Plays a role in pre-mRNA splicing by regulating the release and transfer of U4/U6.U5 tri-small nuclear ribonucleoprotein (tri-snRNP) complexes from their assembly site in Cajal bodies to nuclear speckles, thereby contributing to the assembly of the pre-catalytic spliceosome on target pre-mRNAs (PubMed:34023904). May also participate in recycling of snRNPs back to Cajal bodies during splicing (PubMed:34023904). Plays a role in regulating MAGI2-mediated endocytosis (PubMed:24608321). Anchoring/scaffolding protein that is a part of the functional network formed by USH1C, USH1G, CDH23 and MYO7A that mediates mechanotransduction in cochlear hair cells. Required for normal development and maintenance of cochlear hair cell bundles. Required for normal hearing. Part of a complex composed of USH1C, USH1G and MYO7A (PubMed:21709241, PubMed:21311020). Interacts with USH1C (via the first PDZ domain) (PubMed:20142502, PubMed:12588794). Interacts with PDZD7 (PubMed:19028668). Interacts with CDH23 and PCDH15; these interactions may recruit USH1G to the plasma membrane (By similarity). Interacts with intraflagellar transport proteins IFT20, IFT52 AND IFT57 (PubMed:31637240). Interacts with splicing factors SF3B1, PRPF6, PRPF31 and SON (PubMed:34023904). Interacts with the U4/U6.U5 tri-small nuclear ribonucleoprotein (tri-snRNP) complex in the presence of pre-mRNAs (PubMed:34023904). Interacts (via SAM domain) with MAGI2 (via PDZ 6 domain); the interaction is triggered by phosphorylation of USH1G by CK2 and negatively regulates MAGI2-mediated endocytosis (PubMed:24608321). Detected at the tip of cochlear hair cell stereocilia. Recruited to the cell membrane via interaction with CDH23 or PCDH15 (By similarity). In photoreceptor cilia, detected predominantly at the cilium base (By similarity). Expressed in the pericentriolar region of the centrosome (By similarity). Expressed in vestibule of the inner ear, eye and small intestine. The disease is caused by variants affecting the gene represented in this entry. The first cases with non-syndromic sensorineural hearing loss based on mutations in USH1G. The hearing loss has an onset during early childhood, is progressive, and has a downsloping audiogram configuration. Ophthalmic and vestibular abnormalities are absent.