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RAMSAY HUNT SYNDROME

(Herpes Zoster Oticus)

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INTRODUCTION & HISTORICAL BACKGROUND

1. Otalgia (severe ear pain)
2. Auricular vesicles (herpetic eruption in the ear)
3. Peripheral facial nerve palsy (LMN type)

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DEFINITION

Ramsay Hunt Syndrome is a peripheral facial nerve palsy accompanied by erythematous vesicular eruption of the skin of the ear canal, auricle (herpes zoster oticus), and/or mucous membrane of the oropharynx, caused by reactivation of latent Varicella Zoster Virus (VZV) in the geniculate ganglion of the facial nerve (CN VII).

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EPIDEMIOLOGY

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ETIOLOGY & MICROBIOLOGY

• Causative agent: Varicella Zoster Virus (VZV) — a double-stranded DNA virus, member of Herpesviridae family
• Primary infection: Chickenpox (Varicella) — usually in childhood
• After primary infection, VZV travels retrogradely along sensory nerve fibers and establishes latency in the dorsal root ganglia and cranial nerve sensory ganglia
• Reactivation occurs when cell-mediated immunity wanes → VZV reactivates in the geniculate ganglion of CN VII

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ANATOMY OF RELEVANCE

The Facial Nerve (CN VII) — Intratemporal Course

BRAINSTEM (Pontomedullary junction)
        ↓
Internal Auditory Canal (IAC)
        ↓
  ┌─────────────────────────┐
  │   GENICULATE GANGLION   │ ← Site of VZV latency & reactivation
  └─────────────────────────┘
        ↓ (Greater Petrosal Nerve — parasympathetic to lacrimal gland)
  Tympanic Segment
        ↓ (Nerve to Stapedius — CN VII branch)
  Mastoid/Vertical Segment
        ↓ (Chorda Tympani — taste, submandibular/sublingual glands)
  Stylomastoid Foramen
        ↓
  Extratemporal branches (Temporal, Zygomatic, Buccal, Marginal Mandibular, Cervical)

The geniculate ganglion is the sensory ganglion of CN VII, receiving afferents from:

• External ear (concha, EAC) via auricular branch of CN X (Arnold's nerve) and auricular branch of CN V3
• Soft palate, tonsillar fossa via intermediate nerve (Nerve of Wrisberg)

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PATHOPHYSIOLOGY

┌──────────────────────────────────────────────────────────┐
│          PRIMARY VZV INFECTION (Varicella/Chickenpox)    │
└──────────────────────────────┬───────────────────────────┘
                               ↓
        Retrograde axonal transport → Latency established in
              GENICULATE GANGLION (CN VII sensory ganglion)
                               ↓
         [Trigger: Immunosuppression, aging, stress, malignancy]
                               ↓
              REACTIVATION OF VZV IN GENICULATE GANGLION
                               ↓
         ┌──────────────┬──────────────────┬─────────────────┐
         ↓              ↓                  ↓                 ↓
    Anterograde    Inflammatory        Spread to        Spread to
    spread to      edema →             CN VIII         pharyngeal
    skin → VESICLES compression of     (cochlear/      branches →
    in EAC/pinna   CN VII in bony      vestibular)     oral vesicles
                   fallopian canal     → SNHL/Vertigo
                        ↓
              FACIAL NERVE PALSY (LMN type)

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HUNT'S ORIGINAL CLASSIFICATION (4 Types)

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CLINICAL FEATURES

Cardinal Triad:

1. Severe Otalgia — deep-seated, boring, lancinating ear pain (often precedes vesicles by 2–3 days — "pre-eruptive stage")
2. Vesicular Eruption
3. Peripheral Facial Nerve Palsy (LMN)

Detailed Clinical Features:

1. PRODROMAL PHASE (1–3 days before eruption)

• Burning, intense otalgia (ipsilateral)
• Low-grade fever, malaise
• Hyperesthesia/dysesthesia of the ear

2. VESICULAR ERUPTION

• External auditory canal (EAC)
• Concha of pinna
• Postauricular area
• Soft palate / tonsillar fossa
• Anterior 2/3 of tongue (taste fibers via chorda tympani)
• Occasionally: neck, face (C2/C3 dermatomes)

Vesicles are clear initially → turbid → rupture → crust over 7–10 days (Bailey & Love's 28th Ed., p. 784)

3. FACIAL NERVE PALSY

• LMN type — involves ALL muscles of ipsilateral face (forehead sparing absent, unlike UMN palsy)
• Graded by House-Brackmann Scale (Grade I–VI)
• Features:
  • Loss of wrinkling of forehead
  • Unable to close eye (Lagophthalmos) → Bell's phenomenon (eye rolls up on attempting closure)
  • Flattening of nasolabial fold
  • Drooping of angle of mouth
  • Inability to puff cheeks
  • Loss of taste (anterior 2/3 tongue — chorda tympani involvement)
  • Hyperacusis (nerve to stapedius involved)
  • Reduced lacrimation (greater petrosal nerve involved)

4. AUDIOVESTIBULAR SYMPTOMS (CN VIII involvement)

• Sensorineural hearing loss (SNHL) — high-frequency predominant
• Vertigo — acute, episodic (like vestibular neuritis)
• Tinnitus
• Nystagmus — toward unaffected side

5. OTHER FEATURES

• Dysgeusia (altered taste)
• Dry eye (keratoconjunctivitis)
• Dysphagia (if CN IX, X involved)
• Rarely: contralateral limb weakness (zoster encephalitis)

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INVESTIGATIONS

1. Clinical Diagnosis (primarily)

2. Audiological Assessment

• Pure Tone Audiogram (PTA) — SNHL, predominantly high-frequency
• Tympanometry — Type A (normal middle ear compliance; helps rule out otitis media)
• BERA/ABR — to assess retrocochlear involvement
• Caloric testing / VNG — vestibular function assessment

3. Nerve Function Tests

4. Topognostic (Site of Lesion) Diagnosis

LEVEL OF LESION DETERMINATION:

Above Geniculate Ganglion:
  ↓ Lacrimation (Schirmer's +ve)
  ↓ Stapedial reflex (absent)
  ↓ Taste (anterior 2/3)
  ↓ Salivation

Between Geniculate & Nerve to Stapedius:
  Normal Lacrimation
  ↓ Stapedial reflex (absent)
  ↓ Taste
  ↓ Salivation

Between N. to Stapedius & Chorda Tympani:
  Normal Lacrimation
  Normal Stapedial reflex
  ↓ Taste
  ↓ Salivation

Below Chorda Tympani (Stylomastoid foramen):
  Normal Lacrimation
  Normal Stapedial reflex
  Normal Taste
  Normal Salivation — Motor palsy ONLY

5. Radiological Investigations

• MRI with Gadolinium contrast (investigation of choice):
  • Enhancement of geniculate ganglion, facial nerve
  • Rules out schwannoma, cholesteatoma, parotid malignancy
  • Assesses brainstem
• HRCT Temporal bone: Rules out bony erosion, cholesteatoma, otitis media

6. Virological Tests

• VZV PCR from vesicle fluid, saliva, or CSF — confirmatory
• Tzanck smear from vesicle base: multinucleated giant cells (not specific)
• Serology: Rise in IgM/IgG anti-VZV antibodies
• CSF analysis (if CNS involvement suspected): lymphocytic pleocytosis, raised protein

ZOSTER SINE HERPETE

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CLINICAL PHOTOGRAPH — HALLMARK SIGNS

Image: Composite photograph of Ramsay Hunt Syndrome.

• Left (Image A): Right auricle showing inflammatory swelling with characteristic clustered vesicles and hemorrhagic crusting in the concha and EAC, extending to the preauricular region.
• Right (Image B): Ipsilateral LMN facial nerve palsy — facial asymmetry, drooping of right side, loss of nasolabial fold, and Bell's phenomenon (upward rolling of globe on attempted eye closure = lagophthalmos).

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HOUSE-BRACKMANN GRADING SCALE FOR FACIAL PALSY

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DIFFERENTIAL DIAGNOSIS

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MANAGEMENT

FLOWCHART OF MANAGEMENT

PATIENT WITH ACUTE FACIAL PALSY + EAR PAIN/VESICLES
              ↓
    Clinical Assessment (Triad + Audiovestibular)
              ↓
    CONFIRM DIAGNOSIS (Clinical ± VZV PCR/MRI)
              ↓
  ┌───────────────────────────────────────────────┐
  │              GENERAL MEASURES                 │
  │  - Eye care (Lagophthalmos prevention)        │
  │  - Lubricant eye drops (Methylcellulose)      │
  │  - Eye pad/taping at night                    │
  │  - Analgesics for otalgia                     │
  └──────────────────┬────────────────────────────┘
                     ↓
  ┌──────────────────────────────────────────────────────┐
  │              ANTIVIRAL THERAPY                       │
  │   (MOST IMPORTANT — Start within 72 hours)           │
  │                                                      │
  │  Acyclovir 800 mg 5×/day × 7–10 days (oral)         │
  │      OR                                              │
  │  Valacyclovir 1g TDS × 7 days (better bioavailability)│
  │      OR                                              │
  │  Famciclovir 500 mg TDS × 7 days                    │
  │                                                      │
  │  Severe: IV Acyclovir 10 mg/kg TDS × 7–10 days      │
  └──────────────────┬───────────────────────────────────┘
                     ↓
  ┌──────────────────────────────────────────────────────┐
  │              CORTICOSTEROIDS                         │
  │  Prednisolone 1 mg/kg/day → taper over 10–14 days   │
  │  (Reduces neural edema, improves prognosis)          │
  │  Combined Antiviral + Steroid = Best outcome         │
  └──────────────────┬───────────────────────────────────┘
                     ↓
  ┌────────────────────────────────────┐
  │    AUDIOVESTIBULAR MANAGEMENT      │
  │  • SNHL: Betahistine, Vasodilators │
  │  • Vertigo: Prochlorperazine,      │
  │    Cinnarizine                     │
  │  • Vestibular rehab exercises      │
  └──────────────────┬─────────────────┘
                     ↓
         MONITOR RECOVERY WEEKLY
         (House-Brackmann grading)
                     ↓
        ┌────────────────────────────┐
        ↓                            ↓
   RECOVERY (>70%)           No Recovery by 3 months
   (Most within 6 months)    → Consider Surgical Decompression
                                    ↓
                          ENoG: >90% degeneration within 14 days
                          EMG: Denervation potentials
                                    ↓
                          SURGICAL DECOMPRESSION of
                          Facial Nerve (Transpetrosal route)
                          — middle cranial fossa approach

DETAILED DRUG REGIMENS

Eye Care (Critical — prevents exposure keratopathy)

• Artificial tear drops (Methylcellulose) — during day
• Lubricant eye ointment — at night
• Eye pad taping at night
• Tarsorrhaphy (temporary lateral lid suturing) — if corneal exposure severe
• Regular ophthalmology review

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SURGICAL MANAGEMENT

Indications for Facial Nerve Decompression:

1. ENoG > 90% degeneration within 14 days of onset
2. No clinical or electrical recovery by 3 months
3. Surgical decompression is controversial — evidence is limited

Surgical Approaches:

Other Surgical Procedures:

• Tarsorrhaphy — for lagophthalmos
• Gold weight implant in upper eyelid — for permanent lagophthalmos
• Hypoglossal-facial nerve anastomosis — for permanent complete palsy
• Gracilis free muscle transfer — for long-standing palsy with muscle atrophy

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COMPLICATIONS

Short-term:

Long-term:

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PROGNOSIS

• Complete palsy + treated early: 70% full recovery
• Complete palsy + untreated: ~50% full recovery
• RHS vs Bell's palsy: RHS has significantly worse prognosis than Bell's palsy
• Recovery timeline: Most within 3–6 months; if no recovery by 1 year → permanent

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COMPARISON: RAMSAY HUNT SYNDROME vs BELL'S PALSY

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RECENT ADVANCES (Important for RGUHS)

1. Zoster Vaccine (Shingrix)

• Recombinant subunit vaccine (RZV — Shingrix) — 2 doses, highly effective (~97%) in preventing herpes zoster and RHS in adults ≥50 years
• Superior to older live-attenuated Zostavax vaccine
• Recommended for immunocompetent adults ≥50 years by CDC and WHO (2023 guidelines)
• Reduces incidence of post-herpetic neuralgia by >90%

2. Antiviral Updates

• Valacyclovir is preferred over Acyclovir due to superior oral bioavailability (3–5× higher)
• Combination antiviral + steroid proven superior to either alone (Murakami et al.)
• Early initiation (<72h) significantly improves outcomes

3. VZV PCR in Zoster Sine Herpete

• High-sensitivity VZV PCR from saliva is now the standard non-invasive diagnostic test for RHS without vesicles
• Changing the diagnostic paradigm — up to 16–18% of Bell's palsy cases may actually be VZV-mediated (detected by PCR)

4. MRI Advances

• 3T MRI with gadolinium — detects subtle geniculate ganglion enhancement as early as 48 hours
• Differentiates RHS from Bell's palsy, schwannoma, and other causes of facial palsy
• Predicts extent of nerve damage and correlates with prognosis

5. ENoG as Prognostic Tool

• ENoG performed between day 3–14 of complete palsy onset is most informative

• 90% degeneration → surgical decompression consideration

• Updated evidence suggests transcutaneous electrostimulation may help during recovery

6. Vestibular Rehabilitation

• Evidence-based vestibular rehabilitation exercises (Cawthorne-Cooksey, Brandt-Daroff) show significant improvement in RHS-related vestibular dysfunction

7. Facial Reanimation Surgery

• Selective neurorrhaphy, cross-facial nerve grafting, and dynamic reanimation using free gracilis muscle transfer — newer reconstructive options for permanent palsy
• Botulinum toxin (Botox) — used to treat synkinesis and hemifacial spasm post-recovery

8. Immunocompromised Patients (HIV/AIDS)

• RHS can present with multidermatome zoster and bilateral facial palsy
• IV Acyclovir + long-term oral antivirals recommended
• Higher rates of PHN and incomplete recovery

9. COVID-19 and RHS

• Recent case series (2021–2023) report reactivation of VZV (causing RHS) following COVID-19 infection and COVID-19 mRNA vaccination — proposed mechanism: immune dysregulation
• Not yet established as causative; monitoring ongoing

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SUMMARY FLOWCHART: DIAGNOSTIC APPROACH

ACUTE PERIPHERAL FACIAL PALSY
            ↓
  Is it LMN or UMN type?
            ↓ LMN
  Check for Vesicles in EAC/Pinna?
     ↓ YES                    ↓ NO
  RHS likely         Other causes (Bell's, Otitis Media,
     ↓               Cholesteatoma, Tumor, Lyme, etc.)
  Check:                      ↓
  • CN VIII signs?         VZV PCR (Zoster sine herpete?)
  • Taste loss?
  • Lacrimation?
  • Hyperacusis?
            ↓
   TOPOGNOSTIC TESTING
   (Schirmer's, Stapedial reflex,
    Electrogustometry)
            ↓
   IMAGING: MRI Gadolinium
   + HRCT Temporal Bone
            ↓
   ENoG + EMG (if complete palsy)
            ↓
   CONFIRM RHS → START TREATMENT within 72h

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KEY POINTS FOR VIVA / SHORT NOTES (RGUHS Focus)

1. James Ramsay Hunt — described in 1907
2. VZV — reactivates in geniculate ganglion (sensory ganglion of CN VII)
3. Classic triad: Otalgia + Vesicles + LMN Facial Palsy
4. Bell's phenomenon — eye rolls up when patient tries to close eye (lagophthalmos)
5. House-Brackmann scale — grading facial palsy (Grade I=Normal, VI=Complete palsy)
6. Schirmer's test — reduced if lesion is above/at geniculate ganglion (Greater Petrosal Nerve)
7. ENoG — >90% degeneration = poor prognosis / consider surgical decompression
8. Treatment: Acyclovir/Valacyclovir + Prednisolone — within 72 hours
9. Prognosis of RHS is worse than Bell's palsy (50–70% full recovery vs. 85–90%)
10. Zoster sine herpete — RHS without vesicles; diagnose with VZV PCR
11. Shingrix vaccine — recombinant VZV vaccine; >97% effective; recommended ≥50 years
12. Complications: PHN, synkinesis, crocodile tears, exposure keratopathy
13. Corneal protection is a CLINICAL EMERGENCY — lagophthalmos → corneal ulcer → blindness
14. MRI gadolinium — investigation of choice for imaging

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REFERENCES

1. Scott-Brown's Otorhinolaryngology, Head and Neck Surgery (8th Ed.) — Facial Nerve chapter
2. Cummings Otolaryngology — Head and Neck Surgery (7th Ed., Flint et al.) — Ch. Facial Nerve, Herpes Zoster Oticus
3. Stell and Maran's Head and Neck Surgery — Facial nerve and peripheral neuropathies
4. Zakir Hussain's ENT — Ramsay Hunt Syndrome, Peripheral Facial Palsy
5. Dhingra's Diseases of Ear, Nose and Throat (8th Ed.) — Facial nerve disorders, p. relevant chapters
6. Hazarika's Textbook of ENT & Head-Neck Surgery — Ramsay Hunt Syndrome, facial palsy
7. Bailey & Love's Short Practice of Surgery (28th Ed., p. 784) — Facial nerve palsy
8. Harrison's Principles of Internal Medicine (21st Ed., p. 12502) — Differential diagnosis of facial palsy
9. Murakami S et al. — "Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: significance of early diagnosis and treatment" — Ann Neurol 1997
10. Coulson S et al. — "Herpes zoster oticus (Ramsay Hunt syndrome)" — Cochrane Review
11. CDC/WHO 2023 — Shingrix (RZV) vaccination guidelines
12. Sweeney CJ, Gilden DH — "Ramsay Hunt syndrome" — JNNP 2001
13. Recent articles (2021–2023) — VZV reactivation post-COVID-19 and mRNA vaccination

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