AIM: To evaluate relationship between ocular pseudoexfoliation syndrome(PXF) and sensory-neural hearing loss (SNHL). METHODS: This prospective case-control study was designed on patients who referred to a general ophthalmic clinic at Imam Khomeini Medical Center, Urmia, Iran (March 2010 through November 2010). On routine ophthalmic examination, patients diagnosed with ocular PXF were referred to the ENT department and, selected cases (after evaluating inclusion and exclusion criteria) were referred to Audiometric Department. Pure tone hearing threshold level(HTL) was measured at 1, 2, 3 kHz for each ear and was compared with International Standard (ISO 7029) median age associated hearing loss at 1, 2, 3 kHz (AAHL). RESULTS : Overall 21 of 50 patients (42.0%) had a higher HTL than the ISO 7029 median AAHL at 1, 2 and 3kHz, which included 14 ears of 9 patients in the male group and 21 ears of 12 patients in the female group. Approximately 12.0% of patients had glaucoma at the same time, however; no significant correlation was found in SNHL prevalence and severity between PXF patient and patients with simultaneous glaucoma. SNHL was more common in patients with ocular PXF compared to their age- sex matched controls (P <0.05). CONCLUSION: Most of patients with ocular PXF had SNHL compared to their age-sex matched controls, which could be due to PXF fibrils in the inner ear. These findings suggest PXF could be a systemic disease.