title: Auditory Brainstem Implantation in Neurofibromatosis Type 2: Experience From the Manchester Programme.
abstract: To describe the experience of auditory brainstem implantation (ABI) in patients with Neurofibromatosis type 2 (NF2).
  
  
  
  Retrospective case review.
  
  
  
  Tertiary referral centre.
  
  
  
  Implanted with a Cochlear ABI22 or ABI24M between 1994 and 2009 because of NF2 disease.
  
  
  
  Rehabilitative.
  
  
  
  Surgical complication rate; audiological outcomes.
  
  
  
  There were 50 primary ABI insertions in 49 patients, including 16 inserted at the time of first side tumor removal as a sleeper, and two revision repositionings which failed to improve outcome. Postoperatively three patients had cerebrospinal fluid leaks which did not require reoperation, one patient had meningitis, and eleven patients suffered either temporary or permanent lower cranial nerve dysfunction. Twenty-nine patients became full time users; a further 12 patients became non-users. Three patients died while their device was inactive. Five patients retain serviceable contralateral hearing. Audiological open set testing of users showed means of: environmental sounds discrimination 51%; phoneme discrimination: with ABI alone 22%/lip reading (LR) 45%/ABI with LR 65%; sentence testing: with ABI alone 13%/LR 19%/ABI with LR 54%.
  
  
  
  The majority of patients with NF2 implanted with an ABI find the device a useful aid to communication in conjunction with LR and in recognizing common environmental sounds. A small proportion gain open set discrimination. Almost a third of patients may end up as non-users. There is probably an increased risk of postoperative lower cranial nerve dysfunction so careful preoperative assessment is advised.
authors.affiliation: *Department of Otolarygology †Department of Audiology ‡Manchester Centre for Genomic Medicine, Institute of Human Development, University of Manchester; and Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester §Department of Neurosurgery, Salford Royal Hospital NHS Foundation Trust, Salford ||Institute of Cancer ¶Institute of Cardiovascular Sciences, University of Manchester, Manchester Academic Health Science Centre #Business School, Manchester Metropolitan University, U.K.
authors.forename: Richard Thomas
authors.initials: RT
authors.lastname: Ramsden
authors.forename: Simon Richard Mackenzie
authors.initials: SR
authors.lastname: Freeman
authors.forename: Simon Kingsley Wickham
authors.initials: SK
authors.lastname: Lloyd
authors.forename: Andrew Thomas
authors.initials: AT
authors.lastname: King
authors.forename: Xin
authors.initials: X
authors.lastname: Shi
authors.forename: Charlotte Lucy
authors.initials: CL
authors.lastname: Ward
authors.forename: Susan Mary
authors.initials: SM
authors.lastname: Huson
authors.forename: Deborah Jane
authors.initials: DJ
authors.lastname: Mawman
authors.forename: Martin Paul
authors.initials: MP
authors.lastname: O'Driscoll
authors.forename: Dafydd Gareth
authors.initials: DG
authors.lastname: Evans
authors.forename: Scott Alexander
authors.initials: SA
authors.lastname: Rutherford
mesh_terms: D000328:Adult; D038263:Auditory Brain Stem Implantation; D038262:Auditory Brain Stem Implants; D001307:Auditory Perception; D003638:Deafness / Q000209:etiology / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D016518:Neurofibromatosis 2 / Q000150:complications* / Q000601:surgery; D009464:Neuroma, Acoustic / Q000150:complications* / Q000601:surgery; D012189:Retrospective Studies; D016896:Treatment Outcome