Abstract: Objective To investigate the diagnosis and treatment of neurofibromatosis type2(NF2). Methods The clinical manifestations, magnetic resonance imaging(MRI) findings, and treatment of 4 patients with surgically and pathologically-confirmed NF2-associated bilateral acoustic neufibroma were retrospectively analyzed,along with literature review. Results Bilateral acoustic neuroma was the mainly clinical manifestation in these four NF2 patients,although unilateral acoustic neuroma and schwannoma have also been described in literature.Microsurgery remains the mainstream treatment for NF2. Conclusions NF2 has unique clinical manifestations and MRI findings. It is mainly diagnosed by MRI and treated by surgery.However, the timing of surgery should be individualized and pay attention to the protection of the hearing at one side.

Key words: neurofibromatosis type 2, MRI, treatment experience