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Neurofibromatosis 2
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Neurofibromatosis 2

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NF2; Bilateral acoustic neurofibromatosis; Central bilateral acoustic NF

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

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

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  • Causes

    NF2 is passed down through families in an autosomal dominant pattern. This means that if one 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 include:

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

    Tests include:

    • Genetic testing
    • Medical history
    • MRI
    • Physical examination
  • Treatment

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

    Patients may benefit from genetic counseling.

    Every year, patients with NF2 should be evaluated with:

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

    For information and support, visit www.ctf.org.

Related Information

  Neurofibromatosis-...Autosomal dominant...    

References

Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol. 2007;6:340-351.

Sahin M. Neurocutaneous syndromes. In: Kliegman RM, Stanton BF, St. Geme III JW, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 589.

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Review Date: 10/29/2013  

Reviewed By: Chad Haldeman-Englert, MD, FACMG, Wake Forest School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, 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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