Case Presentation: A 59-year-old male with past medical history of diabetes mellitus and hypertension presented to the ED with complaints of a sharp, pulsatile pain in the left temporal region and jaw that started one week prior and became progressively more severe. This was associated with nausea, vertigo, hearing loss, otalgia and a painful, well defined erythematous, vesicular rash on the left temporal side of the face, left malar eminence and left chin with mild scabbing. He also developed vesicles on the left lateral margin of the tongue and had a red, swollen auricle. His House-Brackmann score was I/VI. He did not have fever, photophobia, blurry vision, tinnitus, motor deficits, syncope or discharge from the ear. His lab work was notable for a WBC of 12,100 cells/µL and glucose of 244 mg/dL. Given his presentation he was diagnosed with Ramsay Hunt Syndrome and started on acyclovir and prednisone. His symptoms rapidly improved, and he was discharged home. At the time of his outpatient ENT follow up appointment, his hearing loss had improved.
Discussion: Ramsay Hunt Syndrome is a rare entity estimated to affect 5 in 100,000 adults each year. It is a clinical diagnosis which typically presents with a triad of otalgia, auricular vesicles, and ipsilateral facial paralysis. Viral studies can detect VZV in saliva, tears, and blood, but are not necessary to establish the diagnosis. The presence of facial paralysis is the dominant feature of this syndrome, occurring with most presentations and often more severe than in ‘usual’ Bell’s Palsy. Our patient was unique because he exhibited the vesicles and otalgia without perceptible facial paralysis. There is evidence in the literature that in immune-competent patients, early administration of acyclovir and prednisone decreases the duration of pain, speeds up the healing of skin lesions, and prevents nerve degeneration. One study found early recognition and treatment of Ramsay Hunt Syndrome reversed or prevented neurological deficits in 75% of the cases.
Conclusions: Recognizing Ramsay Syndrome when there is an atypical presentation of an already rare entity proves to be challenging. It is vital to diagnose the condition early in order to initiate therapy in time to prevent permanent neurological deficits.