title: Neurofibromatosis 2: hearing restoration options.
abstract: Neurofibromatosis 2 (NF2) is an autosomal dominant disease in which hearing loss is predominant. Auditory restoration is possible using cochlear implants (CI) or auditory brainstem implant (ABI).
  
  
  OBJECTIVE
  To assess the auditory results of CI and ABI in NF2 patients and review the literature.
  
  
  METHODS
  Four NF2 patients were prospectively evaluated. They were submitted to tumor resection followed by ipsilateral CI or ABI depending on cochlear nerve preservation. Long term auditory results were described for CI (12 months) and ABI (48 months).
  
  
  RESULTS
  All patients achieved auditory perception improvements in their hearing thresholds. The CI patient does not recognize vowels or sentences. The 3 ABI patients discriminate 70% of vowels and 86% in the 4-choice test. One of them does not recognize sentences. The other two recognize 100% of closed sentences and 10% and 20% of open sentences.
  
  
  CONCLUSIONS
  The choice of implant type to restore hearing to NF2 patients will relay on anatomical and functional cochlear nerve preservation during tumor resection surgery. Although our experience was different, the literature shows that if this condition is achieved, CI will offer better auditory results. If not, ABI is recommended.
authors.affiliation: Department of Otorhinolaryngology - Medical School of the University of São Paulo, SP, Brazil. 　vitatimonteiro@ig.com.br
authors.forename: Tatiana Alves
authors.initials: TA
authors.lastname: Monteiro
authors.forename: Maria Valeria Schmidt
authors.initials: MV
authors.lastname: Goffi-Gomez
authors.forename: Robinson Koji
authors.initials: RK
authors.lastname: Tsuji
authors.forename: Marcos Queiroz Telas
authors.initials: MQ
authors.lastname: Gomes
authors.forename: Rubens Vuono
authors.initials: RV
authors.lastname: Brito Neto
authors.forename: Ricardo Ferreira
authors.initials: RF
authors.lastname: Bento
mesh_terms: D000328:Adult; D038262:Auditory Brain Stem Implants; D019929:Cochlear Implantation; D005260:Female; D005500:Follow-Up Studies; D006312:Hearing Loss, Bilateral / Q000209:etiology / Q000601:surgery*; D006801:Humans; D008297:Male; D016518:Neurofibromatosis 2 / Q000150:complications*; D011446:Prospective Studies; D016896:Treatment Outcome