CBL: Ramsay Hunt Syndrome

Facial paralysis with vesicular rash in the ear canal.

Topic: Neuro-infectious Level: Resident

Learning Objectives

  • Recognize Ramsay Hunt syndrome as VZV reactivation in the geniculate ganglion.
  • Differentiate it from Bell’s palsy by the presence of vesicular rash and more severe prognosis.
  • Understand appropriate diagnostic work-up and treatment with antivirals and steroids.

Case History

Patient: 54-year-old woman with acute onset right facial weakness and ear pain.

Symptoms: Two days of severe right ear pain, followed by vesicular eruption in the external auditory canal and progressive facial weakness. Reports loss of taste on the anterior tongue, hyperacusis, and dizziness.

PMH: Hypertension, otherwise healthy.

Examination

General: Alert, uncomfortable from ear pain.

HEENT: Vesicular rash on right pinna and external canal.

Neuro: Right lower motor neuron facial palsy; incomplete eye closure; decreased taste on anterior 2/3 of tongue; mild sensorineural hearing loss; unsteady gait.

Localization & Pathophysiology

Ramsay Hunt syndrome localizes to the geniculate ganglion of CN VII. Reactivation of varicella zoster virus causes inflammation and damage to the facial nerve, often spreading to CN VIII → hearing loss and vertigo.

Work-up

  • Clinical diagnosis based on triad: ear pain, vesicular rash, ipsilateral LMN facial palsy.
  • Consider PCR/serology for VZV in atypical cases.
  • Audiometry if hearing loss suspected.
  • MRI if diagnosis uncertain or alternative causes considered.

Treatment

  • Antivirals: Acyclovir, valacyclovir, or famciclovir — start promptly.
  • Steroids: Prednisone — improves recovery when combined with antivirals.
  • Eye care: Lubrication, taping eyelid shut if incomplete closure.
  • Pain control: Analgesics or neuropathic pain medications.

Review Questions

Question 1

Which virus causes Ramsay Hunt syndrome?

  • a. Herpes simplex virus
  • b. Varicella zoster virus
  • c. Epstein-Barr virus
  • d. Cytomegalovirus

Question 2

Which clinical triad defines Ramsay Hunt syndrome?

  • a. Facial paralysis, hearing loss, dizziness
  • b. Ear pain, headache, facial weakness
  • c. Ear pain, vesicular rash, ipsilateral LMN facial palsy
  • d. Ear fullness, tinnitus, vertigo

Question 3

What is the most effective treatment strategy?

  • a. Steroids alone
  • b. Antivirals alone
  • c. Antivirals + steroids
  • d. Surgical decompression

Teaching Points

  • Distinguish Ramsay Hunt from Bell’s palsy by rash and severity.
  • Involves CN VII (geniculate ganglion) ± CN VIII.
  • Prompt treatment improves outcomes; delay → incomplete recovery.

References

  1. Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry. 2001.
  2. Guidelines on management of acute facial palsy with vesicular rash. Otolaryngol Clin North Am.