Int Arch Otorhinolaryngol 2013; 17(03): 351-355
DOI: 10.7162/S1809-977720130003000018
Case Report
Thieme Publicações Ltda Rio de Janeiro, Brazil

Retrolabyrinthine approach for cochlear nerve preservation in neurofibromatosis type 2 and simultaneous cochlear implantation

Ricardo Ferreira Bento
1  Otolaryngologist, PhD. Professor and Chairman. Department of Otolaryngology, University of São Paulo School of Medicine, São Paulo, Brazil.
,
Tatiana Alves Monteiro
2  Otolaryngologist. Department of Otolaryngology, University of São Paulo School of Medicine, São Paulo, Brazil.
,
Aline Gomes Bittencourt
3  Otolaryngologist, PhD student. Neurotology Fellow. Division of Otorhinolaryngology, University of São Paulo Medical School, São Paulo, Brazil.
,
Maria Valeria Schmidt Goffi-Gomez
4  Audiologist, PhD in Human Communication Disorders (Speech Pathology). Department of Otolaryngology, University of São Paulo School of Medicine, São Paulo, Brazil.
,
Rubens de Brito
5  Otolaryngologist, PhD. Associate Professor. Department of Otolaryngology, University of São Paulo School of Medicine, São Paulo, Brazil.
› Author Affiliations
Further Information

Publication History

18 March 2013

18 April 2013

Publication Date:
21 January 2014 (online)

  

Summary

Introduction: 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.

Objectives: 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.

Conclusion: 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.