Mutations in POU3F4 (OMIM *300039), which lead to X-linked DFN3/DFNX2 (OMIM #304400) associated with cochlear incomplete partition type 3 (IP3), are rare in EVA cohorts, and from 0 to 50% of POU3F4 patients have been found to have an EVA (de Kok et al. 1995; Pollak et al. 2016; Gong et al. 2014). This evidence concerns the gene POU3F4 and X-linked mixed hearing loss with perilymphatic gusher.