Em. Raynor et Mj. Mulroy, SENSORINEURAL HEARING-LOSS IN THE MDX MOUSE - A MODEL OF DUCHENNE-MUSCULAR-DYSTROPHY, The Laryngoscope, 107(8), 1997, pp. 1053-1056
Sensorineural hearing loss has been identified in several types of mus
cular dystrophy, but few studies have investigated any relationship be
tween Duchenne muscular dystrophy and hearing. An animal model of Duch
enne muscular dystrophy, the mdx mouse, exhibits the same genetic defe
ct as humans. We performed brainstem auditory evoked responses on mdx
and control mice in order to assess sensorineural hearing loss. The am
plitude and latency of wave I for each animal were measured at increas
ing sound pressure levels. A significant increase in threshold and a d
ecrease in wave I amplitude were found in the mdx mice. These results
indicate that significant sensorineural hearing loss is associated wit
h muscular dystrophy in the mdx mouse. Possible cellular mechanisms co
ntributing to the hearing deficit are presented.