A 36-year-old man presented with facial nerve palsy, hearing loss, vertigo and headache. He was initially diagnosed with Ramsay Hunt syndrome and treated with a systemic steroid and valaciclovir; however, his symptoms deteriorated. Serum rapid plasma reagin (RPR) and treponema pallidum hemagglutination tests were positive. Cerebrospinal fluid analysis revealed an elevated white blood cell count and positive RPR, confirming the diagnosis of neurosyphilis. Penicillin G (PCG) was administered, and his facial nerve function and headache improved. However, left-side hearing loss worsened temporarily, which was assumed to be a Jarisch-Herxheimer reaction. Betamethasone was administered along with PCG, and he recovered completely.
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