Combined cochleo-saccular and neuroepithelial abnormalities in the Varitint-waddler-J (VaJ) mouse
Combined cochleo-saccular and neuroepithelial abnormalities in the Varitint-waddler-J (VaJ) mouse
复制标题
DOI：
10.1016/s0378-5955(98)00107-5
复制
发表时间：
1998-09-01
期刊：
影响因子：
2.8
通讯作者：
Steel, KP
中科院分区：
文献类型：
--
作者：
Cable, J;Steel, KP
Varitint-waddler-J(Va(J))小鼠的听力损失是由耳蜗囊和神经上皮成分混合引起的。Va(J)/Va(J)和Va(J)/+突变体均表现出耳蜗功能受损，但纯合子的影响比杂合子更严重。这两组人都没有任何可检测到的复合动作电位。只有一半的杂合子出现了耳蜗微音符，但幅度降低，阈值升高，在任何纯合子中都没有检测到。在大多数杂合子中，在高刺激水平下都可以看到总和电位(SP)反应。纯合子中唯一的反应是在非常高的声级下，一半突变体的SPS为负，而其余的纯合子对声音刺激没有反应。在两组突变中，耳蜗内电位(EP)通常很小或不存在，纯合子受到的影响更严重。血管纹中色素沉着的减少似乎与EP的减少有关，而14天龄小鼠毛细胞中可通过电子显微镜检测到的神经上皮的原发缺陷显著影响诱发电位。(C)1998 Elsevier Science B.V.保留所有权利。
Hearing loss in Varitint-waddler-J (Va(J)) mice is of mixed origin with both cochleo-saccular and neuroepithelial components. Both Va(J)/Va(J) and Va(J)/+ mutants show impaired cochlear function, but the homozygotes are more severely affected than heterozygotes. Neither group have any detectable compound action potential. Cochlear microphonics are only seen in half of the heterozygotes, at a reduced amplitude and raised threshold, and are not detected in any homozygotes. Summating potentials (SP) responses are seen in most of the heterozygotes, at high stimulus levels. The only responses in homozygotes were negative SPs seen in half of the mutants at very high sound levels, while the remaining homozygotes showed no responses to sound stimulation. Endocochlear potentials (EP) were often small or absent in both groups of mutants, with the homozygotes being more severely affected. Reduced pigmentation in the stria vascularis appears to be associated with a reduced EP, while a primary defect of the neuroepithelium, detectable by electron microscopy in hair cells of 14 day old mice, dramatically influences evoked potentials. (C) 1998 Elsevier Science B.V. All rights reserved.