Variants in the ATP6V1B1 or ATP6V0A4 genes diminish the V‐ATPase proton‐secreting function and, since these subunits are also expressed in the inner ear, they can be associated with sensorineural hearing loss (SNHL)[18, 19, 21, 23]. This evidence concerns the gene ATP6V0A4 and sensorineural hearing loss disorder.