Sensorineural Hearing Loss in themdx Mouse: A Model of Duchenne Muscular Dystrophy

Sensorineural hearing loss has been identified in several types of muscular dystrophy, but few studies have investigated any relationship between Duchenne muscular dystrophy and hearing. An animal model of Duchenne muscular dystrophy, themdx mouse, exhibits the same genetic defect as humans. We perf...

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Published in:The Laryngoscope 1997-08, Vol.107 (8), p.1053-1056
Main Authors: Raynor, Eileen M., Mulroy, Michael J.
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Mulroy, Michael J.
description Sensorineural hearing loss has been identified in several types of muscular dystrophy, but few studies have investigated any relationship between Duchenne muscular dystrophy and hearing. An animal model of Duchenne muscular dystrophy, themdx mouse, exhibits the same genetic defect as humans. We performed brainstem auditory evoked responses on mdx and control mice in order to assess sensorineural hearing loss. The amplitude and latency of wave I for each animal were measured at increasing sound pressure levels. A significant increase in threshold and a decrease in wave I amplitude were found in the mdx mice. These results indicate that significant sensorineural hearing loss is associated with muscular dystrophy in the mdx mouse. Possible cellular mechanisms contributing to the hearing deficit are presented.
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title Sensorineural Hearing Loss in themdx Mouse: A Model of Duchenne Muscular Dystrophy
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