Involved in hearing and vision as member of the USH2 complex. In the inner ear, required for the hair bundle ankle formation, which connects growing stereocilia in developing cochlear hair cells. In retina photoreceptors, the USH2 complex is required for the maintenance of periciliary membrane complex that seems to play a role in regulating intracellular protein transport. Interacts with collagen IV and fibronectin via its laminin EGF-like domains. Interaction with collagen may be required for stable integration into the basement membrane. Interacts with NINL (By similarity). Interacts with USH1C. Interacts (via the cytoplasmic region) with PDZD7. Component of USH2 complex, composed of ADGRV1, PDZD7, USH2A and WHRN. Interacts with ADGRV1/MASS1 (via N-terminal PDZ domain). Interacts (via the cytoplasmic region) with WHRN. Interacts (via the cytoplasmic region) with VEZT and MYO7A (via MyTH4-FERM domains); the interaction associates VEZT with the USH2 complex at the stereocilia base (By similarity). Component of the interstereocilia ankle links in the inner ear sensory cells. In photoreceptors, localizes at a plasma membrane microdomain in the apical inner segment that surrounds the connecting cilia called periciliary membrane complex. A number of isoforms are produced. Present in the synaptic terminals of inner ear hair cells (at protein level). Predominantly expressed in the retina and cochlea. Weakly expressed in brain and kidney. Detectable from E17 in the neural epithelium, but not in the retinal pigment epithelium (RPE) of the developing retina. After birth, it is expressed at P7 and remains expressed during adulthood.