TFEB plays pivotal roles in autophagy and lysosomal biogenesis.[12] Diminished nuclear localization of TFEB is linked to the progression of various neurodegenerative diseases.[13, 14, 15] Enhancing TFEB expression and nuclear localization using small‐molecule drugs can improve autophagy, thereby reducing damage to HCs and spiral neurons.[16, 17] Nevertheless, the precise mechanisms by which TFEB controls autophagy in ARHL remain unclear. This evidence concerns the gene TFEB and neurodegenerative disease.