Type II neurofibromatosis has a completely different phenotype than type I disease, with some overlap. Onset of disease is often not until the second or third decade of life. The main aspect of type II neurofibromatosis is the formation of bilateral acoustic neuromas (vestibular schwannomas). These tumors can lead to headaches, vertigo, and various degrees of hearing loss. Schwannomas may occur in any cranial nerve. The criteria used to establish the diagnosis are (1) the presence of bilateral schwannomas; (2) the combination of a first-degree relative with type II neurofibromatosis and a unilateral vestibular schwannoma; or (3) a first-degree relative with type II neurofibromatosis and any two of the following tumors: neurofibroma, glioma, schwannoma, meningioma, or juvenile posterior subcapsular lenticular opacity.
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