Neurofibromatosis 2 (NF2) is an autosomal dominant disorder characterized by the bilateral development of vestibular schwannoma, along with the occurrence of multiple other benign intracranial, spinal cord, and peripheral nerve tumors. Cataracts are nontumorous manifestations of NF2.
Diagnostic Criteria and Clinical Aspects
The diagnostic criteria for NF2 are the observance of bilateral eight-nerve masses (vestibular schwannomas) with appropriate imaging techniques (e.g., CT or MRI) or the existence of a first-degree relative with NF2 and either a unilateral vestibular schwannoma or two of the following: meningioma, neurofibroma, glioma, schwannoma, or juvenile posterior subcapsular lenticular opacity.
Vestibular schwannomas, formerly termed acoustic neuromas, are a universal feature of NF2. They are slow-growing, benign tumors that form in the internal auditory canal where the...