Steel K P, Barkway C, Bock G R
Hear Res. 1987;27(1):11-26. doi: 10.1016/0378-5955(87)90022-0.
Most viable dominant spotting (Wv/Wv) mutant mice, which show cochleo-saccular degeneration, were found to have an endocochlear potential (EP) around zero together with a structurally abnormal stria vascularis. Inner hair cells were well preserved, but outer hair cells in the basal half of the cochlea were degenerating, possibly as a result of primary strial dysfunction. Thresholds for the detection of a compound action potential were raised to around 100 dB SPL in the mutants with no EP, and there was little if any cochlear microphonic at the round window. Of the 20 Wv/Wv mice studied, five partially escaped the effects of the mutation and had measurable positive potentials (15-86 mV) in scala media in the basal turn; responses in these animals were intermediate between control responses and those of mutants with no EP. These findings confirm that the pathological processes in this mutant, with cochleo-saccular abnormalities, are fundamentally different from the pathological processes in animals with neuroepithelial abnormalities reported previously [see Steel and Bock (1983) Arch. Otolaryngol. 109, 22-29, for references].
大多数具有存活能力的显性斑点（Wv/Wv）突变小鼠表现出蜗球囊变性，其耳蜗内电位（EP）接近零，同时血管纹结构异常。内毛细胞保存良好，但耳蜗基部一半的外毛细胞正在退化，这可能是原发性血管纹功能障碍的结果。在没有EP的突变体中，复合动作电位的检测阈值提高到约100 dB SPL，圆窗处几乎没有耳蜗微音器电位。在研究的20只Wv/Wv小鼠中，有5只部分逃脱了突变的影响，在基部转的中阶有可测量的正电位（15 - 86 mV）；这些动物的反应介于对照反应和没有EP的突变体的反应之间。这些发现证实，这种具有蜗球囊异常的突变体中的病理过程与先前报道的具有神经上皮异常的动物中的病理过程有根本不同[参考文献见Steel和Bock（1983年）《耳鼻咽喉科文献》109卷，22 - 29页]。
