In our study, we successfully identified a novel insertion variant of GSDME in exon 8 (GSDME: NM_004403.3: c.1113_1114insGGGGTGCAGCTTACAGGGTGGGTGT: p.P372fs*36) that segregated with the occurrence of hearing loss of the affected family. This evidence concerns the gene GSDME and hearing loss disorder.