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Neurofibromatosis Type 2 (NF2)

A genetic condition in which tumors form on the nerves of the inner ear and cause loss of hearing and balance. Tumors may also occur in the brain and on nerves in the skull and spinal cord, and may cause loss of speech, eye movement, and the ability to swallow.

PubMed Health Glossary
(Source: NIH - National Cancer Institute)

About Neurofibromatosis Type 2 (NF2)

This rare disorder affects about 1 in 25,000 people. Approximately 50 percent of affected people inherit the disorder; in others the disorder is caused by a spontaneous genetic mutation of unknown cause. The hallmark finding in NF2 is the presence of slow-growing tumors on the eighth cranial nerves. These nerves have two branches: the acoustic branch helps people hear by transmitting sound sensations to the brain; and the vestibular branch helps people maintain their balance.

The characteristic tumors of NF2 are called vestibular schwannomas because of their location and the types of cells involved. As these tumors grow, they may press against and damage nearby structures such as other cranial nerves and the brain stem, the latter which can cause serious disability.

Schwannomas in NF2 may occur along any nerve in the body, including the spinal nerves, other cranial nerves, and peripheral nerves in the body. These tumors may be seen as bumps under the skin (when the nerves involved are just under the skin surface) or can also be seen on the skin surface as small (less than 1 inch), dark, rough areas of hairy skin. In children, tumors may be smoother, less pigmented, and less hairy.

Although individuals with NF2 may have schwannomas that resemble small, flesh-colored skin flaps, they rarely have the café-au-lait spots that are seen in NF1....Read more about Neurofibromatosis Type 2
NIH - National Institute of Neurological Disorders and Stroke

What works? Research summarized

Evidence reviews

EOS 2D/3D X-ray Imaging System: A Systematic Review and Economic Evaluation

EOS is a biplane X-ray imaging system manufactured by EOS Imaging (formerly Biospace Med, Paris, France). It uses slot-scanning technology to produce a high-quality image with less irradiation than standard imaging techniques.

Particle Beam Radiation Therapies for Cancer [Internet]

Radiotherapy with charged particles can potentially deliver maximal doses while minimizing irradiation of surrounding tissues. It may be more effective or less harmful than other forms of radiotherapy for some cancers. Currently, seven centers in the United States have facilities for particle (proton) irradiation, and at least four are under construction, each costing between $100 and $225 million. The aim of this Technical Brief was to survey the evidence on particle beam radiotherapy.

Conservative management, gamma-knife radiosurgery, and microsurgery for acoustic neurinomas: a systematic review of outcome and risk of three therapeutic options

This review evaluated the efficacy and risks of three therapeutic options for acoustic neurinomas. It demonstrated that with conservative management and radiologic follow-up the majority of acoustic neurinomas are slow growing but eventually require intervention. Gamma-knife radiosurgery and microsurgery provide tumour control. The analysis was simplistic, and the authors' conclusions about the superior efficacy of microsurgery needs to be interpreted with caution.

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Terms to know

Acoustic
Having to do with sound or hearing.
Equilibrium (Balance)
Biological system that enables individuals to know where their bodies are in the environment and to maintain a desired position. Normal balance depends on information from the labyrinth in the inner ear, from other senses such as sight and touch, and from muscle movement.
Genetic
Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
Hearing Loss
A general term for the complete or partial loss of the ability to hear from one or both ears.
Nerves
A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.
Schwann Cells
Schwann cells function to support neurons in the peripheral nervous system. Schwann cells wrap around axons to form the myelin sheath.

More about Neurofibromatosis Type 2

Photo of a child

Also called: Bilateral acoustic neurofibromatosis, Neurofibromatosis 2, BANF

See Also: Vestibular Schwannoma

Other terms to know: See all 6
Acoustic, Equilibrium (Balance), Genetic

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