Neurofibromatosis Type 2

Approved by the Cancer.Net Editorial Board, 05/2019

What is neurofibromatosis type 2?

Neurofibromatosis type 2 (NF2) is a hereditary condition most commonly associated with bilateral vestibular schwannomas, also known as acoustic neuromas. These are benign (noncancerous) tumors that occur on the nerves for balance and hearing leading to the inner ear. Although these tumors are benign, they can cause hearing and balance problems.


  • Schwannomas of other nerves

  • Meningiomas, a slow-growing tumor that usually develops on the surface of the brain

  • Gliomas, including ependymomas of the brain or spinal cord

People with NF2 are at an increased risk of developing cataracts in the eyes and benign skin tumors. They may have café-au-lait spots, which are light brown pigmentation, the color of “coffee with milk.” This feature is similar to those individuals with Neurofibromatosis type 1, but people with NF2 usually have fewer café-au-lait spots than people with NF1.


What causes NF2?


How is NF2 inherited?

Normally, every cell has 2 copies of each gene: 1 inherited from the mother and 1 inherited from the father. NF2 follows an autosomal dominant inheritance pattern, in which a mutation happens in only 1 copy of the gene. If a copy of the gene with the mutation is inherited, that gene becomes the dominant gene and can cause features of NF2.  A parent with a NF2 may pass along a copy of their normal gene or a copy of the gene with the mutation. Therefore, a child who has a parent with a genetic mutation has a 50% chance of inheriting that mutation. A brother, sister, or parent of a person who has a mutation also has a 50% chance of having the same mutation. However, if the parents test negative for the mutation, the risk to the siblings significantly decreases but their risk may still be higher than an average risk.


How common is NF2?


How is NF2 diagnosed?


  • Bilateral, meaning both sides, vestibular schwannomas, which are tumors that grow from the nerve that controls hearing and balance


    • A single vestibular schwannoma diagnosed before age 30, or

    • Any 2 of the following: a meningioma, glioma, schwannoma, or cataract


  • Multiple meningiomas and a unilateral vestibular schwannoma, meaning affecting only 1 side, diagnosed under age 30, a glioma, schwannoma, or cataract


How are tumors related to NF2 treated?

Although the primary method of treating tumors associated with NF2 is surgery, many people with this disease have tumors that grow slowly or not at all. That means they can be closely monitored in an approach called watchful waiting, watch and wait, or active surveillance. In this approach, active treatment would begin if there are signs that the tumor could cause neurological problems, or the pattern of growth threatens brain or spinal cord function.

Specifically, regarding bilateral acoustic neuromas, a major goal of treatment is to preserve the person’s hearing for as long as possible. However, hearing will eventually be lost at some point. Surgery is usually done in people who can no longer hear or in those with a small tumor where a meaningful attempt can be made at preserving the nerve that makes hearing possible.

In addition, focused radiation therapy is sometimes used for vestibular schwannomas. Like surgery, it carries a risk that the treatment will hurt the person’s hearing in order to control the tumor. The other tumors seen in this disease are usually meningioma and ependymoma, and these types are typically removed only if they grow to an extent that they put enough pressure on the nearby brain or spinal cord to affect its functioning.

Reports of using bevacizumab (Avastin), a drug that interferes with blood vessel formation to stop tumor growth for treating vestibular schwannomas associated with NF2, have been encouraging. Although only a few people have been treated with this drug, the results include both measureable shrinkage of tumor and partial restoration of hearing loss in some, but not all, patients. This is an experimental treatment and needs more testing, but it holds promise that other drugs may be proven effective as well. Talk with your doctor about this option, including the side effects of bevacizumab.  In addition, you should ask about available clinical trials, which are research studies for new treatments.

What are the estimated cancer risks associated with NF2?


What are the screening options for NF2?



  • Hearing evaluations, including audiometry and a brain stem auditory evoked response (BAER) test, which is a test that detects electrical activity in the cochlea and auditory pathways



Questions to ask the health care team

If you are concerned about your risk of cancerous and benign tumors, talk with your health care team. It can be helpful to bring someone along to your appointments to take notes. Consider asking the following questions:

  • What is my risk of developing tumors of the nervous system?

  • What can I do to reduce my risk of this?

  • What are my options for screening?


  • Does my family history increase my risk of developing cancer?

  • Does it suggest the need for a cancer risk assessment?


  • Should I consider genetic testing?

Related Resources

The Genetics of Cancer

Genetic Testing

What to Expect When You Meet With a Genetic Counselor

Collecting Your Family Cancer History

Sharing Genetic Test Results with Your Family

Family Genetic Testing Q&A

More Information

Neurofibromatosis Network

Children’s Tumor Foundation

National Institute of Neurological Disorders and Stroke 

Acoustic Neuroma Association

National Cancer Institute  


National Society of Genetic Counselors