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Video-head-impulse testing (vHIT) offers a quantitative assessment of the angular vestibulo-ocular reflex (aVOR) of all three canals. We evaluated patterns of peripheral-vestibular impairment, its change over time and evaluated correlations with other parameters. PubMed\/Embase were searched for articles reporting vHIT in patients with CANVAS\/RFC1-related ataxia. A multiple linear-regression model was used to analyse relationships between vHIT-gains and clinical parameters (age, disease duration, sex, biallelic RFC1 expansion). A special focus was put on sequential vHIT in individual patients. 23\/64 studies met inclusion criteria; additional 13 studies were identified through reference screening. Twenty-five studies reported individual vHIT-gains and demographic data, suitable for quantitative analysis. Substantial aVOR-gain reductions were found for horizontal (0.32\u2009\u00b1\u20090.02, <jats:italic>n<\/jats:italic>\u2009=\u2009146 patients), anterior (0.39\u2009\u00b1\u20090.03, <jats:italic>n<\/jats:italic>\u2009=\u200927) and posterior (0.29\u2009\u00b1\u20090.03, <jats:italic>n<\/jats:italic>\u2009=\u200927) canals. Linear regression showed an association between horizontal vHIT-gains (<jats:italic>n<\/jats:italic>\u2009=\u2009146 patients; range of gain: 0-1.3) and disease duration (range: 0\u2013444 months, coef. =-0.0048, <jats:italic>p<\/jats:italic>\u2009=\u20090.031) and male sex (coef. =-0.1604, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). A decline in horizontal-canal vHIT-gains at least one side over time was noted in 15\/21 patients after a mean follow-up time of 33.4\u2009\u00b1\u200910.7 months. vHIT is a potential biomarker for monitoring progression of CANVAS\/RFC1-related ataxia. The significant association between reduced vHIT-gains and disease duration, and their intra-individual decline over time emphasize that impairment of the aVOR reflects the underlying neurodegenerative disease process. Multi-centre prospective studies are needed for systematic early screening and longitudinal validation as outcome for future targeted therapy trials.<\/jats:p>","DOI":"10.1007\/s12311-025-01825-y","type":"journal-article","created":{"date-parts":[[2025,3,20]],"date-time":"2025-03-20T04:51:52Z","timestamp":1742446312000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["The Spectrum of Peripheral-Vestibular Deficits and Their Change Over Time in CANVAS\/RFC1-Related Ataxia Systematic Review and Meta-Analysis of Quantitative Head-Impulse Testing"],"prefix":"10.1007","volume":"24","author":[{"given":"Maja","family":"Szymanska Heydel","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix","family":"Heindl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annette","family":"Hartmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Max","family":"Borsche","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Trasch\u00fctz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominik","family":"Straumann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Strupp","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander Andrea","family":"Tarnutzer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,3,20]]},"reference":[{"issue":"1","key":"1825_CR1","first-page":"61","volume":"6","author":"D J Szmulewicz","year":"2016","unstructured":"J Szmulewicz D, et al. 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M. Strupp is Joint Chief Editor of the Journal of Neurology, Editor in Chief of Frontiers of Neuro-otology and Section Editor of F1000. He has received speaker\u2019s honoraria from Abbott, Auris Medical, Biogen, Eisai, Gr\u00fcnenthal, GSK, Henning Pharma, Interacoustics, J&J, MSD, NeuroUpdate, Otometrics, Pierre-Fabre, TEVA, UCB, and Viatris. He receives support for clinical studies from Decibel, U.S.A., Cure within Reach, U.S.A. and Heel, Germany. He distributes \u201cM-glasses\u201d and \u201cPositional vertigo App\u201d. He acts as a consultant for Abbott, AurisMedical, Bulbitec, Heel, IntraBio, Sensorion, Vifor and Vertify. He is investor and share- and patent-holder of IntraBio. The other authors have no relevant financial or non-financial interests to disclose.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing Interests"}}],"article-number":"67"}}