Symptoms of neurofibromatosis type 2 (NF2) typically start during the late teens or early twenties, but may develop at any age.
Most of the problems are caused by non-cancerous (benign) tumours, which grow in various part of the body.
Most people with NF2 develop benign tumours in the nerves used by the brain to help with hearing and sense of balance. These are known as vestibular schwannomas.
Vestibular schwannomas can cause ear problems such as:
These tumours tend to only cause problems in one ear initially, but both ears are often affected eventually.
Less common symptoms of vestibular schwannomas can include a feeling that your environment is spinning around you (vertigo), nausea and vomiting.
It's likely that these tumours will grow larger over time, eventually causing additional symptoms such as:
About two-thirds of people with NF2 develop cataracts. These are cloudy patches in the lens (the transparent structure at the front of the eye).
Cataracts can make a person’s vision blurred or misty. However, they're usually mild in NF2 and rarely disrupt vision significantly.
Cataracts are normally associated with old age, but they can develop in children and young adults with NF2.
Just over half of people with NF2 develop benign tumours on or underneath the surface of their skin. These are called schwannomas.
These often take the form of skin plaques. These are small, coloured, raised patches of skin that are usually less than 2cm across.
Tumours that develop under the skin can grow to around the size of a golf ball and they can be painful if they develop along a section of nerves.
Some people with NF2 also develop one or two coffee-coloured patches on their skin, called café au lait spots. However, having lots of café au lait spots is usually a sign of neurofibromatosis type 1 (NF1).
Many people with NF2 will develop a condition called peripheral neuropathy. Peripheral neuropathy is where a section of the nervous system, known as the peripheral nervous system, becomes damaged.
Peripheral neuropathy can cause a wide range of symptoms, depending on which sections of the peripheral nervous system are damaged. Possible symptoms include:
Around half of people with NF2 develop one or more benign tumours inside their brain. These types of tumours are called meningiomas.
Meningiomas may cause no noticeable problems. However, they can sometimes lead to an increase in pressure in and around the brain, causing symptoms such as:
The tumours can also disrupt certain brain functions. Depending on where in the brain they're located, this may cause:
Around half of people with NF2 develop one or more benign tumours inside their spinal cord. These are known as ependymomas.
The spinal cord is a long band of nerves that runs from your brain down the centre of the spine.
Around half of people who develop ependymomas won't have any noticeable symptoms. The remaining half may experience: