Here, we enrolled 21 Chinese patients with either syndromic or nonsyndromic HL who were previously evaluated and were not found to have hotspot mutations in the common deafness genes GJB2, SLC26A4, MT‐RNR1, and GJB3. Next‐generation sequencing (NGS) technologies, including targeted NGS (TGS) and whole‐exome sequencing (WES), were performed on the probands of each family to identify rare pathogenic mutations. The gene discussed is GJB2; the disease is deafness.