Rare vestibular conditions: Neurofibromatosis type II
What is neurofibromatosis type II?
Neurofibromatosis type II (NF2) is a rare genetic disorder that causes benign (non-cancerous) tumours of the vestibulocochlear nerve (eighth cranial nerve). These tumours are called acoustic neuromas or vestibular schwannomas, and in people with NF2 they occur on both sides affecting both inner ears. NF2 can be inherited (other family members have this condition) or due to a new gene mutation. The incidence of NF2 is estimated to be 1 in 33,000 people worldwide.
What are the symptoms of NF2?
Symptoms depend on the location and size of the vestibular schwannomas. Symptoms usually first arise during adolescence or young adulthood. Symptoms include:
Problems with balance and walking
Dizziness
Oscillopsia (decreased ability to focus the eyes when moving)
Facial weakness or numbness
Ringing or buzzing in the ears (tinnitus)
Gradual hearing loss
Other symptoms of NF2 may include muscle weakness, loss of vision due to cataracts, and other tumours within the central nervous system (like neurofibromas, meningiomas, ependymomas).
How do you know if I have NF2?
The presence of bilateral vestibular schwannomas is diagnostic of NF2. These tumours are seen on MRI of the brain. You may be sent for hearing and vestibular function tests with an audiologist. Genetic testing for NF2 is available.
Treatment for NF2
Treatment for tumours related to NF2 include surgery and radiation, or a conservative approach of monitoring and waiting if the tumours are slow growing.
Vestibular rehab for NF2
A physiotherapist can help by evaluating your strength, balance, and mobility. A physiotherapist with specific training in vestibular rehabilitation will do an assessment of your inner ear balance function, and determine if specific gaze stability exercises to facilitate compensation for vestibular loss are indicated. Physiotherapy can help to improve your balance and mobility after surgical resection of tumours.
Physiotherapy for NF2 is usually focused on exercises to improve your balance and walking, including an individualized home exercise program. A physiotherapist can determine if any mobility devices or special equipment would help improve your walking pattern or endurance. By doing a thorough evaluation of your balance and gait, a physiotherapist can determine if you are at risk of falling and provide advice on how to prevent falls.
Physiotherapy treatment for NF2 is individualized and focused on maximizing your function and achieving your activity goals.
More NF2 resources:
Acoustic Neuroma Association of Canada
Thumbnail image source: radiopaedia.org
