INTRODUCTION: Few cases of cochlear implantation (CI) in neurofibromatosis type 2 (NF2) patients have been reported in the literature. The approaches described were translabyrinthine, retrosigmoid, and middle cranial fossa methods. OBJECTIVES: To describe a case of a NF2Âdeafened patient who underwent vestibular schwannoma resection via RLA with cochlear nerve preservation and CI through the round window during the same surgery. Resumed Report: A 36ÂyearÂold woman with severe bilateral hearing loss from NF2 underwent a vestibular schwannoma resection and simultaneous CI. Functional assessment of the cochlear nerve was performed by electrical promontory stimulation. Complete tumor removal was accomplishment via RLA with anatomic, functional cochlear, and facial nerve preservation. A cochlear electrode array was partially inserted via the round window. Sound field hearing threshold improvements were achieved. The mean tonal threshold was 46.2 dB HL. The patient can only detect environmental sounds and human voice, and cannot discriminate vowels, words, or sentences after 2 years of follow-up. CONCLUSION: Cochlear implantation is a feasible auditory restoration option in NF2 when cochlear anatomic and functional nerve preservation are achieved. RLA is adequate for this purpose and features an option for hearing preservation in NF2 patients.
