Comprehensive management of bilateral acoustic neuromas. Current perspectives

Bilateral acoustic neuromas are pathognomonic for neurofibromatosis 2. Patients with neurofibromatosis 2 present complex and challenging management problems, because growth or surgical removal of the acoustic neuroma may result in total hearing loss. Early diagnosis with gadolinium-enhanced magnetic resonance imaging and refinements in hearing preservation surgery have improved our ability to prevent total hearing loss. For patients with larger tumors or no useful hearing, the auditory brain-stem implant allows restoration of some auditory function when the tumor is removed. We describe our management strategy for patients with bilateral acoustic neuromas, and present case reports to show their diversity. We also discuss the newly identified neurofibromatosis 2 tumor suppressor gene.