Ramsay Hunt Syndrome Type 2

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Article initiated by:

Bayan Al Othman, MD, Subhan Tabba, Yi-Hsien Yeh

All contributors:

Andrew Go Lee, MD, Ashwini Kini, MD, Paul Phelps, MD, FACS, Adam R. Sweeney, MD, Nagham Al-Zubidi, MD, Bayan Al Othman, MD, Michael T Yen, MD, Seong Lee, MD, William Kretzschmar M.D.

Assigned editor:

Bayan Al Othman, MD, Hans Heymann, MD

Review:

Assigned status Update Pending

 by Adam R. Sweeney, MD on June 13, 2025.

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Contributing Editors:	add

Ramsay Hunt Syndrome Type 2

DiseasesDB

11176

ICD-10

[1]02.2

ICD-9

053.11

OMIM

159700

MedlinePlus

001647

MeSH

D016697

Ramsay Hunt syndrome type 2 is caused by reactivation of varicella zoster virus in the geniculate ganglion. Patient often presents with facial nerve paralysis leading to facial droop, dry eyes, dry mouth, and hearing loss. Diagnosis is largely clinical, but PCR of direct immunofluorescent assay analysis of the vesicular fluid can be helpful in determining the diagnosis. Treatment includes methylprednisolone and acyclovir, which achieves a high rate of complete recovery.

Disease Entity

Ramsay Hunt syndrome type 2, also known as herpes zoster oticus, is caused by reactivation of the varicella zoster virus in the geniculate ganglion. The geniculate ganglion is a sensory nerve cell bundle of the facial nerve (CN VII); the inflammatory response triggered by the reactivated virus can lead to extension of inflammation to the motor a lower motor neuron lesions of the facial nerve and/or involvement of other cranial nerves, resulting in paralysis of the facial muscles, dry mouth, dry eyes, and hyperacusis, and a plethora of other symptoms. The classic clinical presentation includes a vesicular rash on the ear (herpes zoster oticus) or the oral mucosa with accompanying acute peripheral facial nerve paralysis. Ramsay Hunt syndrome type 2 accounts for approximately 12% of all facial palsies and carries a worse prognosis compared to Bell's palsy.

Ramsay Hunt Syndrome Type 2
DiseaseDB	11176
ICD-10	B02.2 (ILDS B02.270_, G53.0
ICD-9-CM	053.11
OMIM	159700
MeSH	D016697
MedlinePlus

Epidemiology

The estimated incidence of Ramsay Hunt Syndrome is 5 in 100,000 with no predilection for sex. Patient's must have had chicken pox virus (VZV) at some point in their life with the exceptions of rare cases of Ramsay hunt in patients with no history of VZV infection who have been vaccinated with the live attenuated VZV. The condition predominantly affects adults over 60 years of age, though it can occur across all age groups including children. It can affect both immunocompromised and immunocompetent patients. The syndrome is more common than initially recognized because up to one-third of cases present without the characteristic vesicular rash, making diagnosis challenging and potentially leading to underreporting. It is also thought to be the cause of as many as 20% of clinically diagnosed cases of Bell's Palsy.

Etiology

Ramsay Hunt syndrome type 2 is caused by reactivation of varicella zoster virus in the geniculate ganglion of CN VII. VZV is a double stranded DNA virus of the Human Herpes Virus Family.

Risk Factors

Age (older adults are at higher risk), recent illness, psychological stress, use of immunosuppressive medications (e.g., corticosteroids, chemotherapy), and HIV/AIDS.

Pathophysiology

Ramsay Hunt Syndrome Type 2 occurs due to reactivation of the latent varicella-zoster virus in the geniculate ganglion. After the initial infection, VZV lies dormant in sensory dorsal root ganglia or cranial nerve ganglia. The virus is able to reshape its DNA and control transcription of certain RNA sequences to silence the replication and transcription of its DNA allowing low level expression without triggering strong immune response. At the same time VZV specific cell mediated immunity specifically effector and memory T cells keep the viral expression in check. Immune suppression with aging or various mechanisms results in decreased VZV specific T cells which can result in a loss of suppression allowing the virus to enter the lytic cycle. The reactivated virus travels anterograde down the sensory neuron to the associated dermatome and replicates in the keratinocytes resulting in the classic vesicular rash. The vesicular rash characteristic of the syndrome results from viral replication in sensory neurons innervating the external auditory canal and auricle. The reactivation of the virus in the geniculate ganglion leads to viral neuritis in the facial nerve. The inflammatory process leads to nerve edema, compression, and demyelination, resulting in the characteristic lower motor neuron facial paralysis. The severity of nerve damage correlates with the intensity of the viral immune response. The anatomic proximity of the geniculate ganglion to the vestibulocochlear nerve within the facial canal often results in involvement of cranial nerve VIII, resulting in tinnitus, hearing loss, vertigo, and nystagmus. Direct perineural and trans-axonal spread of viral inflammation between contiguous cranial nerves and hematogenous dissemination between nerves sharing a common blood supply can also occur.

Primary Prevention

Vaccination occurs in both childhood to prevent "Chickenpox" or Primary VZV with the live attenuated vaccine as well as in adulthood with the recombinant zoster vaccine (shingrix) to prevent reactivation of latent virus.

In childhood, the live attenuated vaccine is give for children at ages 12-15 months and 4-6 years. For adults without evidence of immunity, a 2-dose series is given 4-8 weeks apart. This can help prevent infection and the development of the latent infection.

Adults >50 years of age or adults >19 years of age with immunosuppression. The Recombinant Zoster Vaccine (Shingrix) is a two dose series. The second dose should be administered 2 to 6 months after the first. For immunocompromised individuals, the interval may be 1 to 2 months. Vaccination with Shingrix is recommended for all patients >50 even those without a history of Chickenpox.

Diagnosis

Ramsay Hunt syndrome type 2 is typically diagnosed based on clinical features. In ambiguous cases, PCR or direct immunofluorescence assay of vesicular fluid can aid in confirming the diagnosis. Laboratory studies including WBC count, ESR, and serum electrolytes should be obtained to help distinguish between infectious and inflammatory etiologies.

History and Physical Examination

The classic triad of Ramsay Hunt Syndrome Type II includes: ipsilateral peripheral facial nerve palsy, otalgia, and an erythematous vesicular eruptions in the external auditory canal or auricle (herpes zoster oticus). As mentioned previously the rash can be absent in about one third of cases which are referred to as zoster sine herpetica. On history, patients often report ear pain that may precede the facial weakness and rash, and many describe additional symptoms reflecting vestibulocochlear nerve involvement, including tinnitus, hearing loss, vertigo, nausea, and vomiting. If chorda tympani is involved, patients can report taste disturbances, as well as a reduction of tear, nasal, and saliva secretions. Other symptoms include hyperacusis, lacrimation, ocular pain.

Physical examination will reveal lower motor neuron facial paralysis with inability to close the eye, raise the eyebrow, or wrinkle the forehead on the affected side, distinguishing it from central facial palsy. Careful inspection of the external ear, auditory canal, and oral cavity (particularly the soft palate) may reveal characteristic vesicular lesions, though these may appear as ulcers rather than intact vesicles. Examination may also demonstrate nystagmus, hearing impairment on Weber and Rinne testing, and postural instability when the vestibulocochlear nerve is involved. In rare cases, multiple cranial neuropathies may be present, affecting cranial nerves beyond VII and VIII.

The degree of nerve palsy can be graded based on House-Brackmann facial nerve grading scale.

House-Brackmann Facial Nerve Grading Scale
Grade I	Normal
Grade II	Mild Dysfunction
Grade III	Moderate Dysfunction
Grade IV	Moderately Severe dysfunction
Grade V	Severe Dysfunction
Grade VI	Total Paralysis

Labs and Imaging

Ramsay Hunt Syndrome type 2 can usually be diagnosed based on clinical features and history. However, laboratory confirmation becomes essential in atypical presentations, particularly in zoster sine herpete where vesicles are absent. In a prospective study, 14% of patients developed vesicles after the onset of facial weakness, meaning Ramsay Hunt syndrome may initially be indistinguishable from Bell's palsy.

Polymerase chain reaction (PCR) is the most sensitive and specific method for confirming VZV reactivation, with sensitivity and specificity of 95% and 100%, respectively, compared to 82% and 76% for direct immunofluorescence antigen testing.

• VZV DNA can be detected in multiple specimen types: lesion swabs (obtained by vigorously rubbing the base of an unroofed vesicle), saliva, tear fluid, blood mononuclear cells, or cerebrospinal fluid.
• In patients with classic Ramsay Hunt syndrome, VZV DNA was detected in saliva in up to 72% of submandibular samples and 57% of parotid samples.

Serologic testing has a more limited role. While VZV IgM positivity may indicate recent reactivation, the IDSA/ASM guidelines note that IgM evaluation is not recommended as a primary means to establish acute infection—NAAT or culture is preferred.

If central nervous system complications such as meningitis, ventriculitis or meningoencephalitis are suspected, prompt lumbar puncture with spinal fluid analysis and imaging (CT head) are recommended.

Gadolinium enhanced MRI of the internal auditory canal can be helpful in atypical presentations, demonstrating post-contrast enhancement of cranial nerve VII, VIII, and the internal auditory canal dura, which may facilitate early diagnosis even before vesicles appear. Imaging primarily is used to confirm diagnosis and assess the severity of cranial nerve involvement. It can also help distinguish from bell's palsy as in RHS the inflammation is often more intense.

WBC count, erythrocyte sedimentation rate, and serum electrolytes to differentiate infectious and inflammation etiologies.

Differential diagnosis

• Bell's Palsy
• Temporomandibular joint pain dysfunction syndrome
• Trigeminal Neuralgia
• Migraine
• Benign Paroxysmal Positional Vertigo
• Persistent idiopathic facial pain

Management

The management of Ramsay Hunt Syndrome Type II centers around early initiation of combination antiviral and corticosteroid therapy, ideally within 72 hours of symptom onset, along with supportive care for ocular protection and pain management. One retrospective studies found that when treatment is initiated within 3 days of symptom onset, complete resolution of facial nerve paralysis occured in 75% of the cohort. However, when therapy was started on patients 7 days after symptom onset, only 30% of the cohort achieved complete resolution of facial paralysis, as assessed by the House-Brackmann score.

The recommended antiviral regimens include acyclovir 800 mg orally five times daily, valacyclovir 1,000 mg three times daily, or famciclovir 500 mg three times daily for 7-10 days. One study found the complete recovery rate of patients without hypertension and diabetes was significantly higher in patients treated with famciclovir than acyclovir. Antiviral agents such as acyclovir and famciclovir improve acute pain and recovery of lesions from herpes zoster and prevent the occurrence of postherpetic neuralgia.

For moderate cases, Oral prednisone (1mg/kg/day) up to 60 mg/day for 5 days should be given with antiviral regimens. For severe cases (those with multiple cranial nerve neuropathies), patients can start on IV methylprednisone and transition to oral when lesions crust over. For patients with severe (Grade VI House Brackmann), there has been evidence that high dose corticosteroids up to 200mg/day had the best outcomes, however this was not statistically significant due to sample size. There have also been recent studies on intratympanic injection of steroids to reduce non recovery in facial nerve palsy in Ramsay Hunt Syndrome. At this time no prospective studies have been performed to confirm the effectiveness and to identify an adequate method of intratympanic corticosteroid injection.

No effective treatment has been identified for hearing loss. Vertigo can be treated with diazepam or carbamazepine for vestibulocochlear suppression.

It is also important to consider supportive care for complications of RHS. Eye care should be considered in lagophthalmos and reduced corneal protection including: artificial tears, lubricating ointments at night, and protective eyewear or taping the eyelid closed during sleep.

Medical follow up

Monitor degree of facial nerve damage using the House–Brackmann score. From initiation of medical therapy, follow-up on patients in 2 weeks, 6 weeks and 3 months.

Surgical Treatments

If lagophthalmos persists and corneal damage is not amenable to medical management, physicians can consider surgical interventions such as tarsorrhaphy, gold weight implantation, or and horizontal lid tightening (e.g., lateral tarsal strip).

Complications

Incomplete facial nerve recovery is the most significant complication. Patients can develop synkinesis (involuntary facial movements during voluntary actions), contracture, and incomplete eye closure that leads to severe dry eye or exposure keratopathy. Up to 45% of the patients with Ramsay Hunt Syndrome can experience synkinesia or abnormal movement with voluntary facial movements during recovery.

In addition, patients could develop postherpetic neuralgia, or pain present for longer than three months after onset. This is more likely to develop in patients >50 years old and those who experience facial numbness in the acute period.

Prognosis

Fortunately, studies have shown that all patient's with Ramsay Hunt syndrome improve to some degree with the mean gain at 1 year after onset being 3 House Brackmann grade units. Patient's who present with incomplete eye closure and dry eye had worse facial nerve outcomes in this study. Compared to Bell's palsy, patients with Ramsay Hunt syndrome have more severe paralysis at onset and are less likely to recover completely. The degree of facial nerve paralysis resolution can be predicted by severity of initial paralysis. Studies show the rate of complete resolution to range from 10-22% to 66%. In patients with Bell's palsy, the spontaneous recovery rate is about 70% and the post-therapeutic recovery rate is over 90%. In contrast, the spontaneous recovery rate and post-therapeutic recovery rate in Ramsay Hunt syndrome are about 30% and 55%, respectively.

Risk factors for non-recovery include age over 50 years old, a greater degree of axonal damage on electrodiagnostic testing, involvement of multiple cranial neuropathies, the presence of oropharyngeal lesions, and diabetes.

Patients without hypertension, diabetes and with a initial House-Brackann grade II or better presentation have higher chance of complete recovery (84.6%).