Clinical and genetic data were examined for a total of 76 probands and affected family members (audiometric, ocular, and electron microscopy bioptic data were available for a minority of them) (Table 1 and Table S1): In the 11 families with COL4A5 variants, out of the 32 patients (14 males and 18 females) there was an evolution to renal failure in 50% of the males and 11% of the females at a mean age of 35 and 63 years, respectively (Table 1). Here, COL4A5 is linked to acute kidney injury.