Neurofibromatosis, type II

Overview

What is Neurofibromatosis Type II?

Neurofibromatosis Type II, also called NF2, is a genetic condition that causes a person to develop tumors in their nervous system. Most of the time these tumors are benign, meaning they don't have the potential to spread throughout the body, however they can still cause health problems and be life-threatening depending on where they are located.

The most common type of tumor seen in people with NF2 forms on one of nerves of the brain called the Vestibulocochlear nerve. This nerve is also called cranial nerve VIII or the eighth cranial nerve. Its two functions are helping with balance and sending messages from the ear to the brain. These tumors are called vestibular schwannomas because they affect balance and hearing. Another name for these tumors are acoustic neuromas since they affect our hearing.

Because we have two ears, we also have two sets of eighth cranial nerves. When someone has NF2 they usually develop a vestibular schwannoma on both of their auditory nerves by the time they reach their 30's, and often develop one of these tumors by their 20's. People with NF2 often develop hearing loss in at least one ear but sometimes both ears as a result of these tumors. NF2 can also cause other types of tumors called meningiomas, ependymomas and astrocytomas to form in the tissue around the brain and spinal cord. Another type of tumor called a retinal hamartoma can form in the back of the eye and cause vision problems. People with NF2 can also have balance problems and seizures. A small percentage of people with NF2 can go blind or develop cataracts which can also affect vision.

SOURCE: Emory University - Department of Human Genetics in collaboration with ThinkGenetic • https://www.thinkgenetic.com/diseases/neurofibromatosis-type-ii/overview/5524 • DATE UPDATED: 2016-06-01

References

St. Jude Children's Research Hospital. Neurofibromatosis Type 2 Information Page: https://www.stjude.org/disease/neurofibromatosis-type-2.html

Evans, Gareth D. Gene Reviews: Neurofibromatosis 2. August 2011. Bookshelf ID: NBK1201; PMID: 20301380 http://www.ncbi.nlm.nih.gov/books/NBK1201/

McColin, Mia. About NF2. NF Clinic- Neurology and Neurosurgery. Mass General Hospital. http://neurosurgery.mgh.harvard.edu/nfclinic/nf2.htm

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