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Neurofibromatosis type II (NF2) is a genetic disease and is transmitted as an autosomal dominant trait. NF2 is characterized by bilateral acoustic neuroma with multiple tumors of central nervous system. Progressive hearing loss is a significant problem in patients with NF2, and hearing preservation is an important factor for choice of treatment plan, especially in a NF2 patient with only one hearing ear. We report an experience of preservation of hearing in a NF2 patient with only one hearing ear through decompression of the internal auditory canal via middle cranial fossa approach. (Korean J Otolaryngol 2006;49:439-42)