Cerebellar Ataxia with Bilateral Vestibulopathy Syndrome

Cerebellar ataxia with bilateral vestibulopathy syndrome is a rare brain and inner-ear disorder where two main problems happen together: damage in the cerebellum (the part of the brain that controls coordination) and loss of function in both vestibular systems (the balance organs in the inner ears). This combination causes serious problems with balance, walking, and keeping vision clear when the head moves.Vestibular Health+1

Cerebellar ataxia with bilateral vestibulopathy (CABV) is a rare brain and inner-ear balance disorder. “Cerebellar ataxia” means poor coordination because the cerebellum (the balance and coordination part of the brain) is not working normally. “Bilateral vestibulopathy” means both inner ears have lost much of their balance function. People feel very unsteady, especially in the dark or on uneven ground. They may have shaky vision when they walk (oscillopsia), clumsy leg and arm movements, and trouble walking in a straight line.Frontiers+4PubMed+4Vestibular Health+4

CABV is usually slowly progressive over many years. At the moment, doctors do not have a cure. Treatment focuses on reducing dizziness, improving balance and walking, preventing falls, managing anxiety and depression, and treating any related problems such as neuropathy or chronic cough. Vestibular rehabilitation and other forms of physiotherapy are the main evidence-based treatments. Medicines are mostly used for symptoms, not to stop the disease itself.Wikipedia+4Vestibular Health+4Frontiers+4

People may feel very unsteady, especially in the dark or on uneven ground, sway when standing still, and often need to widen their feet to keep balance. Over many years, walking can become harder, and some people may eventually need a cane, walker, or wheelchair, although progression is usually slow.Vestibular Health+1

In many patients this syndrome overlaps with, or is part of, a condition called CANVAS (Cerebellar Ataxia, Neuropathy, and Vestibular Areflexia Syndrome), which is usually genetic and starts in mid-life.PubMed+2Wikipedia+2

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Other names and types

Other names you may see

Doctors and researchers may use several names for very similar or overlapping conditions:

• Cerebellar ataxia with bilateral vestibulopathy (CABV) – the classic name for this combined syndrome.OUP Academic

• Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) – when sensory nerve damage (neuropathy) is clearly present along with cerebellar ataxia and vestibular loss.Wikipedia+1

• Cerebellar ataxia with bilateral vestibular areflexia – stresses that the vestibular reflexes are absent or very weak.PMC

• RFC1-related CANVAS / RFC1-related ataxia – used when genetic testing finds the RFC1 repeat expansion that is now known to cause many CANVAS-like cases.PubMed+1

Types (ways doctors may classify it)

1. Idiopathic CABV – people who have the typical combination (cerebellar ataxia + bilateral vestibulopathy) but no clear cause even after tests.OUP Academic

2. RFC1-related CANVAS type – cases caused by biallelic AAGGG repeat expansion in the RFC1 gene; usually late-onset and slowly progressive.PubMed+2Wikipedia+2

3. Other genetic ataxia with bilateral vestibulopathy – some spinocerebellar ataxias and other hereditary ataxias can show very similar combined cerebellar and vestibular problems.Frontiers+1

4. Secondary / syndromic forms – where the combined cerebellar and vestibular damage happens as part of a wider degenerative or systemic disease (for example multiple system atrophy or other complex neurodegenerative syndromes).Frontiers+1

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Causes

For many people, the exact cause of cerebellar ataxia with bilateral vestibulopathy is still not found even after many tests. But research shows several important possible causes or associated conditions.

1. RFC1 gene expansion (CANVAS)
   A very common cause of CANVAS and many cases of late-onset ataxia is a specific expansion (many extra repeats) of the AAGGG sequence in the RFC1 gene. This is inherited from both parents and leads to slow degeneration of cerebellum, peripheral nerves, and vestibular system.PubMed+2Wikipedia+2

2. Other inherited cerebellar ataxias (e.g., spinocerebellar ataxias)
   Some genetic ataxias, like spinocerebellar ataxias, can involve both the cerebellum and vestibular system, giving a CABV-like picture. In these cases, mutations affect proteins that keep neurons healthy.Frontiers+1

3. Idiopathic degenerative disease
   In some patients, no genes or external triggers are found. Doctors then call it idiopathic, meaning “of unknown cause.” Over time, cells in the cerebellum and vestibular system slowly degenerate, leading to the combined syndrome.OUP Academic

4. Multiple system atrophy (MSA)
   MSA is a progressive disorder that can affect many brain regions, including cerebellum and brainstem pathways linked to the vestibular system. Some people with MSA show ataxia and vestibular problems similar to CABV.Frontiers

5. Other neurodegenerative disorders
   Conditions like late-onset hereditary ataxias or complex multisystem degenerations may damage both cerebellum and vestibular pathways over time, giving overlapping features with CABV or CANVAS.Frontiers+1

6. Toxic damage from certain medicines (e.g., aminoglycosides)
   Some antibiotics (like gentamicin) and chemotherapy drugs are known to damage vestibular hair cells in the inner ear. If a person already has mild cerebellar problems, added vestibular loss from medications can produce a CABV-like syndrome.Frontiers

7. Autoimmune cerebellar ataxia
   In some autoimmune diseases, the immune system attacks the cerebellum and sometimes the vestibular nerves or nuclei. Autoimmune ataxias, including gluten ataxia or paraneoplastic cerebellar degeneration, can contribute to combined balance problems.Frontiers+1

8. Paraneoplastic syndromes (due to cancer-related antibodies)
   Certain cancers can trigger antibodies that mistakenly attack parts of the nervous system, including cerebellum and vestibular pathways. This “paraneoplastic ataxia” can give severe imbalance and unsteady gait.Frontiers

9. Chronic alcohol-related cerebellar degeneration
   Long-term heavy alcohol use can damage the cerebellum, leading to ataxia. If there is also vestibular damage (for example from associated nutritional problems or toxicity), the picture can resemble CABV.Frontiers

10. Vitamin B1 (thiamine) deficiency
    Very low thiamine can cause cerebellar ataxia and vestibular symptoms as seen in Wernicke’s encephalopathy and related conditions. Replacement of the vitamin is essential, but some damage may remain.Frontiers

11. Vitamin B12 deficiency
    Low B12 levels damage the spinal cord and peripheral nerves, causing loss of sensation and imbalance. When combined with cerebellar dysfunction, this can worsen coordination and gait.Frontiers+1

12. Vitamin E deficiency
    Vitamin E protects nerve cells from oxidative damage. Severe deficiency can cause progressive ataxia and neuropathy, sometimes mistaken for hereditary ataxia, and can contribute to CABV-like symptoms.Frontiers+1

13. Chronic small-vessel ischemia or stroke affecting cerebellum and brainstem
    Repeated small strokes or one larger stroke involving cerebellum and vestibular nuclei can cause long-lasting ataxia and vestibular deficits if both sides are affected.Frontiers

14. Infections involving the brain or inner ear
    Certain infections (like viral or bacterial labyrinthitis, meningitis, or encephalitis) can damage both the vestibular organs and cerebellar structures, leaving permanent imbalance and dizziness.Frontiers

15. Inflammatory demyelinating conditions
    Diseases that destroy myelin (the coating of nerves), such as some variants of multiple sclerosis, can affect cerebellar pathways and brainstem vestibular tracts, causing combined symptoms.Frontiers+1

16. Tumors in the cerebellum or cerebellopontine angle
    Tumors near the cerebellum and vestibular nerve (such as vestibular schwannoma or metastases) may damage both structures directly or by pressure, leading to ataxia and vestibular loss.Frontiers

17. Head trauma involving posterior fossa or temporal bone
    Severe trauma can injure the cerebellum and inner ears together. After healing, some people are left with permanent imbalance and oscillopsia.Frontiers

18. Toxic exposure to industrial chemicals or heavy metals
    Long-term exposure to certain solvents or metals can harm the nervous system, including cerebellum and vestibular system, although this is rarer and often hard to prove.Frontiers+1

19. Mitochondrial disorders
    Some mitochondrial diseases (conditions affecting cellular energy production) can involve many brain regions at once, including cerebellum, peripheral nerves, and vestibular structures, producing mixed ataxia and vestibular symptoms.Frontiers

20. Unknown or mixed causes
    Even with advanced testing, some people never get a clear answer. In these cases, doctors may suspect a mix of genetic vulnerability and environmental factors that slowly damage cerebellar and vestibular systems over time.OUP Academic+1

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Common symptoms

1. Unsteady, wide-based walking (gait ataxia)
   People often walk with their feet far apart and may sway side to side. This wide base is the body’s way of trying to keep balance when the cerebellum and vestibular system are not working well.Vestibular Health+1

2. Trouble walking in the dark or on uneven ground
   Vision normally helps balance. In this syndrome, when it is dark or the floor is soft or uneven, the person feels much more unsteady because both vestibular input and cerebellar coordination are impaired.Vestibular Health+1

3. Frequent falls
   Falls may happen when turning quickly, walking on slopes, or in busy environments. Bilateral vestibular loss and cerebellar ataxia both increase fall risk, so fall prevention is a big focus of care.Vestibular Health+2ScienceDirect+2

4. Oscillopsia (blurry or “bouncy” vision when moving)
   Because the vestibulo-ocular reflex is weak or absent, the eyes cannot stay steady when the head moves. People see the environment jump or blur with each step or head movement.Vestibular Health+2PubMed+2

5. Dizziness or sense of imbalance, especially when walking
   Many patients do not feel spinning vertigo but instead describe a constant feeling of being off-balance, like walking on a boat or trampoline.Vestibular Health+1

6. Difficulty coordinating arms and hands (limb ataxia)
   Fine tasks like buttoning clothes, writing, or picking up small objects can become clumsy because cerebellar control of limb movements is affected.Wikipedia+1

7. Slurred or slow speech (dysarthria)
   The cerebellum helps coordinate the muscles used for speech. Damage can make speech sound slow, slurred, or “scanning,” especially when the person is tired.Wikipedia+1

8. Involuntary eye movements (nystagmus)
   Quick, jerky eye movements may be seen when the person looks to the side or when they try to fix their gaze. This comes from cerebellar and vestibular imbalance.Vestibular Health+1

9. Numbness or tingling in feet and hands (if neuropathy is present)
   In CANVAS and related conditions, the sensory nerves often become damaged. People may feel pins and needles, burning, or numbness, especially in the feet.PubMed+2Wikipedia+2

10. Poor sense of joint position (loss of proprioception)
    Some patients cannot tell where their feet are without looking. This makes standing with eyes closed almost impossible and greatly worsens balance.PubMed+2Wikipedia+2

11. Difficulty swallowing (dysphagia)
    The muscles and coordination needed for swallowing can be affected in these degenerative syndromes, leading to choking or coughing when eating or drinking.Wikipedia+1

12. Chronic dry cough (especially in CANVAS)
    Many people with CANVAS have a long-lasting, unexplained dry cough that can appear years before balance problems. This cough is thought to be related to nerve dysfunction.Wikipedia+1

13. Autonomic symptoms (blood pressure or bladder problems)
    Some individuals have dizziness when standing up, bowel or bladder changes, or temperature regulation issues because the autonomic nervous system can be involved.Wikipedia+2www.elsevier.com+2

14. Fatigue and reduced stamina
    Constant effort to stay upright and focus visually is exhausting. People may feel tired after short walks or normal daily activities.ScienceDirect+1

15. Emotional impact (anxiety, low mood)
    Living with chronic imbalance, fear of falling, and gradual loss of independence can lead to anxiety or depression. Psychological support and reassurance can be very helpful.Wikipedia+1

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Diagnostic tests

Doctors use a mix of clinical examination, bedside maneuvers, lab tests, and imaging to diagnose cerebellar ataxia with bilateral vestibulopathy and to search for the cause.

Physical exam tests

1. Full neurological examination
   The doctor looks at strength, reflexes, coordination, eye movements, and sensation. In this syndrome, they often find limb ataxia, abnormal eye movements, and signs of sensory loss, which together suggest a combined cerebellar and vestibular problem.Wikipedia+1

2. Gait and balance assessment
   The doctor watches how the patient walks, turns, and stands. A wide-based, unsteady gait with difficulty on uneven surfaces and during head turns is typical of combined cerebellar and vestibular disease.Vestibular Health+1

3. Romberg test
   The person stands with feet together, first with eyes open, then closed. In bilateral vestibulopathy and sensory loss, closing the eyes usually makes sway and instability much worse, helping confirm the diagnosis.Vestibular Health+1

4. Heel-to-toe (tandem) walking
   The doctor asks the patient to walk in a straight line placing the heel of one foot just in front of the toes of the other. Strong wobbling or inability to do this supports the presence of cerebellar ataxia.Vestibular Health+1

5. Finger-to-nose and heel-to-shin tests
   These tests check arm and leg coordination. Overshooting, shaky movements, or difficulty hitting the target show limb ataxia caused by cerebellar involvement.Wikipedia+1

Manual bedside vestibular tests

6. Head impulse test (HIT)
   The doctor rapidly turns the patient’s head while the patient tries to keep looking at a fixed target. In bilateral vestibulopathy, the eyes cannot stay on target, and “catch-up” saccades appear, showing loss of vestibulo-ocular reflex.PubMed+2Frontiers+2

7. Visually enhanced vestibulo-ocular reflex (VVOR) / doll’s head manoeuvre
   This is a key sign in CABV. The patient fixes eyes on a moving target while the head is rotated. In normal people, the eyes stay locked on the target; in CABV, the VVOR is impaired and the eyes slip, confirming combined cerebellar and vestibular dysfunction.PubMed+2PMC+2

8. Dynamic visual acuity test
   The examiner checks how well the person can read letters on a chart while the head is still and then while the head is moving. A big drop in visual acuity during head movement supports bilateral vestibular loss.Vestibular Health+1

9. Positional tests (e.g., lying to sitting, turning in bed)
   The doctor observes dizziness, nystagmus, and balance when the patient changes position. Lack of typical positional vertigo patterns but strong imbalance can point towards bilateral vestibulopathy rather than benign positional vertigo.Frontiers+1

10. Timed functional balance tasks
    Simple tasks such as standing on foam, walking with head turns, or the Timed Up and Go test help measure how much daily function is affected and monitor progression or response to therapy.Vestibular Health+1

Lab and pathological tests

11. Routine blood tests (CBC, metabolic panel, thyroid)
    These tests screen for common, treatable causes of ataxia and vestibular symptoms, such as anemia, electrolyte problems, liver or kidney disease, and thyroid disorders, and help rule out other illnesses.Frontiers+1

12. Vitamin level tests (B1, B12, E, folate)
    These check for nutritional deficiencies that can cause or worsen ataxia and neuropathy. Treating any deficiency is important even if a genetic cause like RFC1 expansion is also present.Frontiers

13. Autoimmune and paraneoplastic antibody panels
    Blood tests may look for antibodies against cerebellar or neuronal antigens, which could suggest autoimmune ataxia or paraneoplastic syndromes and sometimes change the treatment plan.Frontiers+1

14. Infection screening (e.g., syphilis, HIV, Lyme disease, hepatitis)
    Certain infections can affect the nervous system and inner ear. Screening helps detect treatable infectious causes of ataxia and vestibular loss.Frontiers

15. Genetic testing (including RFC1 repeat expansion)
    Genetic tests can confirm RFC1-related CANVAS or other hereditary ataxias. In CANVAS, doctors specifically look for a biallelic AAGGG repeat expansion in the RFC1 gene, often using genome or targeted repeat testing.PubMed+2Wikipedia+2

Electrodiagnostic tests

16. Nerve conduction studies (NCS)
    These tests measure how fast and how strongly electrical signals travel along peripheral nerves. In CANVAS and related conditions, they often show sensory neuronopathy or neuropathy, which helps explain numbness and imbalance.PubMed+2Wikipedia+2

17. Electromyography (EMG)
    EMG studies can show whether muscles and motor nerves are working normally. They help separate muscle disease from nerve or cerebellar problems and may show changes in chronic neuropathy.Frontiers

18. Videonystagmography (VNG) or electronystagmography
    These tests record eye movements while the head is moved or while visual and positional stimuli are given. They document abnormal nystagmus patterns and poor vestibulo-ocular reflex, supporting bilateral vestibular failure.Frontiers+1

Imaging and vestibular function tests

19. MRI of the brain (especially cerebellum and brainstem)
    MRI often shows cerebellar atrophy (shrinkage), especially of the vermis and hemispheres, and helps rule out tumors, strokes, or other structural causes. In CANVAS, characteristic cerebellar changes are frequently seen.Wikipedia+2Global Genes+2

20. Formal vestibular testing (caloric tests, rotational chair, head impulse gain)
    Specialized vestibular tests measure each ear’s function. In bilateral vestibulopathy, responses are reduced or absent on both sides, confirming that the vestibular organs are not working properly.PubMed+2Frontiers+2

Non-pharmacological treatments

1. Vestibular rehabilitation therapy (VRT)
Vestibular rehabilitation is a special type of physical therapy program for people with balance and dizziness problems. Exercises usually include balance training, walking practice, and head–eye coordination tasks. For CABV, programs are adapted because both inner ears and the cerebellum are involved. The goal is to teach the brain to use vision and body-sense more effectively to keep balance. Strong clinical guidelines recommend vestibular rehab for people with bilateral vestibular hypofunction because it improves balance, walking, and quality of life.Aan+4PubMed+4Vestibular Disorders Association+4

2. Gaze-stabilization (VOR) exercises
Gaze-stabilization exercises train your eyes to stay focused while your head is moving. A common exercise is to stare at a letter or thumb in front of you while slowly turning your head side to side or up and down. In CABV, these exercises help the remaining visual and brain pathways work harder to keep vision steady. Over time, this can reduce “bouncy” or blurry vision when walking, a key problem in bilateral vestibulopathy.avcphysio.com.au+4umc.edu+4brainandspine.org.uk+4

3. Balance and gait training
Balance and gait (walking) training use step practice, narrow-base walking, turning, and obstacle walking to improve stability. In CABV, people often sway or fall when they turn or walk in dim light. Therapists may use dual-task practice (walking while counting or turning the head) to make walking safer in real life. Repeated practice helps the brain learn safer movement patterns even though the cerebellum and vestibular organs are damaged.PMC+2SAGE Journals+2

4. Coordination exercises for arms and legs
Coordination exercises target the cerebellar component of CABV. Tasks like finger-to-nose, heel-to-shin, rapid alternating hand movements, and stepping drills are practiced slowly and then more quickly. The purpose is to reduce clumsiness, mis-reaching, and overshooting movements. Even though cerebellar cells are damaged, practicing smooth, slow patterns can help remaining circuits work more efficiently and reduce falls caused by mis-stepping.PubMed+1

5. Strength and conditioning programs
Weak hip, leg, and core muscles make balance problems worse. A structured strengthening program (for example, sit-to-stand practice, step-ups, resistance bands, or light weights) helps the body support itself better. For CABV, extra muscle strength gives a “safety margin” when the brain’s balance systems fail, so the person is less likely to buckle or collapse when pushed or when they trip.

6. Sensory substitution and strategy training
Because the inner ears are weak, the brain must depend more on other senses like vision and the feeling from feet and joints. Therapists teach patients to use good lighting, solid shoes, and visual reference points. People learn to look at the horizon or at nearby objects while walking, and to feel the ground through firm footwear. This “sensory substitution” helps compensate for vestibular loss.Physiopedia+1

7. Use of assistive devices (canes, walkers, trekking poles)
Walking aids are not a failure; they are safety tools. A cane, walker, or trekking pole provides an extra point of contact with the ground. In CABV, this can be the difference between staying upright and falling badly. Devices are chosen and adjusted by a therapist to avoid over-reliance and to keep posture as natural as possible. The mechanism is simple: extra weight-bearing points increase stability and widen the base of support.

8. Home and environmental modifications
Simple environment changes greatly cut fall risk. This includes removing loose rugs, adding grab bars, improving bathroom lighting, using non-slip mats, and keeping walkways clear. For CABV, dark hallways and uneven outdoor paths are especially dangerous. The purpose is to reduce sudden surprises to the balance system. The mechanism is prevention: fewer hazards means fewer situations where the damaged cerebellar and vestibular systems are overwhelmed.

9. Fall-prevention education and planning
Education teaches people how to turn slowly, avoid quick head movements, and use handrails. Patients and families learn what to do if a fall happens, how to get up safely, and when to call for help. For CABV, this training reduces fear of falling and real injury risk. It works by changing everyday habits so that movements stay within safe limits for the damaged balance system.

10. Occupational therapy for daily activities
Occupational therapists focus on dressing, bathing, cooking, and other daily tasks. They may suggest shower seats, raised toilet seats, and kitchen rearrangement so items are within easy reach. For CABV, they also train energy-saving strategies and safe transfers (for example, getting in and out of bed). These changes reduce the brain load needed for balance during complex tasks.

11. Vision and eye-movement training
Many people with CABV have nystagmus and difficulty keeping their eyes fixed on a target. Vision-focused exercises train smooth pursuit, saccades, and reading strategies like using a line guide. Although they cannot fully correct eye movement pathways, they help the person find positions and strategies that reduce visual blurring and discomfort.

12. Habituation exercises for motion sensitivity
Habituation means repeated exposure to movements that cause dizziness, but in a controlled way. Patients practice turning their head, bending over, or lying down and sitting up, starting with small movements and building up. For CABV, this helps the brain become “less alarmed” by certain motions, reducing the intensity of dizziness even though the vestibular damage remains.Neuropt+1

13. Task-specific gait training (stairs, outdoor surfaces)
Therapists often practice stair climbing, curb stepping, walking on grass, gravel, or ramps. CABV patients are especially unstable on these surfaces, so supervised practice makes a big difference. The purpose is to copy real-world challenges in a safe environment. With repetition, the brain learns more efficient patterns and the person gains confidence.Medbridge+1

14. Tai chi and slow balance exercises
Tai chi and similar slow movement practices can improve balance, ankle strength, and body awareness. Studies in older adults and people with balance disorders show better stability and fewer falls with regular practice. For CABV, the slow, focused movements are useful because they allow the damaged cerebellar and vestibular systems more time to process, while still challenging posture control.

15. Yoga and flexibility training
Gentle yoga builds flexibility, core strength, and breathing control. Poses are modified to avoid sudden head movements or positions that trigger dizziness. The goal is better posture and muscle control. For CABV, improved posture can make walking less tiring and reduce strain on joints and muscles that are compensating for poor balance.

16. Aquatic (water-based) therapy
Exercising in warm water lets people practice balance and walking with reduced risk, because the water supports body weight. In CABV, water therapy can allow larger stepping and turning movements without fear of falling. The buoyancy and resistance also strengthen muscles, and the gentle sensory input from water helps the brain integrate body-position signals.

17. Vibrotactile or audio biofeedback systems
Some rehab centers use belts, insoles, or headphones that give vibration or sound feedback when the body tilts too far. For bilateral vestibulopathy, these devices act as an “artificial balance sense.” Early studies show that such sensory augmentation can improve stability in people with chronic vestibular loss.Frontiers

18. Psychological support and cognitive-behavioural therapy (CBT)
Living with CABV can cause anxiety, depression, and fear of falling. Psychological therapy, including CBT, helps people manage worry, low mood, and social withdrawal. The purpose is not to “fix” the brain damage but to improve coping skills. Better mental health supports motivation to continue rehab, which indirectly improves physical outcomes.

19. Education and self-management programs
Clear explanations about the disease, prognosis, and realistic goals reduce confusion and fear. Education also covers medication side effects, safe activity levels, and how to monitor symptoms. In CABV, understanding that the condition is chronic but manageable can encourage long-term engagement in exercise and healthy habits.

20. Social and community support, including support groups
Meeting others with balance disorders, in person or online, reduces isolation. Sharing tips on coping with dizziness, fatigue, and lifestyle changes can be very helpful. Social support improves mood and adherence to therapy. For CABV, where the disease is rare, support groups for vestibular disorders or ataxia in general may still be valuable.

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Drug treatments

Important: Doses and schedules below must always be decided by a neurologist or ENT specialist. Many of these medicines are used off-label for CABV, but are approved by regulators such as the U.S. FDA for other conditions like multiple sclerosis, migraine, or neuropathic pain.Springer+4PMC+4www.elsevier.com+4

1. 4-aminopyridine / dalfampridine
4-aminopyridine (sustained-release dalfampridine) is a potassium-channel blocker originally approved to improve walking in multiple sclerosis. In some cerebellar ataxias, low doses can improve gait and reduce nystagmus by making nerve signals in the cerebellum fire more regularly. Studies show benefit in certain cerebellar gait ataxias and nystagmus, and small reports suggest it may help some people with CABV-like syndromes. It is prescription-only, and can cause seizures or tingling at high doses, so careful specialist supervision is essential.epub.ub.uni-muenchen.de+4PMC+4Springer+4

2. Acetyl-DL-leucine
Acetyl-DL-leucine is an amino-acid derivative used in some countries for vertigo. Small studies and case series in cerebellar ataxia suggest it may improve coordination, walking stability, and dizziness for some patients. It appears to change how nerve cells handle certain metabolic pathways, possibly stabilizing their firing. Evidence is still limited, and it is often used off-label in specialist centers. Side effects can include mild stomach upset or headache.

3. Gabapentin
Gabapentin is an anti-seizure medicine widely used for nerve pain. In CABV, it may help if the person also has painful sensory neuropathy, a problem seen in related syndromes like CANVAS. By calming over-active pain signals, it can improve sleep and activity levels. Common side effects include sleepiness, dizziness, and weight gain, so doses are started low and increased slowly.

4. Pregabalin
Pregabalin is similar to gabapentin and is approved for neuropathic pain and anxiety disorders. For CABV, it can reduce burning or shooting pains from sensory nerve damage and may also ease generalized anxiety about dizziness. It works by modulating calcium channels in nerve cells, reducing abnormal signalling. Side effects can include drowsiness, swelling of legs, and blurred vision.

5. Duloxetine
Duloxetine is an antidepressant that also treats neuropathic pain. It increases serotonin and norepinephrine in the brain and spinal cord. For people with CABV who have both low mood and nerve pain, duloxetine can address both problems at once. It may improve energy and participation in rehab. Side effects can include nausea, dry mouth, and changes in sleep.

6. Amitriptyline
Amitriptyline is a tricyclic antidepressant often used at low doses for nerve pain, migraine, and poor sleep. In CABV, it may help if there is neuropathic pain or chronic headache. It works by changing how pain pathways process signals. Common side effects include dry mouth, constipation, drowsiness, and sometimes weight gain, so doctors choose dose carefully and avoid in people with certain heart problems.

7. Clonazepam
Clonazepam is a benzodiazepine that can reduce certain types of nystagmus and severe motion-triggered vertigo by calming brain activity. In CABV, it may be used short term for intense oscillopsia or anxiety. However, long-term use can cause dependence, daytime sleepiness, worse balance, and falls, so specialists usually keep doses low and time-limited.

8. Baclofen
Baclofen is a muscle relaxant used for spasticity. Some people with ataxia-plus syndromes have stiff muscles or spasms. Baclofen acts on GABA-B receptors in the spinal cord to reduce abnormal muscle contractions. This can make walking smoother and less tiring. Side effects include weakness, drowsiness, and dizziness, which must be weighed carefully in someone who already has balance problems.

9. Meclizine or similar vestibular suppressants
Meclizine and related antihistamines can reduce acute vertigo and nausea. For CABV, they may be used briefly during sudden worsening or during travel. However, long-term use is usually avoided because they can slow down vestibular compensation and cause drowsiness and blurred vision. The mechanism is blocking certain histamine receptors in the brain’s balance centers.

10. Antiemetics (such as ondansetron)
When dizziness leads to severe nausea and vomiting, antiemetic medicines like ondansetron can help. They block serotonin or dopamine receptors in brain areas that control vomiting. In CABV, they do not treat the cause of imbalance but make severe episodes more tolerable so people can keep drinking, eating, and participating in therapy.

11. SSRIs (selective serotonin reuptake inhibitors)
Depression and anxiety are common in chronic neurological diseases. SSRIs such as sertraline or escitalopram increase serotonin levels and can improve mood, energy, and social participation. In CABV, better mood usually means better motivation to do exercises and stay active. Side effects may include stomach upset, sleep changes, and sexual side effects.

12. SNRIs and other anti-anxiety medicines
Besides duloxetine, other serotonin-noradrenaline medicines or short-term anti-anxiety drugs may be used when worry about falling becomes disabling. These medicines work on brain chemical messengers to dampen over-reactive fear circuits. Careful medical supervision is needed so sedation does not worsen balance.

13. Acetazolamide (for specific ataxia subtypes)
Acetazolamide is a carbonic anhydrase inhibitor used in some episodic cerebellar ataxias. It alters ion balances in nerve cells and may stabilize firing. In classical CABV due to degenerative causes, evidence is limited, but in selected genetic ataxias with overlapping features, it can reduce attack frequency. Side effects include tingling, kidney stone risk, and taste changes.

14. Riluzole (experimental in ataxia)
Riluzole is approved for amyotrophic lateral sclerosis, but small trials have tested it in cerebellar ataxias. It may have neuroprotective effects by reducing glutamate-related toxicity. For CABV, its use is experimental and limited to research or highly specialized clinics. Liver function tests are usually required because of possible toxicity.

15. Amantadine
Amantadine is used in Parkinson’s disease and for fatigue in some neurological conditions. It may reduce exhaustion and improve motor activation in some patients with ataxia. It works through dopaminergic and NMDA-receptor effects. Side effects can include ankle swelling, skin color changes, and vivid dreams.

16. Beta-blockers (for co-existing tremor)
If a person with CABV also has essential tremor or anxiety-related trembling, beta-blockers (such as propranolol) may be used. They reduce the effect of adrenaline on the heart and muscles, calming tremor and palpitations. However, they can lower blood pressure and may worsen dizziness when standing, so doses must be carefully adjusted.

17. Midodrine or fludrocortisone (for orthostatic intolerance)
Some patients with ataxia syndromes have blood pressure drops on standing. Medicines like midodrine (a vasoconstrictor) or fludrocortisone (a mineralocorticoid that retains salt and water) can stabilize blood pressure. More stable blood flow to the brain reduces light-headedness and falls. These medicines require close monitoring for high blood pressure and fluid overload.

18. Painkillers for musculoskeletal pain
Chronic abnormal posture and stiff walking can cause back or joint pain. Simple pain relievers may help, but long-term use of strong painkillers, especially opioids, is avoided. Opioids can worsen dizziness and cause dependence. Doctors aim for the lowest effective dose and prefer non-drug strategies like physio when possible.

19. Sleep medicines (only with caution)
Poor sleep is common in chronic neurological illness. Low-dose sleep aids may be used short term to break cycles of insomnia. However, many sleeping tablets increase fall risk, confusion, and dependence. Non-drug sleep strategies are always preferred first.

20. Treatment of associated conditions (diabetes, thyroid disease, vitamin deficiencies)
If CABV occurs together with problems like diabetes or vitamin B12 deficiency, careful drug treatment of those conditions is essential. Good blood sugar control and replacement of missing vitamins may prevent further nerve and balance damage. This “indirect” treatment is often one of the most important parts of long-term care.Frontiers+2Wikipedia+2

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Dietary molecular supplements

(Evidence for supplements is generally weaker than for physiotherapy or prescription medicines. They should only be used with medical advice, especially if other drugs are already prescribed.)

1. Vitamin B12
Vitamin B12 is essential for healthy nerves and blood cells. Low B12 can cause neuropathy and unsteady walking. In someone with CABV, a deficiency would worsen symptoms, so doctors often check and correct it. Replacement can be by tablets or injections, depending on the cause. The main function is supporting myelin, the protective coat of nerve fibers, and DNA synthesis in nerve cells.

2. Thiamine (Vitamin B1)
Thiamine is vital for energy production inside neurons. Severe deficiency causes serious neurological problems including ataxia. If a person with CABV also has poor intake, alcohol misuse, or absorption problems, thiamine supplementation may protect remaining cerebellar and peripheral nerves. It helps enzymes in carbohydrate metabolism work properly and supports mitochondrial function.

3. Vitamin E
Vitamin E is a fat-soluble antioxidant. Inherited vitamin E deficiency can itself cause ataxia, so low levels must be corrected. Supplementation may help protect nerve membranes from oxidative damage. In CABV, normal vitamin E levels are important to avoid extra damage, although high-dose supplementation without deficiency has limited evidence.

4. Vitamin D
Vitamin D supports bone strength and muscle function. Low levels are common in people who are less active or avoid going outside because of imbalance. For CABV, correcting deficiency may reduce fracture risk after falls and slightly improve muscle performance. It works by regulating calcium absorption and muscle cell function.

5. Omega-3 fatty acids
Omega-3 fatty acids from fish oil or algae have anti-inflammatory and possible neuroprotective effects. They may support general brain and nerve health and have modest benefits for mood and cardiovascular risk. In CABV, they will not repair damaged vestibular organs, but they may support overall neurological resilience and reduce vascular risks that could worsen brain function.

6. Alpha-lipoic acid
Alpha-lipoic acid is an antioxidant sometimes used in diabetic neuropathy. It may reduce oxidative stress and improve blood flow in small nerves. For CABV with co-existing neuropathy, it could be considered as an adjunct under supervision. Side effects may include stomach upset or low blood sugar in some people with diabetes.

7. Coenzyme Q10 (CoQ10)
CoQ10 is involved in mitochondrial energy production. Some hereditary ataxias respond to CoQ10 replacement when deficiency is present. In CABV, it may support mitochondrial health in surviving neurons. It is usually taken as a capsule with food, and side effects are usually mild, like stomach discomfort.

8. Magnesium
Magnesium plays a role in nerve signalling and muscle relaxation. Mild deficiency can cause cramps and fatigue. For CABV, supplementation may ease muscle tightness and improve sleep quality. Too much magnesium, especially in people with kidney problems, can cause diarrhoea or more serious side effects, so doses must be reasonable.

9. Curcumin (from turmeric)
Curcumin is a plant molecule with anti-inflammatory and antioxidant properties in experimental models. It may help general brain health and reduce inflammation related to chronic disease. In CABV, it is purely supportive and should not replace standard treatment. It is often taken with fat or black pepper extract to improve absorption.

10. L-carnitine
L-carnitine helps transport fatty acids into mitochondria for energy production. In some metabolic and neuromuscular disorders, it is used to support muscle and nerve function. In CABV, it may slightly improve fatigue and exercise tolerance. Possible side effects include stomach upset or body-odour changes.

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Immunity-boosting, regenerative, and stem-cell-related drugs

These approaches are specialist or research-level and not self-treatments. Many are still experimental for ataxia and vestibular disease.

1. Intravenous immunoglobulin (IVIG)
IVIG is a blood product containing pooled antibodies from healthy donors. It is used for immune-mediated neuropathies and some autoimmune ataxias. If a patient with CABV also has evidence of autoimmune attack on nerves or cerebellum, doctors may try IVIG to reduce immune damage. It works by modulating immune responses but is expensive and given only in hospital settings.

2. Corticosteroids and other immunosuppressants
Steroids and immunosuppressants like azathioprine or mycophenolate may be used when immune-mediated cerebellar ataxia or vestibulopathy is suspected. They reduce inflammation and antibody-mediated attack on nervous tissue. In classic degenerative CABV without autoimmunity they are usually not helpful. Long-term side effects include weight gain, infection risk, and bone thinning.

3. Rituximab and targeted biologic therapies
Rituximab is a monoclonal antibody that removes certain B-cells from the immune system. It is used in some autoimmune neurological diseases. For CABV-like syndromes with strong autoimmune features, it might be considered in specialist centers. The idea is to stop antibody production that harms cerebellar or vestibular structures. It carries serious potential risks like infections and infusion reactions.

4. Experimental stem cell therapies
Mesenchymal or neural stem cell infusions are being studied in various neurodegenerative diseases. Early research explores whether these cells can release growth factors or replace damaged neural cells. For CABV, evidence is very limited, and such treatments should only be considered within regulated clinical trials, not commercial “stem cell clinics.” The main mechanism is hoped neuroprotection and limited regeneration.

5. Neurotrophic and growth-factor approaches
Some experimental drugs aim to increase brain-derived neurotrophic factor (BDNF) or other growth factors to support neuron survival. At present, most of this work is in animal models or early-phase human trials in other ataxias or vestibular conditions. For CABV, these approaches remain future possibilities.

6. Lifestyle-based “immune support”
The safest “immune booster” for CABV is not a drug but a bundle of habits: adequate sleep, vaccination against common infections, treatment of chronic diseases, balanced diet, and regular moderate exercise. These measures support the immune system’s normal function and may reduce infections that could temporarily worsen neurological symptoms.

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Surgeries

1. Surgery for structural brain or spinal lesions
If imaging finds a tumor, vascular malformation, or other compressive lesion affecting the cerebellum or brainstem, neurosurgery may be needed. Removing or reducing the lesion can prevent further damage and sometimes improve symptoms. This is not typical for idiopathic CABV, but it is important to consider in the differential diagnosis.

2. Shunt surgery for hydrocephalus
If a person has hydrocephalus (too much cerebrospinal fluid causing pressure on the cerebellum), neurosurgeons may insert a shunt to drain fluid. Reducing pressure can improve gait and balance. Again, this is done when imaging clearly shows hydrocephalus contributing to ataxia and not for routine CABV.

3. Decompression surgery in cervical spine disease
Some patients with ataxia-like symptoms also have severe cervical spinal cord compression. Decompression surgery can protect the spinal cord and improve leg function. If CABV co-exists with such compression, surgery may prevent additional walking problems, even though it does not fix the vestibular damage.

4. Cochlear implantation (when severe hearing loss is present)
CABV can overlap with other conditions causing hearing loss. Cochlear implants improve hearing but not vestibular function. Still, better hearing can help with orientation, social connection, and safety (for example, hearing traffic or warnings). ENT specialists decide if this is appropriate.

5. Orthopaedic surgery after serious injuries
Because of high fall risk, some patients may break hips or other bones and need orthopaedic surgery. The purpose is to repair the injury and restore mobility. After surgery, careful rehab is crucial, because underlying CABV still affects balance.

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Preventions

1. Avoid falls by using walking aids, good lighting, and removing home hazards.

2. Control chronic illnesses such as diabetes or high blood pressure to avoid further nerve and brain damage.Frontiers+1

3. Avoid ototoxic medicines (drugs that damage the inner ear) when alternatives exist; examples include some antibiotics and chemotherapy agents, managed by doctors.

4. Limit alcohol because heavy drinking can worsen cerebellar damage and neuropathy.

5. Do regular vestibular and balance exercises as prescribed, to maintain gains from therapy.Vestibular Disorders Association+1

6. Protect your head by using helmets when cycling and avoiding risky activities that could cause head injury.

7. Keep vaccinations up to date to reduce infections that might temporarily worsen symptoms or cause hospital admissions.

8. Maintain healthy body weight and fitness so muscles and heart can support compensation for balance loss.

9. Manage medications carefully, reviewing them with doctors to remove unnecessary sedating drugs.

10. Attend regular check-ups with neurology, ENT, and rehabilitation teams so changes in symptoms are picked up early.

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When to see doctors

You should see a doctor promptly if you notice new or worsening balance problems, such as suddenly being unable to walk without support, new double vision, new severe headache, or sudden weakness or numbness. These changes could mean something new is happening in the brain or spinal cord. You should also see your doctor if you have more frequent falls, major changes in speech or swallowing, or new bladder or bowel problems.

If you already have CABV, regular follow-up with your neurologist or ENT specialist is important to adjust medication, review therapy needs, and check for treatable complications like depression, neuropathic pain, or blood pressure problems. Any serious injury from a fall, such as hitting your head or breaking a bone, is an emergency and needs urgent medical care.

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What to eat and what to avoid

1. Eat a balanced, whole-food diet rich in vegetables, fruits, whole grains, and lean proteins to support general brain and muscle health.

2. Include sources of B-vitamins (whole grains, beans, eggs, dairy, meat if you eat it) to support nerve function, or discuss supplements if you are vegetarian or vegan.

3. Choose healthy fats like fish, nuts, and seeds, which provide omega-3 fatty acids that may support brain and nerve health.

4. Stay well hydrated, because dehydration can worsen dizziness and low blood pressure.

5. Limit alcohol, as it directly harms the cerebellum and can worsen unsteadiness.

6. Avoid very high-sugar, highly processed foods, which can harm vascular and nerve health over time, especially if you have diabetes risk.

7. Avoid crash diets or extreme fasting, which can cause low blood pressure and low blood sugar, making dizziness and falls more likely.

8. Reduce excessive caffeine, which can worsen tremor, anxiety, and sleep problems in some people.

9. Watch salt intake if you have high blood pressure or heart problems, but do not change salt drastically without medical advice, especially if you take blood-pressure medicines.

10. Discuss any supplement plan with your doctor, to avoid interactions with prescription drugs and to focus on real deficiencies rather than many overlapping products.

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Frequently asked questions

1. Is cerebellar ataxia with bilateral vestibulopathy curable?
At present, CABV is considered a chronic condition without a known cure. Treatment focuses on symptom control, balance training, and prevention of complications. Many people remain independent for years with good rehab and safety planning.PubMed+2Vestibular Health+2

2. Is CABV the same as CANVAS?
CABV and CANVAS are related but not identical. CANVAS includes cerebellar ataxia, neuropathy, and vestibular areflexia and is usually due to a genetic change in the RFC1 gene. CABV describes the combination of cerebellar ataxia and bilateral vestibulopathy, with or without neuropathy, and can appear in different underlying diseases.Frontiers+2Wikipedia+2

3. Can exercises really help if my inner ears are badly damaged?
Yes. Studies show that vestibular rehabilitation improves balance and walking even in bilateral vestibular hypofunction. The brain learns to rely more on vision and body-sense. You may not become “normal,” but you can usually become safer and more confident.Vestibular Disorders Association+4Vestibular Disorders Association+4Lippincott Journals+4

4. Will I eventually need a wheelchair?
CABV usually progresses slowly. Some people need walking aids or a wheelchair for long distances after many years. Early and continuous rehab, plus prevention of other illnesses, can delay disability. Your individual course depends on the exact cause, age, and other health problems.PubMed+2Frontiers+2

5. Are there specific medicines that stop CABV from getting worse?
Currently there is no medicine proven to stop or reverse CABV itself. Drugs like 4-aminopyridine, acetyl-DL-leucine, and others mainly help symptoms such as gait instability or nystagmus. Research into genetic and regenerative treatments is ongoing.Frontiers+3PMC+3Nature+3

6. Can I do vestibular exercises on my own at home?
Many exercises are done at home, but it is safer to first be assessed by a vestibular physiotherapist. They can choose the right level and make sure exercises do not cause falls. Once you know the routine, home practice becomes a key part of long-term management.Vestibular Disorders Association+3umc.edu+3brainandspine.org.uk+3

7. Is it safe to play sports or exercise?
Moderate exercise is usually helpful, but you need activities with low fall risk, such as stationary cycling, supervised treadmill walking with rails, or water therapy. High-speed or contact sports are usually unsafe. Your rehab team can guide you based on your stability.

8. Why is my vision shaky when I walk?
This symptom, called oscillopsia, happens because the vestibulo-ocular reflex (VOR) cannot stabilise your eyes when your head moves. The cerebellar and vestibular damage in CABV affects this system. Gaze-stabilisation exercises and walking aids can reduce the impact but may not fully remove it.PubMed+2PMC+2

9. Does CABV affect thinking or memory?
CABV mainly affects coordination and balance. Most people keep normal thinking and memory, although chronic dizziness, poor sleep, and depression can make concentration harder. If you notice major changes in memory or behaviour, they should always be checked by a doctor, because they may have another cause.

10. Can diet alone treat CABV?
No. A healthy diet supports overall brain and body health but cannot repair damaged cerebellar or vestibular tissue. Diet is still important to protect bones, muscles, and nerves, and to control diseases like diabetes that could worsen symptoms.

11. Is CABV always genetic?
Not always. Some cases are linked to degenerative conditions, RFC1-related CANVAS spectrum, or other neurological diseases, while others remain idiopathic (unknown cause). Genetic testing is sometimes used when CANVAS or other hereditary ataxias are suspected.Frontiers+2Wikipedia+2

12. Will medicine for vertigo cure my imbalance?
Short-term vestibular suppressants can reduce vertigo and nausea but do not fix the underlying problem. In chronic bilateral vestibulopathy, long-term use can even slow compensation. Rehab exercises and safety strategies are more important for long-term stability.Neuropt+2Physiopedia+2

13. Can CABV cause hearing loss?
Vestibular organs and hearing organs sit close together in the inner ear, and some causes of bilateral vestibulopathy also affect hearing. However, CABV itself mainly describes balance and coordination problems. Hearing tests are often done to check for co-existing loss, which may be treated separately with hearing aids or implants.Frontiers+1

14. Is it safe to travel if I have CABV?
Travel is usually possible with planning. Many people find busy visual environments (like stations or airports) and moving platforms (boats, buses) more challenging. Using a cane, allowing extra time, and having a companion can improve safety. Talk with your doctor if you have severe oscillopsia or motion sickness.

15. What is the most important thing I can do right now?
For most people with CABV, the single most helpful step is to work regularly with a vestibular rehabilitation therapist and follow a daily home exercise program, combined with fall-prevention steps and good management of other health problems. This approach has the strongest evidence for improving function and quality of life over time.Vestibular Disorders Association+2SAGE Journals+2

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: December 19, 2025.