title: Auditory rehabilitation of patients with neurofibromatosis Type 2 by using cochlear implants.
abstract: OBJECTIVE
  The aim of this study was to determine whether patients with neurofibromatosis Type 2 (NF2) who have intact ipsilateral cochlear nerves can have open-set speech discrimination following cochlear implantation.
  
  
  METHODS
  Records of 7 patients with documented NF2 were reviewed to determine speech discrimination outcomes following cochlear implantation. Outcomes were measured using consonant-nucleus-consonant words and phonemes; Hearing in Noise Test sentences in quiet; and City University of New York sentences in quiet and in noise.
  
  
  RESULTS
  Preoperatively, none of the patients had open-set speech discrimination. Five of the 7 patients had previously undergone excision of ipsilateral vestibular schwannoma (VS). One of the patients who received a cochlear implant had received radiation therapy for ipsilateral VS, and another was undergoing observation for a small ipsilateral VS. Following cochlear implantation, 4 of 7 patients with NF2 had open-set speech discrimination following cochlear implantation during extended follow-up (15-120 months). Two of the 3 patients without open-set speech understanding had a prolonged period between ipsilateral VS resection and cochlear implantation (120 and 132 months), and had cochlear ossification at the time of implantation. The other patient without open-set speech understanding had good contralateral hearing at the time of cochlear implantation. Despite these findings, 6 of the 7 patients were daily users of their cochlear implants, and the seventh is an occasional user, indicating that all of the patients subjectively gained some benefit from their implants.
  
  
  CONCLUSIONS
  Cochlear implantation can provide long-term auditory rehabilitation, with open-set speech discrimination for patients with NF2 who have intact ipsilateral cochlear nerves. Factors that can affect implant performance include the following: 1) a prolonged time between VS resection and implantation; and 2) cochlear ossification.
authors.affiliation: Department of Otolaryngology, New York University School of Medicine, New York, New York 10016, USA. pamela.roehm@nyumc.org
authors.forename: Pamela C
authors.initials: PC
authors.lastname: Roehm
authors.forename: Jon
authors.initials: J
authors.lastname: Mallen-St Clair
authors.forename: Daniel
authors.initials: D
authors.lastname: Jethanamest
authors.forename: John G
authors.initials: JG
authors.lastname: Golfinos
authors.forename: William
authors.initials: W
authors.lastname: Shapiro
authors.forename: Susan
authors.initials: S
authors.lastname: Waltzman
authors.forename: J Thomas
authors.initials: JT
authors.lastname: Roland
mesh_terms: D000328:Adult; D001299:Audiometry; D019929:Cochlear Implantation; D003054:Cochlear Implants; D003056:Cochlear Nerve / Q000601:surgery; D005260:Female; D006312:Hearing Loss, Bilateral / Q000503:physiopathology / Q000534:rehabilitation* / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D016518:Neurofibromatosis 2 / Q000503:physiopathology / Q000534:rehabilitation* / Q000601:surgery; D009464:Neuroma, Acoustic / Q000601:surgery; D013067:Speech Perception / Q000502:physiology*; D016896:Treatment Outcome