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).

Images:

Alternative Names

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

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

Lots More Information:
•Neurofibromatosis-1
•Autosomal dominant
Review Date:11/21/2005
Reviewed By: Christos Ballas, M.D., Attending Psychiatrist, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network.