Bilateral Acoustic Neuroma in a Large Kindred

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Abstract
A kindred originally described 40 years ago now includes 97 individuals with definite or possible bilateral acoustic neuroma. The trait is autosomal dominant with high penetrance. Onset is at about 20 years of age and survival without surgery varies from 2 to 42 years. Severe accidents, including drowning, are common in the family, possibly due to neurological deficits. In screening such individuals, vestibular function is probably most sensitive, but complete audiometric studies, special roentgenologic examinations, and brain scan have proved useful. No single study can be relied upon to diagnose this disease or to exclude the diagnosis. Peripheral signs suggesting neurofibromatosis are rare but other central nervous system tumors, often asymptomatic, are common, which suggests that this trait represents a "central" form of neurofibromatosis. It is distinct from unilateral acoustic neuroma based on natural history and genetic etiology.

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