Neurofibromatosis 2 | UF Health, University of Florida Health

Neurofibromatosis 2

Definition

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.

Alternative Names

NF2; Bilateral acoustic neurofibromatosis; Bilateral vestibular schwannomas; Central neurofibromatosis

Causes

NF2 can be 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), less common
  • 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:

  • Physical examination
  • Medical history
  • MRI
  • CT scan
  • Genetic testing

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

The following resources can provide more information on NF2:

Images

Central nervous system

References

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

Slattery WH. Neurofibromatosis 2. In: Brackmann DE, Shelton C, Arriaga MA, eds. Otologic Surgery. 4th ed. Philadelphia, PA: Elsevier; 2016:chap 57.

Varma R, Williams SD. Neurology. In: Zitelli BJ, McIntire SC, Nowalk AJ, eds. Zitelli and Davis' Atlas of Pediatric Physical Diagnosis. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 16.

Review Date: 
10/26/2017
Reviewed By: 
Anna C. Edens Hurst, MD, MS, Assistant Professor in Medical Genetics, The University of Alabama at Birmingham, Birmingham, AL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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