Almost everybody who has NF2 develops benign tumours called vestibular schwannomas (formerly called acoustic neuromas) which grow on both hearing nerves. Each of these nerves has two parts. The cochlear nerve carries information about sound and the vestibular nerve carries information about balance, to the brain.
Over time these tumours are likely to cause deafness. A tumour on one side may grow at a different rate from the one on the other side. The size of the tumours may bear little relationship to the degree of deafness. For example, a small tumour may produce deteriorating hearing whilst a large tumour may only cause minimal hearing loss.
Other benign tumours associated with nerves inside the body may occur, in particular: meningiomas on the lining of the brain or on the spine, schwannomas on the spine or the skin. Cataracts in the eyes, which are often present from an early age but may not cause significant problems with vision.
Most NF2 tumours are slow-growing and may cause minimal problems for years. Although they are not malignant (non-cancerous) their position may produce significant symptoms.
The most common first symptoms of NF2 are:
● gradual hearing loss, tinnitus (ringing or roaring in the ears)
● unsteadiness, particularly when walking on uneven ground or in the dark
These symptoms are caused by tumours (vestibular schwannomas) on the hearing nerves.
Other symptoms may relate directly to the pressure caused by tumours on the spine or on the lining of the brain. For example: headaches, change in vision, change in sensation, pain or weakness of an arm or leg.
While the problems in NF2 can be worrying, treatments are improving and support is available through the various doctors and therapists who you may be in contact with.