Characteristics and Prognosis of Hearing Loss Associated with Vogt-Koyanagi-Harada Disease

Abstract

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.

Vogt&ndash;Koyanagi&ndash;Harada syndrome &ndash; current perspectives

“…Patients typically complain of CNS and auditory symptoms including headaches, neck stiffness, photophobia, fever, orbital pain, scalp or skin sensitivity, and focal neurological deficits such as cranial nerve palsies 1,28. They may also have hearing loss, particularly for higher frequencies, dysacousia, vertigo, and tinnitus,1,37,38 with less frequent auditory features observed in Hispanic patients 39. CSF samples from lumbar punctures show pleocytosis in >80% of cases,40,41 and brain imaging studies can occasionally demonstrate focal parenchymal lesions 42…”

Evaluation of Vestibular Functions in Patients with Vogt-Koyanagi-Harada Disease

“…Although the main symptoms are ocular in nature, hearing loss, tinnitus and vertigo/dizziness can also occur. The characteristics and prognosis of hearing loss associated with VKH disease have been reported [Morita et al, 2014]. This study has shown that an early administration of high-dose systemic corticosteroids is effective in treating the auditory manifestations of VKH disease, and the short-term prognosis is relatively good.…”

“…The abnormal VEMP responses in patients with VKH disease are considered to arise, not from vestibular nerve dysfunction, but from dysfunction of the otolith organs based on the following reasons. Firstly, the responsible site of hearing loss in VKH disease is considered to be the cochlea, with the base of the cochlea [Morita et al, 2014] and inner ear, in particular, thought to be involved in patients with VKH disease. Secondly, the appearance of melanocytes in the vestibular labyrinth including the otolith organs has been reported histologically [LaFerriere et al, 1974;Escobar et al, 1995].…”

“…Audiometry was performed using a pure-tone audiometer (AA-76; Rion Co., Japan) in a silent cabin by experienced audiologists, and our detailed protocol has been described previously [Morita et al, 2014]. Nystagmus tests were performed using an infrared charge-coupled device camera.…”

“…Although some articles reported vestibular as well as cochlear involvement in patients with VKH disease [Al Dousary, 2011;Noguchi et al, 2014], tinnitus is the only inner ear finding included in the current criteria. The characteristics and prognosis of hearing loss associated with VKH disease have been reported from our institute [Morita et al, 2014]. Meanwhile, there have been only a few reports about vestibular findings in patients with VKH disease [Al Dousary, 2011;Noguchi et al, 2014], with no articles presenting the results of vestibular evoked myogenic potential (VEMP) testing, in which the functions of otolith organs and vestibular nerves could be evaluated.…”