Neurofibromatosis 2

Definition

Neurofibromatosis 2 (NF2) is a genetic disorder that causes tumors to form on the nerves of the brain and spine (the central nervous system).

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. This means that if one parent has NF2, any child of that parent has a 50-50 chance of having it.

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 (related to hearing) 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

Illustrations

Central nervous system

Page Content:

NF2; Bilateral Acoustic Neurofibromatosis (BAN); Central Bilateral Acoustic NF ; nf2