Neurofibromatosis Type 2

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Neurofibromatosis type 2 (NF2) affects the eighth cranial nerve, which affects hearing and balance. NF2 creates tumors in the central nervous system.

Causes of NF2

Changes in a specific gene can cause NF2. The gene normally makes proteins that help control growth in the nerves. Since the gene is defective, these proteins are not able to control growth and tumors develop.

You’re more likely to develop NF2 if you inherit the abnormal gene from a parent. You have a 50 percent chance of passing off the gene to your children. However, you can develop NF2 without any family history. 

NF2 Symptoms

Symptoms depend on the exact location and size of the tumor and may include:

  • Hearing loss
  • Tinnitus (ringing in ears)
  • Poor balance
  • Headaches
  • Pain or numbness in the face
  • Visual problems such as double or blurry vision
  • Weakness or tingling in arms or legs
  • Bumps under skin (tumor) or small flesh colored skin flaps — adults may also have dark, rough, hairy patches over tumor


NF2 can take years to diagnose. Your doctor may suspect NF2 if you have a history of:

  • Tumors such as:
    • Vestibular schwannomas — tumor that grows from tissue around eighth cranial nerve
    • Glioma — tumor of brain or spine
    • Meningioma — tumor of tissue that surrounds brain and spine
    • Schwannoma — tumor from tissue that covers all nerves
  • Juvenile cataracts

Your doctor can diagnosis you based on your history, symptoms and a physical exam. Your doctor may order MRI scans to create images of the nerves and brain to look for tumors. A biopsy of tumor samples can also help diagnose NF2.  

NF2 Treatment

There are no current treatments to stop these tumors from growing, but you should visit your doctor regularly to check for new tumors or symptoms.

You may require surgery to remove tumors that cause hearing loss. In this case, you may be suitable for cochlear implants to help correct hearing loss. Other surgeries can help correct additional problems like cataract repairs.

Some tumors may also shrink in response to radiation or certain medication. These treatments can have some side effects and complications. You and your doctor will weigh the risks and benefits of each treatment option.


Content was created using EBSCO’s Health Library. Edits to original content made by Rector and Visitors of the University of Virginia. This information is not a substitute for professional medical advice.