Autosomal dominant nonsyndromic hearing loss caused by mutations in the TJP2 gene was observed in a Chinese family involving the missense mutation 2081G>A (G694E) [20], located within the GuK domain of ZO-2, and in the Korean population due to two pathogenic variations: 334G>A (A112T), which is present in the PDZ1 domain of ZO-2, and 3562A>G (T1188A), which is situated within the carboxyl terminal PDZ binding motif TEL of ZO-2 [19]. Here, TJP2 is linked to autosomal dominant nonsyndromic hearing loss.