{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T18:56:47Z","timestamp":1791831407677,"version":"4.3.4"},"reference-count":0,"publisher":"SAGE Publications","issue":"5-6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["VES"],"published-print":{"date-parts":[[2014,12,17]]},"abstract":"<jats:p>Background: Cerebellar Ataxia with Neuropathy and bilateral Vestibular Areflexia Syndrome (CANVAS) is a multi-system ataxia which results in cerebellar ataxia, a bilateral vestibulopathy and a somatosensory deficit. This sensory deficit has recently been shown to be a neuronopathy, with marked dorsal root ganglia neuronal loss. The characteristic oculomotor clinical sign is an abnormal visually enhanced vestibulo-ocular reflex. Objective: To outline the expanding understanding of the pathology in this condition, as well as diagnostic and management issues encountered in clinical practice. Methods: Retrospective data on 80 CANVAS patients is reviewed. Results: In addition to the triad of cerebellar impairment, bilateral vestibulopathy and a somatosensory deficit, CANVAS patients may also present with orthostatic hypotension, a chronic cough and neuropathic pain. Management of falls risk and dysphagia is a major clinical priority. Conclusions: CANVAS is an increasingly recognised cause of late-onset ataxia and disequilibrium, and is likely to be a recessive disorder.<\/jats:p>","DOI":"10.3233\/ves-140536","type":"journal-article","created":{"date-parts":[[2019,12,2]],"date-time":"2019-12-02T18:03:53Z","timestamp":1575309833000},"page":"465-474","source":"Crossref","is-referenced-by-count":75,"title":["CANVAS an update: Clinical presentation, investigation and management"],"prefix":"10.1177","volume":"24","author":[{"given":"David J.","family":"Szmulewicz","sequence":"first","affiliation":[{"name":"Royal Victorian Eye & Ear Hospital, University of Melbourne, Melbourne, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catriona A.","family":"McLean","sequence":"additional","affiliation":[{"name":"Department of Anatomical Pathology, Alfred Hospital, Melbourne, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hamish G.","family":"MacDougall","sequence":"additional","affiliation":[{"name":"Vestibular Research Laboratory, School of Psychology, University of Sydney, Sydney, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leslie","family":"Roberts","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, St Vincent's Hospital, Melbourne, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elsdon","family":"Storey","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Monash University, Melbourne, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. Michael","family":"Halmagyi","sequence":"additional","affiliation":[{"name":"Department of Neurology, Royal Prince Alfred Hospital, Sydney, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","container-title":["Journal of Vestibular Research"],"original-title":[],"link":[{"URL":"https:\/\/content.iospress.com\/download?id=10.3233\/VES-140536","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T05:22:11Z","timestamp":1777612931000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/full\/10.3233\/VES-140536"}},"subtitle":[],"editor":[{"given":"Marco","family":"Mandal\u00e0","sequence":"additional","affiliation":[{"name":"Otological and Skull Base Surgery Department, S.M. delle Scotte Hospital, Siena, Italy"}],"role":[{"vocabulary":"crossref","role":"editor"}]},{"given":"Stefano","family":"Ramat","sequence":"additional","affiliation":[{"name":"Department of Electrical, Computer and Biomedical Engineering, University of Pavia, Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"editor"}]},{"given":"David S.","family":"Zee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2014,12,17]]},"references-count":0,"journal-issue":{"issue":"5-6"},"URL":"https:\/\/doi.org\/10.3233\/ves-140536","relation":{"is-cited-by":[{"id-type":"doi","id":"10.3389\/fneur.2017.00444","asserted-by":"object"},{"id-type":"doi","id":"10.3389\/fneur.2016.00026","asserted-by":"object"},{"id-type":"doi","id":"10.3389\/fgene.2018.00085","asserted-by":"object"}]},"ISSN":["1878-6464","0957-4271"],"issn-type":[{"value":"1878-6464","type":"electronic"},{"value":"0957-4271","type":"print"}],"subject":[],"published":{"date-parts":[[2014,12,17]]}}}