Sensorineural hearing loss in insulin-like growth factor I-null mice:: a new model of human deafness
Sensorineural hearing loss in insulin-like growth factor I-null mice:: a new model of human deafness
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DOI：
10.1111/j.1460-9568.2005.04584.x
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发表时间：
2006-01-01
影响因子：
3.4
通讯作者：
Varela-Nieto, I
中科院分区：
文献类型：
--
作者：
Cediel, R;Riquelme, R;Varela-Nieto, I
据报道，编码人胰岛素样生长因子-I(IGF-I)的基因突变会导致综合征性听力损失。为了研究IGF-I在听觉功能中的确切作用，并推测人类内耳可能发生的形态和电生理变化，我们分析了IGF-I缺乏的小鼠模型的听性脑干反应。我们在这里表明，纯合子IGF-1(-/-)小鼠表现出所有频率的双侧感音神经性听力损失。IGF-1(-/-)小鼠对声刺激也表现出延迟反应；这种延迟反应沿听觉通路增加，表明中枢神经系统对IGF-1(-/-)小鼠的听力损失起到了作用。这些结果支持使用IGF-1(-/-)小鼠作为研究人类综合征性耳聋的新模型。
It has been reported that mutations in the gene encoding human insulin-like growth factor-I (IGF-I) cause syndromic hearing loss. To study the precise role of IGF-I in auditory function and to hypothesize the possible morphological and electrophysiological changes that may occur in the human inner ear, we have analysed the auditory brainstem response in a mouse model of IGF-I deficiency. We show here that homozygous Igf-1(-/-) mice present an all-frequency involved bilateral sensorineural hearing loss. Igf-1(-/-) mice also present a delayed response to acoustic stimuli; this increases along the auditory pathway, indicating a contribution of the central nervous system to the hearing loss in Igf-1(-/-) mice. These results support the use of the Igf-1(-/-) mouse as a new model for the study of human syndromic deafness.