What could be causing my ear ache and new onset facial weakness?

Ramsay Hunt Syndrome (Herpes Zoster Oticus) Until Proven Otherwise

The combination of ear pain and new-onset facial weakness is Ramsay Hunt syndrome (herpes zoster oticus) until proven otherwise, requiring urgent antiviral therapy with acyclovir or valacyclovir plus corticosteroids within 72 hours of symptom onset to prevent permanent facial paralysis and hearing loss. 1

Immediate Diagnostic Priorities

Critical Physical Examination Findings to Assess

• Examine the pinna and external ear canal for vesicles – the hallmark of Ramsay Hunt syndrome, though vesicles may be absent in up to 20% of cases (zoster sine herpete) 1
• Document the degree of facial weakness using the House-Brackmann grading scale to establish baseline severity 1
• Test all other cranial nerves systematically – involvement of cranial nerves V, VIII, IX, X, XI, or XII indicates more extensive disease requiring urgent neuroimaging 1
• Assess for hearing loss and vertigo – these indicate eighth cranial nerve involvement and predict worse outcomes 1

Why This Presentation Demands Urgent Action

The American Academy of Otolaryngology-Head and Neck Surgery emphasizes that pinna or ear canal vesicles combined with facial weakness are pathognomonic for varicella zoster virus (Ramsay Hunt syndrome), which has significantly worse prognosis than Bell's palsy, with only 20-30% achieving complete recovery without treatment versus 70% with Bell's palsy 1. The window for effective antiviral therapy is narrow – treatment must begin within 72 hours of symptom onset 1.

Differential Diagnosis Algorithm

Primary Considerations Based on Clinical Features

If vesicles are present:

• Ramsay Hunt syndrome is confirmed – begin treatment immediately 1

If no vesicles are visible but facial weakness + ear pain are present:

• Still treat as presumptive Ramsay Hunt syndrome (zoster sine herpete) if symptoms are acute onset (<72 hours) 1
• Consider Bell's palsy as secondary diagnosis, but the presence of otalgia makes Ramsay Hunt more likely 1, 2

If additional cranial nerves are involved:

• Urgent MRI with contrast to evaluate for stroke, meningitis, or cerebellopontine angle tumor 1
• Consider bacterial meningitis if fever, headache, or altered mental status present 1

If bilateral facial weakness:

• This is NOT Bell's palsy or Ramsay Hunt syndrome – consider Lyme disease (especially in endemic areas), Guillain-Barré syndrome, sarcoidosis, or bilateral vestibular schwannomas 1

Red Flags Requiring Immediate Neuroimaging

The American Academy of Otolaryngology-Head and Neck Surgery identifies these features that mandate urgent MRI with contrast 1:

• Any additional cranial nerve involvement beyond CN VII
• Vertigo with dysarthria, ataxia, or nystagmus suggesting brainstem stroke
• Gradual onset over weeks rather than acute onset, suggesting tumor
• Recurrent episodes of facial weakness, suggesting demyelinating disease
• Bilateral facial weakness at presentation

Immediate Treatment Protocol

First-Line Therapy for Presumed Ramsay Hunt Syndrome

Antiviral therapy (must start within 72 hours):

• Valacyclovir 1000 mg three times daily for 7 days, OR
• Acyclovir 800 mg five times daily for 7 days 1

Corticosteroid therapy (combined with antivirals):

• Prednisone 60-80 mg daily for 5-7 days, then taper over 10 days 1

Eye protection (critical to prevent corneal injury):

• Artificial tears during the day
• Ophthalmic ointment at night
• Eye patch or taping the eye closed during sleep 1

Why Antivirals Are Essential Despite Limited Evidence

While the American Academy of Otolaryngology-Head and Neck Surgery notes that evidence for antivirals in Bell's palsy is limited, Ramsay Hunt syndrome has a fundamentally different pathophysiology with active viral replication, making antiviral therapy biologically rational and standard of care 1. The combination of antivirals plus corticosteroids improves recovery rates from 20-30% to approximately 70% when started early 1.

Common Diagnostic Pitfalls to Avoid

Missing Ramsay Hunt Syndrome

• Vesicles may appear 2-3 days after facial weakness begins – if you see a patient with acute facial weakness and ear pain but no vesicles, instruct them to return immediately if vesicles develop, or treat presumptively 1
• Vesicles may be hidden in the ear canal – always examine with an otoscope, not just inspect the external ear 1
• Zoster sine herpete (no vesicles) occurs in up to 20% of cases – the combination of otalgia with facial weakness should trigger treatment even without visible vesicles 1

Misattributing Ear Pain to Otitis Media or Externa

The American Academy of Otolaryngology-Head and Neck Surgery clarifies that ear pain in facial nerve disorders is referred pain from the facial nerve itself (via nervi nervorum), not from middle ear or external ear canal pathology 2, 3. If the tympanic membrane appears normal and there is no external canal inflammation, the ear pain is neurogenic and part of the facial nerve syndrome 2, 3.

Failing to Assess for Lyme Disease in Endemic Areas

In Lyme-endemic regions, up to 25% of facial paralysis cases are due to Lyme disease, which requires doxycycline rather than antivirals 1. The American Academy of Otolaryngology-Head and Neck Surgery recommends Lyme serology for patients in endemic areas or with recent travel to endemic areas 1.

When to Obtain Imaging

Do NOT routinely order imaging for uncomplicated Bell's palsy or Ramsay Hunt syndrome

The American Academy of Otolaryngology-Head and Neck Surgery makes a strong recommendation against routine CT or MRI in patients with isolated facial weakness and no red flags 1.

DO obtain urgent MRI with contrast if:

• Multiple cranial nerves involved 1
• Gradual onset over weeks 1
• Recurrent episodes 1
• Bilateral facial weakness 1
• Vertigo with other brainstem signs (dysarthria, ataxia, nystagmus) 1
• No improvement after 3-4 weeks of appropriate treatment 1

Expected Clinical Course and Follow-Up

Timeline for Recovery

• Pain typically improves within 48-72 hours of starting antivirals and corticosteroids 1
• Facial weakness may initially worsen for the first 3-5 days before stabilizing 1
• Maximum recovery occurs over 3-6 months, with most improvement in the first 3 months 1
• Incomplete recovery is common – only 70% achieve complete recovery even with optimal treatment, compared to 85-90% with Bell's palsy 1

Reassessment Criteria

Reassess at 2-3 weeks:

• If no improvement or continued worsening, obtain MRI with contrast to evaluate for alternative diagnoses 1

Reassess at 3 months:

• If significant residual weakness persists, refer to neurology or facial nerve specialist for consideration of surgical decompression or other interventions 1

Complications to Monitor

• Corneal abrasion or ulceration from incomplete eye closure – requires daily assessment until eye closure improves 1
• Synkinesis (abnormal facial movements) develops in 15-20% of patients with incomplete recovery 1
• Permanent hearing loss occurs in 10-15% of Ramsay Hunt syndrome patients 1