中华耳科学杂志Issue(1)：43-48,6.DOI:10.3969/j.issn.1672-2922.2015.01.008
硫胺素缺乏与听力损失
Thiamine Deficiency and Hearing Loss
摘要
Abstract
Thiamine, also known as vitamin B1, works as a crucial coenzyme facilitating energy production, and lipids and ribose metabolisms. Its deficiency can cause a range of diseases including Wernicke encephalopathy (WE), which is caused by selective and symmetrical damage of the diencephalon and brain stem. Patients with WE usually have prolonged in⁃ter-peak latencies on ABR tests, and occasionally suffer from evident hearing loss. On the other hand, mutations of gene SLC192A can deprive patients of the high affinity thiamine transporter, THTR-1, and cause thiamine-responsive megaloblas⁃tic anemia (TRMA). Due to the insufficient thiamine transported into inner ear tissues, patients with TRMA will suffer from a progressive sensorineural hearing loss. Because WE does not guarantee a symptom of hearing loss, and TRMA is also a rela⁃tively rare genetic disease, they are not very familiar to audiology researchers. But in fact, these two diseases are caused by different responses to thiamine deficiency by different tissues in the auditory pathway. In this paper, for the first time, we sys⁃tematically reviewed thiamine related hearing loss. By describing mechanisms and relevant animal models of these two diseas⁃es, we hope the proposed theories can be applied to auditory studies and help researchers better understand the pathology of central hearing loss and interactions among different tissues of the inner ear.关键词
硫胺素/韦尼克脑病/硫胺响应性巨幼细胞贫血综合征/感音神经性听力损失/内耳Key words
Thiamine/Wernicke encephalopathy/Thiamine-responsive megaloblastic anemia/Sensorineural hearing loss/Inner ear分类
医药卫生引用本文复制引用
高可雷,丁大连,李鹏,孙虹,Richard Salvi..硫胺素缺乏与听力损失[J].中华耳科学杂志,2015,(1):43-48,6.基金项目
国家自然科学基金面上项目81170912 （）
