WHRN (whirlin) is a cytoskeletal scaffold protein essential for hearing and vision, functioning as a core component of the ankle link complex (ALC) in the inner ear and periciliary membrane complex in the retina 1. In cochlear hair cells, WHRN is necessary for elongation and maintenance of stereocilia and organization of the hair bundle ankle region, which mechanically connects stereocilia 2. WHRN orchestrates ALC assembly with USH2A, ADGRV1, and PDZD7 through liquid-liquid phase separation, a process disrupted by deafness-associated mutations 2. The protein's phosphorylation state, regulated by ADGRV1-mediated cAMP-PKA signaling, controls USH2A stability through WDSUB1-dependent ubiquitination 3. In photoreceptors, WHRN maintains the periciliary membrane complex regulating intracellular protein transport 4. Pathogenic WHRN variants cause both non-syndromic deafness and Usher syndrome type 2 (USH2), the most common cause of deaf-blindness 1, affecting approximately 1 in 10,000 individuals worldwide 1. Gene therapy and cell-based therapeutic approaches for USH2 are under investigation 4. WHRN mutations impair stereocilia architecture and mechanoelectrical transduction, ultimately resulting in hearing loss and progressive retinal degeneration 3.