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首页> 外文期刊>Genetics research international >The Sound of Silence: Mouse Models for Hearing Loss
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The Sound of Silence: Mouse Models for Hearing Loss

机译：沉默的声音：听力损失的小鼠模型

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Sensorineural hearing loss is one of the most common disabilities in humans. It is estimated that about 278 million people worldwide have slight to extreme hearing loss in both ears, which results in an economic loss for the country and personal loss for the individual. It is thus critical to have a deeper understanding of the causes for hearing loss to better manage and treat the affected individuals. The mouse serves as an excellent model to study and recapitulate some of these phenotypes, identify new genes which cause deafness, and to study their rolesin vivoand in detail. Mutant mice have been instrumental in elucidating the function and mechanisms of the inner ear. The development and morphogenesis of the inner ear from an ectodermal layer into distinct auditory and vestibular components depends on well-coordinated gene expression and well-orchestrated signaling cascades within the otic vesicle and interactions with surrounding layers of tissues. Any disruption in these pathways can lead to hearing impairment. This review takes a look at some of the genes and their corresponding mice mutants that have shed light on the mechanism governing hearing impairment (HI) in humans.
机译：感音神经性听力丧失是人类最常见的残疾之一。据估计，全世界约有2.78亿人的双耳有轻度至极度听力丧失，这对该国造成经济损失，并给个人造成个人损失。因此，至关重要的是对听力损失的原因有更深入的了解，以更好地管理和治疗受影响的个体。小鼠是研究和概括其中某些表型，鉴定导致耳聋的新基因并研究其体内和详细作用的绝佳模型。突变小鼠在阐明内耳的功能和机制中发挥了作用。内耳从表皮层到不同的听觉和前庭组件的发育和形态发生取决于在耳小泡内协调良好的基因表达和精心设计的信号级联以及与周围组织的相互作用。这些途径的任何破坏都可能导致听力障碍。这篇综述着眼于一些基因及其相应的小鼠突变体，这些突变体阐明了人类听力障碍（HI）的调控机制。

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