One rare neurodegenerative disorder with an autosomal recessive inheritance pattern is Wolfram syndrome (WS), which is caused by biallelic mutations of the Wolframin ER transmembrane glycoprotein (Wfs1) gene and first manifests as diabetes mellitus, followed by optic nerve atrophy, deafness, and symptoms of neurodegeneration1–3. Here, WFS1 is linked to deafness.