A 24-Year-Old Man With Ear Pain, Dizziness, and a Rash

D. Brady Pregerson, MD

Disclosures

December 11, 2019

Ramsay Hunt syndrome, like other zoster syndromes, tends to occur more commonly with increasing age (8-10 times increased risk in those older than 60 years) and with impaired cell-mediated immunity, especially impaired immunity resulting from HIV infection. VZV vaccination is recommended for persons older than 60 years; however, caution is advised in patients with impaired cell-mediated immunity because the vaccine contains live virus. Complications of zoster occur in about 12% of patients and may include chronic pain (postherpetic neuralgia), idiopathic geniculate neuralgia, bacterial superinfection, motor neuropathy, myelitis, encephalitis, central nervous system vasculitis, pneumonitis, and meningitis.[3,4]

The diagnosis of Ramsay Hunt syndrome is made clinically, but polymerase chain reaction examinations of the saliva, tear fluid, and vesicular fluid for VZV can confirm the diagnosis. Serologic tests are often of little use, because a history of varicella infection (chickenpox) will result in a positive test. If necessary, VZV may be isolated from vesicle fluid and inoculated into susceptible human or monkey cells for identification by serologic means. Antibody determinations on paired sera may be helpful in establishing the diagnosis by comparing titers at presentation with those from a few weeks later.[3]

Structural lesions can be ruled out by contrast-enhanced CT or MRI, but the diagnosis can typically be made on clinical grounds. Gadolinium enhancement of the vestibular and facial nerves on MRI has been described. Audiometry usually demonstrates sensorineural hearing loss. Unilateral caloric weakness may be present on electronystagmography.

In the setting of a peripheral facial palsy, cerebrospinal fluid (CSF) analysis is rarely obtained, and lumbar puncture is not recommended for establishing the diagnosis. However, in one study, CSF analysis was nonspecifically abnormal, with pleocytosis in 11% of 239 patients with idiopathic peripheral facial palsy, 60% of 17 patients with Ramsay Hunt syndrome, 25% of 8 patients with Lyme disease, and in all 8 patients with HIV infection. On histology, the affected ganglia of the cranial nerve roots are swollen and inflamed, with an inflammatory reaction chiefly of a lymphocytic nature and with few polymorphonuclear leukocytes or plasma cells.[5]

The differential diagnosis of facial vesicular lesions includes herpes simplex, as well as varicella zoster of nearby dermatomes, such as the second branch of the trigeminal nerve. The differential diagnosis of facial weakness includes idiopathic Bell palsy, Lyme disease, otitis externa, odontogenic infection, cholesteatoma, parotid tumor, mastoiditis, mononucleosis, sarcoidosis, and trauma. Although the presence of facial nerve paralysis in Ramsay Hunt syndrome may particularly be confused with that of idiopathic Bell palsy, the diagnosis of Ramsay Hunt syndrome should be entertained in the presence of significant pain, which is often out of proportion to the physical findings.[1,3]

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