Document Detail

The mitochondrial A3243G mutation involves the peripheral vestibule as well as the cochlea.
MedLine Citation:
PMID:  21792976     Owner:  NLM     Status:  In-Data-Review    
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.
Shinichi Iwasaki; Naoya Egami; Chisato Fujimoto; Yasuhiro Chihara; Munetaka Ushio; Akinori Kashio; Tatsuya Yamasoba
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Laryngoscope     Volume:  121     ISSN:  1531-4995     ISO Abbreviation:  Laryngoscope     Publication Date:  2011 Aug 
Date Detail:
Created Date:  2011-07-27     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8607378     Medline TA:  Laryngoscope     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1821-4     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 The American Laryngological, Rhinological, and Otological Society, Inc.
Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
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