Syphilis is a bacterial infection caused by Treponema pallidum. It spreads usually via sexual contact. Syphilis generally presents as a multisystem disease, with symptoms resembling and often confused with those of other diseases, thus often called "the great mimicker". Neurosyphilis is a rare but late course of the disease process when the meninges and central nervous system (CNS) are involved. Otosyphilis is an even rarer, yet important complication of neurosyphilis and a rare cause of sensorineural deafness often misdiagnosed. We present the case of a 46-year-old Caucasian male admitted for acute onset bilateral hearing loss caused by otosyphilis. We include a discussion about cerebrospinal fluid (CSF) protein analysis in individuals diagnosed with neurosyphilis.