AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Background

Herpes zoster oticus (HZ oticus) is a viral infection of the inner, middle, and external ear. HZ oticus manifests as severe otalgia and associated cutaneous vesicular eruption, usually of the external canal and pinna. When associated with facial paralysis, the infection is called Ramsay Hunt syndrome.

Pathophysiology

Reactivation of the varicella-zoster virus (VZV) along the distribution of the sensory nerves innervating the ear, which usually includes the geniculate ganglion, is responsible for HZ oticus. Associated symptoms such as hearing loss and vertigo are thought to occur as a result of transmission of the virus via direct proximity of cranial nerve (CN) VIII to CN VII at the cerebellopontine angle or via vasa vasorum that travel from CN VII to other nearby cranial nerves.

For a CME/CE activity, see Guidelines Issued on Prevention of Herpes Zoster.

Frequency

United States

Ramsey Hunt syndrome accounts for up to 12% of all facial paralyses.

Mortality/Morbidity

Ramsay Hunt syndrome generally causes more severe symptoms and has a worse prognosis than Bell palsy.

• Return-to-baseline neurologic function is predicted partially by severity of paralysis.
• In several studies, only 10-22% of individuals with significant facial paralysis had complete recovery. In one study, however, 66% of patients with incomplete paralysis had complete recovery.
• An additional complication of herpes zoster viral infection is postherpetic neuralgia.

Sex

Incidence in males and females is equal.

Age

Incidence of HZ oticus increases significantly in patients older than 60 years.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

History

• Typically, patients present with severe otalgia. Complaints include the following:
• • Painful, burning blisters in and around the ear, on the face, in the mouth, and/or on the tongue.
  • Vertigo, nausea, vomiting
  • Hearing loss, hyperacusis, tinnitus
  • Eye pain, lacrimation
• Onset of pain may precede the rash by several hours or days. Also, in patients with Ramsay Hunt syndrome, vesicles may appear before, during, or after facial palsy (zoster sine herpete).
• When asked, patients may recall a distant history, perhaps in childhood, of chickenpox (varicella).
• A minority of patients (<10%) give a history of previous herpes zoster viral infection.

Physical

• Physical examination shows a vesicular exanthem, usually of the external auditory canal, concha, and pinna.
• The rash also may appear on postauricular skin, lateral nasal wall, soft palate, and anterolateral tongue.
• Vertigo and sensorineural hearing loss may be noted.
• Paralysis of the facial nerve, mimicking Bell palsy, may be present.
• Complete loss of the ability to wrinkle the ipsilateral brow distinguishes a peripheral lesion of cranial nerve VII from a central lesion of the same nerve, which spares the forehead.
• Associated findings
• • Dysgeusia (alteration in taste)
  • Inability to fully close the ipsilateral eye, which may lead to the occasional presentation of drying and irritation of the cornea.

Causes

Herpes zoster oticus is caused by the reactivation of latent VZV, which has remained dormant within sensory ganglia (commonly the geniculate ganglion) of the facial nerve.

• Individuals with decreased cell-mediated immunity resulting from carcinoma, radiation therapy, chemotherapy, or HIV infection are at greater risk for reactivation of latent VZV.
• Physical and emotional stress often are cited as precipitating factors.

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Isolated facial nerve trauma

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Lab Studies

• Herpes zoster oticus is primarily a clinical diagnosis in the ED.
• Prior to initiating treatment with acyclovir, consider a baseline set of the following laboratory studies:
• • Blood urea nitrogen (BUN)
  • Creatinine
  • Blood cell counts
  • Electrolytes

Imaging Studies

• If diagnosis of Ramsay Hunt syndrome is not established by physical examination alone, consider a head CT scan to investigate other etiologies of facial paralysis.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Emergency Department Care

• Adequate analgesia is important for individuals with significant pain from herpes zoster.
• Nausea and vomiting may require ED treatment.
• Complications, such as corneal irritation or secondary bacterial infection of the vesicles, should be managed with routine therapies.
• Involvement of more than one dermatome is atypical and should prompt the search for possible immunoincompetence.

Consultations

• Consider an ophthalmologic consultation if corneal involvement with vesicles is noted.
• Consider a neurologic consultation if the etiology of the facial paralysis is unclear.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Until recently, therapy for herpes zoster (HZ) oticus has been generally supportive, including warm compresses, narcotic analgesics, and antibiotics for a secondary bacterial infection. However, antiviral agents clearly play a role in limiting the severity and duration of symptoms if given early in the course of the illness. Early administration (<72 h) of acyclovir showed an increased rate of facial nerve function recovery and prevented further nerve degeneration. Furthermore, use of antivirals has shown to decrease incidence and severity of postherpetic neuralgia. Evidence is accumulating that VZV may be responsible for many cases of Bell palsy that go unrecognized because of a lack of cutaneous findings (zoster sine herpete). Accordingly, the clinician should entertain more liberal use of antivirals such as acyclovir, valacyclovir, and famciclovir. Studies have shown no difference between oral and IV acyclovir in immunocompetent patients with facial nerve paralysis.¹

Systemic corticosteroids are used to relieve acute pain, decrease vertigo, and limit the occurrence of postherpetic neuralgia. Treatment with acyclovir plus prednisone has more effective return to facial nerve function and prevention of nerve degeneration than treatment with prednisone alone. Furthermore, patients treated with acyclovir plus prednisone had better outcomes (time to healing of rash, time to cessation of acute neuritis, time to return to usual activity and sleep, and time to cessation of analgesics) than those treated with either prednisone or acyclovir alone.² No evidence indicates that use of corticosteroids prevents development of post-herpetic neuralgia.^{3, 4} Furthermore, evidence proving benefit attributed specifically to steroids is still limited, with a recent review showing no randomized controlled trials supporting use of steroids as an adjuvant to antiviral medications in the treatment of Ramsay Hunt Syndrome.⁵

For treatment of herpes zoster in patients with HIV, inpatient parenteral regimens should be reserved for those with severe immunosuppression, trigeminal nerve involvement, ocular lesions, or multidermatomal involvement. For acyclovir-resistant VZV, IV foscarnet is an appropriate alternative therapy (famciclovir and valacyclovir are not effective against acyclovir-resistant VZV). For outpatient regimens, famciclovir or valacyclovir for 7-10 days is recommended (both have the advantage of easier dosing regimens). Routine use of steroids is discouraged secondary to its immunosuppressive effects.⁶

Treatment of pregnant women with VZV is the same as for nonpregnant women, and it is the same for both HIV-seronegative and seropositive patients.

When secondary impetigo is present, a suitable antistaphylococcal antibiotic should be prescribed.

Cyclic antidepressants, anticonvulsants, opioids, and topical analgesics are sometimes used in the treatment of postherpetic neuralgia.⁷ These agents are more appropriately started by a pain management specialist in an outpatient setting.

Prevention of herpes zoster by vaccination is recommended for all persons aged older than 60 years, even if they have had chickenpox or zoster in the past. This age group suffers significant morbidity from zoster and may, therefore, benefit from the vaccine.  Contraindications to vaccine administration include age younger than 60 years, current use of antivirals, pregnancy, and certain immunosuppressive conditions.⁸

Drug Category: Antivirals

These agents prevent the replication of viral particles. Antiviral medications can be directed against VZV. Acyclovir is the prototypical antiherpetic. Newer agents, famciclovir and valacyclovir, may be more effective and have more convenient dosing. All of these agents have reduced effectiveness if administered more than 72 h after development of the rash.

Drug Category: Corticosteroids

These agents may help to relieve acute pain, decrease vertigo, and limit occurrence of postherpetic neuralgia.

Drug Category: Analgesics

With complaints of severe pain at any point at or beyond the appearance of crusted vesicles, assume that postherpetic neuralgia has developed. This can be extremely debilitating and requires aggressive management. Use narcotic analgesics liberally. NSAIDs and steroids have limited proven benefit in the treatment of postherpetic neuralgia.

Capsaicin cream applied topically to the affected areas generally is associated with dramatic relief. Capsaicin works by depleting pain fibers of substance P, thereby inhibiting propagation of pain impulses. It must be applied regularly qid and patients should be forewarned that the first application or two will be associated with a mild burning or stinging.

Drug Category: Ocular lubricants

These drugs promote hydration of cornea and conjunctivae. If eyelid paralysis is present, corneal irritation may result due to inadequate maintenance of the protective tear film. Use lubricating eyedrops as needed.

Drug Category: Antibiotics

These agents are for treatment of secondary bacterial infection in HZ oticus. These drugs cover for gram-positive skin flora.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Further Inpatient Care

• For patients with herpes zoster oticus, consider admission for any of the following situations:
• • Severe symptoms
  • Involvement of multiple (>2) dermatomes
  • Immunocompromise

Further Outpatient Care

• Ensure that the patient has adequate and timely outpatient follow-up for management of HZ oticus.
• Consultation with an ear, nose, and throat (ENT) specialist may be appropriate.

Complications

Complications of herpes zoster oticus may include the following:

• Postherpetic neuralgia
• Residual paralysis

Prognosis

• Prolonged or permanent facial paralysis (possible)
• Most patients with partial paralysis fully recover; many with severe symptoms are left with partial deficits.

Patient Education

• Instruct patients how to tape eyes shut if lid paralysis is present.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Failure to recognize a central cause of facial paralysis
• Failure to recognize and treat ocular complications
• Failure to identify immunoincompetence

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

1. Furuta Y, Ohtani F, Mesuda Y. Early diagnosis of zoster sine herpete and antiviral therapy for the treatment of facial palsy. Neurology. Sep 12 2000;55(5):708-10. [Medline].
2. Murakami S, Honda N, Mizobuchi M. Rapid diagnosis of varicella zoster virus infection in acute facial palsy. Neurology. Oct 1998;51(4):1202-5. [Medline].
3. Whitley RJ, Weiss H, Gnann JW. Acyclovir with and without prednisone for the treatment of herpes zoster. A randomized, placebo-controlled trial. The National Institute of Allergy and Infectious Diseases Collaborative Antiviral Study Group. Ann Intern Med. Sep 1 1996;125(5):376-83. [Medline].
4. Wood MJ, Johnson RW, McKendrick MW, Taylor J, Mandal BK, Crooks J. A randomized trial of acyclovir for 7 days or 21 days with and without prednisolone for treatment of acute herpes zoster. N Engl J Med. Mar 31 1994;330(13):896-900. [Medline].
5. Uscategui T, Doree C, Chamberlain IJ, Burton MJ. Corticosteroids as adjuvant to antiviral treatment in Ramsay Hunt syndrome (herpes zoster oticus with facial palsy) in adults. Cochrane Database Syst Rev. Jul 16 2008;CD006852. [Medline].
6. Benson CA, Kaplan JE, Masur H. Treating opportunistic infections among HIV-exposed and infected children: recommendations from CDC, the National Institutes of Health, and the Infectious Diseases Society of America. MMWR Recomm Rep. Dec 17 2004;53(RR-15):1-112. [Medline].
7. Pavan-Langston D. Herpes zoster antivirals and pain management. Ophthalmology. Feb 2008;115(2 Suppl):S13-20. [Medline].
8. Harpaz R, Ortega-Sanchez IR, Seward JF. Prevention of herpes zoster: recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Recomm Rep. Jun 6 2008;57:1-30; quiz CE2-4. [Medline].
9. Adour KK. Otological complications of herpes zoster. Ann Neurol. 1994;35 Suppl:S62-4. [Medline].
10. Bauer CA, Coker NJ. Update on facial nerve disorders. Otolaryngol Clin North Am. Jun 1996;29(3):445-54. [Medline].
11. Murakami S, Hato N, Horiuchi J. Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: significance of early diagnosis and treatment. Ann Neurol. Mar 1997;41(3):353-7. [Medline].
12. Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry. Aug 2001;71(2):149-54. [Medline].

Article Last Updated: Nov 4, 2008