 were reviewed. Demographic data, surgical results, intra-operative incidents, and post-operative complications were retrieved.
  
  
  RESULTS
  Complications occurred in 36% of implantations; 5% hereof major complications. Gushing/oozing from the cochleostoma occurred in 10% of implantations and was related to transient, but not prolonged post-operative vertigo.
  
  
  CONCLUSIONS
  Intra-operative risks of gushing/oozing and post-operative vertigo are the primary clinical issues in PS/NSEVA patients regarding CI. Nonetheless, the surgical success rate is high and the major complication rate is low; similar to studies of unselected series of CI recipients.
authors.affiliation: a Department of Otorhinolaryngology, Head & Neck Surgery, and Audiology , East Danish Center for Cochlear Implantation, Copenhagen University Hospital Rigshospitalet/Gentofte Hospital , Copenhagen , Denmark ;
authors.forename: Kristianna
authors.initials: K
authors.lastname: Mey
authors.affiliation: a Department of Otorhinolaryngology, Head & Neck Surgery, and Audiology , East Danish Center for Cochlear Implantation, Copenhagen University Hospital Rigshospitalet/Gentofte Hospital , Copenhagen , Denmark ;
authors.forename: Michael
authors.initials: M
authors.lastname: Bille
authors.affiliation: a Department of Otorhinolaryngology, Head & Neck Surgery, and Audiology , East Danish Center for Cochlear Implantation, Copenhagen University Hospital Rigshospitalet/Gentofte Hospital , Copenhagen , Denmark ;|b Faculty of Health and Medical Sciences , University of Copenhagen , Copenhagen , Denmark.
authors.forename: Per
authors.initials: P
authors.lastname: Cayé-Thomasen
mesh_terms: D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation / Q000706:statistics & numerical data*; D003718:Denmark / Q000453:epidemiology; D005260:Female; D006044:Goiter, Nodular / Q000150:complications / Q000601:surgery*; D006319:Hearing Loss, Sensorineural / Q000150:complications / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology; D012189:Retrospective Studies; D014723:Vestibular Aqueduct / Q000002:abnormalities* / Q000601:surgery
keywords: Cochlear implantation; Pendred syndrome; enlarged vestibular aqueduct; gusher; malformations; surgical complications