Treatment for neurofibromatosis type 2 (NF2) involves regular monitoring. Any problems are treated by a team of health professionals.
Because of the rarity of NF2, the NHS has created 4 specialist centres where healthcare professionals with expertise in treating the condition are based.
The specialist centres are located at:
- Addenbrooke's Hospital in Cambridge
- Central Manchester University Hospitals
- Guy's and St Thomas' NHS Foundation Trust in London
- Oxford University Hospitals Trust
If you're diagnosed with NF2, you'll usually be referred to 1 of these centres so a treatment plan can be drawn up.
Everyone with NF2 requires regular monitoring to check for signs of any problems developing and to 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 cloudy patches at the front of the eye (cataracts)
- annual hearing tests to check the extent of any hearing loss
Depending on your symptoms, more-frequent tests may sometimes be required.
Contact your specialist centre if any new symptoms develop between examinations or if existing symptoms get worse.
The growth of tumours is 1 of the main problems associated with NF2. 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 significantly affect your life.
You should discuss the best option for you with your care team before deciding on a treatment.
It's possible to surgically remove some tumours, but the risks 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 there's a risk it may damage your brain.
For smaller tumours, a type of radiotherapy known as the "gamma knife" may be an option. No actual knife is involved – rather, a tightly focused beam of gamma radiation is used to shrink a tumour.
As with surgery, this treatment carries some risks. There's a possibility the gamma radiation could result in any new tumours becoming cancerous. The chances of this are thought to be quite small, but it needs to be considered when weighing up your treatment options.
If you have NF2, it's likely your hearing will get worse over time, so you may benefit from a hearing aid or learning to lip read.
A treatment option may be to surgically implant an electrical device called a hearing implant. There are 2 types of hearing implant used in NF2:
- cochlear implants
- auditory brainstem implants (ABIs)
Cochlear 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. The implants restore only some degree of hearing, but they can make lip reading easier.
As with all types of surgery, there's a risk of complications. Some can be serious, such as infection of the outer layer of the brain (meningitis). These risks need to be considered when deciding the best way to manage your hearing problems.
Another option is learning to lip read. Your treatment centre should be able to recommend a hearing therapist or another healthcare professional qualified to teach lip reading.
NF2 can also cause several other health problems that require different treatments. For example:
- childhood cataracts – usually treated with surgery to replace the cloudy lens with an artificial one
- peripheral neuropathy – usually treated with medicine
- tinnitus – this may be treated with various therapies, such as tinnitus retraining therapy to help you tune out the constant buzzing or ringing noise