Because hearing loss is not reported in the patients with a heterozygous deletion of ELMOD3, except for patient 3, the disease mechanism in a three-generation Chinese pedigree reported with ELMOD3-linked autosomal dominant non-syndromic deafness (DNFA81; OMIM #619500) may involve dominant-negative effects rather than haploinsufficiency [14,15,16]. The gene discussed is ELMOD3; the disease is autosomal dominant nonsyndromic hearing loss.