Neurofibromatosis type 2
Neurofibromatosis is the general name for a number of genetic conditions that cause tumours to grow along your nerves.
Tumours are abnormal tissue growths. In neurofibromatosis, the tumours are usually non-cancerous (benign).
Neurofibromatosis type 1 (NF1) is the most common type, affecting about 1 in 3,000 births. This is covered separately read about Neurofibromatosis type 1 .
These pages focus on neurofibromatosis type 2 (NF2) a less common type, affecting about 1 in 33,000 people.
Almost everyone with NF2 develops tumours on the nerves responsible for hearing and balance. These typically cause symptoms such as:
Tumours can also develop inside the brain or spinal cord, or the nerves to the arms and legs. This can cause symptoms such as weakness in the arms and legs, and persistent headaches.
As a result, the growth of nerve tissue isn't properly controlled.
In half of all cases of NF2, the genetic mutation is passed from a parent to their child. In other cases, the genetic mutation appears to develop spontaneously (sporadic mutation).
Although there are genetic tests that can be carried out during pregnancy to check if your child will have NF2, most cases are diagnosed using tests after birth.
Treatment involves regular monitoring and treating any problemsas theyoccur.
Surgery can be used to remove most tumours, althoughit carriesa riskof causing problems, such as complete deafness or facial weakness. Therefore,the risks and potential benefits need to be carefully considered before treatment.
Most people with NF2 eventually develop significant hearingloss and often benefit from learning to lip read or using a hearing aid. Special implants can sometimes be inserted to improve a person's hearing.
NF2tends toget worse over time, although the speed at which this happens can vary considerably. However, most people with NF2 eventually lose their hearing completely, and some people require a wheelchair or other type of mobility device.
Tumours developing inside the brain and spinal cord can place a strain on the body and shorten life expectancy.However, improved techniques used to diagnose, monitor and treatthe conditionare thought to have increased life expectancy and improved the quality of life for many people with NF2.
This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time.
Neurofibromatosis is the name for a number of genetic conditions that cause tumours to grow along your nerves. This article is related to Neurofibromatosis type 2
Symptoms of neurofibromatosis type 2 (NF2) typically start during the late teens or early twenties, but may develop at any age.
Neurofibromatosis type 2 (NF2) is caused by a genetic mutation in a gene called the NF2. Genes are the instructions that are used to produce all human characteristics.
If you or your child develops symptoms of neurofibromatosis type 2 (NF2), a number of tests can be used to look for signs of the condition.
There is currently no cure for NF2, so management focuses on regular monitoring and, if possible, treating problems as and when they arise.