title: Cochlear implantation in patients with neurofibromatosis type 2 and bilateral vestibular schwannoma.
abstract: OBJECTIVE
  To investigate the results of cochlear implantation in patients with neurofibromatosis Type 2 (NF2) and bilateral vestibular schwannoma.
  
  
  METHODS
  Retrospective case review.
  
  
  METHODS
  Three academic tertiary referral centers.
  
  
  METHODS
  Seven patients with NF2 and bilateral vestibular schwannoma who lost hearing in at least one ear after treatment of their tumor (surgery or radiation therapy).
  
  
  METHODS
  Cochlear implantation after treatment of their vestibular schwannoma.
  
  
  METHODS
  Postimplantation audiometric scores (pure-tone average thresholds, consonant-nucleus-consonant (CNC) words/phonemes, Central Institute for the Deaf (CID) sentences, Hearing in Noise Test (HINT) quiet/noise, and Monosyllable, Trochee, Spondee (MTS) recognition/category tests), patient satisfaction, and device use patterns.
  
  
  RESULTS
  The average age at implantation was 40 years (range, 16-57 yr). Follow-up ranged from 6 to 88 months after implantation. Three patients were implanted with residual useful hearing in the contralateral ear, whereas four patients had no hearing in the contralateral ear. Hearing loss was due to surgical excision of tumor (n=5) or gamma-knife radiotherapy (n=2). Postactivation pure-tone average thresholds in the implanted ear ranged from 30 to 55 dB (average, 32.5 dB), although speech reception testing varied considerably among subjects. Despite this variability, all patients continue to use the device on a daily basis.
  
  
  CONCLUSIONS
  In selected cases of deafness in patients with NF2 where there has been anatomic preservation of the auditory nerve after acoustic neuroma resection or radiation therapy, cochlear implantation may offer some improvement in communication skills, including the possibility of open-set speech communication in some patients. These results compare favorably to the auditory brainstem implant offering an alternative for hearing rehabilitation in patients with NF2.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, The Johns Hopkins University, Baltimore, Maryland, and Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, USA. llustig@ohns.ucsf.edu
authors.forename: Lawrence R
authors.initials: LR
authors.lastname: Lustig
authors.forename: Jennifer
authors.initials: J
authors.lastname: Yeagle
authors.forename: Colin L W
authors.initials: CL
authors.lastname: Driscoll
authors.forename: Nikolas
authors.initials: N
authors.lastname: Blevins
authors.forename: Howard
authors.initials: H
authors.lastname: Francis
authors.forename: John K
authors.initials: JK
authors.lastname: Niparko
mesh_terms: D000293:Adolescent; D000328:Adult; D001301:Audiometry, Pure-Tone; D001309:Auditory Threshold / Q000502:physiology; D019929:Cochlear Implantation / Q000379:methods*; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000534:rehabilitation*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D016518:Neurofibromatosis 2 / Q000150:complications* / Q000601:surgery; D009464:Neuroma, Acoustic / Q000150:complications* / Q000601:surgery; D016634:Radiosurgery / Q000009:adverse effects; D012189:Retrospective Studies; D016896:Treatment Outcome