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Neurofibromatosis 2

Neurofibromatosis 2 (NF2) is a disorder in which tumors form on the nerves of the brain and spine (the central nervous system). It is passed down (inherited) in families.

Although it has a similar name to neurofibromatosis type 1, it is a different and separate condition.

Causes

NF2 can be passed down through families in an autosomal dominant pattern. This means that if 1 parent has NF2, any child of that parent has a 50% chance of inheriting the condition.

The main risk factor is having a family history of the condition.

Symptoms

Symptoms of NF2 include:

  • Balance problems
  • Cataracts at a young age
  • Changes in vision
  • Coffee-colored marks on the skin (café-au-lait)
  • Headaches
  • Hearing loss
  • Ringing and noises in the ears
  • Weakness of the face

Exams and Tests

Signs of NF2 include:

  • Brain and spinal tumors
  • Hearing-related (acoustic) tumors
  • Skin tumors

Tests include:

  • Genetic testing
  • Medical history
  • MRI
  • Physical examination

Treatment

Acoustic tumors can be observed, or treated with surgery or radiation.

People with this disorder may benefit from genetic counseling.

People with NF2 should be regularly evaluated with these tests:

  • MRI of the brain and spinal cord
  • Hearing and speech evaluation
  • Eye exam

Support Groups

A support group can be helpful for emotional support and for giving and receiving practical advice. For information and support, visit www.ctf.org.

Alternative Names

NF2; Bilateral acoustic neurofibromatosis; Central bilateral acoustic NF

References

Evans DG. Neurofibromatosis 2. GeneReviews. Seattle, WA; University of Washington; 2011:8. www.ncbi.nlm.nih.gov/books/NBK1201/. Accessed: October 24, 2015.

Sahin M. Neurocutaneous syndromes. In: Kliegman RM, Stanton BF, St Geme III JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 596.

Update Date 10/27/2015

Updated by: Chad Haldeman-Englert, MD, FACMG, Fullerton Genetics Center, Asheville, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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