Expanded familial validation revealed that the sequence variation MYH14 c.541G>A at codon 181 (exon 3) (Fig. 3A) located in the motor domain, near the ATP binding site of the MYH14 gene (Fig. 3C), co-segregates with the deafness phenotype in all affected individuals (IB:2, IIB:2, IIB:3, IIIB:1 and IIIB:2) in part B of the composite pedigree (Fig. 1B), while the variant c.2594C>T; p.T865 M presents only in subjects IIIB:1 and IIIB:2. Here, MYH14 is linked to deafness.