Objective: To determine if exfoliation syndrome (XFS) is associated with hearing loss (HL) or vestibular dysfunction. Data Sources: PubMed, Scopus, CINAHL, and Cochrane Library through April 1, 2022. Review Methods: Two reviewers independently screened abstracts, selected articles for inclusion, and extracted data. Studies included for qualitative analysis conducted audiometric, tympanometric, or vestibular evaluations on all subjects.Results: Twenty-one publications (1148 patients with XFS and 1212 controls) were included in the systematic review, and 16 publications (968 patients with XFS and 1147 controls) in the meta-analysis. Greater severity of HL was seen for patients with XFS compared to controls across all frequencies (odds ratio [OR] 8.8 [7.3-10.2]). Patients with XFS were more likely to have moderate to profound sensorineural HL ), and less likely to have none to mild HL (OR 0.34 [0.17-0.67]) or no HL (OR 0.37 [0.28-0.50]). Three studies found patients with XFS had lower tympanometric peaks. Two studies found that abnormal vestibular testing results could be more common for patients with XFS.Conclusions: HL is associated with XFS. A sensorineural component to HL is confirmed, and mixed HL is possible. Given the high prevalence and infrequent diagnosis of XFS, the authors hypothesize that the current understandings of presbycusis might be improved by further investigation in XFS.