GRXCR1 encodes a 290 amino acid protein containing a glutaredoxin-like domain and a cysteine-rich C-terminal region that plays a critical role in stereocilia morphogenesis in inner ear hair cells 1. The protein localizes along the length of stereocilia, the actin-filament-rich mechanosensory structures essential for hearing and balance 1. GRXCR1 functions to regulate actin cytoskeletal architecture during stereocilia development, with loss of function resulting in abnormally thin and shortened stereocilia 1. Unlike its paralog GRXCR2, GRXCR1 is diffusely distributed throughout stereocilia and has distinct functions in stereocilia morphogenesis, affecting F-actin content and interacting with GRXCR2 2. The gene shows preferential expression in fetal cochlea 3. Mutations in GRXCR1 cause autosomal recessive nonsyndromic hearing loss (DFNB25), characterized by congenital moderate to profound hearing loss that can be progressive 34. Clinical presentations include sensorineural hearing loss with variable vestibular dysfunction, including dizziness and canal paralysis in some patients 4. Various mutation types have been identified, including splice-site, missense, nonsense, and deletion mutations across different populations 345.