CANVAS syndrome

CANVAS Syndrome is named with an easy-to-remember acronym for cerebellar ataxia, neuropathy, and vestibular areflexia. There are only a very few patients reported who have the requisite combination of two rare clinical findings (cerebellar ataxia and vestibular areflexia), and the very common peripheral neuropathy. Patients with CANVAS combine cerebellar ataxia (i.e. coordination problems — the CA), peripheral nerve damage (neuropathy – N), and loss of vestibular function (vestibular areflexia — the VA). This combination causes major disturbances to balance as each of these systems alone contributes to balance. Of course, when all are out at the same time, balance is much worse than when only one or two happens to be malfunctioning.

“Vestibular disorder” is an umbrella term used to encompass many different conditions that affect the inner ear and those parts of the central nervous system involved in maintaining balance.

Vestibular disorders can result from or be worsened by injuries, genetic or environmental conditions, or occur for unknown reasons. There are more than twenty-five known vestibular disorders. Each is unique, but many share common diagnostic traits, which can make it difficult for healthcare professionals to easily differentiate them.

The most commonly diagnosed vestibular disorders include benign paroxysmal positional vertigo (BPPV), labyrinthitis or vestibular neuritis, Ménière’s disease, and secondary endolymphatic hydrops. Vestibular disorders also include superior semicircular canal dehiscence, acoustic neuroma, perilymph fistula, ototoxicity, enlarged vestibular aqueduct, migraine-associated vertigo, and Mal de Sébarquement. Other problems related to vestibular dysfunction include complications from aging, autoimmune disorders, and allergies.

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