Neurology & ENT & Family Medicine
Ramsay Hunt syndrome
Ramsay Hunt syndrome—first described by American neurologist James Ramsay Hunt in 1907 and also known as herpes zoster oticus or geniculate ganglion zoster—is a particularly severe and disabling form of shingles resulting from the reactivation of the varicella-zoster virus (VZV— Varicella-zoster virus, VZV, herpesvirus type 3) in the geniculate ganglion of the facial nerve (VII), following a primary varicella infection decades earlier, during which VZV established permanent latency in the sensory ganglia of the cranial and spinal nerves. Viral reactivation in the geniculate ganglion leads to inflammation and necrosis of the VII nerve fibers, as well as viral diffusion to neighboring anatomical structures innervated by the facial nerve and its branches: the external auditory canal + the pinna + the tympanic membrane + the anterior third of the tongue (tympanic cord) + and, by extension, cochleovestibular structures (VIII - vestibulocochlear nerve) and the oral cavity. The classic clinical triad combines homolateral peripheral facial paralysis (affecting all levels of the face - forehead + eye + mouth - in contrast to central involvement, which spares the forehead) + auricular shingles (painful vesicular eruption on the auricle + external auditory canal + eardrum + sometimes palate and tongue) + cochleo-vestibular symptoms (tinnitus + sensorineural hypoacusis + vertigo). Compared with paralysis a frigore (Bell's palsy - presumed to be caused by a non-vericular virus), Ramsay Hunt syndrome has a much more severe prognosis: only 70 % of complete recovery of facial palsy (vs. 90 to 95 % for Bell's palsy) - justifying even more urgent and aggressive antiviral + corticosteroid treatment. Early treatment (within the first 72 hours of symptom onset, ideally within 24 hours) is the most important modifiable prognostic factor for recovery of facial function.
Clinical presentation
- Intense ear pain (prodrome): Severe ear pain + deep burning pain of the external auditory canal + sometimes radiating to the neck + jaw + occipital region + precedes rash by 1 to 3 days + may be the only initial sign → any patient with intense ear pain + facial paralysis must have a rash actively sought (rash may be mild or absent at first)
- Herpes zoster eruption (ear rash) Vesicles clustered in a bunch on an erythematous background + characteristic location: auricle (helix + antihelix + lobule + concha) + external auditory canal + tympanic membrane + sometimes soft palate + uvula + tongue (anterior two-thirds) + rash may be barely visible if confined to the ear canal → examine carefully with an otoscope
- Contralateral peripheral facial paralysis: Complete or partial paralysis of all facial muscles on the affected side + inability to close the eye (lagophthalmos) → exposure keratitis if not protected + deviation of the mouth to the opposite side + flattening of the nasolabial fold + disappearance of forehead wrinkles + Bell's sign (upward deviation of the eyeball when attempting to close the eyes) + paralysis may precede or follow the rash
- Cochleovestibular (VIII) involvement: unilateral tinnitus + sensorineural hearing loss (distinguish from conductive hearing loss by otoscopy) + vertigo + nystagmus + nausea + vomiting + gait instability → damage to the VIII cranial nerve indicates viral spread and worsens the prognosis
- Other possible manifestations: dysgeusia (altered taste - chorda tympani) + xerostomia + xerophthalmia (damage to lacrimal and salivary glands) + velopalatine paralysis (nasal voice) + very rarely: involvement of adjacent cranial nerves (IX + X + XI + XII) in extensive forms - Garcin's syndrome if multiple cranial nerves are affected
Differential diagnosis of peripheral facial paralysis
| Etiology | Distinctive elements | Frequency |
|---|---|---|
| Bell's palsy (cold-induced) | Idiopathic facial paralysis (presumed viral — HSV-1) + NO visible rash + NO hearing loss + NO vertigo + often after cold exposure + diagnosis of exclusion + favorable prognosis (90–95 % full recovery) | 60–70 % des paralysies faciales périphériques + la plus fréquente |
| Ramsay Hunt syndrome | Auricular vesicular eruption + severe otalgia + possible cranial nerve VIII involvement + VZV + less favorable prognosis than Bell's palsy | 10–15 % of peripheral facial palsies |
| Acute or chronic otitis media | History of otitis + fever + otorrhea + middle ear involvement on otoscopy + facial paralysis due to compression | Rare + complication of untreated otitis |
| Facial nerve tumor (neurinoma + parotid) | Slow, progressive facial paralysis (weeks to months) — facial paralysis developing over more than 3 weeks should suggest a tumor → urgent MRI | Rare + Not to be missed |
| Lyme disease (neuroborreliosis) | Tick exposure history + prior Lyme rash (erythema migrans) + sometimes bilateral facial palsy + Lyme serology + CSF if associated meningitis | Rare in Quebec + risk areas: Estrie + Montérégie + Outaouais |
| Sarcoidosis (Heerfordt's syndrome) | Uveitis + parotiditis + fever + facial paralysis + elevated ACE + chest CT scan (hilar adenopathy) | Rare |
Paraclinical assessment
- Otoscopy essential examination to visualize vesicles in the external auditory canal and on the eardrum + which can be invisible to the naked eye without an instrument + audiogram if hearing loss is suspected
- Brain MRI with gadolinium: Contrast-enhanced MRI of the facial nerve at the geniculate ganglion and internal auditory canal. Rules out tumor causes (VII nerve schwannoma, invasive parotid tumor, cholesteatoma). Not necessary for typical presentations. Indicated for atypical or progressive courses.
- PCR VZV on vesicular swab + VZV serology: Virological confirmation if diagnosis is uncertain + useful if zoster sine herpete (Ramsay Hunt without visible rash — present in 10–15 % of cases)
- Facial EMG Assess the severity of denervation + prognosticate recovery + to perform if complete paralysis persists after 2 to 3 weeks
ℙ️
Zoster sine herpete—a form of Ramsay Hunt syndrome without a visible rash—is present in 10 to 15 % of cases and represents a major diagnostic pitfall. A patient with severe peripheral facial palsy + intense ear pain + cochleovestibular involvement (hearing loss + vertigo) without a rash may nevertheless have Ramsay Hunt syndrome confirmed by VZV PCR on ear swab or VZV seroconversion. Treat as Ramsay Hunt (aciclovir + corticosteroids) if the clinical presentation is suggestive, even without visible vesicles.
Treatment
- Antiviral — acyclovir or valacyclovir (emergency — ideally within 72 hours): valaciclovir 1,000 mg × 3 times/day × 7 days orally (better oral bioavailability than acyclovir) OR IV acyclovir 10 mg/kg every 8 hours for severe forms OR immunosuppression. Mechanism: inhibition of VZV replication → reduction of viral load → limitation of nerve damage. Start even beyond 72 hours if new active lesions OR for severe forms.
- Corticosteroids (prednisone): prednisone 1 mg/kg/day (max 60–80 mg/day) × 5 days then tapering over 5 days + to be systematically combined with antiviral + reduces neural inflammation + improves facial paralysis recovery + reduces pain + the combination of acyclovir + prednisone is superior to each treatment alone for facial recovery
- Eye Protection — PRIORITY if lagophthalmos: lubricant eye drops (artificial tears) every 2 hours during the day + ophthalmic ointment at night + eye occlusion with a moist chamber (goggles) or occlusive eye patch at night + urgent ophthalmological examination if exposure keratitis is suspected (eye pain + photophobia + decreased visual acuity) → risk of neurotrophic keratitis if insensitive cornea + corneal ulcer + blindness if untreated
- Pain relievers for earache: Acetaminophen + NSAIDs + tramadol if insufficient + gabapentin or pregabalin if predominant neuropathic component + postherpetic neuralgia frequent after Ramsay Hunt (more frequent and more severe than classic shingles)
- Facial physiotherapy Massages + facial re-education exercises + electrostimulation + to start as soon as the first signs of motor recovery appear (too soon = counterproductive if the nerve has not recovered) + to continue for 3 to 6 months
- Vertigo Treatment: dimenhydrinate (Gravol®) + prochlorperazine if incapacitating vertigo + vestibular rehabilitation once the acute phase is stabilized
Prognosis and sequelae
- Facial paralysis Full recovery in 70% of cases with early treatment (vs 90–95% for Bell's palsy) + sequelae in 30%: residual paresis + synkinesias (associated involuntary movements—eye closure when smiling) + hemifacial contracture + hemifacial spasm + crocodile tears (tearing during chewing)
- Hearing loss permanent hearing loss in 20-30 % of cases + hearing aids or cochlear implant in severe forms
- Postherpetic neuralgia Persistent neuropathic pain after rash resolution + more frequent and severe in Ramsay Hunt syndrome than in cutaneous shingles + treatment: gabapentin + pregabalin + amitriptyline + lidocaine patch + capsaicin patch
- Poor prognostic factors: age > 60 years + sudden complete facial paralysis + associated cochlear-vestibular compromise + treatment delay > 72 hours + immunodeficiency
Medical emergency — treat within 72 hours
See a doctor urgently—within hours, not days—if intense ear pain plus facial paralysis (deviated mouth, inability to close an eye, facial asymmetry) appear, with or without a visible rash on the outer ear. Ramsay Hunt syndrome is a medical emergency: treatment with valacyclovir + prednisone initiated within the first 72 hours (ideally within 24 hours) doubles or triples the chances of complete facial recovery. Every hour of delay worsens the neurological prognosis. Eye protection (artificial tears + overnight patching) must be initiated immediately if the eye does not close completely, to prevent exposure keratitis. To schedule an appointment, visit cliniqueomicron.ca.
Consult at Clinique Omicron
Clinique Omicron's physicians and nurse practitioners (NPs) diagnose Ramsay Hunt syndrome through clinical examination (otoscope + facial paralysis assessment), immediately initiate treatment with valaciclovir + prednisone, prescribe eye protection and refer to ophthalmology if exposure keratitis is suspected, prescribe an audiogram and refer to ENT for cochleovestibular involvement, and refer to neurology for facial rehabilitation and post-herpetic neuralgia management. Consultations are available at several service points in Quebec and via telemedicine. To book an appointment, visit cliniqueomicron.ca.
The content of this page is provided for informational purposes only and does not replace the advice of a doctor or neurologist. Ramsay Hunt syndrome is a medical emergency for which the prognosis of facial recovery is directly linked to the promptness of antiviral and corticosteroid treatment – any delay in treatment will irreversibly degrade the chances of full recovery.
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