In this study, we have successfully identified a novel insertion variant in exon 8 of the GSDME gene (GSDME: NM_004403.3: c.1113_1114insGGGGTGCAGCTTACAGGGTGGGTGT: p.P372fs*36) associated with hearing loss in a large Chinese pedigree family, thereby expanding the variant spectrum. This evidence concerns the gene GSDME and hearing loss disorder.