Hearing loss, likely confounded by the patients’ marked cognitive decline and therefore reported only in two BPAN cases (Rathore et al. 2014; Kaleka et al. 2019), was consistently observed in Wdr45 KO mice both from the progression cohort and phenotyping cohort, as revealed by the clickbox and ABR tests. This evidence concerns the gene WDR45 and neurodegeneration with brain iron accumulation 5.