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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.

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.

A cirurgia de implante coclear (IC) é método seguro e factível de restaurar audição em pacientes com surdez grave a profunda. OBJETIVO: Descrever complicações do IC. MATERIAL E MÉTODOS: Dados de 591 IC com tempo de seguimento de pelo menos 1 ano foram retrospectivamente analisados. Foram excluídos 41 pacientes por dados incompletos, acesso via fossa media ou perda de seguimento. RESULTADOS: Dos 550 IC analisados, 341 foram realizados em crianças e adolescentes e 209 em adultos. O tempo médio de surdez foi de 6,3 ± 6,7 anos para pré-linguais e 12,1 ± 11,6 anos para os pós-linguais. O tempo médio de seguimento foi 3,9 ± 2,8 anos. Complicações maiores ocorreram em 8,9% casos e menores em 7,8%. Problemas durante a inserção do feixe de eletrodos (3,8%) foi a complicação maior mais frequente, seguido pela deiscência de ferida operatória (1,4%). Paralisia facial temporária (2,2%), lesão muro facial (2,2%) e lesão de membrana timpânica (1,8%) foram as complicações menores mais frequentes. Nenhum óbito foi observado. CONCLUSÃO: Complicações cirúrgicas tiveram baixa ocorrência, sendo a maioria solucionada com sucesso. Esses resultados confirmam que o IC é uma cirurgia segura e a maioria das complicações cirúrgicas pode ser manejada com medidas conservadoras ou intervenções menores.Cochlear implantation is a safe and reliable method for auditory restoration in patients with severe to profound hearing loss. OBJECTIVE:To describe the surgical complications of cochlear implantation. MATERIALS AND METHODS: Information from 591 consecutive multichannel cochlear implant surgeries were retrospectively analyzed. All patients were followed-up for at least one year. Forty-one patients were excluded because of missing data, follow-up loss or middle fossa approach. RESULTS: Of 550 cochlear implantation analyzed, 341 were performed in children or adolescents, and 209 in adults. The mean hearing loss time was 6.3 ± 6.7 years for prelingual loss and 12.1 ± 11.6 years for postlingual. Mean follow-up was 3.9 ± 2.8 years. Major complications occurred in 8.9% and minor in 7.8%. Problems during electrode insertion (3.8%) were the most frequent major complication followed by flap dehiscence (1.4%). Temporary facial palsy (2.2%), canal-wall lesion (2.2%) and tympanic membrane lesion (1.8%) were the more frequent minor complications. No death occurred. CONCLUSION: There was a low rate of surgical complications, most of them been successfully managed. These results confirm that cochlear implant is a safe surgery and most surgical complications can be managed with conservative measures or minimal intervention.

The productive work between otolaryngologists and neurosurgeons has resulted in the emergence of endoscopic endonasal skull base surgery. The goal of the present study is to describe the endoscopic anatomy of the endonasal approach to the sellar region and planum sphenoidale, highlighting the key points of the surgical approach and the neurovascular landmarks. METHOD: Descriptive study of the endoscopic endonasal dissection of 9 fresh cadavers with exposure of the anatomic structures. RESULTS: The endoscopic endonasal ethmoidectomy and sphenoidotomy allows an expanded access to the sellar area and planum sphenoidale. The surface anatomy of the sphenoid sinus is easily identifiable and provides safe landmarks, guiding the intracranial dissection. CONCLUSION: The endoscopic endonasal approach to the skull base by the ENT and neurosurgeon is feasible, but it requires adequate anatomical knowledge and endoscopic skills for its realization, which can be obtained by practicing in cadavers.