USH1G encodes SANS, a multifunctional scaffolding protein central to both splicing and mechanotransduction. In the nucleus, USH1G regulates pre-mRNA splicing by controlling the release and transfer of U4/U6.U5 tri-snRNP complexes from Cajal bodies to nuclear speckles, facilitating pre-catalytic spliceosome assembly 1. USH1G also participates in snRNP recycling during splicing 2. Notably, USH1G functions as a scaffold within a mechanotransduction network with USH1C, CDH23, and MYO7A in cochlear hair cells, where these proteins form condensed assemblies via liquid-liquid phase separation at stereocilia tip-link densities 3. This complex is essential for normal hearing and hair cell bundle development and maintenance. USH1G involvement in MAGI2-mediated endocytosis regulation further expands its functional repertoire 4. Pathogenic USH1G variants cause Usher syndrome type 1G, characterized by combined hearing loss and progressive vision loss 5. However, USH1G mutations appear relatively rare, representing a minor contributor to overall Usher syndrome pathogenesis compared to MYO7A and USH1C 6. Digenic inheritance involving USH1G and PCDH15 has been documented in non-syndromic hearing impairment, demonstrating functional epistasis between mechanotransduction components 7.