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 50-50 chance of having it.

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
• 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.nf.org.

Alternative Names

NF2; Bilateral acoustic neurofibromatosis; Central bilateral acoustic NF

References

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

Haslam RHA. Neurocutaneous syndromes. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 596.

Updated: 4/14/2012

Reviewed by: Chad Haldeman-Englert, MD, 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, Medical Director, A.D.A.M., Inc.