MYO7A encodes an unconventional myosin motor protein that functions as an actin-based ATPase involved in intracellular vesicle trafficking. In the retina, MYO7A plays critical roles in outer photoreceptor disk renewal, retinal pigment epithelial melanosome and phagosome distribution, and opsin transport in photoreceptors 1. In the inner ear, MYO7A is essential for cochlear hair cell bundle differentiation, morphogenesis, and organization, and participates in the USH1 protein interactome mediating mechanotransduction 1. MYO7A dysfunction causes three distinct clinical phenotypes: non-syndromic autosomal dominant hearing loss (DFNA11), autosomal recessive hearing loss (DFNB2), and syndromic Usher syndrome type 1B (USH1B) characterized by congenital hearing loss and progressive retinitis pigmentosa 2. Among Japanese hearing loss patients, MYO7A variants occur in 1.36% of cases 2, while in Chinese populations MYO7A ranks among the top deafness genes 3. Autosomal dominant cases typically present late-onset progressive hearing loss from missense variants, whereas autosomal recessive and Usher syndrome cases show congenital or early-onset hearing loss, with vision loss emerging in ages 5-15 years 2. MYO7A is the most frequently mutated gene in USH1 families (accounting for 53.2%-60% of cases), underscoring its clinical significance in syndromic inherited disorders 45.