Ramsay Hunt syndrome

01OverviewDefinition, clinical context and the essential points that orientate the chapter.

Ramsay Hunt syndrome is a VZV-associated facial neuropathy, classically combining ipsilateral lower motor neurone facial weakness, otalgia and herpetic vesicles around the ear or within the mouth. Vestibulocochlear involvement can add hearing loss, tinnitus, nausea or vertigo. The clinical picture may evolve over several days, so careful inspection of the ear canal and palate is particularly useful when severe ear pain accompanies a new facial palsy.

The syndrome differs from uncomplicated idiopathic Bell palsy because there is an identifiable viral process, often more severe nerve dysfunction and a greater risk of incomplete recovery. Management must cover infection, inflammation, pain, eye protection and functional consequences. Early treatment is generally favoured, ideally within seventy-two hours of onset, but late presentation is not a reason to dismiss significant ongoing neurological or ocular disease. Discuss the actual presentation with ENT or infection specialists, especially when route selection or immunosuppression complicates treatment.

Key points

Look for painful vesicles in the ear canal, pinna and palate.
VZV can cause facial palsy with hearing loss, tinnitus or vertigo.
The rash may follow weakness, so early absence does not exclude the diagnosis.
Arrange same-day specialist advice and aim to begin appropriate treatment promptly.
Antiviral choice, route and dose depend on complication severity and renal function.
Steroids are commonly combined with antivirals after individual risk assessment.
Protect the eye and arrange facial, auditory and vestibular follow-up.

02AetiologyUnderlying causes, associations and risk factors, with why each one matters.

VZV reactivation

Varicella zoster virus remains latent after earlier infection and may reactivate in cranial sensory ganglia, producing painful vesicles and associated nerve dysfunction.

Host susceptibility

Ageing and impaired cellular immunity can increase zoster risk and complication severity, although Ramsay Hunt syndrome can also occur in an otherwise immunocompetent adult.

03PathophysiologyThe causal sequence from the underlying abnormality to symptoms and harm.

1
Facial nerve inflammation
Inflammatory injury involving the facial nerve impairs ipsilateral upper and lower facial movement, with severity reflecting the degree of motor pathway dysfunction.
2
Adjacent nerve involvement
The nearby vestibulocochlear apparatus may be affected, adding hearing loss, tinnitus and vertigo to the facial motor and cutaneous manifestations.
3
Corneal exposure mechanism
Reduced blink and eyelid closure disturb the tear film and expose the cornea, while associated sensory dysfunction can further compromise protective responses.

04Clinical features and red flagsSymptoms, examination findings, patterns of presentation and time-critical warnings.

Pain and vesicles

Examine the pinna, concha, external auditory canal and oral cavity, including the palate. Vesicles may be subtle or already crusting. Severe neuropathic otalgia can precede the eruption and should prompt repeat examination if the initial diagnosis was Bell palsy.

Whole-face motor weakness

Document forehead movement, blink, eyelid closure, smile and lip seal. Weakness can be marked, with drooling, impaired articulation and inability to retain food. Associated taste disturbance or hyperacusis can reflect other facial nerve functions.

Auditory and vestibular involvement

Ask specifically about sudden hearing loss, tinnitus and vertigo. Hearing change requires prompt audiological and ENT assessment; acute sustained vertigo with central signs still needs a stroke evaluation. A visible rash does not exclude a concurrent or alternative emergency.

Ocular and wider neurological disease

Look for incomplete eye closure, ocular redness, altered vision and facial sensory change. Dysphagia, other cranial neuropathies, meningism or encephalopathy indicate a more complicated presentation and may alter admission, investigation and antiviral route.

Immune and renal context

Identify immunosuppressive medicines, malignancy, transplant history and significant systemic illness. Establish renal function, hydration and ability to take oral treatment. Older age and reduced clearance increase the risk of antiviral accumulation and neurological adverse effects.

Red flags requiring action

Facial palsy with red eye, visual change or incomplete closure needs urgent assessment of corneal and ocular risk.
Multiple cranial nerve deficits, meningism, confusion, disseminated vesicles or significant immunosuppression require hospital assessment and consideration of intravenous antiviral treatment.

05InvestigationsWhat to request, why it matters and how to interpret it.

Investigation order

Read from the initial assessment onwards. Tests may run in parallel in urgent care; first-line, preferred, confirmatory, definitive and gold-standard labels appear only when the chapter explicitly states them.

01
Clinical ear, facial and neurological assessmentFirst step
Why
Establish the syndrome, its extent and the urgency of specialist involvement.
Interpretation and limitations
Record distribution and timing of rash, severity of facial weakness, other cranial nerves, hearing and balance. Typical vesicles support VZV, but an atypical course or absent rash may require broader specialist assessment rather than an unqualified exclusion.
02
Vesicle PCR when available
Why
Support the virological diagnosis when sampling can clarify the cause.
Interpretation and limitations
A swab of vesicle fluid for VZV or related viral testing can be useful, especially with atypical lesions. Arrange treatment on clinical grounds when appropriate rather than waiting for the laboratory result. Testing strategy differs when no accessible lesion is present.
03
Renal function and hydration assessment
Why
Select antiviral dose safely and identify circumstances requiring closer monitoring.
Interpretation and limitations
Use the relevant creatinine-clearance bands for the chosen product, accounting for rapidly changing renal function. Review concurrent nephrotoxic medicines. A standard adult dose can accumulate in an older dehydrated patient even when tablet administration appears uncomplicated.
04
Audiometry and ocular assessment
Why
Document threatened sensory function and guide urgent protective treatment.
Interpretation and limitations
Formal hearing testing provides a baseline, while vision and corneal examination assess exposure or ocular VZV. Do not defer urgent ophthalmology for a red painful or visually impaired eye while waiting for routine audiology.
05
Selective imaging or cerebrospinal fluid investigation
Why
Investigate additional central, meningeal or structural features when present.
Interpretation and limitations
The hospital team chooses imaging and other tests for encephalopathy, meningism, additional focal deficits or an atypical persistent palsy. These are not routine confirmatory tests for every clinically typical limited presentation.

06Differential diagnosisRealistic alternatives and the features that help distinguish them.

Idiopathic Bell palsy

An isolated acute facial palsy without another identified cause may be idiopathic. Severe otalgia, vesicles or additional auditory symptoms should prompt reconsideration.

Complicated ear infection

Otitis, mastoid disease or skull-base infection may produce pain and facial weakness, particularly in vulnerable patients, and requires an infection-specific assessment.

Brainstem disease

Central lesions can produce a complete facial motor pattern with other cranial nerve, eye-movement or limb findings, requiring urgent neurological evaluation.

07ManagementImmediate care, first-line treatment, alternatives and escalation.

01Early careArrange treatment and protect functionFirst stepFacial palsy with otalgia and vesicles raises suspicion of Ramsay Hunt syndrome.

1Discuss the patient with ENT the same day, recording onset, eye closure, hearing, neurological findings and immune status.
2Begin the agreed antiviral plan promptly and consider combined oral corticosteroid treatment after the relevant contraindications and risks have been assessed.
3Initiate lubrication and safe eyelid closure support, with urgent ophthalmology when the cornea is inadequately protected or ocular symptoms are present.
4Provide analgesia appropriate to the person's circumstances and plan review of persistent neuropathic pain rather than assuming rash healing ends the illness.

02Route selectionIdentify complicated zosterNeurological extent, immune status, poor intake or product restrictions make oral therapy uncertain.

1Arrange hospital infection or neurological assessment for disseminated or visceral disease, encephalitis, multiple deficits or significant immunosuppression.
2Recognise that the cited valaciclovir SmPC includes motor neuropathies among complicated-zoster indications for intravenous therapy; do not automatically apply its oral schedule to every facial palsy.
3Although NHS regional pathways describe oral regimens for Ramsay Hunt, confirm the route with the treating specialist and document why the selected plan fits the presentation.
4Reassess deterioration or an inadequate oral response promptly, including hydration, renal function and potential antiviral toxicity.

03Follow-upSupport incomplete neurological recoveryThe acute infection is controlled but facial, hearing or balance impairment remains.

1Arrange follow-up of facial movement and eye protection, explaining that recovery can take months and may be incomplete.
2Coordinate audiology for hearing impairment and vestibular rehabilitation when a stable balance deficit persists after the acute illness.
3Refer troublesome synkinesis, persistent pain or oral functional difficulty to the relevant specialist rehabilitation service.
4Discuss safe return to work and contact precautions while lesions remain active, accounting for vulnerable contacts and healthcare duties.

Key medicines and prescribing safety3 treatments · regimens, roles and cautions

An oral zoster regimen listed for Ramsay Hunt in the NHS Grampian antimicrobial pathway, when the clinical route assessment supports it.

Aciclovir 800 mg oral tablets, product 4336

When the specialist selects oral treatment, give 800 mg five times daily, about four hours apart while awake, for seven days. For zoster, reduce to 800 mg every eight hours at creatinine clearance 10–25 mL/min, or every twelve hours below 10 mL/min.

Check aciclovir or valaciclovir hypersensitivity, renal clearance and hydration. Severe immunosuppression or poor intestinal absorption may require intravenous treatment. Watch for renal injury or new confusion, particularly in older adults and with nephrotoxic medicines; pregnancy or breastfeeding requires an individual prescribing assessment.

A less frequent oral alternative in the regional pathway, subject to specialist confirmation of route and the current product's complicated-zoster precautions.

Valaciclovir oral alternative, product 101527

If an oral alternative is specifically agreed, the immunocompetent zoster regimen is 1 g three times daily for seven days. At creatinine clearance 30–49 mL/min use 1 g twice daily; at 10–29 use 1 g daily; below 10 use 500 mg daily.

The SmPC directs intravenous treatment for complicated zoster including motor neuropathies, so assess this warning explicitly. Maintain hydration, monitor renal function and neurological adverse effects, and review nephrotoxic interactions. Stop immediately for suspected DRESS and never restart after confirmed valaciclovir-related DRESS. Avoid with valaciclovir or aciclovir hypersensitivity; seek individual pregnancy or breastfeeding advice. For intermittent haemodialysis, administer the renal-adjusted dose after dialysis on dialysis days.

An example of a regional combined-treatment approach to inflammatory facial nerve dysfunction; it is not a separately proven universal Ramsay Hunt steroid schedule.

Prednisolone alongside antiviral treatment

The NHS Tayside adult facial-palsy pathway uses prednisolone 1 mg/kg orally once daily, capped at 60 mg, for five days, then reduces the daily dose by 10 mg each day until stopped, with its local gastroprotection plan. The pathway adds an antiviral when Ramsay Hunt is suspected. Agree this regional combined regimen and timing with the treating specialist.

Consider diabetes and glucose monitoring, pregnancy, peptic ulceration, glaucoma, psychiatric vulnerability and active infection. Steroids should not substitute for antiviral treatment in suspected VZV palsy. Account for ongoing corticosteroid exposure and obtain specialist advice in immunocompromised patients.

08ComplicationsImportant consequences, why they occur and why they matter clinically.

Incomplete facial recovery

Persistent weakness, contracture or synkinesis may interfere with eye closure, expression, eating and communication after the acute rash has resolved.

Auditory and balance deficits

Vestibulocochlear injury can leave permanent hearing impairment or chronic disequilibrium, requiring audiological support and a tailored balance rehabilitation programme.

Post-herpetic pain and ocular injury

Persistent neuropathic pain can impair sleep and functioning, while inadequately protected corneal exposure or ocular VZV can threaten vision.

09Monitoring and follow-upTreatment response, safety checks and longer-term review.

Check pain, hydration, oral intake and renal function where relevant during antiviral treatment; confusion may represent disease progression or medicine accumulation and needs reassessment.
Review eyelid closure and the ocular surface until protection is reliable, with clear escalation instructions for redness, pain or reduced vision.
Track facial recovery and hearing separately, arranging repeat audiological review and rehabilitation according to the deficits rather than only rash resolution.
Assess persistent neuropathic pain, sleep, synkinesis and social or occupational impact, offering targeted treatment when these limit recovery.

10Special situationsVariants, exceptions and circumstances that change the usual approach.

Rash timing and diagnostic uncertainty

Vesicles may appear after weakness, and VZV-related neuropathy can occasionally occur without the typical visible eruption. Severe ear pain or evolving auditory symptoms should prompt reassessment and specialist advice rather than repeated reassurance based only on the first skin examination.

Oral regimens are not universal

A convenient tablet schedule does not decide the appropriate route. Neurological complications, immune status and the product warning about motor neuropathy must be considered alongside regional practice. The practical action is early specialist route selection, not delayed treatment while uncertainty persists.

Infectious contact advice

VZV from active shingles lesions can cause chickenpox in a susceptible contact. Keep lesions clean, use suitable non-adherent coverage where possible and avoid exposing susceptible pregnant people, significantly immunocompromised people and newborns. Healthcare workers should seek occupational advice about active uncovered lesions.

Functional rehabilitation

Facial therapy and vestibular rehabilitation address different deficits. Exercises should be selected after assessment rather than treating all residual weakness, dizziness and discomfort as one problem. Hearing aids or other communication support may remain useful even when facial movement improves.

11Common pitfallsFrequent interpretation and management errors.

01
Calling the palsy idiopathic without inspecting the ear canal and palate can delay recognition of VZV disease.
02
Using a genital-herpes antiviral dose for cranial zoster under-treats the intended infection and ignores indication-specific dosing.
03
Prescribing an unadjusted antiviral schedule in renal impairment risks accumulation, renal injury and neurological toxicity.
04
Stopping follow-up when vesicles crust overlooks persisting corneal exposure, hearing loss, balance dysfunction and pain.