Symptoms - Neurofibromatosis type 2

The symptoms of neurofibromatosis type 2 (NF2) typically start during the late teens or early 20s, but they may develop at any age.

Most of the problems are caused by non-cancerous (benign) tumours growing in various parts of the body.

Ear problems

Most people with NF2 develop non-cancerous tumours along the nerves used by the brain to help with hearing and balance. The tumours are known as vestibular schwannomas and can cause problems such as:

• hearing loss that gradually gets worse over time
• a constant ringing or buzzing sound (tinnitus)
• balance problems, such as feeling dizzy – usually made worse by walking on uneven ground or moving around in the dark

The tumours tend to only cause problems in 1 ear at first, but both ears are often affected eventually.

Less common symptoms include vertigo – when it feels like you or everything around you is spinning – and feeling and being sick.

It's likely the tumours will grow larger over time, eventually causing additional symptoms such as:

• numbness in parts of your face
• weakness of your tongue – this can cause slurred or unusual-sounding speech and difficulty swallowing (dysphagia)
• facial pain – although this is less common

Cataracts

About 2 in 3 people with NF2 develop cloudy patches in the lens of the eye (cataracts).

Cataracts can make a person's vision blurred or misty. However, they're usually mild in NF2 and rarely cause serious vision problems.

Cataracts are normally associated with old age, but they can develop in children and young adults with NF2.

Read more about childhood cataracts

Skin problems

Just over half of people with NF2 develop benign tumours on or underneath the surface of their skin. These are called schwannomas.

They often take the form of skin plaques: small, coloured, raised patches of skin, usually less than 2cm across.

Tumours that develop under the skin can grow to around the size of a golf ball and can be painful if they develop along a section of nerves.

Some people with NF2 may also develop a small number of coffee-coloured patches on their skin, called café au lait spots. But having lots of these spots is usually a sign of neurofibromatosis type 1 (NF1).

Peripheral neuropathy

Many people with NF2 will develop a condition called peripheral neuropathy. Peripheral neuropathy can cause a wide range of symptoms, including:

• pins and needles in the affected body part
• numbness and a reduced ability to feel pain or temperature changes – particularly in your feet
• a burning pain – usually in the feet and legs, followed by the hands and arms as the neuropathy progresses
• muscle weakness

Read more about peripheral neuropathy

Brain problems

Around 1 in 2 people with NF2 develop 1 or more benign tumours inside their brain. These 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:

• headaches
• being sick
• seizures (fits)
• vision disturbances – such as double vision

The tumours can also disrupt certain brain functions. Depending on where they are, they may cause:

• personality changes
• weakness or numbness in 1 side of the body
• difficulty speaking, understanding words, writing and reading (aphasia)
• vision problems
• fits or blackouts
• memory problems
• a loss of smell (anosmia) or a sensation of strange smells (phantosmia)
• unsteadiness, loss of co-ordination and difficulty walking
• difficulty speaking and swallowing

Spinal cord problems

Around 1 in 2 people with NF2 develop 1 or more benign tumours inside their spinal cord. These are called ependymomas.

Of those who develop ependymomas, about half will not have any noticeable symptoms. But those who do may experience:

• back pain
• muscle weakness
• unpleasant physical sensations in certain parts of the body – such as numbness, tingling, or a "crawling" sensation on the skin