Neurofibromatosis 2

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 http://www.nf.org

Johns Hopkins patient information

Last revised: December 7, 2012
by Sharon M. Smith, M.D.

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