The hallmark of NF2 is the appearance of bilateral vestibular schwannomas. These tumors most often occur on the vestibular portion of the eighth cranial nerve and typically are benign. Like NF1, NF2 is associated with specific diagnostic criteria including: the presence of multiple types of central nervous system tumors (eg, meningioma, glioma, neurofibroma, schwannoma) cerebral calcification, and posterior subcapsular lens opacity. Tuberous sclerosis, NF1, Li-Fraumeni syndrome, and Turcotıs syndrome are not associated with bilateral vestibular schwannomas.
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