Neurofibromatosis-2 and bilateral acoustic neuromas: distinctions from neurofibromatosis-1 (von Recklinghausen's disease).

Although acknowledged as separate entities in the neurologic literature, central neurofibromatosis and peripheral neurofibromatosis, which is also known as von Recklinghausen's disease, have not been commonly distinguished by otologists as two separate diseases. An NIH Conference statement has reclassified these diseases as neurofibromatosis-1 and neurofibromatosis-2. Genetic, biochemical, and clinical variants of these two related diseases are presented and discussed. Patients with bilateral acoustic neuromas may have either the central or peripheral form of the disease; central neurofibromatosis is much more rare than the peripheral form of the disease.