Neurofibromatosis Type 2

Overview

Mayo Clinic specialists are internationally recognized as experts in diagnosing and treating tumors associated with neurofibromatosis type 2 (NF2).

Specialists in neurology, neurosurgery, medical genetics, orthopedics, otorhinolaryngology, dermatology, audiology, developmental pediatrics, plastic surgery, radiology and ophthalmology work together to address the multiple complications and complex management challenges that can accompany this condition. Mayo specialists use state-of-the-art equipment and are at the forefront in research and testing of promising new therapies.

Fortunately, the future for those with neurofibromatosis type 2 looks much brighter than it did even a few years ago. Surgical techniques have improved, and early detection has led to more successful management of the disease. In addition, the discovery of the gene that causes this condition has sparked hope for eventual gene therapies to cure or prevent NF2.

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• Treatment of Children & Adolescents

Diagnosis

The symptoms of NF2 — ringing in the ears (tinnitus), hearing loss, and difficulty with balance — most often appear in young adults. It may be diagnosed earlier in children with a family history of the disease or it may not appear until the 40s in someone with a mild form of the disorder. Magnetic resonance imaging is the preferred diagnostic test for identifying NF2. Read more about neurofibromatosis type 2 diagnosis.

Treatment Options

Treatment options include observation, surgical removal and radiotherapy. It is important that children whose parents have neurofibromatosis type 2 be monitored at an early age in order to detect tumors while hearing-preservation surgery is still possible. Read more about NF2 treatment options.

About Neurofibromatosis Type 2

NF2 is a rare, hereditary disorder that causes tumors along the nerves in the brain and spinal cord. The disease occurs in one in 30,000-40,000 people. It is an autosomal dominant disorder, meaning that it is passed from parent to child. An affected person has a 50 percent risk of passing on the gene to a child. However, about half the people with NF2 do not have a history of the disease in either parent. The disorder ranges from mild to severe, with the severest form usually found in younger children.

One characteristic of the disorder is bilateral acoustic neuromas (vestibular schwannomas) — tumors on the nerves affecting hearing and balance on both sides of the body. Tumors on the brain and spinal cord (gliomas and meningiomas) can also be part of this disease.

The gene that causes NF2 is different from the one that causes neurofibromatosis type 1, which is 10 times more common.

Read more about acoustic neuroma.