Objectives/Hypothesis: The pathophysiology of idiopathic sudden sensorineural hearing loss (ISSNHL) is still unknown, but labyrinthine artery infarction has been proposed. The objective of this study was to perform a systematic review and conduct a meta-analysis assessing the risk of developing stroke and myocardial infarction after presentation with ISSNHL.Methods: A systematic literature review was conducted using Pubmed, Embase, Web of Science, and Cochrane Libraries. All studies investigating an association between ISSNHL and stroke and/or myocardial infarction (MI) were included. Adhering to the MOOSE guideline, two independent reviewers extracted data, assessed risk of bias, and evaluated the relevance and quality of evidence.Results: Three observational studies evaluating the risk of stroke in ISSNHL were included (n = 6,521 patients). All individual study results indicated an increased relative risk of stroke after ISSNHL (unadjusted relative risk range 1.21-1.63). Pooled adjusted hazard ratios revealed a 1.42-fold increased risk of stroke after ISSNHL (hazard ratio [HR] 1.42; 95% confidence interval [CI] 1.15-1.75, I 2 = 55%). Subgroup analysis of one study demonstrated that the increased risk is only present in adults aged above 50 years (HR 1.23; 95% CI 1.07-1.42). Five observational studies evaluating the risk of MI in patients with ISSNHL were included (n = 61,499 patients). Pooled analyses demonstrated that ISSNHL was not associated with MI (HR 1.08, 95% CI 0.87-1.34).Conclusion: ISSNHL may be an independent risk factor for the subsequent development of stroke especially in a subgroup of elderly patients. More studies are needed to confirm this association and to assess whether such patients would benefit from cardiovascular risk assessment and management to prevent future strokes.