Our findings (i.e., the identification of the second family reported globally with HL caused by a variant in <i>NCOA3</i>) further support the involvement of <i>NCOA3</i> in the etiopathogenesis of ADNSHL, which should, thus, be considered as a new gene for autosomal dominant non-syndromic hearing loss. Here, NCOA3 is linked to autosomal dominant nonsyndromic hearing loss.