title: Surgical management of vestibular schwannomas and hearing rehabilitation in neurofibromatosis type 2.
abstract: OBJECTIVE
  To report our approach to the surgical management of vestibular schwannomas (VSs) and hearing rehabilitation in neurofibromatosis Type 2 (NF2).
  
  
  METHODS
  Retrospective cohort study.
  
  
  METHODS
  Tertiary referral NF2 unit.
  
  
  METHODS
  Between 1981 and 2011, seventy-five patients were managed in our NF2 unit, of which, 58 patients are under current review.
  
  
  METHODS
  Patients who underwent VS excision were evaluated for tumor size, surgical approach, and outcomes of hearing and facial nerve function. All current patients were evaluated for NF2 mutation, hearing, and auditory implantation outcomes.
  
  
  RESULTS
  Forty-four patients underwent resection of 50 VS in our unit, of which, 14% had facial neuroma excision and reinnervation during the same operation. At 12 months after surgery, facial nerve outcomes were House-Brackmann (HB) 1 in 33%, HB2 in 21%, and HB3 in 30%. Total VS resection was achieved in 78% of patients using a translabyrinthine approach. Seventy-two percent of the current patients have American Association of Otolaryngology-Head and Neck Surgery class A to C hearing (maximum speech discrimination score over 50%) in the better hearing ear, and a further 14% are full-time users of cochlear implants or auditory brainstem implants. The remaining patients have been assessed for auditory implantation.
  
  
  CONCLUSIONS
  By following a policy of treating VS in NF2 patients where tumor growth is observed, complete tumor resection can be achieved through a translabyrinthine approach while achieving comparable facial nerve outcomes to published series. We advocate proactive hearing rehabilitation in all patients with timely assessment for auditory implantation to maintain quality of life.
authors.affiliation: Department of Otolaryngology, Cambridge University Hospitals NHS Trust, Cambridge, UK.
authors.forename: James R
authors.initials: JR
authors.lastname: Tysome
authors.forename: Robert
authors.initials: R
authors.lastname: Macfarlane
authors.forename: Juliette
authors.initials: J
authors.lastname: Durie-Gair
authors.forename: Neil
authors.initials: N
authors.lastname: Donnelly
authors.forename: Richard
authors.initials: R
authors.lastname: Mannion
authors.forename: Richard
authors.initials: R
authors.lastname: Knight
authors.forename: Frances
authors.initials: F
authors.lastname: Harris
authors.forename: Zebunnisa H
authors.initials: ZH
authors.lastname: Vanat
authors.forename: Yu Chen
authors.initials: YC
authors.lastname: Tam
authors.forename: Katherine
authors.initials: K
authors.lastname: Burton
authors.forename: Anke
authors.initials: A
authors.lastname: Hensiek
authors.forename: F Lucy
authors.initials: FL
authors.lastname: Raymond
authors.forename: David A
authors.initials: DA
authors.lastname: Moffat
authors.forename: Patrick R
authors.initials: PR
authors.lastname: Axon
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001301:Audiometry, Pure-Tone; D038262:Auditory Brain Stem Implants; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation; D003054:Cochlear Implants; D015331:Cohort Studies; D005154:Facial Nerve / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D016515:Genes, Neurofibromatosis 2; D005838:Genotype; D006309:Hearing / Q000502:physiology; D034381:Hearing Loss / Q000534:rehabilitation*; D006320:Hearing Tests; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000502:physiology; D016518:Neurofibromatosis 2 / Q000150:complications / Q000534:rehabilitation* / Q000601:surgery*; D009464:Neuroma, Acoustic / Q000209:etiology / Q000534:rehabilitation* / Q000601:surgery*; D013506:Otologic Surgical Procedures / Q000009:adverse effects / Q000379:methods*; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult