Initial insights into GSDMs emerged in the early 2000s (Fig. 1).15 Saeki and colleagues cloned the mouse gene, GSDM, which bore the signature of the deafness autosomal dominant non-syndromic sensorineural 5 (DFNA5) gene.15 The term GSDM is derived from its selective expression in the mouse gastrointestinal tract and epithelial layers of the skin, an essential step in pinpointing the gene responsible for the Rim3 mutation in mice. The gene discussed is GSDMA; the disease is deafness.