Raynor E M, Mulroy M J
Department of Surgery, Medical College of Georgia, Augusta 30912, U.S.A.
Laryngoscope. 1997 Aug;107(8):1053-6. doi: 10.1097/00005537-199708000-00009.
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.
在几种类型的肌肉萎缩症中已发现感音神经性听力损失，但很少有研究调查杜氏肌营养不良症与听力之间的关系。杜氏肌营养不良症的动物模型——mdx小鼠，与人类存在相同的基因缺陷。为了评估感音神经性听力损失，我们对mdx小鼠和对照小鼠进行了脑干听觉诱发电位测试。在不断增加的声压水平下测量了每只动物I波的振幅和潜伏期。发现mdx小鼠的阈值显著升高，I波振幅降低。这些结果表明，mdx小鼠的感音神经性听力损失与肌肉萎缩症有关。本文还提出了导致听力缺陷的可能细胞机制。
