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A diagnosis of neurofibromatosis type 2 (NF2) comes with many questions and concerns, both for young adults and their families. Although the majority of people with NF2 lead normal lives, there are still many aspects of the disorder that are difficult to predict.
It may comfort you to know that in the Neurofibromatosis Program at Boston Children’s Hospital, we have already helped thousands of children and young adults successfully manage their NF. Our compassionate, experienced clinicians are here to help you every step of the way.
What is NF2?
NF2 is a genetic disorder characterized by certain types of tumors that form within a person’s body or brain.
The most common types of tumors associated with NF2 are:
Are there other types of NF?
Yes. Besides NF2, there are two other distinct forms of neurofibromatosis: neurofibromatosis type 1 (NF1) and schwannomatosis.
What is neurofibromatosis type 1 (NF1)?
NF2 may often be confused with NF1. Like NF2, NF1 is also a genetic disorder characterized by the presence of tumors that form along nerves in the body. However, the disorders are caused by two different genes that are located on two different chromosomes. It’s extremely rare that someone would have both NF1 and NF2.
Here are some notable clinical differences between NF1 and NF2:
What is schwannomatosis?
Schwannomatosis is an extremely rare form of NF that affects about 1 in every 40,000 individuals. Although schwannomatosis shares some clinical similarities with the other forms of NF, it is a separate disorder.
Here are important distinguishing features of schwannomatosis:
Are there any medical complications associated with NF2?
Because NF2 affects the nervous system, the most complications involve problems related to vision, hearing, and balance. Numbness or weakness in the face, arms or legs may also occur. However, there is no evidence that NF2 causes intellectual and learning disabilities, which are very common in individuals with NF1.
What caused my child to get NF2?
In 50 percent of cases, NF2 is inherited from a parent:
In the other 50 percent of cases, NF2 occurs as a result of a spontaneous mutation:
Is there a difference between inherited NF2 and NF2 that occurs from a spontaneous mutation?
No. Aside from its origins, there is no difference between inherited NF2 and NF2 that was caused by a spontaneous mutation.
Did I do anything to cause my child’s NF2?
No, there’s no evidence suggesting that NF2 is caused by environmental factors or something the mother did (or didn’t do) during pregnancy.
When do symptoms of NF2 present?
NF2 is usually detected in early adulthood, with the average age of symptom onset being around 20 years.
What are the first signs of NF2?
Most NF2 tumors grow on the eighth cranial nerve. Located in the inner ear, the eighth cranial nerve is responsible for sending information on both sound and balance to the brain.
As a result, the first symptoms of NF2 are usually caused by the nerve’s impairment:
Other symptoms of NF2 may include:
Are symptoms of NF2 progressive?
Although it’s almost impossible to predict exactly how NF2 will progress, vestibular schwannomas grow slowly and usually cause balance and hearing to deteriorate over time. Fortunately, there are surgical interventions that can preserve hearing. Visit our Treatment tab to learn more about these options.
A diagnosis of NF2 comes with a lot of questions and concerns, for both patients and their families. During your initial appointments with the doctor, it can be easy to become overwhelmed with information and forget what you wanted to ask.
Many parents and young adults find it helpful to write down questions beforehand or jot them down as they arise. That way, when you talk to the doctors, you can be sure that all of your concerns are addressed.
Some questions you might want to ask include:
We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”