Treatment for neurofibromatosis type 2 (NF2) focuses on regular monitoring and, if possible, treating any problems caused by tumours.

Treating Neurofibromatosis Type 2  

Treatment for neurofibromatosis type 2 (NF2) focuses on regular monitoring and, if possible, treating any problems caused by tumours.


Everyone with NF2 requires regular monitoring to check for signs of any problems developing and, if necessary, treatment will be arranged.

Monitoring NF2 usually involves:

  • Annual MRI scans to check whether any new tumours have developed and whether any existing tumours have grown larger.
  • Annual eye tests to check for the presence of cataracts (cloudy patches at the front of the eye).
  • Annual hearing tests to check the extent of any hearing impairment.

Depending on the extent and severity of your symptoms, more frequent tests may sometimes be required.

Contact your specialist centre if any new symptoms develop in between these examinations, or if any existing symptoms get worse.

Treating Tumours

The growth of tumours is one of the main problems associated with NF2 and it's not always obvious what the best treatment is.

Many tumours are small and may not grow large enough to cause any problems, but others can be large and have a significant impact on your life.

You should discuss the best option for you with your care team before deciding on a particular treatment.


It's possible to surgically remove some tumours, but the risks involved can often outweigh the benefits.

For example, removing tumours from the nerve tissue next to your ears could further damage your hearing and cause paralysis of your facial muscles.

Removing tumours from the spinal cord carries a small risk of damaging the spinal cord, which could cause some degree of paralysis.

However, in some cases, surgery may be required to prevent potentially serious complications, such as a tumour growing so large that there's a risk of it damaging your brain.


For smaller tumours, a type of radiotherapy known as the gamma knife may be an option. This treatment doesn't involve the use of an actual knife – it uses a tightly focused beam of gamma radiation to shrink a tumour.

As with surgical removal, this treatment carries some risks. There's a possibility that the gamma radiation could cause biological changes to occur in tissue, which could result in any new tumours becoming cancerous. The chances of this happening are thought to be quite small, but it needs to be considered when weighing up your treatment options.

Treating Hearing Problems

If you have NF2, your hearing will probably become impaired to such an extent that you'll require treatment.

Hearing Aids

One option may be to consider a hearing implant. These are surgically implanted electrical devices used to bypass problems in the hearing mechanism. There are two types of hearing implants used in NF2, called cochlear implants and auditory brainstem implants (ABIs).

Cochlea implants and ABIs have an external microphone that receives and processes sounds. These signals are passed into an internal receiver before being carried through wires to electrodes either in the cochlea (the coiled, spiral tube inside the inner ear) or the brainstem.

If you have an ABI fitted, the surgeon will first remove any tumours from the hearing nerves. These implants only restore some degree of hearing. However, they can make lip reading easier (see below).

As with all types of surgery, there's a risk of complications. Some of these can be serious, such as infection on the outer layer of the brain (meningitis), which can occur in around 1 in every 60 cases. These risks need to be taken into consideration when deciding the best way to manage your hearing problems.

Lip Reading

Another option is learning to lip read. Your treatment centre should be able to recommend a hearing therapist or another healthcare professional who's qualified to teach lip reading.

Treating Other Problems

NF2 can also cause several other health problems, which require different treatments. For example, NF2 can cause:

  • Childhood cataracts - which is usually treated with surgery to remove the cloudy lens and replace it with a clear artificial lens.
  • Peripheral neuropathy - which is usually treated with medication.
  • Tinnitus - which may be treated with a number of different therapies, such as tinnitus retraining therapy to help you tune out the constant buzzing or ringing noise.