In cochlear developing hair cells, essential in organizing the USH2 complex at stereocilia ankle links. Blocks inhibition of adenylate cyclase activity mediated by ADGRV1. Homodimerizes (via PDZ2 domain). Component of USH2 complex, composed of ADGRV1, PDZD7, USH2A and WHRN. Interacts (via PDZ domains) with WHRN; the interaction is direct (By similarity). Interacts with USH1G (PubMed:19028668). Interacts with ADGRV1 (via the cytoplasmic region) (PubMed:20440071). Interacts with USH2A (via the cytoplasmic region) (PubMed:20440071). Interacts with MYO7A (via MyTH4-FERM domains) (By similarity). Localizes at the ankle region of the stereocilia. Weakly expressed in the inner ear. Expressed in the retinal pigment epithelium. The disease is caused by variants affecting the gene represented in this entry. A chromosomal aberration disrupting PDZD7 has been found in patients with non-syndromic sensorineural deafness. Translocation t(10;11),t(10;11). The disease is caused by variants affecting distinct genetic loci, including the gene represented in this entry. A PDZD7 mutation has been found in combination with a mutation in ADGRV1 in a patient affected by Usher syndrome, suggesting PDZD7 mutations contribute to digenic Usher syndrome. The gene represented in this entry acts as a disease modifier.