Surgery for neurofibromatosis 2.

PURPOSE OF REVIEW: This study reviews the latest literature relating to the surgical treatment of otologic manifestations in patients with neurofibromatosis 2 (NF2). The emphasis is on vestibular and other schwannomas. We review surgical approaches, including hearing preservation and nonhearing preservation surgery, as well as outcomes, including hearing and facial nerve function.

RECENT FINDINGS: Vestibular schwannomas in NF2 are difficult to manage because they are bilateral and may be aggressive. Depending on hearing status, tumor size and the presence or absence of compressive symptoms, these tumors can be managed by observation, radiotherapy or surgery. The goal is to maximize the years of useful hearing. Surgery may attempt to preserve hearing or aim for complete tumor resection and preservation of facial nerve function.

SUMMARY: The natural history of vestibular schwannomas and other tumors in patients with NF2 is difficult to predict. The decision between observation and either medical or surgical intervention, as well as the choice of surgical procedure, depend on patient factors and preferences and on the experience of the treating center.