title: English consensus protocol evaluating candidacy for auditory brainstem and cochlear implantation in neurofibromatosis type 2.
abstract: OBJECTIVE
  Hearing loss resulting from bilateral vestibular schwannomas (VSs) has a significant effect on the quality of life of patients with neurofibromatosis Type 2 (NF2). A national consensus protocol was produced in England as a guide for cochlear implantation (CI) and auditory brainstem implantation (ABI) in these patients.
  
  
  METHODS
  Consensus statement.
  
  
  METHODS
  English NF2 Service.
  
  
  METHODS
  Clinicians from all 4 lead NF2 units in England.
  
  
  METHODS
  A protocol for the assessment, insertion and rehabilitation of CI and ABI in NF2 patients.
  
  
  RESULTS
  Patients should undergo more detailed hearing assessment once their maximum aided speech discrimination score falls below 50% in the better hearing ear. Bamford-Kowal-Bench sentence testing scores below 50% should trigger assessment for auditory implantation, as recommended by the National Institute for Clinical Excellence guidelines on CI. Where this occurs in patients with bilateral stable VS or a unilateral stable VS where the contralateral cochlear nerve was lost at previous surgery, CI should be considered. Where VS surgery is planned, CI should be considered where cochlear nerve preservation is thought possible, otherwise an ABI should be considered. Intraoperative testing using electrically evoked auditory brainstem responses or cochlear nerve action potentials may be used to determine whether a CI or ABI is inserted.
  
  
  CONCLUSIONS
  The NF2 centers in England agreed on this protocol. Multisite, prospective assessments of standardized protocols for auditory implantation in NF2 provide an essential model for evaluating candidacy and outcomes in this challenging patient population.
authors.affiliation: *Department of Otolaryngology, Cambridge University Hospitals NHS Foundation Trust, Cambridge; †Department of Genetics, Manchester Royal Infirmary, Manchester; ‡Department of Neurology, and §Department of Otolaryngology, Guy's and St. Thomas' NHS Foundation Trust, London; ∥Department of Otolaryngology, Manchester Royal Infirmary, Manchester; ¶Department of Otolaryngology, National Hospital for Neurology and Neurosurgery, London; #Department of Neurology, John Radcliffe Hospital, Oxford; ** Emmeline Centre for Hearing Implants, Cambridge University Hospitals NHS Foundation Trust, Cambridge; ††Department of Neurosurgery, John Radcliffe Hospital, Oxford; ‡‡Department of Neurosurgery, Manchester Royal Infirmary, Manchester; §§Department of Audiology, and ∥∥Department of Neurosurgery, Cambridge University Hospitals NHS Foundation Trust, Cambridge; ¶¶Department of Audiology, Manchester Royal Infirmary, Manchester; ##Department of Otolaryngology, John Radcliffe Hospital, Oxford; ***Department of Otolaryngology, Royal National Throat Nose and Ear Hospital; and †††Department of Neurosurgery, King's College Hospital NHS Trust, London, U.K.
authors.forename: James R
authors.initials: JR
authors.lastname: Tysome
authors.forename: Patrick R
authors.initials: PR
authors.lastname: Axon
authors.forename: Neil P
authors.initials: NP
authors.lastname: Donnelly
authors.forename: Dafydd Gareth
authors.initials: DG
authors.lastname: Evans
authors.forename: Rosalie E
authors.initials: RE
authors.lastname: Ferner
authors.forename: Alec F Fitzgerald
authors.initials: AF
authors.lastname: O'Connor
authors.forename: Simon R
authors.initials: SR
authors.lastname: Freeman
authors.forename: Michael
authors.initials: M
authors.lastname: Gleeson
authors.forename: Dorothy
authors.initials: D
authors.lastname: Halliday
authors.forename: Frances
authors.initials: F
authors.lastname: Harris
authors.forename: Dan
authors.initials: D
authors.lastname: Jiang
authors.forename: Richard
authors.initials: R
authors.lastname: Kerr
authors.forename: Andrew
authors.initials: A
authors.lastname: King
authors.forename: Richard D
authors.initials: RD
authors.lastname: Knight
authors.forename: Simon K
authors.initials: SK
authors.lastname: Lloyd
authors.forename: Robert
authors.initials: R
authors.lastname: Macfarlane
authors.forename: Richard
authors.initials: R
authors.lastname: Mannion
authors.forename: Deborah
authors.initials: D
authors.lastname: Mawman
authors.forename: Martin
authors.initials: M
authors.lastname: O'Driscoll
authors.forename: Allyson
authors.initials: A
authors.lastname: Parry
authors.forename: James
authors.initials: J
authors.lastname: Ramsden
authors.forename: Richard
authors.initials: R
authors.lastname: Ramsden
authors.forename: Scott A
authors.initials: SA
authors.lastname: Rutherford
authors.forename: Shakeel R
authors.initials: SR
authors.lastname: Saeed
authors.forename: Nick
authors.initials: N
authors.lastname: Thomas
authors.forename: Zebunnisa H
authors.initials: ZH
authors.lastname: Vanat
mesh_terms: D038263:Auditory Brain Stem Implantation / Q000379:methods*; D038262:Auditory Brain Stem Implants; D002985:Clinical Protocols; D003051:Cochlea / Q000601:surgery*; D019929:Cochlear Implantation / Q000379:methods*; D003054:Cochlear Implants; D032921:Consensus; D004739:England; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D006319:Hearing Loss, Sensorineural / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D016518:Neurofibromatosis 2 / Q000503:physiopathology / Q000601:surgery*; D013067:Speech Perception / Q000502:physiology