PJVK (pejvakin) is a peroxisome-associated protein essential for protecting auditory hair cells against noise-induced oxidative damage 1. The protein regulates noise-induced peroxisome proliferation and promotes autophagic degradation of damaged peroxisomes (pexophagy) in auditory hair cells and neurons 1. When exposed to sound overstimulation, PJVK acts as a reactive oxygen species sensor that recruits autophagy machinery to eliminate oxidatively damaged peroxisomes, while simultaneously promoting peroxisome proliferation as an adaptive response 1. At the cellular level, pejvakin localizes to stereociliary rootlets in hair cells, where it binds to and colocalizes with the rootlet component TRIOBP, playing a critical role in maintaining stereocilia architecture and mechanotransduction function 2. Mutations in PJVK cause DFNB59, an autosomal recessive non-syndromic hearing loss with variable phenotypes ranging from cochlear hearing loss to auditory neuropathy spectrum disorder 3. The protein belongs to the gasdermin family (also known as DFNB59), though unlike other family members, PJVK lacks pore-forming activity and does not induce pyroptosis 4. Clinically, patients with PJVK mutations show hypervulnerability to sound exposure and may benefit from cochlear implantation 3.