Ramsay Hunt Syndrome: Herpes Zoster Oticus and Facial Paralysis

After childhood chickenpox (primary VZV infection) the virus lodges in sensory ganglia and remains latent. Years or decades later, with immune compromise (age, immunosuppression, stress, illness), the virus reactivates and produces segmental zoster or Ramsay Hunt syndrome.

In Ramsay Hunt, the target is the geniculate ganglion of the facial nerve. Inflammation involves both the nerve fibres and adjacent structures (cochlea, vestibular system, ear skin). This explains the classic triad: palsy (nerve), hearing / balance (adjacent organs), vesicles (external ear skin).

Annual incidence is around 5 per 100,000. Frequency rises markedly after age 50. In immunosuppressed patients (HIV, leukaemia, chemotherapy) both incidence and severity increase; disseminated zoster or cerebellar involvement can develop. We expand on the clinical framework in our otology and hearing centre.

Classic pattern: ear pain first (sometimes a 1-3 day prodrome before vesicles), then painful vesicles on the auricle, external canal entrance and rarely the tympanic membrane, then ipsilateral facial palsy within hours to days. Hearing loss and / or vertigo may follow.

The classic triad: palsy + vesicles + audiovestibular signs. Ramsay Hunt can be diagnosed without vesicles — "zoster sine herpete". Clinical suspicion is the basis, and VZV PCR (CSF or blood when no vesicle fluid is available) can support the diagnosis.

Difference from Bell palsy: Bell has no vesicles, pain is mild, hearing loss is atypical. Bell recovers spontaneously in 85-90% versus 50-70% in Ramsay Hunt — and this is highly dependent on timing of therapy. Every facial palsy therefore needs an ear exam and audiogram.

Standard grading of facial nerve dysfunction is the House-Brackmann (HB) scale. HB I: normal. HB II: slight dysfunction, asymmetry visible only on careful examination, eye closes completely. HB III: moderate dysfunction, obvious asymmetry but eye closes with effort.

HB IV: moderately severe, marked asymmetry and weakness, eye closure incomplete even with effort. HB V: severe dysfunction, only minimal movement. HB VI: complete paralysis, no movement. The scale is used for both prognosis and response monitoring.

Ramsay Hunt typically presents at HB IV-V. After treatment the grade is reassessed at 6-12 months. Final HB I-II is excellent recovery; HB III is moderate; HB IV or worse is considered permanent sequela.

Timing of treatment is the single strongest predictor of outcome. Patients treated within 72 hours have full recovery in 70-75%; treatment starting beyond 72 hours drops this to 50%, and beyond 7 days to 30-40%.

Antiviral choice: oral aciclovir 800 mg five times daily for 7-10 days. Alternative is valaciclovir 1000 mg three times daily — better bioavailability. In severe disease or immunosuppression, IV aciclovir (10 mg/kg three times daily for 7-10 days). Dose-adjust for renal function.

Corticosteroid: oral prednisone 1 mg/kg/day (max 60-80 mg) for 5-7 days at full dose, then tapered over 7-10 days. Steroids reduce nerve oedema and add benefit on top of antiviral monotherapy. Use with caution in diabetes, hypertension, peptic ulcer disease.

Supportive care: eye protection (artificial tears, ocular ointment at night with tape — to prevent corneal ulceration), pain control (NSAID, gabapentin / pregabalin for postherpetic neuralgia), antiemetic / vestibular suppressant briefly if vertigo. Facial physiotherapy starts 1-2 weeks later. More detail: cholesteatoma page.

Sensorineural hearing loss occurs in 30-50% of Ramsay Hunt patients due to cochleovestibular involvement — VZV causes inflammation in the inner-ear structures. Treatment: alongside antivirals, steroid (part of the systemic dose) and, if needed, intratympanic steroid (dexamethasone injection).

Vertigo / imbalance is reported in 15-25%, resembling vestibular neuritis. Acutely, vestibular suppressants (meclizine, dimenhydrinate) are used for 1-3 days. Then early vestibular rehabilitation accelerates compensation.

Hearing loss recovers partially or fully over 3-6 months; in some patients it remains. Vestibular signs likewise settle through compensation. Postherpetic neuralgia (PHN — prolonged burning pain in the ear and hemi-face) is seen in 20-30% and is managed with gabapentin / pregabalin / TCA.

Permanent facial paresis: 25-30% retain mild-moderate residual asymmetry, typically HB III-IV. Synkinesis (involuntary co-movement — e.g. eye closure with mouth opening) can be partly controlled with rehabilitation; refractory cases consider botulinum toxin or surgery (cross-facial nerve graft, dynamic gracilis transfer).

Corneal complications: with inadequate eye closure, dryness and ulceration develop, with potential for vision loss. Prevention: aggressive artificial tears, ocular ointment at night, gold-weight implant or temporary tarsorrhaphy in selected cases.

Postherpetic neuralgia (PHN): the most common sequela after VZV reactivation — burning, stabbing pain beyond 3 months. Risk factors: older age, severe acute pain, immunosuppression. Treatment: gabapentin starting 300 mg/day, titrated to 1800-3600 mg/day. Topical lidocaine patches can help.

Rare complications: cerebellitis, encephalitis, involvement of other cranial nerves (V, VIII, IX, X), bacterial superinfection. These cases warrant hospital admission and IV antiviral therapy.

Recombinant zoster vaccine (Shingrix) is recommended in adults over 50 and in immunocompromised individuals over 18. Two doses 2-6 months apart. Zoster prevention efficacy is over 90% with 91% protection against postherpetic neuralgia. This lowers Ramsay Hunt incidence as well.

The older live zoster vaccine (Zostavax) is no longer used in most countries. Shingrix is preferred as it is safe in immunosuppressed patients and has high efficacy.

Personal recommendations: vaccinate everyone over 50, vaccinate before planned or ongoing immunosuppression, and vaccinate even after previous zoster (or Ramsay Hunt) — past infection does not provide durable full protection, with a 5% recurrence risk.

Shingrix is available in Türkiye. It is not under national insurance coverage and is acquired privately. Side effects are mostly injection site pain, fatigue and headache — resolving in a few days. Related reading: our patient testimonials.