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About NF-2

NF-2 is sometimes referred to as central neurofibromatosis or bilateral acoustic neuroma disease. NF-2 is different for each person, but because it affects nerves next to the brain or spinal cord, the problems of NF-2 can cause serious disabilities. Even so, many people with NF-2 can lead relatively normal, rewarding lives and learn to compensate for deficits they may have.

In some people signs of the disorder are detectable in childhood, but for the majority of people, NF-2 is not apparent until late teenage years or later. People with NF-2 may only have a few café-au-lait spots--brown oval or circular spots on the skin. Skin tumors are few in number and easily overlooked.

However, everyone with NF-2 has tumors affecting hearing and balance (vestibular schwannoma or acoustic neuroma). Cataracts in the lens or changes in the retina of the eye are present in most people. Tumors, which push on the brain or spinal cord (meningiomas and schwannomas), as well as tumors along the peripheral nerves (schwannomas), or tumors within the spinal cord occur in many people. These problems are serious because they can cause weakness or seizures, but the tumors are benign.

People with NF-2 should realize that while the problems they face can be frightening, there have been important advances in both treating and detection of NF-2, which offer considerable hope.

What to Do If You, or Your Child, Has NF-2

People with NF-2 need regular physical examinations to check their hearing and neurologic function. Hearing tests of pure tones, auditory evoked responses, balance tests, scans of the brain and spinal cord, and eye exams will probably be needed annually. Because NF-2 will ultimately affect your hearing in both ears, you will need to be open to several methods of communication. Sign language opens many opportunities outside of the home for the hearing impaired. It is most useful when the entire family learns to sign. Unfortunately, lip reading is often impractical for people with NF-2. Once the NF diagnosis has been made, it's important to begin a careful, organized program to spot any change in your body that could be caused by NF. Make regular visits to your doctor part of your routine.

People with NF-2 may need complicated surgery on more than one occasion. Because some surgery can lead to permanent disabilities, such as loss of hearing, the timing of surgery needs to be carefully considered. Surgery should be performed only by a neurosurgical or ear, nose, and throat team with special expertise in NF-2 because the surgery can lead to complications in less experienced hands. Specially-trained therapists, audiologists, ophthalmologists, and counselors are also essential in the rehabilitation process. In some instances, surgery should be delayed because some tumors may show very little growth over many years and can be watched carefully. But it is clear that the best results are obtained when problems are identified and treated early.

People with NF-2 need to be cautious of non-standard treatments. Treatment of NF-2 should always be discussed with physicians who treat many patients with NF-2. While the problems in NF-2 can be very frightening, there is evidence of significant improvements in treatment methods, which are very encouraging and indicate real hope for patients to lead extremely functional lives. There is also a community of people who can provide advice and support.

Guidelines for People with NF-2

  • Evaluate other family members for vestibular tumors.
  • Discuss inheritance of NF-2.
  • Learn signing or alternative forms of communication.
  • Have eye exam for cataracts and retinal abnormalities.
  • Have MRI of brain and entire spinal cord annually.
  • Have annual hearing tests.
  • Have careful neurologic exam annually.
  • Discuss potential impact of NF-2 on career and family.
  • Assess resources and support needed for daily living.