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Twenty-seven symptomatic SCA1 patients in various disease stages (SARA score range: 3\u201332 points) and 18 sex and age matched healthy controls underwent a clinical assessment addressing ataxia severity, non-ataxia signs, cognitive functioning, and speech. Speech samples were perceptually rated by trained speech therapists, and acoustic metrics representing speech timing, vocal control, and voice quality were extracted. Perceptual analysis revealed reduced intelligibility and naturalness in speech samples of SCA1 patients. Acoustically, SCA1 patients presented with slower speech rate and diadochokinetic rate as well as longer syllable duration compared to healthy controls. No distinct abnormalities in voice quality in the acoustic analysis were detected at group level. Both the affected perceptual and acoustic variables correlated with ataxia severity. Longitudinal assessment of speech is needed to place changes in speech in the context of disease progression and potential response to treatment.<\/jats:p>","DOI":"10.1007\/s12311-023-01513-9","type":"journal-article","created":{"date-parts":[[2023,1,12]],"date-time":"2023-01-12T07:03:09Z","timestamp":1673506989000},"page":"112-120","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Perceptual and Acoustic Analysis of Speech in Spinocerebellar ataxia Type 1"],"prefix":"10.1007","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6372-0801","authenticated-orcid":false,"given":"Teije","family":"van Prooije","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9277-2643","authenticated-orcid":false,"given":"Simone","family":"Knuijt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Oostveen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5735-1934","authenticated-orcid":false,"given":"Kirsten","family":"Kapteijns","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3505-2631","authenticated-orcid":false,"given":"Adam P.","family":"Vogel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4412-1616","authenticated-orcid":false,"given":"Bart","family":"van de Warrenburg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,1,12]]},"reference":[{"issue":"11","key":"1513_CR1","doi-asserted-by":"publisher","first-page":"1101","DOI":"10.1016\/S1474-4422(15)00202-1","volume":"14","author":"H Jacobi","year":"2015","unstructured":"Jacobi H, du Montcel ST, Bauer P, Giunti P, Cook A, Labrum R, Parkinson MH, Durr A, Brice A, Charles P, Marelli C. 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The study was conducted in accordance with the Declaration of Helsinki. All participants provided written informed consent.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical Approval"}},{"value":"A. P. Vogel is Chief Science Officer of Redenlab Inc.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing Interests"}}]}}