In order to comprehensively evaluate the regional characteristics of deafness-related variants in different regions of Zhejiang province, we analyzed genetic variants between the remote mountain areas (971 cases) and island areas (500 cases) and found no statistical differences in positive rates of GJB2, GJB3, SLC26A4, and MT-RNR1 genes, P > 0.05. This evidence concerns the gene MT-RNR1 and deafness.