【】Vogt-Koyanag-i Harada Syndrome is an autoimmune disease characterized by chronic granulomatous uveitis of both eyes, and can affect multiple tissues throughout the body, including the meninges, inner ear and skin. At the same time, there are symptoms of nervous system stimulation, hearing loss, hair whitening and vitiligo. Patients are usually referred to the ophthalmology department for eye problems, but may be missed or misdiagnosed when presented to the otolaryngology department for ear symptoms. This paper reports two cases of VOGT-Koyanagi Harada syndrome with ear symptoms and a literature review to improve the understanding of otolaryngology head and neck surgeons.