Symptoms of neurofibromatosis type 2

Symptoms of neurofibromatosis type 2 (NF2) typically startduring thelate teensor early twenties, butmay develop at any age.

Most ofthe problems are caused by non-cancerous (benign) tumours, whichgrow in various part of the body.

Ear problems

Most people with NF2 develop benign tumours in the nervesused by the brainto helpwith hearing and sense of balance. These are known as vestibular schwannomas.

Vestibular schwannomas can cause ear problems such as:

  • gradual Hearing impairment that usually gets worse over time
  • a constant ringing or buzzing sound( tinnitus )
  • balance problems, such as feeling dizzy this is usually made worse by walking on uneven ground or moving around in the dark

These tumours tend to only cause problems in one ear initially, butboth ears areoften affected eventually.

Less common symptoms ofvestibular schwannomas can include a feeling that your environment is spinning around you ( vertigo ), nausea and vomiting.

It's likely that these tumours willgrow larger over time, eventually causing additional symptoms such as:

  • numbness in parts of your face
  • weakness of your tonguewhich can cause slurred or unusual-sounding speech anddifficulty swallowing( dysphagia )
  • facial painalthough thisis less common

Cataracts

Abouttwo-thirds ofpeople with NF2 develop cataracts. Theseare cloudy patches in the lens (the transparent structure at the front of the eye).

Cataracts can make a persons vision blurred or misty. However, they're usually mild in NF2 and rarely disrupt vision significantly.

Cataracts are normallyassociated with old age, butthey can develop in children and young adults withNF2.

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 skincan 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, calledcaf au lait spots. However, havinglots ofcaf au lait spotsis usually a sign of neurofibromatosis type 1 (NF1) .

Peripheral neuropathy

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:

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

These types of tumours are called meningiomas.

Meningiomas may cause nonoticeable problems. However,they can sometimes lead to an increase in pressure in and around the brain, causingsymptoms such as:

  • headaches
  • vomiting
  • seizures (fits)
  • disturbances in visionsuch as double vision

The tumours can also disrupt certain brain functions. Depending on where in the brain they're located, this may cause:

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

Spinal cord problems

Around half of people with NF2 develop one or more benign tumours inside their spinal cord. These areknown 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.Theremaining half mayexperience:

  • back pain
  • muscle weakness
  • unpleasant physical sensations in certain parts of the bodysuch as numbness, tingling, or a "crawling" sensation on the skin
Content supplied by the NHS Website

Medically Reviewed by a doctor on 29 Nov 2016