Thiamine Deficiency and Hearing Loss
GAO Kelei
DING Dalian
LI Peng
SUN Hong
Richard Salvi
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.
Keywords：ThiamineWernicke encephalopathyThiamine-responsive megaloblastic anemiaSensorineural hearing lossInner ear
Publication Date：2015-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：6( 43-48 )