What is Ramsey Hunt syndrome?

What is Ramsay Hunt Syndrome?

Ramsay Hunt syndrome is a rare neurological condition caused by reactivation of the varicella-zoster virus (VZV) in the geniculate ganglion of the facial nerve, resulting in the characteristic triad of ipsilateral facial paralysis, ear pain, and vesicular rash around the ear or in the mouth. 1, 2

Etiology and Pathophysiology

• The syndrome occurs when dormant VZV reactivates and infects the geniculate ganglion of the seventh cranial nerve, producing an inflammatory reaction that leads to facial nerve dysfunction 3, 4
• This represents a late manifestation of varicella-zoster virus infection, distinct from typical shingles because it causes motor neuropathy rather than predominantly sensory neuropathy 5
• The virus affects fewer than 1 in 1,500 people, making it significantly rarer than other forms of herpes zoster reactivation 5

Clinical Presentation

The classical triad consists of:

• Ipsilateral facial paralysis (lower motor neuron pattern) 1, 2
• Otalgia (severe ear pain) 6, 2
• Erythematous vesicular rash on the ear (zoster oticus) or in the mouth 1, 2

Additional common features include:

• Sensorineural hearing loss, tinnitus, vertigo, and nystagmus due to vestibulocochlear nerve involvement from the close anatomic proximity of the geniculate ganglion to the eighth cranial nerve within the bony facial canal 1, 7
• These eighth nerve symptoms were recognized by J. Ramsay Hunt himself when he originally described the syndrome 2

Diagnostic Challenges and Pitfalls

A critical pitfall is that 14% of patients develop vesicles after the onset of facial weakness, making Ramsay Hunt syndrome initially indistinguishable from Bell's palsy. 2

• The syndrome can present with vague and non-specific symptoms during early disease onset, requiring high clinical suspicion and frequent follow-up 5
• Some patients develop peripheral facial paralysis without any visible rash (zoster sine herpete), diagnosed only through fourfold rise in VZV antibody titers or detection of VZV DNA in auricular skin, blood mononuclear cells, middle ear fluid, or saliva 2
• This means a proportion of patients diagnosed with "Bell's palsy" actually have Ramsay Hunt syndrome without the characteristic rash 2

Prognosis and Severity

Compared with Bell's palsy, patients with Ramsay Hunt syndrome have more severe paralysis at onset and are less likely to recover completely. 2

• Advanced age, diabetes mellitus, and hypertension are significant poor prognostic features associated with higher complication rates 5
• If untreated, the condition may cause permanent facial muscle weakness and hearing loss 4
• The two most significant long-term complications are persistent facial nerve palsy (occurring in 2-10% of all facial palsy cases) and hearing impairment 3

Transmission and Isolation Considerations

• Ramsay Hunt syndrome is approximately 20% as contagious as chickenpox, with transmission occurring through direct contact with vesicular fluid or rarely through airborne routes 8
• The contagious period begins 1-2 days before rash onset and continues until all lesions have dried and crusted, typically 4-7 days after rash onset 8
• Immunocompetent patients with localized disease require standard precautions with complete covering of lesions, while immunocompromised patients require airborne and contact precautions until disseminated infection is ruled out 8
• Patients must avoid contact with pregnant women, premature infants, immunocompromised persons, and anyone without varicella immunity 8

Treatment Implications

Early recognition is crucial because prompt initiation of antiviral and corticosteroid therapy significantly improves outcomes. 6, 5

• Treatment with acyclovir (800 mg five times daily) or famciclovir (500 mg three times daily) for 7-10 days, combined with oral prednisone (60 mg daily for 3-5 days), should be considered for all patients when started within 7 days of onset 2
• Antiviral monotherapy without corticosteroids is not supported by evidence and should be avoided 7
• Audiometric evaluation should be performed at treatment conclusion, with repeat testing within 6 months to assess hearing recovery 7