Despite a high level of public awareness regarding the importance of hearing preservation and increasingly stringent international occupational health, safety and welfare requirements mandating provision of safer work environments, ONIHL continues to be a significant occupational hazard. ONIHL is permanent and may cause significant disability, for which there currently exists no cure, but is largely overtly-preventable. The impact of ONIHL on the global transition toward dominant communication-rich white-collar employment roles is difficult to quantitate, but is likely to be substantive upon the afflicted individual. In the mainstream setting, exposure-avoidance strategies aimed to reduce the incidence of ONIHL remain the focus of public health and occupational medicine approaches.

This review explores the association between GJB2 gene mutations, encoding connexin 26 (Cx26), and nonsyndromic hearing loss. Connexins are proteins that form intracellular membrane channels and regulate ion movement between contiguous fluid spaces. A family of autosomal gene mutations has been identified that lead to abnormal connexin expression within the inner ear that are associated with hearing loss. The exact mechanism by which this link is elicited remains unclear. We aim to highlight the clinically underestimated prevalence of GJB2 gene mutations, to explore the influential role of ethnic diversity in mutation frequency, and to provide a framework for hearing specialists in considering the differential diagnosis of nonsyndromic hearing loss. By linking an observed phenotype associated with abnormal Cx26 expression to the current understanding of the biological and genetic basis underlying it will allow a more accurate clinical description of associated hearing loss, and therefore enable more effective patient management and genetic counselling.