We screened 127 genes known to be related to deafness by gene capture and next-generation sequencing and identified the novel variant c.5417C > A (p.A1806D) in MYH14 on the tail domain of NMH II C. By reporting novel pathogenic variants, the phenotypic spectrum of the MYH14 gene could be broadened in the field of hereditary hearing loss. This evidence concerns the gene MYH14 and deafness.