The combined effect of the p.Pro2126Leufs*5 mutation identified in South African DFNB2 families on the myosin molecule would likely lead to a non-functional, unregulated protein that fails to form functional tripartite complexes in the hair cell stereocilia and the tip-links, leading to the failure of the MET process, and, we predict, through this, result in hearing loss. Here, MYO7A is linked to hearing loss disorder.