title: Surgical techniques and outcomes of cochlear implantation in patients with radiographic findings consistent with X-linked deafness.
abstract: OBJECTIVE
  X-linked deafness is a potential etiology of sensorineural hearing loss characterized by bulbous dilatation of the fundus of the internal auditory canal (IAC) and the absence of the bony plates separating the basal turn of the cochlea and IAC. These malformations predispose patients to IAC insertion during cochlear implantation (CI). Our objective is to describe the surgical technique, audiometric and speech performance outcomes in a group of patients with this unique cochlear malformation.
  
  
  METHODS
  A retrospective chart review was performed of all patients at a tertiary care facility who underwent CI between January 2006 and July 2011.
  
  
  RESULTS
  A total of five patients were identified with radiographic findings characteristic of X-linked deafness, specifically a deficient modiolous, absent lamina cribrosa and bulbous IAC. A modified cochleostomy was utilized to ensure electrode insertion within the scala tympani avoiding the IAC. In each case, fluoroscopy was utilized to visual electrode progression during insertion and complete insertion was accomplished. All patients demonstrated improved speech performance following implantation.
  
  
  CONCLUSIONS
  Utilizing the techniques described, patients with these unique radiographic findings consistent with X-linked deafness may successfully undergo CI with excellent potential for auditory rehabilitation.
authors.affiliation: Departments of Otolaryngology-Head and Neck Surgery and Neurosurgery, Louisiana State University, Shreveport, LA, USA. Electronic address: mcoset@lsuhsc.edu.
authors.forename: Maura K
authors.initials: MK
authors.lastname: Cosetti
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, New York University School of Medicine, New York, NY, USA.
authors.forename: David R
authors.initials: DR
authors.lastname: Friedmann
authors.affiliation: Division of Otolaryngology, Beth Israel Medical Center, Harvard Medical School, Boston, MA, USA.
authors.forename: Selena E
authors.initials: SE
authors.lastname: Heman-Ackah
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Shaare Zedek Medical Center, Hebrew University Medical School, Jerusalem, Israel.
authors.forename: Ronen
authors.initials: R
authors.lastname: Perez
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, New York University School of Medicine, New York, NY, USA.
authors.forename: Susan B
authors.initials: SB
authors.lastname: Waltzman
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, New York University School of Medicine, New York, NY, USA.
authors.forename: J Thomas
authors.initials: JT
authors.lastname: Roland
mesh_terms: D001299:Audiometry; D002648:Child; D002675:Child, Preschool; D003051:Cochlea / Q000002:abnormalities*; D019929:Cochlear Implantation / Q000379:methods*; D018450:Disease Progression; D007758:Ear, Inner / Q000601:surgery*; D004567:Electrodes, Implanted / Q000009:adverse effects; D005260:Female; D005471:Fluoroscopy; D006319:Hearing Loss, Sensorineural / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D013060:Speech; D013067:Speech Perception; D062606:Tertiary Care Centers; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
keywords: Cochlear implantation; Cochlear malformation