Neurofibromatosis Type 2
Neurofibromatosis is an inherited problem that causes tumors to form in the nervous system. They form in the nerves or the tissue around them. Neurofibromatosis has three types: neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis. The type a person has depends on the genes that are affected.
This article focuses on NF2. These tumors tend to form in the central nervous system. The eighth cranial nerve is the one most affected. This can lead to problems with hearing and balance.
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NF2 is caused by a problem with a gene that makes proteins to help control nerve growth. The gene is not able to make these proteins and tumors form.
In most people, the gene issue passes from a parent to their child. The gene change can also happen in a person who does not have family members with NF2.
This risk is highest in people with other family who have NF2.
The problems a person has depends on the tumor size and where it is. Smaller ones may not cause symptoms.
A person with NF2 may have:
The doctor will ask about symptoms and health history. The doctor may ask if any family members have NF2. A physical exam will be done. It may take a few years before NF2 is diagnosed.
These tests may be done:
- Blood tests to look for signs of the gene problem
- An MRI scan to look for tumors
- Electrodiagnostic testing to check the electrical activity of the muscles and nerves
- A brainstem auditory evoked response (BAER) test to measure brainwave activity
- Manage any problems the tumors cause
- Remove any tumors causing problems
People who do have symptoms may need:
- Radiation and medicines to shrink tumors
- Surgery to remove tumors or repair related problems, such as hearing loss or cataracts
NF2 often cannot be prevented. People who have a family history of NF2 may want to see a genetic counselor before having children.
- About NF2. Children's Tumor Foundation website. Available at: https://www.ctf.org/understanding-nf/nf2.
- Neurofibromatosis type 2. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/neurofibromatosis-type-2.
- Neurofibromatosis. National Institute of Neurological Disorders and Stroke website. Available at: https://www.ninds.nih.gov/health-information/disorders/neurofibromatosis.
- Tamura, R. Current understanding of neurofibromatosis 1, 2, and Schwannomatosis. International Journal of Molecular Sciences, 2021; 22(11): 5850.
- James P. Cornell, MD
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