OTOG encodes otogelin, a glycoprotein component of inner ear acellular membranes, particularly the tectorial and otolithic membranes 1. The protein functions as an extracellular matrix structural constituent [UniProt], with roles in anchoring otoconial membranes and cupulae to the underlying neuroepithelia in the vestibule, and in organizing the fibrillar network of the tectorial membrane in the cochlea. OTOG likely participates in mechanotransduction processes critical for auditory and vestibular function. Biallelic mutations in OTOG cause autosomal recessive non-syndromic hearing loss designated DFNB18B 2. Clinical characterization of 26 probands with OTOG variants revealed predominantly congenital or childhood-onset, non-progressive mild-to-moderate hearing loss without accompanying symptoms 2. However, rare homozygous missense variants can associate with severe-to-profound hearing loss, likely through disruption of disulfide bonds affecting protein stability 1. Beyond isolated deafness, OTOG has emerged as a candidate gene in Meniere's disease pathogenesis. Multiplex rare missense variants in OTOG were identified in 33% of familial Meniere's disease cases 3, and exome sequencing identified OTOG variants in multiple families, suggesting possible digenic inheritance patterns 45. These findings implicate OTOG in both primary hearing loss and complex inner ear disorders involving vestibular dysfunction.