Neurofibromatosis Type 2

Epidemiology

Neurofibromatosis (NF) type 2 is characterized by multiple cranial nerve schwannomas, but associated peripheral and cutaneous neurofibromas are uncommon. These lesions may be associated with multiple meningiomas or ependymomas. The most common site for schwannomas involves the eighth cranial nerve. The reported incidence is about 1:210,000, which is 10 times less frequent than NF type 1. This entity has an autosomal dominant pattern of inheritance and shows no predilection based on race or sex.

Clinical Findings

Patients with vestibular schwannomas typically present with symptoms of hearing loss in the second and third decades and 30s. Young patients with meningiomas may present with symptoms related to raised intracranial pressure. These patients should be suspected to have NF2. In addition, patients may present with juvenile subcapsular lens opacity.