Bilateral acoustic neuromas are diagnostic of neurofibromatosis-2. This disease is caused by mutations in the NF-2 tumor suppressor gene on chromosome 22, and follows an autosomal dominant pattern of inheritance. Treating these patients is always difficult. Bilateral facial palsies, deafness and the prospect of other CNS tumors developing point to a miserable existence. Management options include observation or subcapsular removal to minimize risk to the audiovestibular nerves and stereotactic radiosurgery. Both require careful consideration. Other options include early treatment of the smaller neuroma to maximise the chance of hearing preservation or treatment of the larger tumor first, with the aim of delaying surgery on the contralateral side if it remains the only source of useful hearing after the first procedure. The treatments in each of these unfortunate patients require individualization and supportive aftercare. The development of brain-stem implant technology to augment auditory function may prove useful in these cases in the future.
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