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Case-Based Learning Module

Ramsay Hunt Syndrome

Learning Objectives

By the end of this session, learners should be able to:

  1. Recognize Ramsay Hunt syndrome as a cranial neuropathy caused by reactivation of varicella zoster virus.
  2. Identify the involvement of cranial nerves VII (facial) and VIII (vestibulocochlear) in this condition.
  3. Understand that treatment may require antivirals, corticosteroids, pain management, and therapy for vestibular symptoms.

Case History

A 57-year-old man presents with:

  • Onset: Painful sore in the right ear, later crusted.
  • Progression (days later): Burning ear pain → reduced hearing, dizziness, nausea, and right-sided facial weakness.
  • Current status: Arrives to clinic in a wheelchair.

Past Medical History:

  • Third-degree burn with graft on left forearm (complicated by infection).
  • Moderate knee osteoarthritis.
  • Otherwise unremarkable.

Medications & Allergies:

  • Occasional ibuprofen.
  • Allergic to cephalexin.

Family History:

  • Mother: diabetes.
  • Father: leukemia (died age 48).

Social History:

  • No alcohol or tobacco use.

Review of Systems:

  • Denies fever, chest pain, respiratory or urinary complaints.
  • Rash limited to right ear.
  • No visual symptoms, bleeding, or bruising.

Examination

  • Vitals: T 99.3°F, BP 117/68, P 72.
  • General: Alert, pale man in wheelchair.
  • Ear: Crusted vesicular rash in right external auditory meatus; painful to examine TM.
  • Cranial Nerves:
    • Moderate weakness of right upper & lower face (CN VII).
    • Hearing reduced in right ear (CN VIII).
    • Facial sensation, eye movements, palate, tongue, sternocleidomastoid normal.
    • No nystagmus.
  • Motor/Sensory: Normal strength, reflexes, and sensation in limbs. Plantar responses flexor.
  • Coordination/Gait: Finger–nose normal; Romberg positive. Unable to ambulate without assistance.

Localization & Neuroanatomy

  • Findings: Rash in right ear, peripheral facial weakness, ipsilateral hearing loss, vestibular symptoms.
  • Localization: Involvement of cranial nerve VII at the geniculate ganglion with spread to cranial nerve VIII (cochlear + vestibular branches).
  • Pathophysiology: Reactivation of latent varicella zoster virus in the geniculate ganglion.

Diagnosis

Differential diagnosis:

  • Peripheral VII + VIII lesions: Trauma, cerebellopontine angle tumor (e.g., vestibular schwannoma), viral infection, stroke.
  • Isolated VII: Bell’s palsy, Lyme disease.

Ramsay Hunt syndrome clues:

  • Co-occurrence of facial weakness, vestibular symptoms, hearing loss, and ear vesicles.
  • Symptoms are variable and may not all be present.

Testing:

  • Blister fluid: DFA or culture.
  • Viral DNA: May be detected in CSF.
  • MRI (CN VII/VIII protocol): Can show nerve enhancement but mainly excludes alternative causes.

Treatment

  • Evidence base: Few randomized controlled trials, but consensus supports early combined therapy.
  • First-line (within 72 hrs of symptom onset):
    • Antiviral (acyclovir or valacyclovir).
    • Corticosteroid (prednisone, e.g., 60 mg daily for 7–14 days, then taper).
  • Supportive care:
    • Pain: gabapentin, amitriptyline.
    • Vestibular symptoms: diazepam or meclizine.
    • Physical/occupational therapy for recovery.
  • Course: Symptoms may persist for weeks–months; prognosis depends on early recognition and treatment.

Review Questions

  1. Ramsay Hunt syndrome affects which two cranial nerves?
    a. III and IV
    b. V and VI
    c. VI and VII
    d. VII and VIII ✅
    e. IX and X
  2. The infection associated with Ramsay Hunt syndrome is:
    a. Varicella zoster ✅
    b. Herpes simplex
    c. HIV
    d. Lyme disease
    e. Neisseria meningitidis
  3. Although no large randomized trials exist, providers typically recommend treating Ramsay Hunt syndrome with:
    a. Antiviral medication
    b. Corticosteroids
    c. Broad-spectrum IV antibiotics
    d. Symptomatic pain relief
    e. Both A and B ✅