The patient was a 20-year-old man with the chief complaint of bilateral hearing loss following a cold with headache, fever and hyperemia of both eyes. Two weeks after the onset bilateral hearing loss and tinnitus appeared. Pure tone audiometry revealed moderate sensory neural hearing loss; the ABR wave V detection thres-hold was 60 dB HL in both ears in response to click stimuli at 3000Hz. No abnormal nystagmus was seen on ENG, Plain X-ray films and CT scans did not reveal any abnormality of the ears. Finally, Harada disease was diagnosed by fundoscopy.
Auditory acuity was improved, except at 8000 Hz, within 6 days by steroid treatment. Two weeks were required for restoration to the normal level at 8000Hz.
Inner ear function appeared to respond promptly to steroid treatment, but ophthalmological improvement was slow.
Harada disease is considered to be an autoimmune disease against melanocytes, and it may cause endolymphatic hydrops.