DFNA20/26-related hearing loss shows considerable variability in both severity and age of onset. In this study, the authors discovered a new ACTG1 variant (p.Ile327Met) of DFNA20/26 and found that its location in a protein-binding region of the γ-actin molecule leads to earlier and more severe hearing loss. These findings provide insight into how mutation sites influence hearing loss progression and may help guide future diagnosis and treatment strategies. (https://jmg.bmj.com/content/early/2025/06/16/jmg-2024-110573 )