Ramsay Hunt Syndrome

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Practice Essentials

Ramsay Hunt syndrome, also termed geniculate neuralgia or nervus intermedius neuralgia, is a rare neurologic condition predominantly observed in adults older than 60 years.[1]  It is characterized by acute peripheral facial neuropathy, presenting with unilateral facial weakness or paralysis (facial palsy) and an erythematous vesicular rash affecting the external auditory canal, auricle (herpes zoster oticus), or the mucous membranes of the oropharynx. Additional clinical manifestations may include tinnitus and hearing loss.

The etiology of Ramsay Hunt syndrome is linked to the reactivation of the varicella zoster virus (VZV), which is also the causative agent of chickenpox in children and shingles (herpes zoster) in adults. Occasionally, Ramsay Hunt syndrome may present without the characteristic skin rash, a condition known as zoster sine herpete.[2]



View Image

Herpes zoster oticus, day 6. Image courtesy of Manolette Roque, MD, ROQUE Eye Clinic.

Signs and symptoms

Patients usually present with paroxysmal pain deep within the ear. The pain often radiates outward into the pinna of the ear and may be associated with a more constant, diffuse, and dull background pain. The onset of pain usually precedes the rash by several hours and even days.

Classic Ramsay Hunt syndrome can be associated with the following:

Diagnosis

The diagnosis of Ramsay Hunt syndrome is usually made without difficulty when the clinical characteristics are present. If necessary, varicella-zoster virus (VZV) may be isolated from vesicle fluid and inoculated into susceptible human or monkey cells for identification by serologic means.

WBC count, erythrocyte sedimentation rate (ESR), and serum electrolytes are helpful in distinguishing the infectious and inflammatory nature of this syndrome.

Management

Treatment goals of Ramsay Hunt syndrome are to minimize disability and relieve symptoms. Clinical evidence shows that earlier start of treatment is correlated with better outcomes.[1]

Oral corticosteroids and oral acyclovir are commonly used in the treatment of Ramsay Hunt syndrome. 

After initiation of medical therapy, the patient with Ramsay Hunt syndrome should be seen in follow-up at 2 weeks, 6 weeks, and 3 months.

Background

Ramsay Hunt syndrome is defined as an acute peripheral facial neuropathy associated with erythematous vesicular rash of the skin of the ear canal, auricle (also termed herpes zoster oticus), and/or mucous membrane of the oropharynx.



View Image

Herpes zoster oticus, day 6. Image courtesy of Manolette Roque, MD, ROQUE Eye Clinic.

This syndrome is also known as geniculate neuralgia or nervus intermedius neuralgia. Ramsay Hunt syndrome can also occur in the absence of a skin rash, a condition known as zoster sine herpete.[2]

Ramsay Hunt syndrome was first described in 1907 by James Ramsay Hunt in a patient who had otalgia associated with cutaneous and mucosal rashes, which he ascribed to infection of the geniculate ganglion by human herpesvirus 3 (ie, varicella-zoster virus [VZV]).[3]

The following may be observed:

Pathophysiology

Ramsay Hunt syndrome is defined as VZV infection of the head and neck that involves the facial nerve, often the seventh cranial nerve (CN VII). Other cranial nerves (CN) may also be involved, including CN VIII, IX, V, and VI (in order of frequency). This infection gives rise to vesiculation and ulceration of the external ear and ipsilateral anterior two thirds of the tongue and soft palate, as well as ipsilateral facial neuropathy (in CN VII), radiculoneuropathy, or geniculate ganglionopathy.

VZV infection causes 2 distinct clinical syndromes. Primary infection, also known as varicella or chickenpox, is a common pediatric erythematous disease characterized by a highly contagious generalized vesicular rash. The annual incidence of varicella infection has significantly declined after the introduction of mass vaccination programs in most countries of the world.[4]

After chickenpox, VZV remain latent in neurons of cranial nerve and dorsal root ganglia.[1] Subsequent reactivation of latent VZV can result in localized vesicular rash, known as herpes zoster. VZV infection or reactivation involving the geniculate ganglion of CN VII within the temporal bone is the main pathophysiological mechanism of Ramsay Hunt syndrome. Diminished level of VZV-specific cell-mediated immunity may lead to reactivation of this virus.[5]

Epidemiology

Frequency

Ramsay Hunt syndrome is a rare neurologic disorder that arises as a complication of latent varicella-zoster virus (VZV) infection.[6]  It is estimated to strike approximately 5 out of every 100,000 people annually in the United States and affects both males and females equally.[1]  The syndrome can occur in anyone who has previously had chickenpox, though it predominantly affects adults older than 60 years and is extremely rare in children younger than 6 years.[6]  Ramsay Hunt syndrome accounts for about 16% of all causes of unilateral facial palsies in children and 18% in adults.[6]  It is also thought to be responsible for as many as 20% of clinically diagnosed cases of Bell palsy.[7]

The condition may present without a cutaneous rash, known as zoster sine herpete. Interestingly, VZV has been detected by polymerase chain reaction (PCR) in the tear fluid of patients diagnosed with Bell palsy,[7]  indicating a possible link. Due to the potential for Ramsay Hunt syndrome to go undiagnosed or be misdiagnosed, accurately determining its true prevalence in the general population is challenging.[1]

The incidence of Ramsay Hunt syndrome among patients with HIV infection is not well-documented but may occur at a higher rate than in the general population due to the increased risk of VZV infection in individuals with HIV.[3]

Mortality/morbidity

Ramsay Hunt syndrome is not usually associated with mortality. It is a self-limiting disease; the primary morbidity results from facial weakness. Unlike Bell palsy, this syndrome has a complete recovery rate of less than 50%.

Prognosis

In general, prognosis is good for the resolution of Ramsay Hunt syndrome symptoms.[8] However, fewer than 50% of patients have complete recovery of facial function.

Patient Education

The patient with Ramsay Hunt syndrome should be educated about eye care to prevent corneal irritation or injury.

History

Ramsay Hunt syndrome is characterized by a constellation of symptoms, predominantly unilateral facial nerve paralysis and a vesicular rash affecting the ear, although these manifestations may not appear concurrently.[1] The syndrome typically impacts one side of the face, leading to facial muscle weakness or stiffness, which can impair the ability to perform facial expressions such as smiling or wrinkling the forehead, resulting in a drooping facial appearance. The most pronounced facial weakness generally occurs within one week following the onset of symptoms. Additional complications include the inability to close the eye on the affected side, potentially leading to corneal irritation and, in rare cases, vision impairment due to corneal damage.

A careful history must be obtained in patients with suspected Ramsay Hunt syndrome.

Patients usually present with paroxysmal pain deep within the ear. The pain often radiates outward into the pinna of the ear and may be associated with a more constant, diffuse, and dull background pain. The onset of pain usually precedes the rash by several hours and even days.

Classic Ramsay Hunt syndrome can be associated with the following[1] :

Facial weakness usually reaches maximum severity by 1 week after the onset of symptoms.

Other cranial neuropathies might be present and may involve cranial nerves (CNs) VIII, IX, X, V, and VI.[9, 10]

Ipsilateral hearing loss has been reported in as many as 50% of cases. Vertigo is usually present in those with hearing loss.[11]

Blisters of the skin of the ear canal, auricle, or both may become secondarily infected, causing cellulitis.

Poor prognostic factors for good functional recovery include the following:

Physical

The dermatologic aspect of Ramsay Hunt syndrome involves an erythematous, vesicular rash that predominantly affects the pinna and, possibly, the external auditory canal, observed in up to 80% of patients.[1] This rash may extend to involve the eardrum, oral mucosa, soft palate, and upper throat, primarily on the affected side. Auditory symptoms are prominent, including tinnitus and significant otalgia. Sensorineural hearing loss is a common complication, affecting approximately 50% of patients, where sound transmission to the brain is disrupted due to inner ear or auditory nerve dysfunction. This hearing loss is generally temporary but may persist or become permanent in some cases. Hyperacusis, where normal sounds are perceived as excessively loud, can also occur, contributing to substantial discomfort.

Additional clinical manifestations may include nausea, vomiting, and vertigo, indicative of vestibular involvement.[1] In rare instances, Ramsay Hunt syndrome may lead to severe complications such as persistent facial pain (postherpetic neuralgia), ongoing facial weakness, altered taste perception, and in severe cases, dissemination of the virus to other cranial nerves or the central nervous system, manifesting as confusion, somnolence, limb weakness, headaches, and neuropathic pain. Although Ramsay Hunt syndrome itself is not contagious, the reactivation of the varicella-zoster virus can pose a risk of transmitting chickenpox to individuals who are not immune. Therefore, patients are advised to avoid contact with susceptible individuals until the vesicular rash has adequately scabbed over. In cases where the typical rash is absent, the condition may be diagnosed as zoster sine herpete, confirmed through virological testing but without the associated cutaneous manifestations.

The location of the accompanying rash varies from patient to patient, as does the area innervated by the nervus intermedius. It may include the following:

The patient may have associated ipsilateral hearing loss and/or vertigo. Hearing impairment is usually more severe in patients with vertigo than in those without vertigo.[12]

A thorough physical examination must be performed, including neuro-otologic and audiometric assessment.

Causes

Classic Ramsay Hunt syndrome is ascribed to infection of the geniculate ganglion by herpesvirus 3 (varicella-zoster virus [VZV]).

Complications

Ramsay Hunt syndrome may result in several infrequent but severe complications, including alterations in taste perception, vision impairment due to corneal ulcers and infections, and dyskinesias resulting from aberrant nerve regeneration leading to incorrect muscle innervation.[1] Chronic sequelae such as postherpetic neuralgia and persistent facial weakness are also possible. In rare cases, the varicella-zoster virus may disseminate to other cranial nerves or to central nervous system structures, manifesting as neurological symptoms including confusion, somnolence, limb weakness, headaches, and neuropathic pain.

While Ramsay Hunt syndrome is not directly transmissible, the underlying reactivation of the varicella-zoster virus can lead to chickenpox in individuals who are either unvaccinated or have not previously contracted the virus.[1] It is recommended that patients with Ramsay Hunt syndrome avoid contact with immunocompromised individuals and those who have not been vaccinated against chickenpox until the vesicular rash has fully crusted over. Furthermore, some patients may present with facial palsy and have laboratory evidence of varicella-zoster virus infection, yet lack the characteristic dermatological findings. These cases are classified as zoster sine herpete.

Rare complications of Ramsay Hunt syndrome include:

Approach Considerations

Diagnosing Ramsay Hunt syndrome can be challenging as its hallmark symptoms—earache, facial paralysis, and a distinctive rash—may not manifest simultaneously.[1] The diagnosis primarily relies on a comprehensive clinical evaluation and a detailed patient history, focusing on identifying characteristic symptoms such as unilateral facial palsy and/or a rash around the ear. Confirmation of the diagnosis can be achieved by testing a sample from the fluid-filled blisters typically found around the ear, which helps distinguish Ramsay Hunt syndrome from other conditions like Bell palsy, acoustic neuroma, or trigeminal neuralgia. While viral studies can detect the presence of the varicella-zoster virus in saliva, tears, and blood, these tests are not essential for confirming Ramsay Hunt syndrome.

Laboratory Studies

The diagnosis of Ramsay Hunt syndrome is usually made without difficulty when the clinical characteristics are present. If necessary, varicella zoster virus (VZV) may be isolated from vesicle fluid and inoculated into susceptible human or monkey cells for identification by serologic means.

WBC count, erythrocyte sedimentation rate (ESR), and serum electrolytes are helpful in distinguishing the infectious and inflammatory nature of this syndrome.

When CNS complications are suspected (eg, meningitis, meningoencephalitis, myelitis, arteritis [large and small vessel], and ventriculitis), spinal fluid analysis and CNS imaging studies are recommended.

Viral studies include the following:

Imaging Studies

Structural lesions can be ruled out by CT scan, MRI, or magnetic resonance (MR) angiography. Transcranial magnetic stimulation (TMS) may be clinically useful in gaining additional information about the prognostic status of the facial nerve.[18]

Magnetic resonance imaging

Gadolinium enhancement of the vestibular and facial nerves on MRI has been described in Ramsay Hunt syndrome.

Advances in clinical MRI images (eg, 3-Tesla MRI, multichannel phased array coil, 3-dimensional fluid-attenuated inversion recovery [3D-FLAIR]) allow the evaluation of subtle alterations at the level of the blood-labyrinthine barrier.[19]  Precontrast hyperintesity and postcontrast enhancement 3D-FLAIR in facial nerve, vestibulococlea nerve and inner ear are correlated with clinical presentation.[20]

Audio-vestibular impairments are closely associated with increased signal intensity in the inner ear organs observed in 3D-fluid-attenuated inversion recovery (3D-FLAIR) MRI scans taken 4 hours after contrast administration.[21] This improvement in imaging technology, particularly the 3D-FLAIR sequence, has greatly improved the evaluation of inner ear structures and their diseases. Lee et al conducted research on 18 patients suffering from herpes zoster oticus (HZ oticus); notably, 77% showed high-signal intensity in the inner ear structures on 4-hour post-contrast 3D-FLAIR images. The most significant findings were in the lateral semicircular canal and cochlea, which strongly correlated with the clinical symptoms of canal paresis and hearing loss, respectively. However, the vestibulo-cochlear nerve exhibited less frequent enhancement in post-contrast T1-weighted images, indicating a more intricate relationship between clinical symptoms and MRI results. These findings highlight the critical role of 3D-FLAIR MRI in diagnosing and understanding the full scope of inner ear involvement in HZ oticus, which may lead to more precise treatments.

Herpes zoster oticus may involve vestibular nucleitis rather than being merely a form of neuritis, challenging the conventional perception of it as a cranial nerve variant of shingles.[22] A retrospective analysis of brain MRIs comparing patients with HZ oticus to those with vestibular neuritis (VN) revealed distinct radiological differences within the brainstem. Specifically, signal irregularities were found in the vestibular nuclei of half of the HZ oticus patients, covering multiple vascular territories. In contrast, these abnormalities were not present in patients with VN. This indicates that the pathology of HZ oticus may extend beyond the vestibular nerve to involve the brainstem, which could necessitate a revised approach to its diagnosis and treatment.

Other Tests

Audiometry usually reveals sensorineural hearing loss in high frequency ranges.[12]

Unilateral caloric weakness may be present on electronystagmography (ENG).

Electrodiagnostic methods, such as facial motor nerve conductions studies (electroneurography), electromyography of facial innervated muscles, the blink reflex, and nerve excitability testing, could add information regarding the extent of seventh cranial nerve (CN VII) involvement, as well as prognostic factors.[7, 23, 24]  

Procedures

In the setting of a peripheral facial palsy, cerebrospinal fluid (CSF) rarely is analyzed. Although lumbar puncture is not recommended in the diagnosis of this disease, CSF findings can be helpful in confirming the diagnosis. In one study, CSF findings were abnormal in 11% of 230 patients with idiopathic peripheral facial palsy, in 60% of 17 patients with Ramsay Hunt syndrome (abnormal finding was pleocytosis), in 25% of 8 patients with Lyme disease, and in all 8 patients with HIV infection.[25] Thus, if the CSF is abnormal, a specific cause should be sought.

Temporary relief of otalgia in geniculate neuralgia may be achieved by applying a local anesthetic or cocaine to the trigger point, if in the external auditory canal.

Histologic Findings

The affected ganglia of the cranial nerve roots are swollen and inflamed.

The inflammatory reaction is chiefly of a lymphocytic nature, but a few polymorphonuclear leukocytes or plasma cells may also be present.

Some of the cells of the ganglia are swollen and others degenerated.

Staging

Several scales have been developed to quantify the degree of facial muscle weakness. Of those, the House-Brackmann scale is most commonly used.[7]  However, the Yanagihara facial nerve grading scale is also clinically helpful.[26]

The House-Brackmann facial neuropathy scale is as follows:

The Yanagihara facial nerve grading scale is as follows:

Each function is scored 0 (complete palsy), 2 (partial palsy), or 4 (nearly normal). 

Approach Considerations

Treatment goals of Ramsay Hunt syndrome are to minimize disability and relieve symptoms. Clinical evidence shows that earlier start of treatment is correlated with better outcomes.[1]

After initiation of medical therapy, the patient with Ramsay Hunt syndrome should be seen in follow-up at 2 weeks, 6 weeks, and 3 months.

Synkinesia, abnormal movements that accompany intended voluntary facial movements, may develop during recovery following Ramsay Hunt syndrome. The occurence rate of synkinesia is higher in Ramsay Hunt syndrome than in Bell palsy. Up to 45% of patients with Ramsay Hunt syndrome experience synkinesia. Severe facial weakness documented by electroneurography (ENoG) is a predictor for development of synkinesia.[27]

Medical Care

The management of Ramsay Hunt syndrome primarily involves the administration of antiviral agents such as acyclovir or famciclovir, coupled with corticosteroids like prednisone.[1] This therapeutic approach aims to enhance the efficacy against the varicella zoster virus, with corticosteroids potentially reducing inflammation and alleviating neuropathic pain. However, the clinical effectiveness of antiviral medications in this specific context remains unconfirmed. Despite therapeutic interventions, some patients may sustain permanent sequelae, including facial paralysis and hearing loss.

Evidence from clnical studies indicates that starting treatment in the first week is correlated with the highest rate of improvement, although treatment started later still has some benefit.

Intravenous high-dose methylprednisolone is not commonly used in the treatment of Ramsay Hunt syndrome, however, it may provide some clinical benefit even if administered late.[28]  

Treatment modalities are further individualized based on the specific clinical manifestations of each patient. Analgesic management may include carbamazepine, an anticonvulsant effective in reducing neuralgic pain.[1] Vertigo may be managed with antihistamines and anticholinergics, while anti-anxiety agents such as diazepam are utilized for symptomatic relief of pain and vertigo. Botulinum toxin type A (Botox) is employed for both neuropathic pain and functional improvement in patients experiencing difficulty in eyelid closure. For those with residual facial weakness, targeted physical therapy is recommended to enhance facial muscle function.

A study by Dworkin et al concluded that controlled-release oxycodone was safe and generally well tolerated in patients experiencing acute pain due to herpes zoster.[29]

The US Food and Drug Administration (FDA) has approved capsaicin for the management of neuropathic pain associated with postherpetic neuralgia.[1] Early initiation of treatment, preferably within three days of symptom onset, is critical for optimal therapeutic outcomes.

Protective measures against corneal damage are essential for patients with impaired eyelid closure.[1] This includes the application of artificial tears and lubricating ointments, and in some cases, the use of an eye patch to prevent exposure-related corneal injuries.

Preventive strategies against the varicella zoster virus encompass vaccination with the chickenpox vaccine in children and the shingles vaccine in individuals aged 50 and older. These vaccinations significantly decrease the risk of viral infection, subsequently reducing the incidence of Ramsay Hunt syndrome.

Consultations

Consultation with an infectious disease specialist is recommended.

If a structural lesion is discovered on imaging, consultation with a neurosurgeon or otolaryngologist is recommended.

Consultation with an ophthalmologist to assist with eye care, especially pertaining to the cornea, may be appropriate.

Medication Summary

Oral corticosteroids and oral acyclovir are frequently prescribed in patients with Ramsay Hunt syndrome. Vestibular suppressants may be helpful if vestibular symptoms are severe. Carbamazepine may be helpful, especially in cases of idiopathic geniculate neuralgia.

Prednisone (Deltasone, Prednisone Intensol, Rayos)

Clinical Context:  May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. May be taken during acute inflammatory period (1-2 wk) and then tapered slowly. As an alternative, Dosepaks (ie, several prepackaged tablets with decreasing doses) can be taken. Individualize dose based on response.

Class Summary

These agents reduce the inflammation of the cranial nerves and help alleviate the pain and neurologic symptoms.

Acyclovir (Zovirax)

Clinical Context:  Patients experience less pain and faster resolution of symptoms when used within 48 h from onset of symptoms. May prevent recurrent outbreaks.

Class Summary

Acyclovir can be used to combat infection caused by herpesviruses such as VZV.

Carbamazepine (Carbamazepine Chewtabs, Carbamazepine CR, Carbatrol)

Clinical Context:  DOC that may reduce polysynaptic responses and block posttetanic potentiation. Adjust dose depending on response to treatment and blood levels.

Class Summary

Mechanism of action of antiepileptics in this syndrome is still unknown. Carbamazepine has been shown to help the neuralgic pain associated with this syndrome, especially in cases of idiopathic geniculate neuralgia.

Meclizine (Antivert, Bonine, Dramamine Less Drowsy Formula)

Clinical Context:  Decreases excitability of middle ear labyrinth and blocks conduction in middle ear vestibular-cerebellar pathways. Associated with therapeutic effects in relief of nausea and vomiting.

Dimenhydrinate (Dramamine)

Clinical Context:  A 1:1 salt of 8-chlorotheophylline and diphenhydramine thought to be useful in treatment of vertigo. Through central anticholinergic activity, diminishes vestibular stimulation and depresses labyrinthine function.

Class Summary

These agents prevent histamine responses in sensory nerve endings and blood vessels. They are effective in treating vertigo.

Scopolamine (Maldemar, Scopace, Transderm Scop)

Clinical Context:  Blocks action of acetylcholine at parasympathetic sites in smooth muscle, secretory glands, and the CNS. Antagonizes histamine and serotonin action.

Transdermal scopolamine may be most effective agent for motion sickness. Its use in vestibular neuronitis limited by its slow onset of action.

Class Summary

These agents are thought to work centrally by suppressing conduction in the vestibular-cerebellar pathways.

What is Ramsay Hunt syndrome (herpes zoster oticus)?What can be observed in Ramsay Hunt syndrome (herpes zoster oticus)?Which cranial nerves are involved in the pathogenesis of Ramsay Hunt syndrome?What syndromes are caused by varicella-zoster virus (VZV)?What is the main pathophysiological mechanism of Ramsay Hunt syndrome?What is the prevalence of Ramsay Hunt syndrome?What disease is thought to be caused by Ramsay Hunt syndrome?What is the incidence of Ramsay Hunt syndrome among patients with HIV infection?What is the prognosis of Ramsay Hunt syndrome?What is the presentation of Ramsay Hunt syndrome?What are the signs and symptoms of Ramsay Hunt syndrome?What are prognostic factors for poor recovery in Ramsay Hunt syndrome?What are physical findings characteristic of Ramsay Hunt syndrome?Where do rashes typically occur in Ramsay Hunt syndrome?When is hearing impairment more severe in Ramsay-Hunt syndrome?Which physical exams should be performed in suspected Ramsay Hunt syndrome?What causes Ramsay Hunt syndrome?What are complications of Ramsay Hunt syndrome?What are the differential diagnoses for Ramsay Hunt Syndrome?What is the role of lab testing in the diagnosis of Ramsay Hunt syndrome?Which viral studies are performed in the diagnosis of Ramsay Hunt syndrome?What is the role of imaging studies in the diagnosis of Ramsay Hunt syndrome?What is the role of audiometry, ENG and EDX studies in the diagnosis of Ramsay Hunt syndrome?What is the role of CSF analysis in the diagnosis of Ramsay Hunt syndrome?How is otalgia managed in Ramsay Hunt syndrome?Which histologic findings are characteristic of Ramsay Hunt syndrome?Which scales are used to assess facial muscle weakness in Ramsay Hunt syndrome?What are the classifications of facial muscle weakness in Ramsay Hunt syndrome?What are the treatment goals for Ramsay Hunt syndrome?What are the treatment options for Ramsay Hunt syndrome?Which medications are used in the treatment of Ramsay Hunt syndrome?Which specialists should be consulted in the treatment of Ramsay Hunt syndrome?Which medications are used for treatment of Ramsay Hunt syndrome?Which medications in the drug class Anticholinergics are used in the treatment of Ramsay Hunt Syndrome?Which medications in the drug class Antihistamines are used in the treatment of Ramsay Hunt Syndrome?Which medications in the drug class Anticonvulsants are used in the treatment of Ramsay Hunt Syndrome?Which medications in the drug class Antivirals are used in the treatment of Ramsay Hunt Syndrome?Which medications in the drug class Corticosteroids are used in the treatment of Ramsay Hunt Syndrome?

Author

Sombat Muengtaweepongsa, MD, MSc, Professor, Center of Excellence in Stroke, Associate Dean for Research and Innovations, Department of Neurology, Faculty of Medicine, Thammasat University, Thailand

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Boehringer Ingelheim; Astra Zeneca; Novo Nordisk; Daiichi Sankyo; Bayer<br/>Received research grant from: Faculty of Medicine, Thammasat University.

Coauthor(s)

Puchit Sukphulloprat, MD, Attending Neurologist, Division of Neurology, Department of Internal Medicine, Thammasat University Faculty of Medicine, Thailand

Disclosure: Nothing to disclose.

Specialty Editors

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Florian P Thomas, MD, PhD, MA, MS, Chair, Neuroscience Institute and Department of Neurology, Director, Hereditary Neuropathy Center, Co-Director, Center for Memory Loss and Brain Health, Co-Director, ALS Center, Hackensack University Medical Center; Associate Dean of Faculty, Founding Chair and Professor, Department of Neurology, Hackensack Meridian School of Medicine

Disclosure: Nothing to disclose.

Chief Editor

Niranjan N Singh, MBBS, MD, DM, FAHS, FAANEM, Adjunct Associate Professor of Neurology, University of Missouri-Columbia School of Medicine; Medical Director of St Mary's Stroke Program, SSM Neurosciences Institute, SSM Health

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbie and Pfizer.

Additional Contributors

Augusto A Miravalle, MD, Fellow, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School

Disclosure: Nothing to disclose.

Acknowledgements

Deepak Awasthi, MD Clinical Professor, Department of Neurosurgery, Louisiana State University School of Medicine; Consulting Staff, Louisiana Brain and Spine Clinic

Deepak Awasthi is a member of the following medical societies: Alpha Omega Alpha and Phi Beta Kappa

Disclosure: Nothing to disclose.

Augusto A Miravalle, MD Fellow, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School

Augusto A Miravalle, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Marion Priscilla Short, MD Assistant Professor, Departments of Neurology, Pediatrics, and Pathology, University of Chicago Hospitals and Clinics

Marion Priscilla Short, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuropathologists, American College of Medical Genetics, American Medical Association, and American Society of Human Genetics

Disclosure: Nothing to disclose.

References

  1. NORD. Ramsay Hunt Syndrome. National Organization for Rare Disorders. Available at https://rarediseases.org/rare-diseases/ramsay-hunt-syndrome/. Last updated 7/25/2025; Accessed: March 8, 2025.
  2. Bhupal HK. Ramsay Hunt syndrome presenting in primary care. Practitioner. 2010 Mar. 254(1727):33-5, 3. [View Abstract]
  3. Goldani LZ, Ferreira da Silva LF, Dora JM. Ramsay Hunt syndrome in patients infected with human immunodeficiency virus. Clin Exp Dermatol. 2009 Jun 1. [View Abstract]
  4. Kleinschmidt-DeMasters BK, Gilden DH. The expanding spectrum of herpesvirus infections of the nervous system. Brain Pathol. 2001 Oct. 11(4):440-51. [View Abstract]
  5. Haginomori S, Ichihara T, Mori A, Kanazawa A, Kawata R, Tang H, et al. Varicella-zoster virus-specific cell-mediated immunity in Ramsay Hunt syndrome. Laryngoscope. 2016 Jan. 126 (1):E35-9. [View Abstract]
  6. Sandoval C C, Nunez F A, Lizama C M, Margarit S C, Abarca V K, Escobar H R. [Ramsay Hunt syndrome in children: four cases and review]. Rev Chilena Infectol. 2008 Dec. 25(6):458-64. [View Abstract]
  7. [Guideline] Gilchrist JM. Seventh cranial neuropathy. Semin Neurol. 2009 Feb. 29(1):5-13. [View Abstract]
  8. Monsanto RD, Bittencourt AG, Bobato Neto NJ, Beilke SC, Lorenzetti FT, Salomone R. Treatment and Prognosis of Facial Palsy on Ramsay Hunt Syndrome: Results Based on a Review of the Literature. Int Arch Otorhinolaryngol. 2016 Oct. 20 (4):394-400. [View Abstract]
  9. Gunbey HP, Kutlar G, Aslan K, Sayit AT, Incesu L. Magnetic Resonance Imaging Evidence of Varicella Zoster Virus Polyneuropathy: Involvement of the Glossopharyngeal and Vagus Nerves Associated With Ramsay Hunt Syndrome. J Craniofac Surg. 2016 May. 27 (3):721-3. [View Abstract]
  10. Kim CH, Kang SI, Kim YH. A case of ramsay hunt syndrome with cranial polyneuropathy. Korean J Audiol. 2012 Sep. 16 (2):80-2. [View Abstract]
  11. Shin DH, Kim BR, Shin JE, Kim CH. Clinical manifestations in patients with herpes zoster oticus. Eur Arch Otorhinolaryngol. 2016 Jul. 273 (7):1739-43. [View Abstract]
  12. Kim CH, Choi H, Shin JE. Characteristics of hearing loss in patients with herpes zoster oticus. Medicine (Baltimore). 2016 Nov. 95 (46):e5438. [View Abstract]
  13. Shen YY, Dai TM, Liu HL, Wu W, Tu JL. Ramsay Hunt Syndrome Complicated by Brainstem Encephalitis in Varicella-zoster Virus Infection. Chin Med J (Engl). 2015 Dec 5. 128 (23):3258-9. [View Abstract]
  14. Sun WL, Yan JL, Chen LL. Ramsay Hunt syndrome with unilateral polyneuropathy involving cranial nerves V, VII, VIII, and XII in a diabetic patient. Quintessence Int. 2011 Nov-Dec. 42 (10):873-7. [View Abstract]
  15. Ganesan V, Bandyopadhyay D, Kar SS, Choudhury C, Choudhary V. Herpes Zoster Infection Involving Mandibular Division of Trigeminal Nerve and Ramsay Hunt Syndrome with Meningitis in an Immunocompetent Patient: A Rare Association. J Clin Diagn Res. 2016 Jun. 10 (6):OD05-7. [View Abstract]
  16. Coffin SE, Hodinka RL. Utility of direct immunofluorescence and virus culture for detection of varicella-zoster virus in skin lesions. J Clin Microbiol. 1995 Oct. 33(10):2792-5. [View Abstract]
  17. Lindström J, Grahn A, Zetterberg H, Studahl M. Cerebrospinal fluid viral load and biomarkers of neuronal and glial cells in Ramsay Hunt syndrome. Eur J Neurosci. 2016 Sep 19. [View Abstract]
  18. Hur DM, Kim SH, Lee YH, Kim SH, Park JM, Kim JH, et al. Comparison of Transcranial Magnetic Stimulation and Electroneuronography Between Bell's Palsy and Ramsay Hunt Syndrome in Their Acute Stages. Ann Rehabil Med. 2013 Feb. 37 (1):103-9. [View Abstract]
  19. Naganawa S, Nakashima T. Cutting edge of inner ear MRI. Acta Otolaryngol Suppl. 2009 Feb. 15-21. [View Abstract]
  20. Chung MS, Lee JH, Kim DY, Lim YM, Ahn JH, Sung YS, et al. The clinical significance of findings obtained on 3D-FLAIR MR imaging in patients with Ramsay-Hunt syndrome. Laryngoscope. 2015 Apr. 125 (4):950-5. [View Abstract]
  21. Lee J, Choi JW, Kim CH. Features of Audio-Vestibular Deficit and 3D-FLAIR Temporal Bone MRI in Patients with Herpes Zoster Oticus. Viruses. 2022 Nov 20. 14 (11):[View Abstract]
  22. Yacovino DA, Perez Akly MS, Ibañez T, Cherchi M. Vestibular Nucleus Involvement in Patients With Acute Vertigo Due to Herpes Zoster Oticus or Vestibular Neuritis. Neurology. 2023 Oct 3. 101 (14):e1461-e1465. [View Abstract]
  23. Psillas, George Constantinidis, Jiannis Vital, Victor Tsalighopoulos, Miltiadis. Ramsay Hunt syndrome: Clinical analysis of 15 cases. Aristotle University Medical Journal. 2016. 35:43-49.
  24. Byun H, Cho YS, Jang JY, Chung KW, Hwang S, Chung WH, et al. Value of electroneurography as a prognostic indicator for recovery in acute severe inflammatory facial paralysis: a prospective study of Bell's palsy and Ramsay Hunt syndrome. Laryngoscope. 2013 Oct. 123 (10):2526-32. [View Abstract]
  25. Kohler A, Chofflon M, Sztajzel R, Magistris MR. Cerebrospinal fluid in acute peripheral facial palsy. J Neurol. 1999 Mar. 246(3):165-9. [View Abstract]
  26. Hato N, Fujiwara T, Gyo K, Yanagihara N. Yanagihara facial nerve grading system as a prognostic tool in Bell's palsy. Otol Neurotol. 2014 Oct. 35 (9):1669-72. [View Abstract]
  27. Morishima N, Yagi R, Shimizu K, Ota S. Prognostic factors of synkinesis after Bell's palsy and Ramsay Hunt syndrome. Auris Nasus Larynx. 2013 Oct. 40 (5):431-4. [View Abstract]
  28. Donati D, De Santi L, Ginanneschi F, Cerase A, Annunziata P. Successful response of non-recovering Ramsay Hunt syndrome to intravenous high dose methylprednisolone. J Neurol Sci. 2012 Jul 15. 318 (1-2):160-2. [View Abstract]
  29. Dworkin RH, Barbano RL, Tyring SK, et al. A randomized, placebo-controlled trial of oxycodone and of gabapentin for acute pain in herpes zoster. Pain. 2009 Apr. 142(3):209-17. [View Abstract]

Herpes zoster oticus, day 6. Image courtesy of Manolette Roque, MD, ROQUE Eye Clinic.

Herpes zoster oticus, day 6. Image courtesy of Manolette Roque, MD, ROQUE Eye Clinic.

Herpes zoster oticus, day 6. Image courtesy of Manolette Roque, MD, ROQUE Eye Clinic.