Targeted Therapies in the Treatment of Vestibular Schwannomas: Current State and New Horizons

Vestibular schwannomas continue to cause hearing loss, facial nerve paralysis, imbalance, and tinnitus. These symptoms are compounded by germline neurofibromatosis type 2 (NF2) gene loss and multiple intracranial and spinal cord tumors associated with NF2-related schwannomatosis. The current treatments of observation, microsurgical resection, or stereotactic radiation may prevent catastrophic brainstem compression but are all associated with the loss of cranial nerve function, particularly hearing loss. Novel targeted treatment options to stop tumor progression include small molecule inhibitors, immunotherapy, anti-inflammatory drugs, radio-sensitizing and sclerosing agents, and gene therapy.