{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,27]],"date-time":"2026-07-27T23:57:52Z","timestamp":1785196672135,"version":"3.55.0"},"reference-count":37,"publisher":"MDPI AG","issue":"24","license":[{"start":{"date-parts":[[2022,12,13]],"date-time":"2022-12-13T00:00:00Z","timestamp":1670889600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"the Ministry of Science and Technology","award":["MOST-110-2622-8-075-001"],"award-info":[{"award-number":["MOST-110-2622-8-075-001"]}]},{"name":"the Ministry of Science and Technology","award":["V111C-162"],"award-info":[{"award-number":["V111C-162"]}]},{"name":"the Ministry of Science and Technology","award":["VGHUST111C-140"],"award-info":[{"award-number":["VGHUST111C-140"]}]},{"DOI":"10.13039\/501100011912","name":"Taipei Veterans General Hospital","doi-asserted-by":"publisher","award":["MOST-110-2622-8-075-001"],"award-info":[{"award-number":["MOST-110-2622-8-075-001"]}],"id":[{"id":"10.13039\/501100011912","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100011912","name":"Taipei Veterans General Hospital","doi-asserted-by":"publisher","award":["V111C-162"],"award-info":[{"award-number":["V111C-162"]}],"id":[{"id":"10.13039\/501100011912","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100011912","name":"Taipei Veterans General Hospital","doi-asserted-by":"publisher","award":["VGHUST111C-140"],"award-info":[{"award-number":["VGHUST111C-140"]}],"id":[{"id":"10.13039\/501100011912","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Veterans General Hospitals and University System of Taiwan Joint Research Program","award":["MOST-110-2622-8-075-001"],"award-info":[{"award-number":["MOST-110-2622-8-075-001"]}]},{"name":"Veterans General Hospitals and University System of Taiwan Joint Research Program","award":["V111C-162"],"award-info":[{"award-number":["V111C-162"]}]},{"name":"Veterans General Hospitals and University System of Taiwan Joint Research Program","award":["VGHUST111C-140"],"award-info":[{"award-number":["VGHUST111C-140"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Hearing loss and the related otologic manifestations are receiving increased scrutiny as significant causes of morbidity in Fabry disease. However, the relative risks of auditory deficits among patients with Fabry disease relative to the general population without a diagnosis of Fabry disease have not been studied. This study aims to explore the associations between Fabry disease and hearing-related manifestations using a nationwide population-based dataset. We identified study patients for this cross-sectional study from the 2015\u20132017 claims databases of the Taiwan Longitudinal Health Insurance Database 2005. We first identified 2312 patients aged over 20 years with a diagnosis of Fabry disease. We used propensity score matching to select five comparison patients per patient with Fabry disease and 11,560 comparison patients without Fabry disease. We used multivariable logistic regressions to estimate the odds ratios (ORs) and corresponding 95% confidence intervals (CIs) for tinnitus, hearing loss, and sudden deafness among Fabry disease patients vs. comparison patients. Chi-square tests showed statistically significant differences between patients with and without Fabry disease in the prevalence rates of tinnitus (16.7% vs. 11.7%, p &lt; 0.001), hearing loss (7.5% vs. 6.2%, p = 0.014) and sudden deafness (1.7% vs. 1.0%, p = 0.005). Multiple logistic regression revealed that patients with Fabry disease were more likely to suffer from tinnitus, hearing loss and sudden deafness, with adjusted odds ratios of 1.513 (95% CI = 1.336\u20131.713), 1.246 (95% CI = 1.047\u20131.483), and 1.681 (95% CI = 1.166\u20132.423), respectively. We found that Fabry disease is significantly associated with certain auditory manifestations, including hearing loss, sudden deafness, and tinnitus.<\/jats:p>","DOI":"10.3390\/jcm11247396","type":"journal-article","created":{"date-parts":[[2022,12,14]],"date-time":"2022-12-14T03:21:52Z","timestamp":1670988112000},"page":"7396","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Association of Fabry Disease with Hearing Loss, Tinnitus, and Sudden Hearing Loss: A Nationwide Population-Based Study"],"prefix":"10.3390","volume":"11","author":[{"given":"Yen-Fu","family":"Cheng","sequence":"first","affiliation":[{"name":"Department of Medical Research, Taipei Veterans General Hospital, Taipei 112, Taiwan"},{"name":"Department of Otolaryngology-Head and Neck Surgery, Taipei Veterans General Hospital, Taipei 112, Taiwan"},{"name":"Department of Otolaryngology-Head and Neck Surgery, School of Medicine, National Yang Ming Chiao Tung University, Taipei 112, Taiwan"},{"name":"Institute of Brain Science, National Yang Ming Chiao Tung University, Taipei 112, Taiwan"},{"name":"Research Center of Sleep Medicine, College of Medicine, Taipei Medical University, Taipei 110, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sudha","family":"Xirasagar","sequence":"additional","affiliation":[{"name":"Department of Health Services Policy and Management, Arnold School of Public Health, University of South Carolina, Columbia, SC 29208, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chin-Shyan","family":"Chen","sequence":"additional","affiliation":[{"name":"Research Center of Sleep Medicine, College of Medicine, Taipei Medical University, Taipei 110, Taiwan"},{"name":"Department of Economics, National Taipei University, 151 University Rd. San Xia, New Taipei City 237, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dau-Ming","family":"Niu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Taipei Veterans General Hospital, Taipei 112, Taiwan"},{"name":"Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei 112, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4661-959X","authenticated-orcid":false,"given":"Herng-Ching","family":"Lin","sequence":"additional","affiliation":[{"name":"Sleep Research Center, Taipei Medical University Hospital, Taipei 110, Taiwan"},{"name":"School of Health Care Administration, Taipei Medical University, Taipei 110, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,12,13]]},"reference":[{"key":"ref_1","unstructured":"Bokhari, S.R.A., Zulfiqar, H., and Hariz, A. (2017). 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