This systematic review commissioned by the UK Department for the Environment, Food and Rural Affairs (Defra), considers how the evidence base for noise effects on health has changed following the recent reviews undertaken for the WHO Environmental Noise Guidelines. This systematic review assesses the quality of the evidence for environmental noise effects on mental health, wellbeing, and quality of life; birth and reproductive outcomes; and cognition for papers published since the WHO reviews (mid-2015 to March 2019), as well as for cancer and dementia (January 2014 to March 2019). Using the GRADE methodology (Grading of Recommendations Assessment, Development and Evaluation) most evidence was rated as low quality as opposed to very low quality in the previous reviews. There is now low-quality evidence for a harmful effect of road traffic noise on medication use and interview measures of depression and anxiety and low quality evidence for a harmful effect of road traffic noise, aircraft noise, and railway noise on some cancer outcomes. Many other conclusions from the WHO evidence reviews remain unchanged. The conclusions remain limited by the low number of studies for many outcomes. The quantification of health effects for other noise sources including wind turbine, neighbour, industrial, and combined noise remains a research priority.