MYH14 encodes a nonmuscle myosin heavy chain II that plays critical roles in cellular processes and auditory function. The protein exists in two alternatively spliced isoforms (NMHCII-C1 and NMHCII-C0) that differ by an 8-amino acid insertion in the globular head domain 1. MYH14 functions as a master regulator of actin dynamics and contributes to cytokinesis, cell shape regulation, and specialized cellular functions 2. The protein interacts with MYH9 to regulate GSK3β-mediated β-catenin signaling pathways 3. Pathogenic variants in MYH14 cause autosomal dominant nonsyndromic hearing loss (DFNA4A), with clinical presentations ranging from mild-to-moderate progressive hearing loss to severe prelingual deafness 4 5. Additionally, MYH14 mutations are associated with a complex neuromuscular syndrome characterized by peripheral neuropathy, myopathy, hoarseness, and hearing loss 6. Recent cases have expanded the phenotypic spectrum to include severe distal myopathy with early respiratory failure 6. In myotonic dystrophy type 1, aberrant MYH14 splicing and reduced expression contribute to disease pathogenesis 1. MYH14 represents one of the significant contributors to autosomal dominant hearing loss in European populations, accounting for approximately 4% of cases 7.