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.
Part of: Neurofibromatosis (NF)
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