MYO15A encodes myosin XVA, an unconventional myosin motor protein with ATPase activity that functions as an actin-based motor molecule 1. The protein is essential for organizing stereocilia in mature hair bundles of the inner ear, where it binds actin filaments and facilitates actin-based cellular projections critical for mechanotransduction 1. MYO15A mutations cause autosomal recessive non-syndromic sensorineural hearing loss (NSHL), classified as deafness autosomal recessive 3 (DFNB3) 1. Clinically, MYO15A ranks among the most common deafness genes globally, accounting for 3.58% of Chinese NSHL cases and appearing among the top five causative genes in multiple populations including Japanese, Chinese, and Taiwanese cohorts 123. Pathogenic variants predominantly cause early-onset, bilateral, symmetric, severe-to-profound hearing loss, typically congenital in presentation 1. Notably, biallelic non-truncating variants commonly result in profound hearing loss, while variants affecting the N-terminal, motor, and FERM domains may produce partial deafness with preserved low-frequency hearing 1. Additionally, MYO15A variants have been implicated in age-related hearing loss pathways involving apoptosis and oxidative stress mechanisms 4. The high prevalence of novel variants (76.47% in one cohort) underscores significant genetic heterogeneity requiring population-specific molecular screening strategies 1.