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When Your Child Has Neurofibromatosis Type 2 (NF2)

Technician preparing girl for CT scan.
With NF2, your child may have an imaging test, such as an MRI or CT scan, to check for tumors.
Neurofibromatosis type 2 (NF2) is a condition that makes certain nerve cells more likely to develop tumors. With NF2, the most common location for tumors is along the nerves in the ears (auditory nerves). This can lead to hearing loss and problems with balance. Sometimes these tumors happen in both ears. Tumors can also develop along nerves in the brain and spinal cord. Your child’s healthcare provider will tell you more about your child’s condition and treatment choices for your child. 

What causes NF2?

NF2 is caused by an abnormality (mutation) in specific genes that affect nerve growth. This mutation often happens by chance. It can also be passed down in families. If a diagnosis is confirmed, parents and siblings should be evaluated for the condition as well.

What are the symptoms of NF2?

Symptoms vary for each child. They can also be hard to recognize during childhood, so the diagnosis is often delayed until the child is in his or her 20s or 30s. The symptoms may also be affected by the location of the tumors. Your child may have:

  • Hearing loss

  • Ringing in the ears (tinnitus)

  • Problems with balance

  • Problems with vision

  • Numbness or tingling in the arms or legs

How is NF2 diagnosed?

Your child will likely see a pediatric neurologist for diagnosis and treatment. This is a healthcare provider who specializes in neurologic problems. If NF2 is suspected, the following may be done:

  • Neurologic exam to check how well your child’s nervous system is working. During the exam, the healthcare provider checks your child’s muscle strength, balance, coordination, and reflexes. He or she also checks skills such as hearing or vision

  • Ophthalmologic exam to evaluate for cataracts and ocular pressure 

  • Auditory exam to evaluate hearing 

  • Health history to learn more about your child’s symptoms, other health problems, and family medical history

  • Imaging tests such as an MRI or CT scan to check tumor size and location

  • Genetic tests to check for the mutation

Diagnosis of NF2 requires one of the following:

  • Bilateral vestibular schwannoma, also known as an acoustic neuroma. This slow-growing tumor can lead to hearing loss, ringing in the ears, or facial muscle weakness

  • Parent or sibling with NF2

Or any two of the following:

  • Meningioma

  • Glioma

  • Schwannoma

  • Juvenile posterior lenticular opacities    

How is NF2 treated?

Surgery to remove any tumors is the most common treatment. Surgery holds various risks and benefits that the healthcare provider will discuss with you in detail. In rare cases, a tumor can become malignant (cancerous). This may require other treatments such as radiation or chemotherapy. In certain cases, a cochlear implant can help restore hearing. Your healthcare provider will explain if this is a consideration if your child's hearing becomes affected. 

What are the long-term concerns?

The outcome for your child varies depending on the severity of his or her condition. After treatment, many children can be as active and independent as other children. If a tumor was removed along the auditory nerve, your child may have permanent hearing loss after treatment. Ask your child’s healthcare provider about supportive care that can help your child learn to manage his or her disability.

Coping with your child’s condition

A positive outlook helps while supporting your child. Encourage your child to be active and to try new things. Consider counseling, which can help you and your child deal with any fears or concerns. And seek help from friends, community resources, and support groups. The more you learn about your child’s condition and its treatments, the more in control you may feel. For more information about neurofibromatosis, contact the following organizations:

  • Children’s Tumor Foundation, www.ctf.org

  • Neurofibromatosis, www.nfinc.org

© 2000-2017 The StayWell Company, LLC. 800 Township Line Road, Yardley, PA 19067. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional's instructions.