Vestibular disease
CANVAS syndrome
This information is intended as a general introduction to this topic. As everyone is affected differently by balance and dizziness problems, you should speak to your doctor for individual advice.
For reasons of better readability, the generic masculine is used and the simultaneous use of the language forms male, female and diverse (m/f/d) is dispensed with. Unless otherwise indicated, the personal designations used in this patient information refer to all genders.

What is CANVAS syndrome?
CANVAS is an acronym that stands for cerebellar ataxia (CA), neuropathy (N) and vestibular areflexia (VA) syndrome.
CANVAS syndrome is a neurological disorder in which there is a disorder of the brain and/or nerves. It usually begins in adults over the age of 50 and is slowly progressive over 10 years or more.
A syndrome is a collection of problems or symptoms that often occur together. This means that CANVAS syndrome consists of three components:
- The cerebellum is the part of the brain that controls muscle coordination. In the case of ataxia, muscle control or coordination is impaired. Cerebellar ataxia is therefore a disorder of muscle control or coordination that originates in the cerebellum.
- Neuropathy is a disorder of the nerves. Sensory neuropathy is when the affected nerves transmit signals about touch, pressure, pain and temperature. Motor neuropathy is when the affected nerves control muscles and movements. Autonomic neuropathy is when the affected nerves control the functions of your internal organs.
- The vestibular system in the inner ear is an important part of the balance system. Areflexia literally means the absence of reflexes. Vestibular areflexia therefore means that the reflexes of the vestibular system do not function normally. The vestibulo-ocular reflex (VOR) in particular is affected. This reflex ensures that your eyes remain still when your head moves. For example, it allows you to focus on the words on this page, even if you nod your head up and down or turn it back and forth. And when you walk, the VOR keeps your gaze stable while your head bobs up and down. Patients with CANVAS syndrome have bilateral vestibular loss, which means that both inner ears are affected.
CANVAS syndrome was first described in 2004 and scientists are still researching it. It is still unclear whether CANVAS syndrome is a disease in its own right or a collection of symptoms that sometimes occur together. CANVAS syndrome appears to be quite rare
Summary
- CANVAS stands for cerebellar ataxia (CA), neuropathy (N) and vestibular areflexia (VA).
- Is a rare neurological syndrome that affects balance.
- Occurs most frequently in adults over 50 years of age.
- A slowly progressing (progressive) disease.
- There are 3 areas affected: (1) cerebellar ataxia (disorder of muscle control, which is controlled by the cerebellum), (2) neuropathy (disorder of the nerves), (3) vestibular areflexia (the reflexes of the inner ear do not function properly)
- Balance problems can be worse if all 3 areas are active and not just 1 or 2 areas.
- Rotational vertigo is not normally a symptom.
- Treatment mainly consists of controlling the symptoms and avoiding factors that could exacerbate the symptoms.
- Vestibular rehabilitation can help the brain to compensate for the missing inner ear signals.
What causes CANVAS syndrome?
The causes of CANVAS syndrome are still being researched. People with CANVAS syndrome have atrophy (shrinkage) of the cerebellum. This is the part of the brain that controls muscle coordination. They also have damage to certain nerves.
CANVAS syndrome has been found to run in families in some cases, and recently a genetic change (mutation) has been found in some families with CANVAS syndrome. But it is still not clear how this mutation causes CANVAS syndrome. It is also possible that more than one gene may cause CANVAS syndrome in these families.
People with CANVAS syndrome have symptoms in all three areas: Cerebellar ataxia, neuropathy and vestibular areflexia. Sometimes people have symptoms in one or two areas for many years before developing symptoms in all three areas.
The symptoms of cerebellar ataxia can include
- Problems walking; for many people this is the first symptom of CANVAS syndrome
- Balance disorders
- Coordination difficulties, e.g. difficulty picking up objects
- Difficulties with fine motor control, such as writing or eating
- Slow, slurred speech (dysarthria)
- Rapid, involuntary eye movements (nystagmus)
- Difficulty swallowing or the feeling that things are stuck in your throat (dysphagia)
The symptoms of neuropathy can include
- Chronic cough
- Muscle weakness, numbness or pain in the hands and feet (peripheral neuropathy)
- Pain from stimuli that do not normally cause pain (allodynia)
- Unusual sensations such as burning or electric shocks in response to stimuli that do not normally trigger such sensations (dysesthesia)
- Difficulty swallowing (dysphagia)
- Balance problems
- Dizziness or presyncope when sitting or standing up (orthostatic hypotension)
- Feet that are cooler and sweat less than the rest of the body
- Dry mouth or dry eyes
- Constipation
- Erectile dysfunction
The symptoms of vestibular areflexia can include
- Loss of balance (also known as postural instability or loss of postural control)
- Unsteady gait (when walking or running)
- Feelings of instability or dizziness
- In some people, blurred or jumpy vision when the head moves, even when walking or running (oscillopsia)
Balance and gait problems worsen in the dark, on uneven or bouncy ground or on a moving surface. These problems usually disappear when you are sitting or lying still.
People with CANVAS syndrome do not normally have rotary vertigo.
Vestibular problems in CANVAS syndrome always affect both sides of the body.
Balance problems can be worse when all three areas of CANVAS syndrome (cerebellar ataxia, neuropathy and vestibular areflexia) are affected than when only one or two of the areas are affected.
Diagnosis of CANVAS syndrome
CANVAS syndrome can be diagnosed by a neuro-otologist or ENT specialist who specializes in vertigo.
CANVAS syndrome is diagnosed when all three areas are affected: Cerebellar ataxia, neuropathy and vestibular areflexia. Diagnosis is difficult when only one or two areas are affected. It can take more than 10 years before all areas are affected and become apparent. Only then is it diagnosed in most patients.
These specialized doctors will take a thorough medical history, perform a neurological examination and various tests to assess the function of your vestibular system.
You will probably have some of the following diagnostic tests:
- Balance tests
- Vestibular functional examination
- Nerve conduction tests
- Magnetic resonance imaging (MRI)
If you have problems swallowing (dysphagia), your doctor may suggest an examination by a speech therapist. Speech therapists specialize in the assessment and treatment of speech, communication, swallowing and feeding problems.
Your doctor may also suggest genetic testing to rule out other genetic mutations that cause ataxia, such as Friedrich ataxia and spinocerebellar ataxia type 3.
Treatment of CANVAS syndrome
There are currently no treatments available to reverse or slow down the progression of CANVAS syndrome. Treatment mainly consists of reducing the symptoms of CANVAS syndrome and avoiding factors that could exacerbate the symptoms.
Therapy option

Treatment of hypotension
Some people with CANVAS syndrome experience a sudden drop in blood pressure when they sit up or stand up (orthostatic hypotension). This can lead to dizziness, lightheadedness, blurred vision and other problems. To prevent this, your doctor may recommend some or all of the following measures:
- Avoiding certain medications that can exacerbate the problem
- Drink plenty of fluids and make sure you drink enough, especially in warm weather
- Wearing compression stockings
- Taking steroids or medications that increase blood pressure
The IVRT has compiled a list of measures that you can download from our website here.
Therapy option

Treatment of swallowing difficulties
If you have problems swallowing, a speech therapist can help you. Often a certain head position when eating can help with swallowing problems. In some cases, you may need to eat foods with a certain texture or consistency that are easier to swallow.
Therapy option

Treatment of balance problems
If the vestibular system is not working properly, this means that the brain's balance system receives little or no information from the vestibular organ in the inner ear. You then feel unsteady and have balance problems.
If you suffer from neuropathy, your balance disorder is even greater. The brain's balance system receives little or no information from the vestibular and proprioceptive systems (skin, muscles and joints). You will have to rely on the information from your eyes (visual system) to keep your balance.
It is a challenge for your brain to compensate for the missing information from the vestibular and proprioceptive systems. Vestibular rehabilitation can help with this.
Vestibular rehabilitation is an exercise therapy. The goal for people with CANVAS syndrome is to help the brain compensate for the missing signals. A certified IVRT® vertigo and vestibular therapist can help you set treatment goals and create an appropriate program.
A consultation with an occupational therapist can help you to identify factors in your home that could pose a risk of tripping and falling. They can also suggest aids and modifications such as grab rails to help you stand safely.
Your doctor may recommend neurological physiotherapy. This is a form of exercise therapy for people with problems affecting the brain, spinal cord and nerves. It can help to improve walking, strength, endurance and balance. A physiotherapist will work with you to create an exercise program.
What happens next?
What you can expect in the future.
As researchers learn more about CANVAS syndrome in the future, it may become easier to diagnose. Treatments may also become available that can prevent, reverse or slow down CANVAS syndrome.
It is also possible that the definition of CANVAS syndrome will change as we learn more about it. For example, the diagnosis of CANVAS syndrome may one day be limited to people with a specific genetic mutation and specific symptoms.
In order to keep this patient information as short as possible, we have not included a detailed list of references. However, this can be requested at any time at info@ivrt.de.