Neurofibromatosis type 2 (NF2) is the official name for the syndrome whose hallmark is bilateral vestibular schwannomas (VS) (Figure 63–1). NF2 replaces a variety of synonyms that have been associated with this entity: central neurofibromatosis, bilateral acoustic neurofibromatosis, cranial neuromatosis, central schwannomatosis, neurofibromatosis universalis, familial bilateral acoustic neuroma syndrome, familial bilateral acoustic neurofibromas, Wishart–Gardner–Eldridge syndrome, neurinomatosis, and neurofibrosarcomatosis. The first known description of the clinical course and postmortem findings of NF2, almost two centuries ago, was of a patient who developed bilateral deafness, had intractable headaches and vomiting, and died at age 21.