What are the typical exam findings and treatment for Ramsay Hunt syndrome?

Exam Findings of Ramsay Hunt Syndrome

Ramsay Hunt syndrome presents with ipsilateral facial paralysis accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth, along with other cranial nerve symptoms due to varicella-zoster virus reactivation in the geniculate ganglion. 1

Key Clinical Features

• Facial nerve palsy: Unilateral peripheral facial weakness or paralysis, which may be severe at onset and less likely to recover completely compared to Bell's palsy 1

• Vesicular rash: Painful fluid-filled blisters on the ear (zoster oticus) or in the mouth; importantly, these vesicles may:

  • Precede or follow the facial palsy rather than occurring simultaneously 2
  • Be located in hidden areas such as the ear canal or mouth (in approximately 20% of cases) 2
  • Develop after the onset of facial weakness in about 14% of patients, making initial presentation indistinguishable from Bell's palsy 1

• Eighth cranial nerve symptoms: Due to the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal 1

  • Tinnitus 1
  • Hearing loss (sensorineural) 3, 4
  • Vertigo 3, 4
  • Nystagmus 1

• Other symptoms:

  • Severe ear pain 5
  • Nausea and vomiting 1

Diagnostic Considerations

• Diagnosis is primarily clinical, based on the presence of facial palsy with vesicular eruptions 1

• In patients with facial paralysis without visible rash, consider "zoster sine herpete" - Ramsay Hunt syndrome without the characteristic rash 1

• The diagnosis may be delayed as blisters can appear before or after the onset of facial paralysis 2

• Always examine hidden areas like the ear canal and oral cavity for vesicles 2

Treatment Approach

• Early treatment is crucial for optimal outcomes and should be initiated within 72 hours of symptom onset 2, 6

• Antiviral therapy:

  • Acyclovir (800 mg, five times daily for 7-10 days) 1
  • Valacyclovir (1000 mg three times daily for 7 days) 4
  • Famciclovir (500 mg, three times daily for 7-10 days) 1

• Corticosteroids:

  • Prednisone (60 mg daily for 3-5 days) 1
  • Other options include methylprednisolone, prednisolone, or hydrocortisone 6

• Combination therapy:

  • Combined antiviral and steroid therapy shows better outcomes than steroids alone (70.5% vs 68% achieving House-Brackmann grade I or II) 6

Prognosis

• When treated early (within 72 hours), over 80% of patients recover completely or with only slight sequelae 2

• Among patients with complete facial palsy (House-Brackmann grades V or VI), approximately 51.4% recover to grades I or II 6

• Recovery rates vary based on the steroid used in combination with antivirals:

  • 81.3% for methylprednisolone
  • 76.3% for hydrocortisone
  • 69.2% for prednisone
  • 61.4% for prednisolone 6

• Patients with Ramsay Hunt syndrome generally have more severe paralysis at onset and are less likely to recover completely compared to those with Bell's palsy 1

Special Considerations

• Immunocompromised patients (such as those with HIV) may be at higher risk for developing Ramsay Hunt syndrome 4

• Consider Ramsay Hunt syndrome in all cases of facial palsy, even without visible vesicles initially, as the rash may develop later 1

• Early diagnosis and treatment are essential to prevent long-term complications such as permanent facial nerve dysfunction 5, 6