New research has discovered that mutations in the TMTC4 gene lead to the activation of the unfolded protein response (UPR), causing the death of hair cells in the inner ear. This UPR activation also plays a role in hearing loss from loud noise or certain drugs, indicating its involvement in various forms of deafness. Inhibiting the UPR in sensory hair cells may present a potential approach to slowing down the loss of hearing or protecting against the damaging effects of loud noise and ototoxic drugs. However, this does not address the restoration of lost hearing capacity, which would require a means of replacing the lost hair cells and their connections to the brain. The findings highlight the potential for testing UPR-blocking drugs in individuals at risk of hearing loss. For more information, visit: Link to the article.