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How is neurofibromatosis type 2 (NF2) treated?

Neurofibromatosis type 2 (NF2) is best managed at a specialty clinic, with an initial screening and annual follow-up evaluations (more frequent if the disease is severe). Improved diagnostic technologies, such as magnetic resonance imaging (MRI), can reveal tumors of the vestibular nerve as small as a few millimeters in diameter. Vestibular schwannomas grow slowly, but they can grow large enough to engulf a branch of the eighth cranial nerve and cause brain stem compression and damage to surrounding cranial nerves. Surgical options depend on the tumor size and the extent of hearing loss. There is no general agreement among doctors about when surgery should be performed or which surgical option is the best. Individuals considering surgery should carefully weigh the risks and benefits of all options so as to determine which treatment is right for them. Surgery to remove the entire tumor while it's still small might help preserve hearing. If hearing is lost during this surgery but the auditory nerve is maintained, surgical placement of a cochlear implant (a device placed in the inner ear, or cochlea, that processes electronic signals from sound waves to the auditory nerve) may be an option to improve hearing. As the tumors grow larger, it becomes harder to surgically preserve hearing and the auditory nerve. The development of the penetrating auditory brain stem implant (a device that stimulates the hearing portions of the brain) can restore some hearing in individuals who have completely lost hearing and do not have an auditory nerve present. Surgery for other tumors associated with NF2 is aimed at controlling or relieving symptoms. Surgery also can correct cataracts and retinal abnormalities.

This answer is based on source information from the National Institute of Neurological Disorders and Stroke.

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Important: This content reflects information from various individuals and organizations and may offer alternative or opposing points of view. It should not be used for medical advice, diagnosis or treatment. As always, you should consult with your healthcare provider about your specific health needs.