Neurofibromatosis type 2

Neurofibromatosis is the 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).

Types of neurofibromatosis

There are two main types of neurofibromatosis:

• Neurofibromatosis type 1 (NF1) is the most common type of neurofibromatosis, affecting about one in 3,000 people.
• Neurofibromatosis type 2 (NF2) is less common, affecting about one in 35,000 people.

Despite sharing the same name, the two types of neurofibromatosis are separate conditions that have different causes and symptoms.

This information is about neurofibromatosis type 2 (NF2). 

Find out more about neurofibromatosis type 1.

How it affects you

Almost everyone with NF2 will develop tumours on the nerves responsible for hearing and balance. These typically cause symptoms such as:

• gradual hearing loss, that usually gets worse over time 
• ringing or buzzing in the ears (tinnitus)
• balance problems, particularly when moving in the dark or walking on uneven ground

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.

Read more about the symptoms of neurofibromatosis type 2.

Why it happens

NF2 is caused by a genetic mutation. This is where the instructions that are carried in all living cells become scrambled in some way. As a result, the growth of nerve tissue is not controlled properly.

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 on its own. This is known as a 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.

Read more about the causes of neurofibromatosis type 2 and diagnosing neurofibromatosis type 2.

How it's treated

There is currently no cure for NF2. Treatment involves regular monitoring and treating any problems that occur.

Surgery can be used to remove most tumours, although there is a risk this will cause problems such as complete deafness or facial weakness. Therefore, the risks and potential benefits will need to be carefully considered before treatment.

Most people with NF2 eventually develop significant hearing loss and often benefit from learning to lip read or using a hearing aid. Sometimes, special implants can be inserted to aid hearing.

NF2 tends to get worse over time, although the speed at which this happens can vary considerably. However, most people with NF2 will eventually lose their hearing and some people will require a wheelchair or other type of mobility device.

Tumours that develop inside the brain and spinal cord can place a strain on the body and shorten life expectancy. The average life expectancy for someone with NF2 is 65 years of age.

Read more about treating neurofibromatosis type 2.