Introduction 

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.

What are the symptoms of NF2?

Almost everyone with NF2 develops 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 NF2.

What is the cause?

NF2 is caused by a genetic mutation, which is a permanent change in the DNA sequence that makes up a gene. 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.

Read more about the causes of NF2 and diagnosing NF2.

How is it treated?

There's currently no cure for NF2. Treatment involves regular monitoring and treating any problems as they occur.

Surgery can be used to remove most tumours, although it carries a risk of 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 hearing loss 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.

NF2 tends to get 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 treat the condition are thought to have increased life expectancy and improved the quality of life for many people with NF2.

Read more about treating NF2.

The Neuro Foundation

The Neuro Foundation is a UK charity that aims to improve the lives of people with neurofibromatosis (either type 1 or type 2).

For more information, you can visit The Neuro Foundation website, call its helpline on 020 8439 1234, or email info@nfauk.org.

Information about you

If you or your child has NF2, your clinical team will pass information about you/your child on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS).

This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time.

Find out more about the register.

Tinnitus

A common symptom of NF2 is tinnitus. Tinnitus causes people to hear constant sounds in one ear, both ears or in their head. An audiologist explains the possible causes and effects it often has, and how to deal with them. Ashleigh, who was diagnosed with tinnitus in 2005, describes how she copes with it.

Media last reviewed: 12/05/2016

Next review due: 12/05/2018

Page last reviewed: 13/03/2015

Next review due: 13/03/2017