Wolfram syndrome is a neurodegenerative disease characterized by neurological symptoms, as well as diabetes, optic atrophy and hearing loss, among others. The WFS1 gene encodes a protein named Wolframin and it accounts for about 99% of cases, 60% of which present with hearing loss. To date, of all animal models of Wolfram syndrome developed carrying a variant or deletion of one exon of Wfs1, none mimics the early-onset hearing loss.