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Neurofibromatosis Type 2

Reviewed by: Karen W. Gripp, MD

What Is Neurofibromatosis Type 2?

Neurofibromatosis type 2 (NF2) is a genetic condition. NF2 causes benign (not cancerous) tumors to grow in the nervous system.

The growths can cause problems that range from mild to severe. There's no cure for NF2, but doctors use different treatments to manage symptoms. Experts are hopeful that new, more effective treatments will be available soon.

There are two main types of neurofibromatosis:

  • Neurofibromatosis type 1 (NF1): The most common type, it affects 1 of every 3,000 births.
  • NF2: NF2 is much rarer, and is found in about 1 in 25,000 births.

They're very different conditions. NF1 can never become NF2 or the other way around, and a person can have only one type of neurofibromatosis.

A third type of NF, schwannomatosis, is very rare. Most people with this type don't have symptoms until they're adults.

What Are the Signs & Symptoms of Neurofibromatosis 2?

The growths of neurofibromatosis type 2 are:

  • vestibular schwannomas (or acoustic neuromas): growths that affect nerves in the inner ear that control balance and hearing
  • ependymomas: a type of brain tumor
  • meningiomas: tumors in the membrane that surrounds the brain and spinal cord

These are benign, slow-growing tumors. The problems they cause take time to develop and cause symptoms. While almost all children with NF1 have symptoms by the time they're 8, NF2 symptoms usually don't start until a child is older.

Hearing loss in the late teens and early twenties is often the first symptom of the disorder. It happens because vestibular schwannomas (shwah-NOE-muhs) grow on the vestibular, which carry electrical impulses from the ear to the brain, allowing us to hear. Some people with NF2 have total hearing loss, while others have a partial loss. The growths can affect hearing in one ear or both (called bilateral vestibular schwannomas).

Someone with NF2 also might have:

  • ringing in the ears (tinnitus)
  • headaches
  • facial pain or weakness
  • a feeling of being unsteady or off-balance
  • seizures
  • trouble swallowing
  • cataracts, which can affect vision

What Causes Neurofibromatosis?

NF1 and NF2 are part of a group of rare conditions called RASopathies (raz-OP-uh-thees). These happen when there's a problem in the way cells communicate in one of the body's pathways. Changes in the genes (our genetic "blueprints") in the RAS pathway cause the RASopathies.

Children with neurofibromatosis type 2 have cells that don't make merlin as they should. Merlin (also called schwannomin) is a protein that keeps cells from growing and dividing too quickly or out of control.

The faulty gene that causes NF2 can:

  • pass from a parent to a child
  • happen because of a spontaneous gene change during pregnancy. This means that a child has the condition but the parents do not. This is called a new mutation.

How Is Neurofibromatosis 2 Diagnosed?

To diagnose neurofibromatosis type 2, doctors will:

  • do audiometry (hearing tests) to check for hearing loss or balance problems
  • order brain and spine imaging studies to look for tumors in the nerves of the ears, spinal cord, or brain
  • ask about family members who may have NF2
  • refer a child to an eye doctor to check for cataracts and other problems

Genetic testing can confirm a diagnosis of NF2. For women with NF2 who are pregnant, amniocentesis or chorionic villus sampling can sometimes help doctors see if the fetus has the condition.

How Is Neurofibromatosis Treated?

A health care team treats people with NF2. Specialists usually include a:

  • neurologist: for brain and nervous system problems
  • neuro-otologist: for neurological ear disorders 
  • neuroradiologist: to review X-rays, MRIs, and CT scans of the brain, spine, and spinal cord
  • audiologist: for hearing and balance problems
  • geneticist: to explain how the gene passes to children

The condition can affect different parts of the body, so someone might need:

Someone with significant hearing loss from NF2 can benefit from learning other ways to communicate, such as sign language or lip reading.

They also might get MRIs of the spinal cord and brain to check for growths. Some people with NF2 might be candidates for clinical trials that test for new treatment options.

What Else Should I Know?

  • Doctors often do surgery to treat tumors that cause problems or get bigger. 
  • People with neurofibromatosis type 2 should have regular exams that check their hearing and vision.
  • For families with a history of NF2, genetic screening can help identify NF2 as early as possible. Supportive treatments and therapies work best when started early.

How Can Parents Help?

If your child has neurofibromatosis type 2, you can help by:

Learning about NF2. Talk to the care team about how it might affect your child. Find out about treatments that can help.

Getting the right care. Medical treatments for NF2 can be complicated. Be sure your child sees doctors with experience in caring for those with NF2.

Show love and acceptance. Help your child deal with the emotions that can come from problems like hearing loss, balance problems, and other symptoms. When your child needs extra support, talking with a therapist or meeting other people who have NF2 can help too.

Finding support. It takes time to adjust to a new diagnosis. Reach out to family and friends you feel close to. Ask your child's care team for recommendations. You also can find information and support online at:

Reviewed by: Karen W. Gripp, MD
Date reviewed: September 2019