| The mitochondrial A3243G mutation involves the peripheral vestibule as well as the cochlea. | |
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MedLine Citation:
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PMID: 21792976 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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OBJECTIVES/HYPOTHESIS: To evaluate vestibular function in patients with the mitochondrial A3243G mutation. STUDY DESIGN: Data from patients with the A3243G mutation attending an academic tertiary referral center were prospectively recorded. METHODS: The clinical histories of 13 unrelated patients with the mitochondrial A3243G mutation (six mitochondrial encephalomyopathy, lactic acidosis, and stroke-like 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-conducted sound (ACS-VEMPs) were performed. In seven patients who showed abnormal ACS-VEMP, VEMP in response to galvanic stimuli (galvanic-VEMP) were also recorded. RESULTS: 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 (P < .05). Ten of the 13 patients showed abnormal caloric responses, whereas 12 patients showed abnormal ACS-VEMPs on one or both sides. All of the seven patients who underwent galvanic-VEMP testing showed normal responses. CONCLUSIONS: The A3243G mutation is associated with vestibular dysfunction involving both the superior and inferior vestibular nerve systems. Furthermore, our results from galvanic-VEMP testing suggests that a labyrinthine lesion is primarily responsible for the symptoms of vestibular dysfunction. |
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Authors:
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Shinichi Iwasaki; Naoya Egami; Chisato Fujimoto; Yasuhiro Chihara; Munetaka Ushio; Akinori Kashio; Tatsuya Yamasoba |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: The Laryngoscope Volume: 121 ISSN: 1531-4995 ISO Abbreviation: Laryngoscope Publication Date: 2011 Aug |
Date Detail:
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Created Date: 2011-07-27 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8607378 Medline TA: Laryngoscope Country: United States |
Other Details:
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Languages: eng Pagination: 1821-4 Citation Subset: IM |
Copyright Information:
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Copyright © 2011 The American Laryngological, Rhinological, and Otological Society, Inc. |
Affiliation:
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Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Tokyo, Japan. iwashin-tky@umin.ac.jp. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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