Abstract
Vogt–Koyanagi–Harada (VKH) disease is an autoimmune disorder that occurs in the melanocytes present in the uvea, leptomeninges, skin and inner ear. Clinically, this disease is characterized by bilateral uveitis and retinal detachment and is associated with meningismus and hearing loss. Gadolinium (Gd)-enhanced MRI may aid in demonstrating bilateral choroidal thickening and central nervous system involvement. We present a case of VKH where Gd-enhanced three-dimensional (3D) fluid attenuation inversion recovery (FLAIR) imaging showed abnormal bilateral enhancement in the inner ears. A 36-year-old female was referred to our institution with symptoms of visual disturbance, headache and tinnitus, and was diagnosed with VKH based on fundus examination and clinical presentations. MRI findings revealed bilateral enhancement in the choroid, leptomeninges, and inner ears. In particular, Gd-enhanced 3D FLAIR showed more conspicuous enhancement of the leptomeninges and inner ear compared with Gd-enhanced 3D T1 weighted image. Therefore, Gd-enhanced 3D FLAIR imaging can be used when leptomeningeal or inner ear pathology is clinically suspected.
Highlights
Vogt–Koyanagi–Harada (VKH) disease is one of the causes of uveo-meningeal syndromes and is an autoimmune disease of the melanocytes present in the uvea, leptomeninges, skin, and inner ear.[1]
We present a case of VKH in which gadolinium (Gd)-enhanced threedimensional (3D) fluid attenuation inversion recovery (FLAIR) showed abnormal bilateral enhancement in the choroid, leptomeninges, and inner ears
VKH is a systemic inflammatory disorder characterized by bilateral uveitis associated with serous retinal detachment and accompanied by neurologic, auditory, and integumentary manifestations
Summary
Vogt–Koyanagi–Harada (VKH) disease is one of the causes of uveo-meningeal syndromes and is an autoimmune disease of the melanocytes present in the uvea, leptomeninges, skin, and inner ear.[1] The revised diagnostic criteria of VKH, which was established at an international workshop on VKH, is based on clinical findings and ancillary ophthalmic tests.[2] MRI findings are not included in the criteria, several studies have suggested that MRI can aid in diagnosing VKH.[3,4] On MRI, in addition to the bilateral ocular findings, central nervous system (CNS) involvement, such as focal brain parenchymal, pachymeningeal and leptomeningeal lesions, can be demonstrated. We present a case of VKH in which gadolinium (Gd)-enhanced threedimensional (3D) fluid attenuation inversion recovery (FLAIR) showed abnormal bilateral enhancement in the choroid, leptomeninges, and inner ears. To the best of our knowledge, there is only one report that described inner ear involvement on MRI,[5] and no report on inner ear enhancement on Gd-enhanced 3D FLAIR imaging
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