Neurofibromatosis type 2
Treating neurofibromatosis type 2
Your treatment plan
Due to the rarity of neurofibromatosis type 2 (NF2), the NHS has created four specialist centres where healthcare professional with expertise in treating the condition are based.
The specialist centres are located at:
- Guy’s & St Thomas' Hospital in London
- Addenbrooke’s Hospital in Cambridge
- Central Manchester University Hospitals
- Oxford Radcliffe Hospital
- Addenbrooke’s Hospital in Cambridge
If you are diagnosed with NF2, it is likely that you will be referred to one of these centres so that a treatment plan can be drawn up.
There is currently no cure for NF2. A treatment plan will be based on regularly monitoring how the condition progresses and, if possible, treating symptoms as and when they arise.
Monitoring
Monitoring the progression of NF2 usually involves:
- having yearly MRI scans to check whether any new tumours have developed and whether any existing tumours have grown larger
- having yearly eye tests to check for the presence of cataracts (cloudy patches at the front of the eye)
- having yearly hearing tests to check the extent of any hearing impairment
Depending on the extent and severity of your symptoms, more frequent tests may be required.
Treating symptoms
Tumours
It is possible to surgically remove 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.
For smaller tumours, a non-surgical option is a type of radiotherapy known as the gamma knife. This treatment does not involve the use of an actual knife. The "knife" refers to a tightly focused beam of gamma radiation that can be used to shrink a tumour.
As with surgery, this type of non-surgical treatment is not without risks. There is a possibility that the gamma radiation could cause biological changes to occur in tissue, which could mean that any future tumours that develop are cancerous rather than benign. However, this risk is thought to be very small.
One study found that people with NF2 who were treated with radiotherapy were 10 times more likely to develop cancerous tumours at a later date, compared with people who were not treated. However, as the initial risk of developing a cancerous tumour is relatively small, this increase in risk, although significant, is not as large as it may first appear.
There is no single treatment approach for tumours associated with NF2. A number of factors may influence your recommended treatment plan. These include the number, size and location of any tumours, as well as any other symptoms you have.
Each type of treatment has potential benefits and risks. Discuss these carefully with your care team before deciding on a particular treatment.
Hearing impairment
If you have NF2, your hearing will probably become impaired to such an extent that you will require treatment.
One option is to learn 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.
A type of hearing aid called an auditory brain stem (ABS) implant can also benefit people with NF2.
Before fitting the implant, the surgeon will remove any tumours from the nerves that connect the ears to the brain. Electrodes will then be implanted in the part of the brain that processes sound information.
The electrodes will be connected to a small sound processor chip that is placed outside your ear. The chip captures sound waves as they enter your ear and converts them into electrical impulses, which are transmitted to the electrodes. The ABS implant essentially replicates the work of the hearing nerves.
The implant will not fully restore your hearing. It will only restore some degree of hearing. However, the implant can make lip reading easier. The surgery carries a small risk of serious complications, such as:
- infection on the outer layer of the brain (meningitis), which can occur in around 1 in every 60 cases
- a blood clot inside the lung (pulmonary embolism), which can occur in around 1 in 55 cases
Additional conditions
Click on the links below for more information about more conditions that may affect people with NF2:
