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Introduction: Pseudoexfoliation syndrome (PXS) occurs due to the deposition of extracellular fibrillar materials on the anterior chamber of the eye. This syndrome has been considered to be part of a systemic disease with the potential involvement of the inner ear called sensoroneural hearing loss (SNHL).  In this study, we aimed on evaluating SNHL within PXS patients in Iran to compare them with other international reports. Materials and Methods: In total, 33 patients with PXS and 33 age and sex matched controls were enrolled prospectively in a case-control study. Both groups underwent complete ophthalmologic and otorhinolaryngologic examinations and pure tone audiometry (PTA) testing. Six frequencies (0.25, 0.5, 1, 2, 3, 4 and 6 KHz) were evaluated for PTA in the same ethnic group in order to select the case and control individuals. Data were analyzed using t-test and chi-square test. Results: Forty-nine out of 66 ears (75.2%) in the PXS group and 27 ears (40.9%) in the control group had SNHL (P