CLINICAL OVERVIEW

Ramsay Hunt Syndrome

OUTLINE
Fort, Glenn G., MD, MPH Open all Close all

Released January 1, 2024.

BASIC INFORMATION

DEFINITION

BASIC INFORMATION
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SYNONYMS
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DEFINITION
EPIDEMIOLOGY & DEMOGRAPHICS
(https://clinicalkey.udemproxy.elogim.com/#!/content/derived_clinical_overview/76-
Ramsay Hunt syndrome is a localized herpes zoster infection involving the seventh nerve and geniculate ganglia, resulting in
s2.0-B9780323755764007766#hl0000042)
hearing loss, vertigo, and facial nerve palsy.
Predominant Sex
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s2.0-

SYNONYMS
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Predominant Age
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Herpes zoster oticus
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Geniculate herpes
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PHYSICAL FINDINGS & CLINICAL
Herpetic geniculate ganglionitis
PRESENTATION
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ETIOLOGY
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ICD-10CM CODE

B02.21 Postherpetic geniculate ganglionitis

EPIDEMIOLOGY & DEMOGRAPHICS

Predominant Sex
Equal sex distribution

Predominant Age

• Increasingly common with advancing age

• Rare in childhood

PHYSICAL FINDINGS & CLINICAL PRESENTATION

• Characteristic vesicles:

1. On pinna

2. In external auditory canal ( Fig. E1 (https://clinicalkey.udemproxy.elogim.com/#!/content/derived_clinical_overview/76-s2.0-

B9780323755764007766#f0010) )

3. In distribution of the facial nerve (VII cranial nerve) and, occasionally, adjacent cranial nerves can also be involved,
such as V, IX, X

• Facial paralysis on the involved side

• Auditory symptoms include mild to severe tinnitus, deafness, vertigo, and nystagmus

FIG. E1

Herpes zoster of the geniculate ganglion resulting in vesicles on the ear (as shown here) and tympanic
membrane occurs in Ramsay Hunt syndrome. Both seventh and eighth cranial nerve functions may be affected.

From White GM, Cox NH [eds]: Diseases of the skin: color atlas and text, ed 2, St Louis, 2006, Mosby.
ETIOLOGY

• Reactivation of dormant infection with varicella-zoster virus after primary varicella.

• Herpetic inflammation of the geniculate ganglion is thought to be the cause of this syndrome.

DIAGNOSIS

• Usually made by recognition of the clinical features detailed previously

• Viral culture and/or microscopic examination of specimens taken from active vesicles

DIFFERENTIAL DIAGNOSIS
• Herpes simplex

• External otitis

• Impetigo

• Enteroviral infection

• Guillain-Barre syndrome

• Bell palsy of other etiologies, such as Lyme disease

• Acoustic neuroma (before appearance of skin lesions)

The differential diagnosis of headache and facial pain is described in Section II.

WORKUP
If the diagnosis is in doubt, confirm varicella-zoster virus infection.

LABORATORY TESTS

• Generally not necessary

• Viral culture of specimens of vesicular fluid and scrapings of the vesicle base

• Tzanck preparation, which may reveal multinucleated giant cells but has low sensitivity

• Direct immunofluorescent staining of scrapings

IMAGING STUDIES
MRI may demonstrate enhancement of the facial and vestibulocochlear nerves before appearance of vesicles.

TREATMENT

ACUTE GENERAL Rx

• Use of corticosteroids is controversial.

• Acyclovir (800 mg by mouth [PO] 5× qd for 10 days), famciclovir (500 mg tid for 7 days), or valacyclovir (1 g q8h for 7 days)
may hasten healing.

• Use of prednisone (60 mg PO qd for 7 days or on a tapering regimen, 40 mg PO for 2 days, 30 mg for 7 days, followed by
tapering course) is recommended by some authors, but its use remains controversial.

• Analgesics should be used as indicated.

 CHRONIC Rx

• Duloxetine and amitriptyline are effective in postherpetic pain.

• Other agents for postherpetic pain include gabapentin and pregabalin.

• Narcotic analgesics may occasionally be necessary.

DISPOSITION
Recurrences are unusual.

REFERRAL
To otolaryngologist: Patients with persistent facial paralysis for potential surgical decompression of the facial nerve

PEARLS & CONSIDERATIONS

To prevent reactivation of herpes zoster, the herpes zoster vaccine is recommended. The Zoster Vaccine Recombinant,
Adjuvanted (SHINGRX) is 90% effective after 2 doses and can be given to persons >50 yr of age.

COMMENTS
Immunodeficiency states, particularly HIV infection, should be considered in:

• Younger patients

• Severe cases

• Patients with a history of specific risk behavior

RELATED CONTENT
Ramsay Hunt Syndrome (Patient Information)

Herpes Zoster (Related Key Topic)

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