Preservation and restitution of auditory function in neurofibromatosis type 2

Abstract

Neurofibromatosis type 2 (NF2) is defined by bilateral vestibular schwannomas; 86% of patients suffer from additional spinal tumours, schwannomas and meningiomas and, in rare cases, also gliomas and ependymomas. Furthermore, most patients present with additional cranial meningiomas or even meningiomatosis of the sinuses. As a consequence, these patients are at risk of bilateral deafness, facial and motor nerve palsies and para- or tetraparesis. Fifty-eight percent of affected patients become symptomatic before the age of 20 years, but first manifestation at over 40 years is possible. About 50% exhibit a familial history of NF2, while the other 50% have a new mutation. © 2008 Springer-Verlag.