Neurofibromatosis 2
Key points
- Neurofibromatosis 2 (NF2) is a genetic disorder that causes tumors to form on the nerves of the central nervous system, in the brain, and on the spine.
- NF2 is inherited, and the main risk factor is having a family history of NF2.
- If a parent has NF2, the chances of a child having it are 50-50.
Alternative names
NF2; Bilateral Acoustic Neurofibromatosis (BAN); Central Bilateral Acoustic NF
Definition
Neurofibromatosis 2 (NF2) is a genetic disorder that causes tumors to form on the nerves of the central nervous system, in the brain, and on the spine.
See also neurofibromatosis 1 (NF1).
Causes, incidence, and risk factors
NF2 is inherited, and the main risk factor is having a family history of NF2. Inheritance is autosomal dominant. If a parent has NF2, the chances of a child having it are 50-50.
Symptoms
- Hearing loss
- Ringing and noises in the ears
- Balance problems
- Cataracts at a young age
- Changes in vision
- Headaches
- Facial weakness
- Coffee-colored marks on the skin
Signs and tests
Signs include:
- Brain and spinal tumors
- Acoustic tumors
- Skin tumors
Tests include:
- Physical examination
- Medical history
- MRI
- Genetic testing
Treatment
Most patients require surgery to remove tumors. Tumors also can be treated with radiation.
Support Groups
For information and support, see www.nf.org
Written and revised by
Gerald Hurowitz, M.D.
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