Neurofibromatosis type 2 - Treating neurofibromatosis type 2

There is currently no cure for NF2, so management focuses on regular monitoring and, if possible, treating problems as and when they arise.

Your specialist care team

Due to the rarity of neurofibromatosis type 2 (NF2), the NHS has created four specialist centres where healthcare professionals with expertise in treating the condition are based.

The specialist centres are located at:

• Guy's and St Thomas' NHS Foundation Trust in London
• Addenbrooke's Hospital in Cambridge
• Central Manchester University Hospitals
• Oxford University Hospitals Trust

If you are diagnosed with NF2, it is likely you will be referred to one of these centres so that a treatment plan can be drawn up.

Monitoring

Everyone with NF2 will require regular monitoring. This is needed to check for signs of any problems developing and arrange treatment if necessary.

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 causes 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.

Surgery

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. These could include a tumour that grows so large that there is a risk of it damaging your brain.

Radiotherapy

For smaller tumours, a type of radiotherapy, known as the gamma knife, may be an option. This treatment does not 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 is not without risks. There is a possibility the gamma radiation could cause biological changes to occur in tissue, which could result in any new tumours becoming cancerous. Although the chances of this happening are thought to be quite small, it will need 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 will 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 then 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 will not fully restore your hearing, they will only restore some degree of hearing. However, they can make lip reading easier (see below).

As with all types of surgery, there is 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 one in every 60 cases. These risks will need to be taken into consideration when deciding on 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 is 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