Acoustic neuroma in von Recklinghausen's disease

Bilateral acoustic neuromas are common in von Recklinghausen's disease. Their surgical removal likely will produce total hearing loss unless the lesions are small and intracanalicular. Therefore, a posterior fossa myelogram is indicated in all patients when a diagnosis of neurofibromatosis is made. To preserve hearing in resection of small tumors, a middle fossa approach is appropriate. In lesions slightly larger than intracanalicular size, hearing may be preserved with a two-stage middle fossa and transsigmoid approach. The prospect of total hearing loss and even facial diplegia predisposes to surgical procrastination. However, the morbidity and mortality associated with removal of medium and large tumors may be greater for neurofibromatosis than for patients with singular acoustic neuromas. Therefore, we believe it is not advisable to postpone operation when tumors extend into the cerebral pontine angle, even though it means loss of hearing.