Neurofibromatosis type II(NF2) is an autosomal dominant disorder that clinically characterized by the presence of bilateral vestibular schwannomas. NF2 is benign tumors that arise from the eighth cranial nerve. The initial presenting symptoms of unilateral vestibular schwannomas, including hearing loss, have been well documented in the literature. The exclusive goal in management of NF2 patients is hearing preservation. Profound hearing loss is a serious complication of NF2. In this case, we report a patient with NF2 associated with unilateral hearing loss. A 15-year-old girl, that previous unknown NF2 was detected on brain MRI to Multiple cranial nerve schwannoma. It was interesting fact that presented with gradual otologic symptoms as first. It is within this context that otologic findings in this patient of subjects will be discussed.