Signs and Symptoms of Ramsay Hunt Syndrome
Ramsay Hunt syndrome presents with the classical triad of ipsilateral facial paralysis, otalgia (ear pain), and vesicular rash on the ear or in the mouth, frequently accompanied by vestibulocochlear nerve symptoms including hearing loss, tinnitus, vertigo, and nystagmus. 1
Core Clinical Features (Classical Triad)
The diagnosis is based on three cardinal features that define the syndrome:
- Ipsilateral facial nerve palsy - peripheral facial paralysis on the same side as the rash 1, 2
- Otalgia - severe ear pain that often precedes other symptoms 1, 3
- Vesicular rash - erythematous, fluid-filled blisters appearing on the ear (zoster oticus) or in the mouth 1, 3, 2
Vestibulocochlear Nerve Involvement
Due to the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal, patients frequently experience eighth nerve symptoms:
- Hearing loss - sensorineural hearing impairment occurs commonly 4, 1, 2
- Tinnitus - ringing or buzzing in the affected ear 4, 1, 2
- Vertigo - spinning sensation or dizziness 4, 1, 2
- Nystagmus - involuntary eye movements 4, 1, 2
- Nausea and vomiting - secondary to vestibular involvement 4, 2
Critical Diagnostic Pitfall
A crucial clinical caveat is that 14% of patients develop vesicles AFTER the onset of facial weakness, making Ramsay Hunt syndrome initially indistinguishable from Bell's palsy. 2 This delayed rash appearance can lead to missed or delayed diagnosis, which significantly impacts prognosis since patients with Ramsay Hunt syndrome have more severe paralysis at onset and are less likely to recover completely compared to Bell's palsy. 2
Temporal Presentation Pattern
The typical sequence of symptom development:
- Ear pain often appears first, sometimes 1-2 days before other symptoms 3
- Facial paralysis develops acutely 3, 2
- Vesicular rash may appear simultaneously with paralysis or up to several days later 2
- Vestibulocochlear symptoms can manifest at any point during the acute phase 2
Rare Presentations
Some patients may present with atypical features:
- Ramsay Hunt syndrome zoster sine herpete - facial paralysis without visible rash but with serologic or molecular evidence of VZV reactivation 2
- Multiple cranial nerve involvement - rare retrograde transaxonal spread affecting additional cranial nerves 5
- Brainstem encephalitis - extremely rare complication from viral spread into brain parenchyma 5
Severity Indicators
The initial clinical presentation predicts outcome: