title: Retrolabyrinthine approach for cochlear nerve preservation in neurofibromatosis type 2 and simultaneous cochlear implantation.
abstract: BACKGROUND
  Few cases of cochlear implantation (CI) in neurofibromatosis type 2 (NF2) patients had been reported in the literature. The approaches described were translabyrinthine, retrosigmoid or middle cranial fossa.
  
  
  OBJECTIVE
  To describe a case of a NF2- deafened-patient who underwent to vestibular schwannoma resection via RLA with cochlear nerve preservation and CI through the round window, at the same surgical time. Resumed Report: A 36-year-old woman with severe bilateral hearing loss due to NF2 was submitted to vestibular schwannoma resection and simultaneous CI. Functional assessment of cochlear nerve was performed by electrical promontory stimulation. Complete tumor removal was accomplishment via RLA with anatomic and functional cochlear and facial nerve preservation. Cochlear electrode array was partially inserted via round window. Sound field hearing threshold improvement was achieved. Mean tonal threshold was 46.2 dB HL. The patient could only detect environmental sounds and human voice but cannot discriminate vowels, words nor do sentences at 2 years of follow-up.
  
  
  CONCLUSIONS
  Cochlear implantation is a feasible auditory restoration option in NF2 when cochlear anatomic and functional nerve preservation is achieved. The RLA is adequate for this purpose and features as an option for hearing preservation in NF2 patients.
authors.affiliation: Otolaryngologist, PhD. Professor and Chairman. Department of Otolaryngology, University of São Paulo School of Medicine, São Paulo, Brazil.
authors.forename: Ricardo Ferreira
authors.initials: RF
authors.lastname: Bento
authors.affiliation: Otolaryngologist. Department of Otolaryngology, University of São Paulo School of Medicine, São Paulo, Brazil.
authors.forename: Tatiana Alves
authors.initials: TA
authors.lastname: Monteiro
authors.affiliation: Otolaryngologist, PhD student. Neurotology Fellow. Division of Otorhinolaryngology, University of São Paulo Medical School, São Paulo, Brazil.
authors.forename: Aline Gomes
authors.initials: AG
authors.lastname: Bittencourt
authors.affiliation: Audiologist, PhD in Human Communication Disorders (Speech Pathology). Department of Otolaryngology, University of São Paulo School of Medicine, São Paulo, Brazil.
authors.forename: Maria Valeria Schmidt
authors.initials: MV
authors.lastname: Goffi-Gomez
authors.affiliation: Otolaryngologist, PhD. Associate Professor. Department of Otolaryngology, University of São Paulo School of Medicine, São Paulo, Brazil.
authors.forename: Rubens
authors.initials: R
authors.lastname: de Brito
keywords: Cochlear Implantation; Hearing Loss; Neurofibromatosis 2