{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T06:05:40Z","timestamp":1792821940260,"version":"4.5.0"},"reference-count":13,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2024,1,18]],"date-time":"2024-01-18T00:00:00Z","timestamp":1705536000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2024,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Nonreversible hearing loss (HL) is the main sequelae of Susac syndrome (SuS). We aimed to identify risk factors for HL in SuS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The CARESS study is a prospective national cohort study that started in December 2011, including all consecutive patients with SuS referred to the French reference center. The CARESS study was designed with a follow\u2010up including fundoscopy, audiometry, and brain magnetic resonance imaging at 1, 3, 6, and 12\u2009months after diagnosis and then annually for 5\u2009years. The primary outcome was the occurrence at last follow\u2010up of severe HL defined as the loss of 70\u2009dB in at least one ear on audiometry or the need for hearing aids.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirty\u2010six patients (female 66.7%, median age 37.5 [range 24.5\u201342.5] years) included in the clinical study were analyzed for the primary outcome. Thirty\u2010three patients (91.7%) had cochleovestibular involvement at SuS diagnosis including HL &gt;20\u2009dB in at least one ear in 25 cases. At diagnosis, 32 (88.9%), 11 (30.6%), and 7 (19.4%) patients had received steroids, intravenous immunoglobulin, and\/or immunosuppressive (IS) drugs, respectively. After a median follow\u2010up of 51.8 [range 29.2\u201377.6] months, 19 patients (52.8%) experienced severe HL that occurred a median of 13 [range 1.5\u201329.5] months after diagnosis. Multivariable analysis showed that the odds of severe HL were lower in patients who received IS drugs at diagnosis (OR 0.15, 95% CI 0.01\u20131.07, <jats:italic>p<\/jats:italic>\u2009=\u20090.058).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Severe HL in SuS is associated with the absence of IS drugs given at diagnosis. Our findings support the systematic use of IS drugs in SuS.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.16211","type":"journal-article","created":{"date-parts":[[2024,1,18]],"date-time":"2024-01-18T12:21:51Z","timestamp":1705580511000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Risk factors for severe hearing loss in Susac syndrome: A\u00a0national cohort study"],"prefix":"10.1111","volume":"31","author":[{"given":"Marion","family":"Peyre","sequence":"first","affiliation":[{"name":"Department of Internal Medecine, Hospital Bichat\u2010Claude Bernard, Assistance Publique H\u00f4pitaux de Paris Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arthur","family":"Mageau","sequence":"additional","affiliation":[{"name":"Department of Internal Medecine, Hospital Bichat\u2010Claude Bernard, Assistance Publique H\u00f4pitaux de Paris Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie\u2010C\u00e9cile","family":"Henry\u00a0Feugeas","sequence":"additional","affiliation":[{"name":"Department of Radiology, Hospital Bichat\u2010Claude Bernard, Assistance Publique H\u00f4pitaux de Paris Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Serge","family":"Doan","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Hospital Bichat\u2010Claude Bernard, Assistance Publique H\u00f4pitaux de Paris Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Halimi","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology \u2010 Head and Neck Surgery, Hospital Bichat\u2010Claude Bernard, Assistance Publique H\u00f4pitaux de Paris Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Klein","sequence":"additional","affiliation":[{"name":"Department of Radiology Clinique Alleray\u2010Labrouste  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiphaine","family":"Goulenok","sequence":"additional","affiliation":[{"name":"Department of Internal Medecine, Hospital Bichat\u2010Claude Bernard, Assistance Publique H\u00f4pitaux de Paris Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chrystelle","family":"Fran\u00e7ois","sequence":"additional","affiliation":[{"name":"Department of Internal Medecine, Hospital Bichat\u2010Claude Bernard, Assistance Publique H\u00f4pitaux de Paris Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie\u2010Paule","family":"Chauveheid","sequence":"additional","affiliation":[{"name":"Department of Internal Medecine, Hospital Bichat\u2010Claude Bernard, Assistance Publique H\u00f4pitaux de Paris Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Papo","sequence":"additional","affiliation":[{"name":"Department of Internal Medecine, Hospital Bichat\u2010Claude Bernard, Assistance Publique H\u00f4pitaux de Paris Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6544-234X","authenticated-orcid":false,"given":"Karim","family":"Sacr\u00e9","sequence":"additional","affiliation":[{"name":"Department of Internal Medecine, Hospital Bichat\u2010Claude Bernard, Assistance Publique H\u00f4pitaux de Paris Universit\u00e9 Paris Cit\u00e9  Paris France"},{"name":"Centre de Recherche sur l'Inflammation, INSERM UMR1149, CNRS ERL8252, Facult\u00e9 de M\u00e9decine site Bichat Laboratoire d'Excellence Inflamex  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the French Susac Study Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,1,18]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2022.103097"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.revmed.2021.05.014"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.revmed.2011.11.002"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2023.578032"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.14836"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-019-13593-5"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2016-314295"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0b013e3180404c99"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.15062"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2015.06.063"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1055\/a-1766-6243"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/biomet\/80.1.27"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2023.1105461"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.16211","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,8]],"date-time":"2024-04-08T04:43:21Z","timestamp":1712551401000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.16211"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,1,18]]},"references-count":13,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2024,5]]}},"alternative-id":["10.1111\/ene.16211"],"URL":"https:\/\/doi.org\/10.1111\/ene.16211","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"value":"1351-5101","type":"print"},{"value":"1468-1331","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,1,18]]},"assertion":[{"value":"2023-08-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-01-02","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-01-18","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e16211"}}