LOXHD1 (lipoxygenase homology PLAT domains 1) is essential for normal hearing function, primarily through its role in maintaining auditory mechanotransduction in inner ear hair cells. LOXHD1 is indispensable for localizing and stabilizing TMC1, the pore-forming subunit of mechanosensitive ion channels at the stereocilial tip link, the site where sound-induced forces are transmitted 1. It selectively interacts with TMC1, CIB2, LHFPL5, and the tip-link protein PCDH15 to maintain functional mature auditory channels, though it is dispensable for the developmental TMC2-driven channels 1. Mutations in LOXHD1 cause autosomal recessive deafness (DFNB77), with LOXHD1 variants accounting for 33.3% of genetically diagnosed down-sloping sensorineural hearing loss cases in young adults, emerging as a signature causative gene in this population 2. LOXHD1 has been identified as a promising candidate for gene therapy approaches to hearing loss 3, offering potential clinical intervention for genetic deafness. Outside the auditory system, LOXHD1 shows restricted expression in the testis and serves as an oncofusion-regulated tumor-associated antigen in Ewing sarcoma 4. While LOXHD1 variants have been reported in Fuchs corneal dystrophy studies, its causal pathogenic role in corneal disease remains unconfirmed 5.