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Sensorineural Hearing Loss in the mdx Mouse: A Model of Duchenne Muscular Dystrophy
Author(s) -
Raynor Eileen M.,
Mulroy Michael J.
Publication year - 1997
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1097/00005537-199708000-00009
Subject(s) - duchenne muscular dystrophy , mdx mouse , sensorineural hearing loss , hearing loss , audiology , muscular dystrophy , medicine , auditory brainstem response , brainstem , dystrophin
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, the mdx 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.