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Six months later he developed a peripheral neuropathy and muscle fatigue followed by a slowly progressive aphasia and cortical blindness as well as increased seizure activity. A computed tomography scan showed bilateral basal ganglion calcification. The serum lactate level was elevated at 3.4 mEq\/dL. A muscle biopsy enabled the diagnosis of mitochondrial myopathy. This disorder is presented as an unusual cause of progressive sensorineural hearing loss in adults.<\/jats:p>","DOI":"10.1177\/000348949510401007","type":"journal-article","created":{"date-parts":[[2014,10,3]],"date-time":"2014-10-03T14:38:05Z","timestamp":1412347085000},"page":"786-792","source":"Crossref","is-referenced-by-count":5,"title":["Mitochondrial Encephalomyopathy: A Rare Genetic Cause of Sensorineural Hearing Loss"],"prefix":"10.1177","volume":"104","author":[{"given":"Thomas J.","family":"Donovan","sequence":"first","affiliation":[{"name":"St Louis, Missouri"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[1995,10,1]]},"reference":[{"key":"bibr1-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.43.12.2471"},{"key":"bibr2-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1016\/S0733-8619(18)30859-4"},{"key":"bibr3-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/109.5.915"},{"key":"bibr4-000348949510401007","first-page":"161","volume":"13","author":"Shapira Y","year":"1977","journal-title":"First J Med Sci"},{"key":"bibr5-000348949510401007","first-page":"95","volume-title":"Advances in contemporary neurology","author":"Pavlakis SG","year":"1988"},{"key":"bibr6-000348949510401007","first-page":"141","volume-title":"Mitochondrial pathology in muscular diseases","author":"Rowland LP","year":"1983"},{"key":"bibr7-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410160409"},{"key":"bibr8-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(80)90031-3"},{"key":"bibr9-000348949510401007","first-page":"408","volume":"23","author":"Tsairis P","year":"1973","journal-title":"Neurology"},{"key":"bibr10-000348949510401007","first-page":"89","volume-title":"Mitochondrial pathology in muscular diseases","author":"Fukuhara N","year":"1983"},{"key":"bibr11-000348949510401007","first-page":"1119","volume":"14","author":"Barkovich AJ","year":"1993","journal-title":"Am J Neuroradiol"},{"key":"bibr12-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.29.2.86"},{"key":"bibr13-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.28.2.79"},{"key":"bibr14-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(89)92145-4"},{"key":"bibr15-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1001\/archopht.1985.01050120059020"},{"key":"bibr16-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.25.7.614"},{"key":"bibr17-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.29.5.717"},{"key":"bibr18-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(91)90093-M"},{"key":"bibr19-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(91)90088-O"},{"key":"bibr20-000348949510401007","first-page":"322","volume":"38","author":"Marangros N","year":"1990","journal-title":"HNO"},{"key":"bibr21-000348949510401007","first-page":"533","volume":"42","author":"Kawasaki Y","year":"1990","journal-title":"No To Shinkei"},{"key":"bibr22-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1007\/BF00314383"},{"key":"bibr23-000348949510401007","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1987.00520210065021"},{"key":"bibr24-000348949510401007","first-page":"1080","volume":"26","author":"Yamamoto M","year":"1986","journal-title":"Rinsho Shinkeigaku"}],"container-title":["Annals of Otology, Rhinology &amp; Laryngology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/000348949510401007","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/000348949510401007","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T11:14:11Z","timestamp":1777374851000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/000348949510401007"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1995,10]]},"references-count":24,"journal-issue":{"issue":"10","published-print":{"date-parts":[[1995,10]]}},"alternative-id":["10.1177\/000348949510401007"],"URL":"https:\/\/doi.org\/10.1177\/000348949510401007","relation":{},"ISSN":["0003-4894","1943-572X"],"issn-type":[{"value":"0003-4894","type":"print"},{"value":"1943-572X","type":"electronic"}],"subject":[],"published":{"date-parts":[[1995,10]]}}}