硫胺素缺乏与听力损失

发布时间：2018-11-09 09:08

【摘要】：硫胺素即维生素B1,在机体中会被转化为辅酶参与能量、脂类及核酸代谢等生理过程。机体缺乏硫胺素可以导致韦尼克脑病(WE),其患者由于双侧对称的间脑和脑干损害,常常出现ABR的波间期延长并时有听力下降的症状。SLC19A2基因变异会导致硫胺响应性巨幼细胞贫血综合征(TRMA),其患者由于缺乏该基因编码的硫胺转运体THTR-1,使得内耳组织无法转运硫氨素,造成源于内耳的逐渐加重的感音神经性听力损失。由于听力下降不是WE中最典型的症状,TRMA也是不常见的遗传疾病,使得听力学研究者们对它们不甚熟悉,但其实两者的听力损失恰好是由于听觉通路的不同环节对硫胺素的依赖程度不同而造成的。本文首次系统地将缺乏硫胺素所造成的听力损失串联起来。通过介绍其中听力损失的发生机制以及相关的研究模型,我们希望能将这些理论应用于听力学研究,帮助研究者们加深对中枢性听力损失的发病机制以及内耳不同组织间的相互作用的理解。
[Abstract]:Thiamine, vitamin B1, is converted into coenzymes to participate in physiological processes such as energy, lipids and nucleic acid metabolism. Lack of thiamine in the body can cause (WE), to cause bilateral symmetrical diencephalon and brainstem damage. SLC19A2 gene mutation can lead to thiamine responsive megaloblastic anemia syndrome (TRMA), patients due to the lack of the gene encoded thiamine transporter THTR-1, The internal ear tissue is unable to transport thiamine, resulting in a gradual increase in sensorineural hearing loss from the inner ear. Because hearing loss is not the most typical symptom of WE and TRMA is an uncommon genetic disorder, audiology researchers are not familiar with them. In fact, the hearing loss is caused by the different dependence on thiamine in different parts of auditory pathway. For the first time, the hearing loss caused by thiamine deficiency is systematically connected in this paper. By introducing the mechanism of hearing loss and the relevant research models, we hope to apply these theories to audiology research. It helps the researchers to understand the pathogenesis of central hearing loss and the interaction between different tissues of the inner ear.
【作者单位】：中南大学湘雅医院耳鼻喉头颈外科;Center
【基金】：国家自然科学基金面上项目:81170912
【分类号】：R764

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