{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T18:18:44Z","timestamp":1787681924767,"version":"build-2784847793"},"reference-count":0,"publisher":"Caleo","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Int Adv Otol."],"abstract":"<jats:p>The Brown\u2013Vialetto\u2013Van Laere syndrome or the riboflavin transporter deficiency syndrome is a neurodegenerative disorder initially reported by Brown in 1894, by Vialetto in 1936, and by Van Laere in 1966. The syndrome has been described in more than 100 patients since then. Hearing loss is the most common symptom of the syndrome, as most individuals have it through the development of the disease. Although there is a variation between the onset of hearing loss and the other possible symptoms, hearing loss usually begins in early childhood. Nevertheless, there are some cases describing hearing loss starting in adults. Hereby, we present a case report of a patient who started having the symptoms at the age of 14 and who had a mutation in the SLC52A3 gene, presenting with sensorineural hearing loss associated with cerebellar ataxia, who also underwent successful cochlear implant surgery.\n\nCite this article as: do Amaral MSA, Massuda ET, Mitikami Fen\u00f3lio GH, Barbosa Reis ACM, Angelo Hyppolito M. Cochlear implant in brown\u2013vialetto\u2013 van laere syndrome patient. J Int Adv Otol. 2022;18(2):192-195.<\/jats:p>","DOI":"10.5152\/iao.2022.21159","type":"journal-article","created":{"date-parts":[[2022,4,7]],"date-time":"2022-04-07T03:26:50Z","timestamp":1649302010000},"page":"192-195","source":"Crossref","is-referenced-by-count":8,"title":["Cochlear Implant in Brown\u2013Vialetto\u2013Van Laere Syndrome Patient"],"prefix":"10.65717","volume":"18","author":[{"given":"Maria","family":"Stella Arantes do Amaral","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo T.","family":"Massuda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guilherme Henrique","family":"Mitikami Fen\u00f3lio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Cl\u00e1udia Mir\u00e2ndola Barbosa Reis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel","family":"Angelo Hyppolito","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"55248","published-online":{"date-parts":[[2022,3,30]]},"container-title":["The Journal of International Advanced Otology"],"original-title":[],"link":[{"URL":"https:\/\/www.advancedotology.org\/index.php\/pub\/article\/download\/1624\/1622","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.advancedotology.org\/index.php\/pub\/article\/download\/1592\/1589","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.advancedotology.org\/index.php\/pub\/article\/download\/1592\/1589","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T10:37:50Z","timestamp":1769078270000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.advancedotology.org\/index.php\/pub\/article\/view\/1592"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,3,30]]},"references-count":0,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2022,3,30]]}},"URL":"https:\/\/doi.org\/10.5152\/iao.2022.21159","relation":{},"ISSN":["2148-3817"],"issn-type":[{"value":"2148-3817","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,3,30]]}}}