In our study, we identified a variant of uncertain significance p.P101T in the KCNJ10 gene along with a likely pathogenic variant p.D754Ifs*5 in the SLC26A4 gene and a shortened CEVA haplotype, which involved only three SNPs in a proband with hearing impairment and EVA. Here, KCNJ10 is linked to Hearing impairment.