Abstract Background It is estimated that hearing difficulties will be one of the top ten leading burdens of disease by 2030. Knowledge of mortality among individuals on sick leave or disability pension due to hearing diagnoses is virtually non-existent. We aimed prospectively to examine the associations of diagnosis-specific sick leave and disability pension due to different otoaudiological diagnoses with risks of all-cause and cause-specific mortality. Methods A cohort, based on Swedish registry data, including all 5 248 672 individuals living in Sweden in 2005, aged 20–64, and not on old-age pension, was followed through 2010. Otoaudiological diagnoses were placed in the following categories: otological, hearing, vertigo, and tinnitus. Hazard ratios (HRs) with 95% confidence intervals (CIs) were estimated using Cox proportional hazard models; individuals on sick leave or disability pension due to different otoaudiological diagnoses during 2005 were compared with those not on sick leave or disability pension. Results In multivariable models, individuals with sickness absence due to otoaudiological diagnoses showed a lower risk of mortality, while individuals on disability pension due to otoaudiological diagnoses showed a 14% (95% CI 1-29%) increased risk of mortality, compared with individuals not on sick leave or disability pension. The risk increase among individuals on disability pension was largely attributable to otological (HR 1.56; 95% CI = 1.04-2.33) and hearing diagnoses (HR 1.20; 95% CI = 1.00-1.43). Conclusion This large nationwide population-based cohort study suggests an increased risk of mortality among individuals on disability pension due to otoaudiological diagnoses.