Medical therapy for vestibular schwannoma

Vestibular schwannomas (VS) symptomatically affect between 1 in 500 and 1 in 1000 people in their lifetime. Most VS cases are sporadic, with no associated tumors or family history, but ∼5% occur in the context of NF2-related schwannomatosis (formerly neurofibromatosis 2). The mainstays of the current management strategy are observation, surgery, and radiosurgery/radiotherapy. However, in the last 12 years, bevacizumab, an antivascular endothelial growth factor (VEGF) antibody, has been utilized for patients with NF2 and rapidly growing tumors or deteriorating hearing. Other drugs have been studied in clinical trials but none have produced clinically important improvements in tumor size or hearing function. While morbidity and mortality rates related to VS treatment have improved in recent decades, significant improvements could still be made, in particular with regard to long-term facial nerve and hearing outcomes.