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.

Introduction  The exclusive use of a cochlear implant (CI) in one ear allows patients to effectively hear speech in a quiet environment. However, in environments with competing noise, the processing of multiple sounds becomes complex. In an attempt to promote binaural hearing in a noninvasive manner, the use of a hearing aid in the nonimplanted ear is suggested for patients with a unilateral CI. Aims  To identify the prevalence of hearing aid use in the contralateral ear in adults who already have a CI; to determine the reasons why some patients do not use contralateral hearing aids (CHAs); and to analyze the effects of residual hearing in CHA users. Materials and Methods  This is a clinical study in 82 adult patients with CI implants who responded to a questionnaire designed to determine current use of CHA. Results  In our patient sample, 70 CHA nonusers were identified. The prevalence of CHA users was determined to be 12% with a 95% confidence interval of 11 to 13%. About 58.2% of the CHA nonusers reported a lack of noticeable benefit even after wearing hearing aids, and 23.6% reported not having received the option to use a CHA. CHA users had a pure tone average of 107-dB hearing level, whereas CHA nonusers had a pure tone average of 117-dB hearing level. Conclusion  The prevalence of the use of a CHA is low in our study. We attribute the low use of a CHA to either a lack of residual hearing or to a lack of benefit from the amplification.

Neurofibromatose 2 (NF2) é uma doença autossômica dominante que, invariavelmente, cursa com surdez. Restauração auditiva pode ser realizada por meio do implante coclear (IC) ou implante auditivo de tronco encefálico (ABI). OBJETIVO: Avaliar resultados auditivos de IC e ABI em pacientes com NF2 e revisão de literatura. MÉTODO: Foram avaliados prospectivamente quatro pacientes com NF2 submetidos à exérese tumoral e implantação de IC ou ABI ipsilateral simultâneo, dependendo se o nervo coclear foi preservado ou não cirurgicamente. São descritos resultados auditivos em longo prazo (12-48 meses). RESULTADOS: Todos pacientes implantados obtiveram percepção sonora, com melhora dos limiares tonais. A paciente com IC não discrimina vogais nem sentenças. Os três pacientes com ABI apresentam discriminação média de vogais de 70% e teste 4-choice 86%. Um paciente não discrimina sentenças. Os outros dois discriminam 100% de sentenças em formato fechado e em formato aberto 10% e 20%. CONCLUSÃO: A escolha do tipo de implante usado na restauração auditiva de portadores NF2 dependerá da preservação anatômica e funcional do nervo coclear na cirurgia de ressecção tumoral. Embora nossa experiência mostre o contrário, a literatura mostra que, se essa condição for alcançada, IC fornecerá melhores resultados auditivos. Caso contrário, ABI trará maiores benefícios.

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. CONCLUSION: 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.