9-year-old with sudden left SNHL, ear pain, vesicles on pinna and canal.
Q1
Identify the most likely diagnosis based on the clinical presentation and lab findings.
Audiometry
Severe SNHL left ear
Physical exam
Vesicles in ear canal, concha; facial palsy (lower motor neuron)
MRI IAC
Enhancement of facial/vestibulocochlear nerves
✅ Model Answer:
• Diagnosis: Ramsay Hunt syndrome (herpes zoster oticus) — VZV reactivation in the geniculate ganglion causing sudden severe SNHL, ear pain, vesicles on pinna and ear canal, facial nerve palsy (lower motor neuron), and MRI enhancement of facial/vestibulocochlear nerves. Positive VZV PCR confirms diagnosis.
• Any other test: VZV IgM/IgG serology , audiometry (already done), facial nerve EMG/ENoG (to assess nerve degeneration), vestibular testing (if dizziness), comprehensive neurologic exam.
• What to do next: Start IV acyclovir (10-15 mg/kg TID) + oral prednisone (1 mg/kg/day, taper over 2 weeks). Urgent ENT/neurology consultation. Pain management (analgesics). Eye care (artificial tears, eye patch if corneal exposure).
• Follow-up plan: Serial audiometry (hearing may partially recover). Facial nerve recovery assessment (EMG if poor). Consider vestibular rehabilitation if vertigo. Monitor for postherpetic neuralgia. Ophthalmology consult if eye involvement.
Q2
What is Ramsay Hunt syndrome and what causes it?
✅ Model Answer:
• Ramsay Hunt syndrome (herpes zoster oticus) is a reactivation of the varicella-zoster virus (VZV) in the geniculate ganglion (cranial nerve VII ganglion).
• Also known as: Herpes zoster oticus.
• Predisposing factors: Immunosuppression, stress, older age (but can occur in children).
• Classic triad:
- Vesicles: On the pinna, external auditory canal, and/or tympanic membrane
- Facial paralysis: Lower motor neuron (peripheral) facial palsy (ipsilateral)
- Hearing loss: Sensorineural hearing loss (SNHL) on the same side
• Other cranial nerves: Can also affect CN VIII (vestibular), CN IX, CN X.
Q3
How does Ramsay Hunt syndrome differ from Bell's palsy?
✅ Model Answer:
• Bell's palsy:
- Cause: Idiopathic (often viral, HSV-1 reactivation in the facial nerve)
- Vesicles:Absent (no vesicles)
- Hearing loss: Usually absent (no SNHL)
- Ear pain: May have post-auricular pain, but no vesicles
- Treatment: Corticosteroids (prednisone) alone
- Prognosis: Good (85% recover fully)
• Ramsay Hunt syndrome:
- Cause: VZV reactivation in the geniculate ganglion
- Vesicles:Present (pinna, canal, TM)
- Hearing loss:SNHL (common, may be severe)
- Ear pain: Severe, often with vesicles
- Treatment: Antivirals + corticosteroids
- Prognosis: Worse than Bell's (hearing may not recover fully)
Q4
What is the pathophysiology of hearing loss in Ramsay Hunt syndrome?
✅ Model Answer:
• Pathophysiology: VZV reactivation in the geniculate ganglion → inflammation and viral spread to the adjacent vestibulocochlear nerve (CN VIII) and facial nerve (CN VII).
• Mechanism of SNHL:
- Viral invasion of the cochlea (inner ear) or the vestibulocochlear nerve
- Inflammatory response → edema, ischemia, and damage to hair cells or the auditory nerve
- MRI findings: Enhancement of the facial and vestibulocochlear nerves (indicating inflammation)
• Why sudden? Viral reactivation is acute and causes rapid damage to the inner ear structures.
• Prognosis: Hearing may partially recover with early treatment, but often permanent if severe.
Q5
What is the management of Ramsay Hunt syndrome?
✅ Model Answer:
• Antiviral therapy:
- Acyclovir: IV 10-15 mg/kg TID (or 800 mg PO 5 times daily) for 7-10 days
- Valacyclovir: 1 g PO TID (alternative)
- Start early: Within 72 hours of symptom onset for best outcomes
• Corticosteroids:
- Prednisone: 1 mg/kg/day (max 60-80 mg) for 5-7 days, then taper over 2 weeks
- Evidence: Combination of antiviral + corticosteroid improves facial nerve and hearing outcomes
• Supportive care:
- Pain management: Analgesics (NSAIDs, opioids if severe)
- Eye care: Artificial tears, eye lubrication, eye patch (if corneal exposure due to incomplete eye closure)
- Vestibular rehabilitation: If dizziness or balance issues
- ENT/Neurology consultation: Urgent for severe cases
- Facial nerve monitoring: EMG/ENoG to assess severity
Q6
What are the complications of Ramsay Hunt syndrome?
✅ Model Answer:
• Hearing loss: Can be permanent (severe or profound) — especially if treatment is delayed
• Facial paralysis: May be incomplete recovery (synkinesis, contracture, facial weakness)
• Postherpetic neuralgia: Persistent pain in the affected area after vesicles heal (more common in older adults)
• Corneal exposure/keratitis: Due to incomplete eye closure (facial palsy) → can lead to corneal ulceration
• Vestibular dysfunction: Dizziness, vertigo, imbalance (due to CN VIII involvement)
• Tinnitus: Ringing in the affected ear
• Other cranial nerve involvement: CN IX (glossopharyngeal), CN X (vagus) — palatal weakness, voice changes
• Secondary infection: Bacterial superinfection of vesicles
• Psychological: Anxiety, depression, social isolation (facial disfigurement, hearing loss)
Q7
What is the role of MRI in Ramsay Hunt syndrome?
✅ Model Answer:
• MRI with contrast (gadolinium) of the IAC (internal auditory canal):
- Findings: Enhancement of the facial nerve (CN VII) and/or vestibulocochlear nerve (CN VIII) in the internal auditory canal or labyrinthine segment
- Why? Indicates inflammation due to VZV reactivation
- Rule out: Other causes of sudden SNHL + facial palsy (e.g., vestibular schwannoma, other neoplasms)
• Timing: Usually performed if atypical presentation, no vesicles, or no improvement with treatment
• Prognostic value: Enhancement extent may correlate with severity of nerve damage and clinical outcomes
• Limitations: Normal MRI does NOT rule out Ramsay Hunt
Q8
What is the prognosis and long-term outcome for children with Ramsay Hunt syndrome?
✅ Model Answer:
• Prognosis:
- Facial nerve recovery: 70% recover well if treated early with antivirals + steroids; 30% have residual weakness/synkinesis
- Hearing recovery: 50-60% improve, but many have permanent SNHL (especially if severe initially)
- Tinnitus: May persist
- Vestibular: Variable; may improve with vestibular rehabilitation
- Children: Generally better prognosis than adults (better healing capacity)
• Long-term management:
- Hearing aids: If permanent SNHL
- Facial reanimation surgery: If significant residual facial weakness (rare in children)
- Vestibular rehabilitation: If balance issues persist
- Pain management: If postherpetic neuralgia
- Ophthalmology: Annual eye exams (corneal protection)
- Psychological support: Coping with facial paralysis, hearing loss
- Follow-up: ENT, audiology, neurology