What is the treatment for Ramsay Hunt syndrome?

Diagnosis and Treatment of Ramsay Hunt Syndrome

Diagnosis

Ramsay Hunt syndrome should be diagnosed based on the presence of ipsilateral facial paralysis accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth, caused by varicella zoster virus reactivation in the geniculate ganglion. 1

Key diagnostic features include:

• Peripheral facial nerve palsy
• Vesicular eruptions on the ear, ear canal, or mouth
• Ear pain (often severe and preceding the rash)
• Associated symptoms may include:
  • Tinnitus
  • Hearing loss
  • Vertigo
  • Nausea and vomiting
  • Nystagmus

Important clinical note: In approximately 14% of cases, vesicles may develop after the onset of facial weakness, making Ramsay Hunt syndrome initially indistinguishable from Bell's palsy 1.

Treatment

First-Line Treatment

All patients with Ramsay Hunt syndrome should receive prompt treatment with a combination of antiviral therapy and corticosteroids within the first 7 days of symptom onset for optimal recovery of facial nerve function. 1, 2

1. Antiviral therapy:

   • Acyclovir 800 mg orally five times daily for 7-10 days 1
   • OR Famciclovir 500 mg orally three times daily for 7-10 days 1
   • Antiviral therapy alone has been shown to significantly improve recovery rates of facial nerve function 2

2. Corticosteroid therapy:

   • Prednisone 60 mg daily for 3-5 days, followed by a taper 1
   • OR Methylprednisolone equivalent dose
   • Early administration of corticosteroids is crucial to reduce inflammation and edema of the facial nerve

Treatment for Non-Responding Cases

For patients who fail to respond to standard oral therapy:

• Intravenous high-dose methylprednisolone should be considered even as a late treatment option, particularly in patients with poor prognostic factors (older age, high-grade facial weakness, absence of blink reflex) 3

Timing of Treatment

Early intervention is critical:

• Treatment should ideally begin within the first 7 days of symptom onset 2
• Delayed treatment is associated with poorer outcomes
• Even in cases initially diagnosed as Bell's palsy, antiviral therapy should be considered if there is suspicion of zoster sine herpete (Ramsay Hunt without visible rash) 1

Monitoring and Follow-up

1. Audiometric evaluation:

   • Should be performed after completion of treatment 4
   • Follow-up within 6 months of initial diagnosis 4

2. Facial nerve function assessment:

   • Regular evaluation of facial voluntary movement
   • Nerve excitability testing may help assess prognosis 2

Prognosis

Compared to Bell's palsy, Ramsay Hunt syndrome:

• Often presents with more severe paralysis at onset
• Has a lower rate of complete recovery
• Has better outcomes with early combined antiviral and corticosteroid therapy 1

Negative prognostic factors include:

• Advanced age
• Complete facial paralysis at onset
• Absence of blink reflex
• Delayed treatment initiation
• Associated eighth nerve symptoms (vertigo, hearing loss)

Rehabilitation

For patients with residual deficits:

• Audiologic rehabilitation including counseling and discussion of amplification options if hearing does not fully recover 4
• Facial rehabilitation exercises may be beneficial for patients with persistent facial weakness

Special Considerations

1. Immunocompromised patients:

   • May require more aggressive or prolonged therapy
   • Consider intravenous antiviral treatment in severe cases

2. Zoster sine herpete:

   • Some patients with apparent Bell's palsy may actually have Ramsay Hunt syndrome without visible vesicles
   • Consider antiviral therapy in all cases of facial palsy, especially with ear pain 1

Early diagnosis and prompt initiation of combined antiviral and corticosteroid therapy are the most important factors for improving outcomes in patients with Ramsay Hunt syndrome.