{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T03:24:17Z","timestamp":1788405857357,"version":"build-2803163510"},"reference-count":21,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2011,7,25]],"date-time":"2011-07-25T00:00:00Z","timestamp":1311552000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Laryngoscope"],"published-print":{"date-parts":[[2011,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives\/Hypothesis:<\/jats:title><jats:p>To evaluate vestibular function in patients with the mitochondrial A3243G mutation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Design:<\/jats:title><jats:p>Data from patients with the A3243G mutation attending an academic tertiary referral center were prospectively recorded.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods:<\/jats:title><jats:p>The clinical histories of 13 unrelated patients with the mitochondrial A3243G mutation (six mitochondrial encephalomyopathy, lactic acidosis, and stroke\u2010like episodes; and seven maternally inherited diabetes and deafness) were recorded, in particular their history of vestibular symptoms. Vestibular examinations including caloric testing and vestibular evoked myogenic potentials in response to air\u2010conducted sound (ACS\u2010VEMPs) were performed. In seven patients who showed abnormal ACS\u2010VEMP, VEMP in response to galvanic stimuli (galvanic\u2010VEMP) were also recorded.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p>Eleven of the 13 patients had vestibular symptoms. The age of onset of vestibular symptoms was significantly later than the ages of onset of hearing loss and diabetes mellitus (<jats:italic>P<\/jats:italic> &lt; .05). Ten of the 13 patients showed abnormal caloric responses, whereas 12 patients showed abnormal ACS\u2010VEMPs on one or both sides. All of the seven patients who underwent galvanic\u2010VEMP testing showed normal responses.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions:<\/jats:title><jats:p>The A3243G mutation is associated with vestibular dysfunction involving both the superior and inferior vestibular nerve systems. Furthermore, our results from galvanic\u2010VEMP testing suggests that a labyrinthine lesion is primarily responsible for the symptoms of vestibular dysfunction.<\/jats:p><\/jats:sec>","DOI":"10.1002\/lary.21879","type":"journal-article","created":{"date-parts":[[2011,7,26]],"date-time":"2011-07-26T16:16:02Z","timestamp":1311696962000},"page":"1821-1824","source":"Crossref","is-referenced-by-count":21,"title":["The mitochondrial A3243G mutation involves the peripheral vestibule as well as the 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