Most children who harbor an acoustic neuroma do so in the setting of neurofibromatosis type 2 (NF2). These patients should be evaluated for other intracranial or spinal neoplasms. Occasional adolescents are found to have a solitary tumor with no family history of NF2. They may represent truly solitary tumors, or be a form fruste of the disease, only to develop a contralateral tumor at another time.

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