Connexin 26-related hearing loss is a genetic condition caused by mutations in the GJB2 gene, which encodes the connexin 26 protein. This protein is essential for the proper functioning of the inner ear, where it helps in the communication between cells. When the protein is defective, it can lead to sensorineural hearing loss, which affects the ability to hear sounds clearly. This type of hearing loss is often present at birth or develops in early childhood. It can vary in severity, ranging from mild to profound. Early diagnosis and intervention, such as hearing aids or cochlear implants, can significantly improve communication abilities for those affected.