Family 2 (Figure 1B) had two candidate genes with possible pathogenic mutations: A unique heterozygous POU4F3 frameshift mutation, c.1007delC (p.A336Vfs), was detected in subjects III:1 and IV:3 with hearing loss, and a unique heterozygous DFNA5 nonsense mutation, c.781C >T (p.R261X), was detected in subjects III:2 and IV:3 with hearing loss, whereas subject IV:1 with normal hearing had neither of these mutations. Here, GSDME is linked to hearing loss disorder.