The ABCC1 gene was first proposed as a pathogenic gene in hereditary deafness,mainly causing non-syndromic deafness.The ABCC1 gene encodes multidrug resistance-associated protein 1,which is involved in substance transport and effection across the blood-labyrinthian barrier.It maintains the stability of the labyrinthine microenviron-ment in the inner ear.However,the function of ABCC1 and its roles in pathogenesis in inner ear disorders have not been fully elucidated.This paper reviews the progress of research on the ABCC1 gene in hereditary deafness to provide a new basis for the research on theABCC1 gene.