When Your Child Has NF2-Related Schwannomatosis (NF2-SWN) )

NF2-related schwannomatosis (NF2-SWN) makes certain nerve cells more likely to develop tumors. Most of these tumors are not cancer (benign). But in very rare cases, they may turn into cancer (malignant). This health problem may also be called neurofibromatosis type 2.

With NF2-SWN, tumors are most often found along the nerves to the ears (auditory nerves). They can lead to hearing loss and problems with balance. Often these tumors happen in both ears. Tumors can also grow along nerves coming out of the brain and spinal cord. Eyesight problems may occur. Your child's doctor will tell you more about your child's condition and their treatment choices.

NF2-SWN is caused by a change (mutation) in certain genes that affect nerve growth. This mutation often happens by chance. It can also be passed down in families (inherited). If a child has NF2-SWN, parents and siblings should be checked for the condition, too.

Symptoms vary for each child. They can also be hard to recognize during childhood. So the diagnosis is often not made until the child is in their 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
• Eyesight problems
• Numbness or tingling in the arms or legs
• Skin sores or tumors

Your child will likely see a pediatric neurologist for diagnosis and treatment. This doctor specializes in nervous system problems in children. If the doctor thinks your child may have NF2-SWN, your child may have tests, such as:

• Nervous system exam. During this exam, the doctor checks how well your child's nervous system is working. They check your child's muscle strength, balance, coordination, and reflexes. They also check skills, such as hearing or vision.
• Eye exam. This checks for eyesight changes, cataracts, and eye pressure.
• Hearing exam. This checks your child's hearing.
• Health history. The doctor will ask about your child's symptoms, other health problems, and family health history.
• Imaging tests. These may include an MRI or CT scan to check tumor size and location.
• Genetic tests. These blood tests check for the gene mutation.

For your child to be diagnosed with NF2-SWN, they need to have one of these:

• Bilateral vestibular schwannomas. These slow-growing tumors are also known as acoustic neuromas. They can lead to hearing loss, ringing in the ears, or weak facial muscles. "Bilateral" means both sides – so in this case, it means that there is a tumor on the main nerve leading from the inner part of each ear to the brain.
• An identical gene variation in two different NF2-related schwannoma, meningioma, or ependymoma tumors.
• Certain combinations of major and minor criteria:
  • Major criteria are a unilateral vestibular schwannoma, a parent with NF2-related schwannomatosis, two or more meningiomas, or a gene variation in an unaffected tissue, such as blood.
  • Minor criteria include more than one schwannoma or ependymoma, or a certain type of cataract.

Your child will be followed closely to watch for changes in their health, such as new or growing tumors or hearing or vision problems.

Surgery is often done to remove any tumors. Surgery has many risks and benefits. The doctor will talk about these with you. In some cases, a child may have other treatments like radiation or chemotherapy.

If your child has a hearing problem, they may need a cochlear implant. It can help fix hearing. Your child's doctor will go over this surgery if needed.

The outcome for your child varies based on how bad their condition is. After treatment, many children can be as active and independent as other children. Other tumors in the nervous system can occur. But they are rare. So your child may need routine checkups.

If a tumor was taken out along the auditory nerve, ask their doctor about support care to help your child learn to manage their disability.

NF2-SWN is linked to a risk for tumors. These include schwannomas, meningiomas, and low-grade ependymomas. Children with NF2 must be checked often. Doing so helps find tumors at an early stage.

A positive outlook helps while supporting your child. Encourage your child to be active and to try new things. Think about getting counseling. It can help you and your child deal with any fears or concerns. And get help from friends, local resources, and support groups. Learning more about your child's condition may help you feel more in control. These resources may also help:

• Children's Tumor Foundation, ctf.org 800-323-7938
• National Institute of Neurological Disorders and Stroke, ninds.nih.gov 800-352-9424