Ramsay Hunt syndrome (also known as herpes zoster oticus or auricular herpes zoster) is a rare neurologic condition characterized by reactivation of the varicella zoster virus involving the facial nerve, and less commonly other cranial nerves (e.g., V, IX, and X).[1] Patients often present with a triad of symptoms: sudden-onset (<72 hours) ipsilateral peripheral facial palsy, severe ear/facial pain, and vesicular ear rash, including blisters.[1] However, many patients do not present with all three classic features as these do not always develop at the same time; vesicles can precede, coexist with, or follow facial palsy. The most suggestive symptom is unilateral facial palsy. Reactivation of varicella zoster, including Ramsay Hunt syndrome, can occur at any age but is most common in people ages >50 years.[1] The condition is a diagnosis of exclusion. Patients typically see improvement in facial movement within a few weeks to months of symptom onset. However, changes in facial symmetry and function can continue for up to a year before a patient's exam stabilizes. Recurrent Ramsay Hunt syndrome is exceedingly rare.[2]
Highlights & Basics
Key Highlights
- Ramsay Hunt syndrome typically presents with sudden-onset (<72 hours) unilateral peripheral facial palsy, severe ear/facial pain, and a vesicular ear rash. Other presenting symptoms include vertigo, hearing loss, tinnitus, dry eye, altered taste, and oral lesions.
- It is caused by reactivation of latent varicella zoster virus (VZV) and spread to the facial nerve. Previous exposure to VZV, age >50 years, and immunosuppression are key risk factors.
- Diagnosis is typically clinical. Other serious causes of facial paralysis such as cerebrovascular accidents and tumors must be ruled out. If there is uncertainty regarding etiology, the vesicular lesions, if present, can be swabbed directly for confirmation by VZV polymerase chain reaction.
- Prompt combination treatment (within 72 hours) with high-dose oral corticosteroids and antivirals is recommended in all patients. Patients with ocular symptoms should be given eye lubricants. In patients who can't close their eyes, taping the eye shut is recommended to protect the cornea at night.
- The majority of patients will recover their facial movement at least partially with treatment.
Quick Reference
History & Exam
Key Factors
sudden-onset (<72 hours) unilateral facial weakness
ipsilateral severe ear/facial pain
ipsilateral vesicular rash
absence of constitutional symptoms
Other Factors
dry eye
vertigo
hearing loss
tinnitus
epiphora
altered taste
oral lesions
keratitis
Diagnostics Tests
1st Tests to Order
clinical diagnosis
varicella zoster virus (VZV) polymerase chain reaction (PCR)
Other Tests to consider
electroneurography (evoked electromyography)
MRI head and neck with contrast
serology for Borrelia burgdorferi
Treatment Options
acute
acute symptoms
oral corticosteroid
antiviral
eye protection
ongoing
chronic symptoms
referral to specialist
Definition
Vignette
Common Vignette
A 67-year-old woman presents with right-sided severe ear pain, room-spinning dizziness, and a painful rash on her right ear. She says that the ear pain and dizziness started the night before, and in the morning on awakening water spilled out of the right corner of her mouth when brushing her teeth. She notes that she could not move the right side of her face when looking in the mirror. She presents to the emergency department because she thinks she is having a stroke. She reports no prior history of facial paralysis. She is otherwise healthy. On physical exam, she has a complete right-sided facial paralysis. There are a few vesicular lesions along her concha and helical rim, and her pinna is diffusely tender to the touch. Otoscopy is unremarkable except for a few vesicular lesions along the floor of the cartilaginous ear canal.
Epidemiology
The incidence of Ramsay Hunt syndrome is approximately 5 per 100,000 people per year.[3] This is in comparison to Bell's palsy, which has an incidence around 3 to 6 times greater at 15 to 30 per 100,000 people per year.[4] It is estimated that 3% to 18% of cases of peripheral facial paralysis are caused by Ramsay Hunt syndrome.[2]
Reactivation of varicella zoster, including Ramsay Hunt syndrome, can occur at any age but is most common in people age >50 years.[1] [5] [6]
The syndrome can affect people of any age, but it is more common in people ages >50 years, and it is extremely rare in children.[5]
Etiology
Ramsay Hunt syndrome is caused by reactivation of latent varicella zoster virus (VZV), which is the same virus that causes chickenpox and shingles, and spreads to the facial nerve. VZV may remain dormant for decades in the geniculate ganglion (cranial nerve VII) or sensory ganglion of the face (cranial nerve V) following primary infection.[7] As adaptive immunity wanes, with aging or in times of physiological stress or immunocompromised state, the virus can be reactivated, causing facial paralysis, facial pain, and vesicular lesions in a dermatomal pattern.[1] [6] [8] Although it has been reported that people without prior VZV vaccination or known infection have been diagnosed with Ramsay Hunt syndrome, this is uncommon (0.5% to 1.0% of patients with herpes zoster reported no prior vaccination or known infection in one small population-based study of people ages <20 years).[9]
Pathophysiology
The pathophysiology underlying Ramsay Hunt syndrome is believed to be due to reactivation of VZV following earlier primary infection or VZV vaccination leading to inflammation and compression of the facial nerve, especially in the labyrinthine segment. This results in loss of axonal transmission and decreased nerve signal to the muscles of facial expression and sudden-onset facial palsy.[2] [10] During primary VZV infection, the virus is carried down the axons to the areas of the skin innervated by the affected ganglion, causing local inflammation.
Images
Functional anatomy of the facial nerve. Proximally, the four cranial nerve nuclei involved in facial nerve functions are shown at the pontomedullary junction: the motor nucleus of VII, the nucleus of the solitary tract, the superior salivatory nucleus, and the spinal nucleus of V. Special visceral efferent motor fibers from the motor nucleus of VII (solid red line) exit the brainstem and travel through the internal acoustic meatus to enter the bony facial canal and exit through the stylomastoid foramen to supply facial muscles. In Ramsay Hunt syndrome, these fibers are affected as they pass through the geniculate ganglion, disrupting motor functions of the seventh cranial nerve. The solitary tract receives special visceral afferent taste fibers (solid blue line) emanating from the anterior two thirds of the tongue. These fibers travel with the chorda tympani through the petrotympanic fissure (not shown). The cell bodies of these special visceral afferent fibers are in the geniculate ganglion which is the site of varicella zoster virus (VZV) reactivation when vesicles erupt on the tongue. The fibers reach the brainstem via the nervus intermedius and can be affected by local inflammation as they pass the geniculate ganglion. Special visceral efferent parasympathetic fibers (thin dotted red line) to the lacrimal and salivary glands emanate from the superior salivatory nucleus, travel in the nervus intermedius, and branch at the geniculate ganglion into the greater petrosal and chorda tympani nerves. Decreased lacrimation may result from involvement of these fibers as they branch at the level of the geniculate ganglion. Special visceral efferent sympathetic fibers (thick dotted red line) emanate from the carotid plexus on the internal carotid artery and join the greater petrosal nerve as these structures pass through the foramen lacerum (not shown). The sympathetic fibers parallel the parasympathetic fibers as they supply the same areas. The spinal nucleus of V receives general somatic afferent fibers from the geniculate zone of the ear via the chorda tympani. Cell bodies of these neurons are located in the geniculate ganglia and are the site of VZV reactivation in classic Ramsay Hunt syndrome causing vesicular eruptions in geniculate zones
Right facial palsy in a man with Ramsay Hunt syndrome. Note the inability to close the right eye (A) and significant smile asymmetry (B), both frequently seen in acute Ramsay Hunt syndrome
Painful vesicular rash in ear in a patient with Ramsay Hunt syndrome
External acoustic meatus with multiple vesicular lesions in a patient with Ramsay Hunt syndrome
A) Vesicular rash on the tongue and B) the palate in a patient with Ramsay Hunt syndrome
Patient with acute Ramsay Hunt syndrome (A and C) that progressed to chronic Ramsay Hunt syndrome (B and D). Photos B and D were taken 14 months after symptom onset. Note the patient's inability to close right eye (A) and significant smile asymmetry (C), which are both frequently seen in acute Ramsay Hunt syndrome. The patient recovered, but had some signs of aberrant facial nerve regeneration; specifically, note right ocular-oral synkinesis (B) and improved but persistent smile asymmetry (D)
Diagnostic Approach
Ramsay Hunt syndrome involves a reactivation of the varicella zoster virus (VZV) along the course of the facial nerve, leading to sudden-onset (<72 hours) unilateral facial paralysis, severe ear/facial pain, and a vesicular rash, including blisters, involving the ear.[1] [6] [13] Additionally, the patient may have associated otologic symptoms, including vertigo, tinnitus, and hearing loss.[1] [10] Ramsay Hunt syndrome is a diagnosis of exclusion: consider other causes of sudden-onset facial paralysis, such as Bell's palsy, cerebrovascular accident, and Lyme disease, when evaluating patients.
The majority of patients presenting with Ramsay Hunt syndrome will recover their facial movement at least partially. Patients typically see improvement in facial movement within a few weeks to months of symptom onset. However, changes in facial symmetry and function can continue for up to a year before a patient's exam stabilizes. Recurrent Ramsay Hunt syndrome is exceedingly rare.[2]
History
- Sudden-onset (<72 hours) unilateral peripheral facial weakness (partial or complete, present in approximately 90% of patients)
- Severe ear/facial pain
- Vesicular lesions involving the pinna (present in approximately 41% of patients).
However, note that many patients do not present with all three classic symptoms as these do not always develop at the same time; vesicles can precede, coexist with, or follow facial palsy. The most suggestive symptom of Ramsay Hunt syndrome is unilateral peripheral facial palsy. Other presenting symptoms are vertigo, hearing loss, tinnitus, epiphora, altered taste, and oral lesions.[1] [10]
Take a careful history. Ask about the following symptoms and features:
- Onset and progression of the palsy
- Otologic symptoms (hearing loss, tinnitus, vertigo) or prior otologic surgery
- Changes in the sense of taste, oral lesions
- Dry eye.
Consider differential diagnoses such as Lyme disease if the patient reports presence of fever, general malaise, myalgia, erythema migrans (i.e., bull's eye) rash, recalled tick bite, or recent travel to a Lyme disease-endemic region. Consider diagnoses such as facial nerve tumor if any of the following red flags are present in the history:
- Gradual onset (>72 hours) facial weakness
- Prior history of ipsilateral facial paralysis.
Also ask about risk factors for Ramsay Hunt syndrome:
- Prior exposure to VZV (chickenpox, shingles), particularly in patients ages >50 years
- Vaccination status - note that VZV vaccination was not widely available until the 1990s
- Immunosuppression - people who are immunosuppressed are more susceptible to VZV infection
- Physiological stress - a known risk factor for viral reactivation.[11]
Physical exam
Perform a focused neurologic exam, a complete head and neck exam, and a detailed cranial nerve exam.
- Ensure that parotid and/or neck masses are not present.
- Document any cranial neuropathies, most notably dermatomal rash, facial weakness, and ocular findings. Sparing of brow function (i.e., ability to raise the eyebrow on the affected side) indicates an upper motor neuron lesion, as the dorsal division of the facial motor nucleus receives bilateral supranuclear efferent input.
- Neural insult in Bell's palsy occurs at the meatal foramen deep within the temporal bone; consequently all branches of the facial nerve are affected.
- Uneven distribution of weakness across facial zones in the acute phase is highly suggestive of a tumor in the parotid or elsewhere along the course of the facial nerve and should prompt imaging studies.
- Examination of the parotid gland includes palpation and visual examination of the oropharynx to rule out a deep lobe parotid tumor, which may displace the tonsil medially.
Perform a thorough visual and otoscopic evaluation to assess involvement of the external ear and ear canal.
- The presence of vesicles, including blisters, in the external ear and auditory canal is highly suggestive of VZV reactivation in Ramsay Hunt syndrome. The vesicles may be seen on the external ear alone (around 41% of patients) or on both the external ear and external ear canal (around 25% of patients).[13] They may be associated with pain affecting the outer portion of the pinna and outer third of the external ear canal.[6] Some patients may present solely with redness of the skin in the ear canal and ear drum. Diffuse swelling of the soft tissue in the external ear canal may be present.
Examine the oral cavity, looking for vesicular eruptions on the cheek, tongue, and palate.
- Vesicles, including blisters, are most commonly seen on the ear but may also be present on the above-mentioned areas.[6]
Perform a thorough ophthalmic evaluation, including slit-lamp exam.[14]
- Patients with ocular symptoms, especially if there is significant involvement in the V1 and V2 dermatomes in addition to facial weakness, are at risk for corneal injury. Keratitis may be present.
- Some patients may present with a dry eye, due to the inability to close the eye.[6]
European expert consensus guidelines recommend referring the patient to an ear, nose, and throat (ENT) specialist and a neurologist if there is involvement of the facial nerve and/or vestibulocochlear nerve, ear pain, and vertigo.[1] This allows for ENT and neurologic follow-up and for treatment to be adjusted accordingly.[1]
Consider other diagnoses if any of the following red flags are present in the physical exam:
- Uneven distribution of facial weakness across facial zones (e.g., preserved eyebrow movement suggests central paralysis from a cerebrovascular accident [CVA] or other causes)
- Bilateral facial weakness
- Presence of other cranial or peripheral neuropathies
- Persistence of complete flaccid paralysis at 4 months from onset.
Initial investigations
The diagnosis is typically clinical and no confirmatory tests are required. However, if there is uncertainty regarding etiology, the vesicular lesions, if present, can be swabbed directly for confirmation by polymerase chain reaction (PCR). A VZV DNA PCR has nearly 100% sensitivity and specificity.[1]
Other investigations
Consider electroneurography (ENoG), also known as evoked electromyography, in acute patients who present with near-complete or complete facial paralysis (i.e., House-Brackmann scale V or VI) to quantify the degree of neural degeneration.[15]
Request magnetic resonance imaging (MRI) of the head and neck in patients presenting with unilateral facial palsy and any red flag sign/symptom to rule out CVA and malignancy. Red flags include gradual onset weakness, other cranial or peripheral neuropathies, uneven distribution of weakness across facial zones, and prior history of ipsilateral facial weakness. CVA is the most important cause of facial paralysis that needs to be identified rapidly so treatment can be initiated. See Overview of stroke .
If the patient has been exposed to ticks or is in or has recently traveled to a Lyme-prevalent region, order Lyme serology (enzyme-linked immunosorbent assay [ELISA] and Western blot). This is especially indicated if they present with suggestive symptoms such as fever, myalgia, and erythema migrans (i.e., bull's eye rash).[16] See Lyme disease .
Risk Factors
History & Exam
- However, patients do not always present with the classic triad of unilateral facial paralysis, severe ear/facial pain, and vesicular ear rash. Consider other causes of sudden-onset facial paralysis such as Bell's palsy, cerebrovascular accident, and Lyme disease when evaluating these patients.
- The presence of vesicles in the external ear and auditory canal is highly suggestive of varicella zoster virus (VZV) reactivation in Ramsay Hunt syndrome. The vesicles, including blisters, can precede, coexist with, or follow facial palsy. The vesicles may be seen on the external ear alone (around 41% of patients) or on both the external ear and external ear canal (around 25% of patients).[13] They may be associated with pain affecting the outer portion of the pinna and outer third of the external ear canal.[6] Some patients may present solely with redness of the skin in the ear canal and ear drum.
- However, patients do not always present with the classic triad of facial paralysis, severe ear/facial pain, and vesicular ear rash. Consider other causes of sudden-onset facial paralysis such as Bell's palsy, cerebrovascular accident, and Lyme disease when evaluating these patients.
Tests
Differential Diagnosis
Bell's palsy
Differentiating Signs/Symptoms
- Most common cause of sudden-onset unilateral facial palsy.
- Absence of vesicular ear rash, vertigo, or hearing loss.
- Pain in Bell's palsy is typically less severe than that in Ramsay Hunt syndrome.
Differentiating Tests
- Clinical diagnosis.
Differentiating Signs/Symptoms
- Gradual onset facial paralysis.
- Prior history of cutaneous squamous cell carcinoma of ipsilateral face/scalp.
Differentiating Tests
- MRI head and neck with contrast: malignancy in the facial nerve course.
- CT temporal bone with or without contrast: widened canal suggests facial schwannoma.
Benign facial nerve tumor (e.g., facial nerve schwannoma)
Differentiating Signs/Symptoms
- Waxing and waning or slowly progressive facial palsy.
- May demonstrate uneven distribution of weakness across facial zones, with elements of synkinesis and fasciculations.
Differentiating Tests
- Contrast-enhanced MRI of the course of the facial nerve, with fine-cut CT of the temporal bones: mass lesion.
Cerebrovascular accident
Differentiating Signs/Symptoms
- Presence of central nervous system deficits, such as unilateral weakness or sensory changes, aphasia, ataxia.
Differentiating Tests
- MRI or CT of the head shows evidence of cerebral infarct or hemorrhage.
Differentiating Signs/Symptoms
- Recent history of blunt force trauma to the cranium (i.e., temporal bone fracture) or penetrating trauma involving the course of the facial nerve.
Differentiating Tests
- CT head (CT temporal bone preferred): transverse temporal bone fracture, often involving the otic capsule.
Differentiating Signs/Symptoms
- Classical presentation is a triad of recurrent unilateral facial swelling, facial palsy, and fissured tongue (lingua plicata).[18]
Differentiating Tests
- Clinical diagnosis.
Malignant otitis externa
Differentiating Signs/Symptoms
- Uncontrolled diabetes or underlying immunosuppression.
- History of ear pain, chronic ear drainage, and other cranial nerve deficits.
Differentiating Tests
- Physical exam: granulation tissue at the bony cartilaginous junction in the ear canal.
- Nuclear bone scan (MRI with Technetium-99 radiotracer): enhancement along the lateral skull base.
Guillain-Barre syndrome
Differentiating Signs/Symptoms
- Typically bilateral presentation.
- Involvement of upper and lower extremities, usually an ascending paralysis from the toes and fingers moving toward the torso.
- May have facial involvement usually as later sequela.
Differentiating Tests
- Lumbar puncture: elevated cerebrospinal fluid protein.
- Electroneurography (ENoG): delayed nerve conduction across involved structures.
Cholesteatoma
Differentiating Signs/Symptoms
- History of recurrent ear infections and ear discharge. Remote history of ear trauma or prior ear surgery.
- Vertigo is associated with labyrinthine fistula.
Differentiating Tests
- CT scan of the petrous temporal bones: soft tissue density in the middle ear and possible ossicular erosion. May show dehiscent facial nerve in tympanic or mastoid segment, with or without erosion of the horizontal semicircular canal and resultant labyrinthine fistula.
Complicated otitis media
Differentiating Signs/Symptoms
- On otoscopy there is perforation of the pars tensa but no evidence of cholesteatoma.
Differentiating Tests
- Diagnosis is clinical.
Lyme disease
Differentiating Signs/Symptoms
- Skin rash (erythema migrans or other), frontal headache, fever, malaise, fatigue, myalgia, arthralgia, known tick exposure, or recent travel to Lyme disease-endemic region.
Differentiating Tests
- Elevated immunoglobulins (IgM and/or IgG) to Borrelia burgdorferi by enzyme-linked immunosorbent assay (ELISA) or indirect fluorescent antibody titers are demonstrated.
- Western blot is then performed for confirmation.
Criteria
House-Brackmann scale[19]
The House-Brackmann scale is a grading tool developed by two neuro-otologists to assist with facial nerve evaluation after lateral skull base surgery.[19] The scale is used widely to determine the severity of facial paralysis; however, it does not account for different degrees of severity between regions of the face, and does not adequately assess changes in facial symmetry after interventions.[20]
Classification using the scale is as follows:[19]
- Grade I = normal
- Grade II = slight weakness/asymmetry
- Grade III = obvious weakness with movement but absence of disfigurement at rest; intact ability to close the eye
- Grade IV = obvious weakness with movement and disfigurement at rest; inability to fully close the eye
- Grade V = barely perceptible movement
- Grade VI = no movement.
Sunnybrook facial grading system[21]
The Sunnybrook facial grading system is slightly more nuanced grading tool than the House-Brackmann scale and is preferred by facial palsy researchers.[21] [22] [23] It consists of ordinal-scale scoring of multiple parameters within three domains:[21]
- Resting symmetry (eye, nasolabial fold, and mouth)
- Symmetry of voluntary movement (brow elevation, gentle eye closure, open mouth smile, snarl, and lip pucker)
- Synkinesis (same parameters as voluntary movement).
Sunnybrook facial grading system domain scores are combined to form a weighted composite score from 0 (complete flaccid paralysis) to 100 (normal function).[21] While the complexity of the grading scale typically requires use of a paper or digital form for scoring and documentation, its use permits tracking of clinical recovery with higher precision over time, making it better suited for research use compared with the House-Brackmann scale.[21]
Electronic facial paralysis assessment (eFACE)[24]
The eFACE is a grading system that allows for comparative scoring across time, and for high fidelity analysis by incorporating variation in paralysis between regions of the face.[24]
It consists of clinician-graded continuous visual analog scales within three domains:[24]
- Static symmetry (resting brow, resting palpebral fissure width, resting nasolabial fold depth, oral commissure resting position)
- Dynamic symmetry (brow elevation, gentle and full eye closure, nasolabial fold depth and orientation with smile, oral commissure excursion with smile, lower lip movement with "eeeee" [the photographer will ask the person to say "eeeee" when capturing movement of the lower lip])
- Synkinesis (ocular, midfacial, mentalis, platysmal).
The tool is available as a downloadable application with a graphical user interface that allows for touch-screen scoring and, when linked to a database, immediate graphical comparison to previous scores. This grading tool has also been validated for use with standardized patient photos and videos.[25] [26] One systematic review concluded that eFACE was the most comprehensive tool available for the assessment of blink function.[27]
Treatment Approach
- Provide immediate pain and inflammation relief
- Provide eye protection to prevent corneal damage in patients unable to close their eye on the affected side
- Minimize the severity of chronic facial palsy
The majority of patients presenting with Ramsay Hunt syndrome will recover their facial movement at least partially with treatment. Patients typically see improvement in facial movement within a few weeks to months of symptom onset. However, changes in facial symmetry and function can continue for up to a year before a patient's exam stabilizes. Recurrent Ramsay Hunt syndrome is exceedingly rare.[2]
Management of acute phase
Corticosteroids and antivirals
Give combination therapy with high-dose oral corticosteroids and high-dose antivirals early (i.e., <72 hours from symptom onset) to all patients with Ramsay Hunt syndrome.[1] [29] [30]
This recommendation is based on the efficacy of corticosteroid treatment shown in studies in patients with Bell's palsy, which is based on reduction of inflammatory edema.[1] [29] [30] Expert consensus guidelines consider that corticosteroids are still the best treatment option for viral inflammation of the facial nerve; however it is not clear how corticosteroids work on patients with Ramsay Hunt syndrome.[1] The recommendation to use antiviral treatment is based on its demonstrated efficacy in patients with herpes zoster. Antivirals are believed to reduce acute pain, improve herpes zoster lesions, and reduce the risk of postherpetic neuralgia.[31]
Data in patients with Ramsay Hunt syndrome are of low quality and have shown mixed efficacy overall.
- One retrospective case review (n=128) of patients with Ramsay Hunt syndrome with complete facial paralysis (House-Brackmann scale VI) showed highest rates of recovery in patients receiving early administration of high-dose corticosteroid and antiviral treatment, compared with normal-dose corticosteroid and antiviral treatment, or with high-dose corticosteroid alone (71%, 60%, and 57%, respectively). However, the results were not statistically significant.[32]
- One small study (n=91) evaluating a combination of corticosteroids plus antiviral treatment showed higher rates of good nerve excitability (a promising sign of nerve function) in patients with Ramsay Hunt syndrome receiving combination treatment than in those receiving corticosteroids alone (75% and 53%, respectively).[33]
- One Cochrane review found one randomized controlled trial (n=15) comparing intravenous acyclovir and corticosteroids with corticosteroids alone in patients with Ramsay Hunt syndrome and found no statistically significant difference between the two groups.[34]
Despite the lack of randomized prospective studies, collective data from retrospective studies show that patients with Ramsay Hunt syndrome treated with corticosteroids and antivirals have better recovery rates than those receiving no medication.
General warnings and cautions concerning short-course corticosteroids should be followed. Temporary or permanent adverse effects can occur with high doses (e.g., blurry vision, weight gain, Cushingoid appearance, altered glucose metabolism, dyslipidemia, hypertension, cataracts, glaucoma, osteoporosis, diabetes). Monitor patients closely for adverse effects. Consider adding a proton-pump inhibitor for gastric protection when prescribing high-dose corticosteroids.
Assess kidney function in patients on high-dose antiviral therapy. Use caution in patients with renal impairment; a dose adjustment may be necessary.
Eye protection
Keratoconjunctivitis sicca (dry eye) is common and may lead to exposure keratopathy. For patients with facial paralysis and symptomatic eye irritation, give moisture-based therapy (e.g., preservative-free methylcellulose ophthalmic drops) and a lubricant eye ointment. Additionally, advise patients with eye irritation when waking up in the morning to tape the eye shut using non-irritant tape at night after ointment has been applied to prevent corneal damage.[28] [35] Eye patches are contraindicated because the eye may easily open under the patch, leading to corneal abrasion.
Surgical decompression
The use of surgical decompression in patients with acute Ramsay Hunt syndrome and complete paralysis who do not show any signs of recovery after treatment is controversial. Neural decompression is rarely employed as there is significant potential morbidity with this procedure. There are limited data supporting surgery over optimal medical management and expectant management.[36] [37]
Management of chronic phase
Facial reanimation
Many patients will develop chronic Ramsay Hunt syndrome, despite prompt treatment, with chronic facial paralysis manifested by facial asymmetry, facial tightness, and facial synkinesis (i.e., nonflaccid facial paralysis, postparalytic facial paralysis).[38] Long-term treatment for these patients is individualized and highly specialized. Refer the patient to a specialist in facial reanimation to discuss both surgical and nonsurgical options. Chronic facial paralysis can be a significant source of depression, anxiety, and social withdrawal for patients, and close monitoring is warranted.
Postherpetic neuralgia (defined as pain that persists for >3 months after the cutaneous herpes zoster lesions have resolved) is rare in patients with Ramsay Hunt syndrome. Refer affected patients to a pain specialist.
Treatment Options
acute symptoms
oral corticosteroid
Primary Options
- prednisone
1 mg/kg/day orally for at least 7 days, taper gradually over 5-7 days, maximum 60 mg/day
- prednisone
Comments
- This recommendation is based on the efficacy of corticosteroid treatment shown in studies in patients with Bell's palsy, which is based on reduction of inflammatory edema.[1] [29] [30] Expert consensus guidelines consider that corticosteroids are still the best treatment option for viral inflammation of the facial nerve; however, it is not clear how corticosteroids work on patients with Ramsay Hunt syndrome.[1] The recommendation to use antiviral treatment is based on its demonstrated efficacy in patients with herpes zoster. Antivirals are believed to reduce acute pain, improve herpes zoster lesions, and reduce the risk of postherpetic neuralgia.[31]
- Data in patients with Ramsay Hunt syndrome are of low quality and have shown mixed efficacy overall.
- One retrospective case review (n=128) of patients with Ramsay Hunt syndrome with complete facial paralysis (House-Brackmann scale VI) showed highest rates of recovery in patients receiving early administration of high-dose corticosteroid and antiviral treatment, compared with normal-dose corticosteroid and antiviral treatment, or with high-dose corticosteroid alone (71%, 60%, and 57%, respectively). However, the results were not statistically significant.[32]
- One small study (n=91) evaluating a combination of corticosteroids plus antiviral treatment showed higher rates of good nerve excitability (a promising sign of nerve function) in patients with Ramsay Hunt syndrome receiving combination treatment than in those receiving corticosteroids alone (75% and 53%, respectively).[33]
- One Cochrane review found one randomized controlled trial (n=15) comparing intravenous acyclovir and corticosteroids with corticosteroids alone in patients with Ramsay Hunt syndrome and found no statistically significant difference between the two groups.[34]
- Despite the lack of randomized prospective studies, collective data from retrospective studies show that patients with Ramsay Hunt syndrome treated with corticosteroids and antivirals have better recovery rates than those receiving no medication.
- General warnings and cautions concerning short-course corticosteroids should be followed. Temporary or permanent adverse effects can occur with high doses (e.g., blurry vision, weight gain, Cushingoid appearance, altered glucose metabolism, dyslipidemia, hypertension, cataracts, glaucoma, osteoporosis, diabetes). Monitor patients closely for adverse effects. Consider adding a proton-pump inhibitor for gastric protection when prescribing high-dose corticosteroids.
antiviral
Primary Options
- valacyclovir
1000 mg orally three times daily for 7 days
- valacyclovir
- acyclovir
800 mg orally five times daily for 7 days
- acyclovir
Comments
- This recommendation is based on the efficacy of corticosteroid treatment shown in studies in patients with Bell's palsy, which is based on reduction of inflammatory edema.[1] [29] [30] Expert consensus guidelines consider that corticosteroids are still the best treatment option for viral inflammation of the facial nerve; however, it is not clear how corticosteroids work on patients with Ramsay Hunt syndrome.[1] The recommendation to use antiviral treatment is based on its demonstrated efficacy in patients with herpes zoster. Antivirals are believed to reduce acute pain, improve herpes zoster lesions, and reduce the risk of postherpetic neuralgia.[31]
- Data in patients with Ramsay Hunt syndrome are of low quality and have shown mixed efficacy overall.
- One retrospective case review (n=128) of patients with Ramsay Hunt syndrome with complete facial paralysis (House-Brackmann scale VI) showed highest rates of recovery in patients receiving early administration of high-dose corticosteroid and antiviral treatment, compared with normal-dose corticosteroid and antiviral treatment, or with high-dose corticosteroid alone (71%, 60%, and 57%, respectively). However, the results were not statistically significant.[32]
- One small study (n=91) evaluating a combination of corticosteroids plus antiviral treatment showed higher rates of good nerve excitability (a promising sign of nerve function) in patients with Ramsay Hunt syndrome receiving combination treatment than in those receiving corticosteroids alone (75% and 53%, respectively).[33]
- One Cochrane review found one randomized controlled trial (n=15) comparing intravenous acyclovir and corticosteroids with corticosteroids alone in patients with Ramsay Hunt syndrome and found no statistically significant difference between the two groups.[34]
- Despite the lack of randomized prospective studies, collective data from retrospective studies show that patients with Ramsay Hunt syndrome treated with corticosteroids and antivirals have better recovery rates than those patients receiving no medication.
- Assess kidney function in patients on high-dose antiviral therapy. Use caution in patients with renal impairment; a dose adjustment may be necessary.
eye protection
Comments
- Keratoconjunctivitis sicca (dry eye) is common and may lead to exposure keratopathy. For patients with facial paralysis and symptomatic eye irritation, give moisture-based therapy (e.g., preservative-free methylcellulose ophthalmic drops) and a lubricant eye ointment.
- Advise patients with eye irritation when waking up in the morning to tape the eye shut at night after ointment has been applied to prevent corneal damage.[28] Eye patches are contraindicated because the eye may easily open under the patch, leading to corneal abrasion.
chronic symptoms
referral to specialist
Comments
- Many patients will develop chronic Ramsay Hunt syndrome, despite prompt treatment, with chronic facial paralysis manifested by facial asymmetry, facial tightness, and facial synkinesis (i.e., nonflaccid facial paralysis, postparalytic facial paralysis).[38] Long-term treatment for these patients is individualized and highly specialized.
- Refer the patient to a specialist in facial reanimation to discuss both surgical and nonsurgical options. Chronic facial paralysis can be a significant source of depression, anxiety, and social withdrawal for patients, and close monitoring is warranted.
- Postherpetic neuralgia (defined as pain that persists for >3 months after the cutaneous herpes zoster lesions have resolved) is rare in patients with Ramsay Hunt syndrome. Refer affected patients to a pain specialist.
Follow-Up Overview
Prognosis
The majority of patients presenting with Ramsay Hunt syndrome will recover their facial movement at least partially. Patients typically see improvement in facial movement within a few weeks to months of symptom onset. However, changes in facial symmetry and function can continue for up to a year before a patient's exam stabilizes. Recurrent Ramsay Hunt syndrome is exceedingly rare.[2]
In general, patients with partial paresis have a better recovery than those with complete paralysis. In one study, 93% to 98% of patients with Bell's palsy presenting with partial paresis had a spontaneous complete recovery, whereas those with complete paralysis, ages >60 years, immunocompromised, or pregnant, had a poorer prognosis.[39] Studies have shown that Ramsay Hunt syndrome has a less favorable recovery pathway than Bell's palsy.[39][40]
Citations
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Jeon Y, Lee H. Ramsay Hunt syndrome. J Dent Anesth Pain Med. 2018 Dec;18(6):333-7.[Abstract][Full Text]
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Key Articles
Referenced Articles
Guidelines
Diagnostic
Summary
Includes an overview of the clinical and molecular diagnostic workup, special diagnostic situations and complicated disease courses, and recommendations on the treatment of Ramsay Hunt syndrome.Published by
German Herpes Management Forum
Published
2020
Treatment
Summary
Includes an overview of the clinical and molecular diagnostic workup, special diagnostic situations and complicated disease courses, and recommendations on the treatment of Ramsay Hunt syndrome.Published by
German Herpes Management Forum
Published
2020