Objective: To clarify the characteristics and prognosis of hearing loss associated with Vogt-Koyanagi-Harada (VKH) disease.
Methods: We retrospectively examined 85 patients diagnosed with VKH disease between January 1996 and December 2012. The control group included age- and gender-matched individuals without definitive ear disease. The patients with VKH disease were treated with high-dose systemic corticosteroids, which were tapered off gradually over a period of 6 months or more by the treating ophthalmologists according to the severity of the ocular inflammation. The features of hearing loss were analyzed based on pure tone audiometric data obtained at the initial presentation according to diagnostic criteria based on the ISO 7029 standard. The efficacy of corticosteroid therapy was evaluated by audiometry at the initial presentation and during therapy for 3-6 months.
Results: In patients with VKH disease, the rate of hearing loss detected by audiometry was significantly higher than that of either subjective hearing loss (p < 0.001) or tinnitus (p < 0.001). Bilateral symmetrical hearing loss was the most common type of auditory disturbance associated with VKH disease. The degree of hearing loss was generally low, with no patients showing profound hearing loss. Hearing thresholds were significantly elevated at high frequencies compared with those at low-to-mid frequencies (p < 0.001). Hearing thresholds at all frequencies after high-dose corticosteroid therapy were significantly better than those at initial presentation (p < 0.001), and the rate of patients who returned to within normal-range pure tone thresholds at all frequencies was 74.8%.
Conclusions: As auditory manifestations cannot be detected through history taking alone, audiometry should be performed to evaluate hearing loss associated with VKH disease. Early administration of high-dose systemic corticosteroids is effective for treating the auditory manifestations, which generally show a relatively good short-term prognosis.