Individuals with mutations in WFS1 present with three different phenotypes: AR Wolfram syndrome (WS, MIM #222300) featuring sensorineural HL, diabetes mellitus (DM), optic atrophy (OA) and various forms of neurologic impairment, diabetes insipidus and urinary dysfunctions, AD progressive HL with OA or impaired glucose regulation also called Wolfram‐like syndrome (WLS, MIM #614296), and DFNA6/14/38‐associated AD nonsyndromic sensorineural HL (NSHL, MIM #600965) (Grenier et al., 2016; Niu et al., 2017). Here, WFS1 is linked to optic atrophy.