What are the clinical features of Ramsay Hunt syndrome?

Clinical Features of Ramsay Hunt Syndrome

Ramsay Hunt syndrome is characterized by peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth, caused by reactivation of varicella-zoster virus in the geniculate ganglion of the facial nerve. 1

Core Clinical Manifestations

• Peripheral facial nerve palsy (facial weakness/paralysis) on the affected side 1
• Erythematous vesicular rash (herpetic vesicles) on the ear (zoster oticus) or in the mouth 1
• Ear pain (otalgia) that can be severe and precede other symptoms 1, 2

Neurological Features

• Facial weakness or paralysis that may be more severe at onset compared to Bell's palsy 1
• Tinnitus (ringing in the ears) due to eighth cranial nerve involvement 3, 1
• Hearing loss (typically sensorineural) due to proximity of the geniculate ganglion to the vestibulocochlear nerve 3, 4
• Vertigo and nystagmus due to vestibular nerve involvement 3, 4
• Nausea and vomiting (related to vestibular dysfunction) 4, 1

Additional Features

• Dysgeusia (altered taste sensation) due to involvement of the chorda tympani branch of the facial nerve 1
• Pain in the distribution of the facial nerve 1, 2
• In some cases (14%), vesicles may develop after the onset of facial weakness, initially making it indistinguishable from Bell's palsy 1

Expanded Clinical Presentations

• Multiple cranial nerve involvement can occur in immunocompromised patients (such as those with HIV), affecting cranial nerves V, VII, VIII, IX, and X 5
• Dysphagia (difficulty swallowing) when cranial nerves IX and X are involved 5
• Headache may be present, especially in cases with more extensive involvement 5
• Rare complications include retrograde transaxonal spread of the virus from the ganglion into the brain parenchyma, leading to encephalitis 6

Diagnostic Considerations

• The diagnosis is primarily clinical, based on the triad of facial nerve palsy, vesicular rash, and ear pain 2
• Some patients may develop "zoster sine herpete" - facial paralysis without the characteristic rash, but with serological evidence of VZV infection 1
• Audiometric evaluation should be performed to assess hearing loss 4

Prognostic Factors

• Patients with Ramsay Hunt syndrome typically have more severe paralysis at onset and are less likely to recover completely compared to Bell's palsy 1
• Early diagnosis and treatment with antivirals and corticosteroids within 7 days of symptom onset significantly improves outcomes 1, 7
• Older patients may have a higher risk of persistent facial weakness and hearing impairment 7

Clinical Pitfalls

• Delayed diagnosis is common and can lead to increased risk of complications with long-term effects 2
• Initial presentation may mimic Bell's palsy if vesicles have not yet appeared 1
• In immunocompromised patients, the presentation may be more severe with multiple cranial nerve involvement 5
• Facial weakness may persist despite treatment, particularly if therapy is initiated late 1, 7

The American Academy of Otolaryngology-Head and Neck Surgery recommends audiometric evaluation at the conclusion of treatment and within 6 months to assess hearing recovery 4.