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This study aimed to analyse the development of the most likely aetiologies during the last 100\u00a0years by a systematic review of the relevant medical literature.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>A systematic review of medical literature in PubMed<jats:sup>\u00ae<\/jats:sup>, PubMed Central<jats:sup>\u00ae<\/jats:sup>, and Web of Science<jats:sup>\u00ae<\/jats:sup> for the period 1920\u20132020 was conducted. Medical publications between 1920 and 1935 were additionally searched manually by review of reference lists. Studies were eligible when a statement regarding the most likely aetiology of the hearing loss of Beethoven was the aimed objective of the publication.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>48 publications were included. The following aetiologies were supposed: otosclerosis (<jats:italic>n<\/jats:italic>\u2009=\u200910), syphilis (<jats:italic>n<\/jats:italic>\u2009=\u20099), Paget\u2019s disease (<jats:italic>n<\/jats:italic>\u2009=\u20096), neural deafness (<jats:italic>n<\/jats:italic>\u2009=\u20095), immunopathy with inflammatory bowel disease, neural deafness with otosclerosis, sarcoidosis or lead intoxication (<jats:italic>n<\/jats:italic>\u2009=\u20092), and systemic lupus erythematosus, trauma, labyrinthitis or inner ear disease (<jats:italic>n<\/jats:italic>\u2009=\u20091). There is an ongoing effort with a mean publication frequency in this topic of 0.48\/year. From 1920 to 1970, otolaryngologists were the group with the highest interest in this field (67%), whereas since 1971 most authors have belonged to non-otolaryngologic subspecialities (81%).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>Over the past 100\u00a0years, otosclerosis and syphilis were predominantly supposed to be the underlying causes. The hypothesis of syphilis\u2014although rejected for a long time\u2014has had a remarkable revival during the past 20\u00a0years. Regarding the outcome following therapeutic intervention by cochlear implantation, the differential diagnosis of neural deafness would be relevant today.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s00405-020-06467-w","type":"journal-article","created":{"date-parts":[[2020,11,23]],"date-time":"2020-11-23T20:55:04Z","timestamp":1606164904000},"page":"2703-2712","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Aetiology of Ludwig van Beethoven\u2019s hearing impairment: hypotheses over the past 100\u00a0years \u2013 A systematic review"],"prefix":"10.1007","volume":"278","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7858-3080","authenticated-orcid":false,"given":"Jan Peter","family":"Thomas","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Dazert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Prescher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christiane","family":"Voelter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,11,23]]},"reference":[{"key":"6467_CR1","doi-asserted-by":"publisher","first-page":"1301","DOI":"10.1288\/00005537-198907000-00021","volume":"99","author":"SL Liston","year":"1989","unstructured":"Liston SL, Yanz JL, Preves D et al (1989) Beethoven\u2019s deafness. 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