What is the recommended treatment for Ramsay Hunt syndrome, including antiviral and corticosteroid dosing, timing of initiation, and adjustments for immunocompromised patients or those who cannot receive steroids?

Ramsay Hunt Syndrome Treatment

Initiate combination therapy with oral prednisolone 1 mg/kg/day (maximum 60 mg) plus acyclovir 800 mg five times daily (or valacyclovir 1000 mg three times daily) within 72 hours of symptom onset for 7-10 days to maximize facial nerve recovery and reduce hearing loss.

First-Line Pharmacologic Therapy

Corticosteroid Regimen

• Standard dose: Prednisolone 1 mg/kg/day (maximum 60 mg/day) as a single daily dose for 7-10 days, followed by a 5-day taper 1, 2
• Alternative high-dose protocol: Prednisolone 200 mg/day may be considered for House-Brackmann grade VI (complete facial paralysis), which showed 71.1% recovery rates versus 60% with standard dosing, though evidence is limited 3
• Timing is critical: Must begin within 72 hours of symptom onset for optimal facial nerve recovery 1
• Treatment initiated after 72 hours shows diminished but still meaningful benefit up to 2 weeks 1, 2

Antiviral Therapy

• Acyclovir: 800 mg orally five times daily for 7-10 days 1, 4
• Alternative: Valacyclovir 1000 mg three times daily for 7-10 days (better bioavailability) 1
• Intravenous option: Acyclovir 10-15 mg/kg three times daily for severe cases or patients unable to take oral medications, adjusted for renal function 1
• Antiviral monotherapy alone is not recommended—must be combined with corticosteroids 1, 5

Treatment Algorithm by Severity

House-Brackmann Grade I-III (Mild to Moderate Palsy)

• Oral prednisolone 60 mg daily for 5 days, then taper over 5 days 1
• Plus oral acyclovir 800 mg five times daily for 7-10 days 4

House-Brackmann Grade IV-VI (Severe to Complete Palsy)

• Consider high-dose prednisolone 200 mg daily for 5 days, then taper 3
• Plus oral acyclovir 800 mg five times daily for 7-10 days 4
• If no improvement after 2-3 weeks, consider salvage therapy with intravenous methylprednisolone 1000 mg daily for 3-5 days 6

Special Populations and Contraindications

Immunocompromised Patients

• Prolonged antiviral course: Extend intravenous acyclovir beyond 7-10 days due to risk of viral reactivation 1
• Standard corticosteroid dosing: Use prednisolone 1 mg/kg/day; avoid high-dose protocols unless benefits clearly outweigh risks 1
• Monitor closely for opportunistic infections and consider prophylaxis 1

When Systemic Corticosteroids Are Contraindicated

Absolute contraindications include:

• Uncontrolled diabetes mellitus
• Active peptic ulcer disease
• Active tuberculosis
• Severe hypertension
• Glaucoma
• Myasthenia gravis 2, 7

Alternative approach: Offer intratympanic corticosteroid injections as initial therapy 2, 7

• Dexamethasone 24 mg/mL or methylprednisolone 40 mg/mL
• 0.4-0.8 mL injected into middle ear via anterosuperior myringotomy
• Up to 4 injections over 2 weeks
• Patient maintains head in otologic position for 15-30 minutes per side 2

Management of Hearing Loss in Ramsay Hunt Syndrome

Acute Hearing Loss at Presentation

• Obtain baseline audiometry within 14 days to document sensorineural hearing loss 2, 7
• The standard prednisolone-acyclovir regimen addresses both facial palsy and cochlear involvement 4, 3

Late-Onset Hearing Loss (After Completing Initial Treatment)

• Treat as a new episode of sudden sensorineural hearing loss with its own 14-day therapeutic window 2
• Restart oral prednisolone 1 mg/kg/day for 7-14 days with taper 2, 7
• Do not add additional antiviral therapy—the hearing loss is immune-mediated, not from active viral replication 2

Salvage Therapy for Incomplete Hearing Recovery

• If hearing remains impaired at 2-6 weeks post-treatment, offer intratympanic steroid injections 2, 7
• Methylprednisolone 62.5 mg/mL: 5 injections over 12 days achieved 73.6% improvement in refractory cases 2
• Perform audiometry before each injection to monitor response 2

Monitoring and Follow-Up

Baseline Assessment

• Audiometry: Pure-tone audiometry and speech discrimination within 14 days of symptom onset 2, 7
• Facial nerve function: Document House-Brackmann grade 3
• Vestibulocochlear testing: Assess for vertigo and balance dysfunction 8, 4

During Treatment

• Monitor blood glucose in diabetic patients receiving corticosteroids 1
• Assess for corticosteroid adverse effects: hyperglycemia, hypertension, insomnia, mood changes, gastric irritation 2
• Renal function monitoring if using intravenous acyclovir 1

Post-Treatment

• Audiometry at completion of treatment and again at 6 months regardless of perceived recovery 2, 7
• Facial nerve function assessment at 3 months and 9 months 1
• 54.5% of patients who recover show improvement within 10 days; 78% reach final hearing level by 1 month 2

Prognostic Indicators

Poor Prognosis Factors

• Complete facial paralysis (House-Brackmann grade VI) at presentation 3
• Age >60 years 6
• Absence of R1 and R2 responses on blink reflex testing 6
• Vertigo at onset predicts poorer hearing recovery 2, 7
• Delayed treatment beyond 72 hours 1, 3
• Vesicles appearing after facial palsy (worse prognosis than vesicles appearing first) 3

Favorable Indicators

• Recovery of at least 50% of hearing loss within first 2 weeks 2, 7
• Early treatment initiation (within 72 hours) 1, 3
• Incomplete facial paralysis at presentation 3

Salvage Therapy for Non-Recovering Cases

Intravenous High-Dose Methylprednisolone

• Indication: Persistent severe facial palsy 2-3 weeks after standard oral therapy 6
• Dosing: Methylprednisolone 1000 mg IV daily for 3-5 days 6
• This approach showed near-complete recovery in two case reports with multiple poor prognostic factors 6

Hyperbaric Oxygen Therapy

• May be offered as adjunctive therapy within 1 month of symptom onset for hearing loss, though evidence is weak 1, 2
• Not a strong recommendation and should not delay standard corticosteroid-antiviral therapy 1

Rehabilitation and Counseling

Audiologic Rehabilitation

• Do not delay counseling about hearing aids, cochlear implantation, or assistive listening devices 2, 7
• Postponement leads to auditory deprivation and poorer long-term outcomes 2
• Begin discussions when hearing loss is first identified 2

Facial Nerve Rehabilitation

• Physical therapy and facial exercises may be beneficial during recovery phase 1
• Eye protection (lubricating drops, nighttime taping) for incomplete eyelid closure 1

Common Pitfalls to Avoid

• Do not use antiviral monotherapy—always combine with corticosteroids 1, 5
• Do not delay treatment waiting for confirmatory VZV testing—initiate empirically based on clinical presentation 8, 4
• Do not assume prior corticosteroid treatment covers late-onset hearing loss—treat as a distinct episode 2
• Do not obtain routine CT head—it adds no diagnostic value and delays treatment 1
• Do not obtain routine laboratory tests unless clinically indicated for specific comorbidities 1
• Do not overlook VZV vasculopathy in patients with new focal neurologic deficits—obtain MRI brain and CSF analysis for VZV DNA/IgG 4