SENSORINEURAL HEARING-LOSS IN THE MDX MOUSE - A MODEL OF DUCHENNE-MUSCULAR-DYSTROPHY

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.