Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Herpes Zoster Oticus Overview of Herpes Zoster Oticus

  • Author: Christina Bloem, MD, MPH; Chief Editor: Steven C Dronen, MD, FAAEM  more...
 
Updated: May 06, 2015
 

Overview of Herpes Zoster Oticus

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. (See the image below.)

Herpes zoster oticus. Image courtesy of Manolette Herpes zoster oticus. Image courtesy of Manolette Roque, MD, Ophthalmic Consultants Philippines Co, EYE REPUBLIC Ophthalmology Clinic.

Ramsay Hunt syndrome accounts for up to 12% of all facial paralyses and generally causes more severe symptoms and has a worse prognosis than Bell palsy.[1, 2, 3] 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.

The incidence rates of HZ oticus in males and females are equal, and incidence increases significantly in patients older than 60 years.

Next

Pathophysiology of Herpes Zoster Oticus

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 herpes zoster (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.

Previous
Next

Clinical Manifestations of Herpes Zoster Oticus

Patient 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 (see the image below)
    Herpes zoster oticus. Image courtesy of Manolette Herpes zoster oticus. Image courtesy of Manolette Roque, MD, Ophthalmic Consultants Philippines Co, EYE REPUBLIC Ophthalmology Clinic.
  • 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 examination

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, and 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 include the following:

  • 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.

Standardized assessment of facial function

The following House-Brackmann facial nerve grading scale provides a standardized way to quantify facial nerve function and objectively track recovery[4, 5] :

  • Grade I - Normal function
  • Grade II - Mild dysfunction
  • Grade III - Moderate dysfunction
  • Grade IV - Moderately severe dysfunction
  • Grade V - Severe dysfunction
  • Grade VI - Total paralysis

Complications

Complications of HZ oticus may include the following[6, 7, 8] :

  • Postherpetic neuralgia
  • Residual paralysis
  • Rarely, herpes zoster encephalitis [7]
Previous
Next

Etiology of Herpes Zoster Oticus

Herpes zoster (HZ) oticus is caused by the reactivation of latent varicella-zoster virus (VZV) that 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 stress and emotional stress often are cited as precipitating factors.

Previous
Next

Laboratory Studies

Herpes zoster oticus (HZ 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

Screening for anti-VZV antibodies (IgM and IgA) should be considered in at-risk immunocompromised patients.[9]

Previous
Next

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.

Previous
Next

Treatment of Herpes Zoster Oticus

Until recently, therapy for herpes zoster (HZ) oticus has been generally supportive, including warm compresses, narcotic analgesics, and antibiotics for a secondary bacterial infection.

Antiviral agents

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 been shown to decrease the incidence and severity of postherpetic neuralgia.[6, 10, 11, 12]

Evidence is accumulating that varicella-zoster virus (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.[2, 3] Studies have shown no difference between oral and IV acyclovir in immunocompetent patients with facial nerve paralysis.[13]

Valacyclovir and famciclovir have been shown to be more effective than acyclovir in reducing risk of pain, with comparable lesion healing and safety profile. Furthermore, patient compliance is likely to be higher with valacyclovir and famciclovir because each has an easier dosing regimen (3 times per day) compared with acyclovir (5 times per day).[14, 15]

Corticosteroids

Systemic corticosteroids are used to relieve acute pain, decrease vertigo, and limit the occurrence of postherpetic neuralgia. The prevailing wisdom states that treatment with acyclovir plus prednisone has more effective return to facial nerve function and prevention of nerve degeneration than treatment with prednisone alone; however, a recent review uncovered very little data to support or negate this theory.[10] 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.[16]

No evidence indicates that use of corticosteroids prevents development of postherpetic neuralgia.[11, 12] Furthermore, evidence proving benefit attributed specifically to steroids is still limited, with one review showing no randomized controlled trials supporting use of steroids as an adjuvant to antiviral medications in the treatment of Ramsay Hunt syndrome.[17]

Treatment in HIV patients

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. Treatment of VZV is the same for both HIV-seronegative and seropositive patients. 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.[18]

Treatment in other situations

Treatment of pregnant women with VZV is the same as that of nonpregnant women.

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.[6] 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 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.[19]

Ensure that the patient has adequate and timely outpatient follow-up for management of HZ oticus.

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.

Consider admission for any of the following situations:

  • Severe symptoms
  • Involvement of multiple (>2) dermatomes
  • Immunocompromise

Consultations

Consider an ophthalmologic consultation if corneal involvement with vesicles is noted, and consider a neurologic consultation if the etiology of the facial paralysis is unclear. Consultation with an ear, nose, and throat (ENT) specialist may be appropriate.

Previous
Next

Prognosis

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

Patients with HZ oticus have poorer prognoses than do those with Bell palsy. HZ oticus may result not only in permanent unilateral facial nerve paralysis, but also present as a polycranial neuropathy. Common disabilities may include hearing loss, vertigo, incomplete eye closure with dry eye, and speech disturbances.[20, 21]

While diplopia and swallowing abnormalities are rare symptoms, their presence suggests a trend toward a worse outcome. These findings are suggestive of a more widespread herpetic polyneuropathy with possible brainstem involvement by the zoster virus. More common cochleovestibular symptoms such as sensorineural hearing loss and vestibular disturbance are not significantly related to prognosis overall.

Other factors, including initial House-Brackmann grades V or higher, time before commencement of treatment, age, and the presence of comorbid disease, influence recovery. Patients with House-Brackmann grade II or better had recovery rates of 84.6%. Furthermore, patients without vertigo, diabetes mellitus, or hypertension have a higher likelihood of complete recovery.[22] Patients with diabetes mellitus have poor outcomes overall, which may be further compounded by the presence of diabetic neuropathy.

Previous
Next

Patient Education

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

Previous
 
Contributor Information and Disclosures
Author

Christina Bloem, MD, MPH Assistant Clinical Professor of Emergency Medicine, George Washington University

Disclosure: Nothing to disclose.

Coauthor(s)

Christopher I Doty, MD, FAAEM, FACEP Associate Professor of Emergency Medicine, Residency Program Director, Vice-Chair for Education, Department of Emergency Medicine, University of Kentucky-Chandler Medical Center

Christopher I Doty, MD, FAAEM, FACEP is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, Council of Emergency Medicine Residency Directors, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Jon Mark Hirshon, MD, MPH, PhD Professor, Department of Emergency Medicine, University of Maryland School of Medicine

Jon Mark Hirshon, MD, MPH, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, American Public Health Association, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Maria M Diaz, MD Staff Physician, Department of Emergency Medicine, Parkland Medical Center

Maria M Diaz, MD is a member of the following medical societies: American College of Emergency Physicians, Phi Beta Kappa, Emergency Medicine Residents' Association

Disclosure: Nothing to disclose.

Allison J N Harriott, MD, MPH Fellow in Critical Care Medicine, Pulmonary Critical Care Medicine, Penn State Milton S Hershey Medical Center

Allison J N Harriott, MD, MPH is a member of the following medical societies: American College of Emergency Physicians, Society for Academic Emergency Medicine, Emergency Medicine Residents' Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Eric L Weiss, MD, DTM&H Medical Director, Office of Service Continuity and Disaster Planning, Fellowship Director, Stanford University Medical Center Disaster Medicine Fellowship, Chairman, SUMC and LPCH Bioterrorism and Emergency Preparedness Task Force, Clinical Associate Professor, Department of Surgery (Emergency Medicine), Stanford University Medical Center

Eric L Weiss, MD, DTM&H is a member of the following medical societies: American College of Emergency Physicians, American College of Occupational and Environmental Medicine, American Medical Association, American Society of Tropical Medicine and Hygiene, Physicians for Social Responsibility, Southeastern Surgical Congress, Southern Oncology Association of Practices, Southern Clinical Neurological Society, Wilderness Medical Society

Disclosure: Nothing to disclose.

Chief Editor

Steven C Dronen, MD, FAAEM Chair, Department of Emergency Medicine, LeConte Medical Center

Steven C Dronen, MD, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Robin R Hemphill, MD, MPH Associate Professor, Director, Quality and Safety, Department of Emergency Medicine, Emory University School of Medicine

Robin R Hemphill, MD, MPH is a member of the following medical societies: American College of Emergency Physicians, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

References
  1. Bauer CA, Coker NJ. Update on facial nerve disorders. Otolaryngol Clin North Am. 1996 Jun. 29(3):445-54. [Medline].

  2. Murakami S, Hato N, Horiuchi J, Honda N, Gyo K, Yanagihara N. Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: significance of early diagnosis and treatment. Ann Neurol. 1997 Mar. 41(3):353-7. [Medline].

  3. Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry. 2001 Aug. 71(2):149-54. [Medline]. [Full Text].

  4. Vrabec JT, Backous DD, Djalilian HR, Gidley PW, Leonetti JP, Marzo SJ. Facial Nerve Grading System 2.0. Otolaryngol Head Neck Surg. 2009 Apr. 140(4):445-50. [Medline].

  5. House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg. 1985 Apr. 93(2):146-7. [Medline].

  6. Pavan-Langston D. Herpes zoster antivirals and pain management. Ophthalmology. 2008 Feb. 115(2 Suppl):S13-20. [Medline].

  7. Eskiizmir G, Uz U, Taskiran E, Unlü H. Herpes zoster oticus associated with varicella zoster virus encephalitis. Laryngoscope. 2009 Apr. 119(4):628-30. [Medline].

  8. Adour KK. Otological complications of herpes zoster. Ann Neurol. 1994. 35 Suppl:S62-4. [Medline].

  9. Gross G, Schöfer H, Wassilew S, Friese K, Timm A, Guthoff R, et al. Herpes zoster guideline of the German Dermatology Society (DDG). J Clin Virol. 2003 Apr. 26(3):277-89; discussion 291-3. [Medline].

  10. Uscategui T, Dorée C, Chamberlain IJ, Burton MJ. Antiviral therapy for Ramsay Hunt syndrome (herpes zoster oticus with facial palsy) in adults. Cochrane Database Syst Rev. 2008 Oct 8. CD006851. [Medline].

  11. Whitley RJ, Weiss H, Gnann JW Jr, Tyring S, Mertz GJ, Pappas PG, et al. 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. 1996 Sep 1. 125(5):376-83. [Medline].

  12. 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. 1994 Mar 31. 330(13):896-900. [Medline].

  13. Furuta Y, Ohtani F, Mesuda Y, Fukuda S, Inuyama Y. Early diagnosis of zoster sine herpete and antiviral therapy for the treatment of facial palsy. Neurology. 2000 Sep 12. 55(5):708-10. [Medline].

  14. McDonald EM, de Kock J, Ram FS. Antivirals for management of herpes zoster including ophthalmicus: a systematic review of high-quality randomized controlled trials. Antivir Ther. 2012. 17(2):255-64. [Medline].

  15. Pavan-Langston D. Herpes zoster antivirals and pain management. Ophthalmology. 2008 Feb. 115(2 Suppl):S13-20. [Medline].

  16. Murakami S, Honda N, Mizobuchi M, Nakashiro Y, Hato N, Gyo K. Rapid diagnosis of varicella zoster virus infection in acute facial palsy. Neurology. 1998 Oct. 51(4):1202-5. [Medline].

  17. 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. 2008 Jul 16. CD006852. [Medline].

  18. Benson CA, Kaplan JE, Masur H, Pau A, Holmes KK. Treating opportunistic infections among HIV-infected adults and adolescents: recommendations from CDC, the National Institutes of Health, and the HIV Medicine Association/Infectious Diseases Society of America. MMWR Recomm Rep. 2004 Dec 17. 53:1-112. [Medline].

  19. Harpaz R, Ortega-Sanchez IR, Seward JF. Prevention of herpes zoster: recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Recomm Rep. 2008 Jun 6. 57:1-30; quiz CE2-4. [Medline].

  20. Coulson S, Croxson GR, Adams R, Oey V. Prognostic factors in herpes zoster oticus (ramsay hunt syndrome). Otol Neurotol. 2011 Aug. 32(6):1025-30. [Medline].

  21. Ryu EW, Lee HY, Lee SY, Park MS, Yeo SG. Clinical manifestations and prognosis of patients with Ramsay Hunt syndrome. Am J Otolaryngol. 2012 May. 33(3):313-8. [Medline].

  22. Yeo SW, Lee DH, Jun BC, Chang KH, Park YS. Analysis of prognostic factors in Bell's palsy and Ramsay Hunt syndrome. Auris Nasus Larynx. 2007 Jun. 34(2):159-64. [Medline].

 
Previous
Next
 
Herpes zoster oticus. Image courtesy of Manolette Roque, MD, Ophthalmic Consultants Philippines Co, EYE REPUBLIC Ophthalmology Clinic.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.