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Herpes Zoster Oticus

Ramsay Hunt Syndrome Type 2

Herpes zoster oticus, a term denoting the infection of the ear by varicella zoster virus (VZV), is one of the many clinical types of VZV described in the literature. In addition to ear pain and the characteristic vesicular eruptions on the skin, herpes zoster oticus frequently manifests with facial nerve palsy, and the term Ramsey Hunt syndrome is often used as a synonym. The diagnosis is made primarily on the basis of clinical criteria.


Presentation

Herpes zoster oticus (HZO) is one of the clinical syndromes which develops after the initial colonization of varicella virus in the ganglia of the cranial nerves [1] [2]. Defined as an infection of the ear that appears after reactivation of VZV from the geniculate ganglion, this clinical entity is now a rare occurrence in clinical practice, and is predominantly encountered among elderly individuals [1] [3] [4]. The clinical presentation comprises of a typical rash in the form of erupting vesicles in the external auditory canal, the eardrum, pinna, and cavum conchae, whereas otalgia (ear pain that is described as severe), tinnitus, vertigo, and even hearing impairment are reported [1] [2] [4] [5] [6]. Higher frequencies are more severely affected than lower frequencies, and in a number of patients, hearing loss can be severe enough to result in deafness [4] [5]. Paresis of the facial nerve on the ipsilateral side (manifesting as unilateral facial paralysis and asymmetry of the face) quite often accompanies the typical features of herpes zoster oticus and the term Ramsay Hunt syndrome (named after the first physician who described the condition) is used for this condition in the literature [2] [5] [6]. Other notable symptoms are nausea, vomiting, nystagmus, and distribution of lesions in the oral cavity, while pain may spread throughout the face and neck area [1] [2].

Fever
  • It is usually a concomitant of fever, but may also develop in situations of other stresses without fever or prior illness. The virus is carried by most people but usually lies quiescent.[medical-dictionary.thefreedictionary.com]
  • These early symptoms can include: a headache; burning, tingling, numbness or itchiness of the skin in the affected area; a feeling of being generally unwell and fever Eventually, most people with shingles experience a localised "band" of pain in the affected[humanitas.net]
  • Additional symptoms may include abdominal pain, chills, fever, joint pain, taste and vision problems, headaches or even loss of eye motion or a drooping eye.[ozarkderm.com]
  • Haug Browse recently published Learning/CME Learning/CME View all learning/CME CME Case 3-2019: A 70-Year-Old Woman with Fever, Headache, and Progressive Encephalopathy Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura Randomized[nejm.org]
Malaise
  • Other symptoms include malaise, fever, and anorexia. There also can be involvement of neural structures and the manifestation of neurologic symptoms.[medical-dictionary.thefreedictionary.com]
  • Clinical Presentation: Patients with Ramsay Hunt syndrome typically present with fever, malaise, tinnitus, vertigo, nystagmus, hearing loss, peripheral facial nerve palsy, and a painful vesicular rash with burning blisters around the ear and on the face[ajnr.org]
  • In addition the patient may have general signs of a viral infection, including fever, headache and general malaise.[physio-pedia.com]
  • Symptoms HZO begins with flu-like symptoms including fever, Myalgia, and malaise for nearly a week.[athena.targetwoman.com]
Rigor
  • However, until this procedure is subjected to a rigorous, controlled trial comparing it with maximal medical therapy, it is difficult to justify the very high costs and risk.[ncbi.nlm.nih.gov]
Nausea
  • Other notable symptoms are nausea, vomiting, nystagmus, and distribution of lesions in the oral cavity, while pain may spread throughout the face and neck area. With a thorough clinical workup, herpes zoster oticus can be easily diagnosed.[symptoma.com]
  • […] are accompanied by: Unilateral facial palsy (VZV may overlap CN 7 & 8) Vesicles on external ear or in ear canal Combined Ear Pain and Vertigo If Dx secure & pt If no telltale findings as above, it mimics Acute Labyrinthitis Sudden onset vertigo, often nausea[diagnosisdude.com]
  • Other symptoms may include hearing loss , vertigo (feeling that the room is spinning), tinnitus (hearing abnormal sounds), nausea, vomiting, loss of taste in the tongue, and dry mouth and eyes. [1] [2] Some cases of herpes zoster oticus do not require[rarediseases.info.nih.gov]
  • Similarly as the result of involvement of the vestibular nerve there are cases of vertigo, associated with nausea and vomiting and nystagmus. This disease is not rare and we could find several reported cases in Korea.[kjorl.org]
  • 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.[slideshare.net]
Regurgitation
  • He also gave history of associated nasal regurgitation and ringing sensation in his left ear. Patient also complained of giddiness on the day of his presentation to us.[medresearch.in]
Jaundice
  • He had cervical lymphadenopathy and did not have any pallor, clubbing and jaundice.[biopublisher.ca]
Dental Abscess
  • Referred pain (eg, dental abscess ). Carcinoma of the nasopharynx. Investigations Virological studies, both serological and molecular, are available but usually the diagnosis is clinical. Audiometry may be performed.[patient.info]
  • Other conditions are viral labyrinthitis, possibly a stroke of the posterior inferior cerebellar artery region, -trigeminal neuralgia, postherpetic neuralgia, persistent idiopathic facial pain and temporomandibular disorders, referred pain (dental abscess[biopublisher.ca]
Vesicular Rash
  • It presents classically with severe otalgia, a vesicular rash in the Concha or on the Pinna of the affected ear in association with a lower motor neurone lesion of the homolateral facial nerve.[ncbi.nlm.nih.gov]
  • For example, it can be misdiagnosed as a kissing bug bite (which raises the spectre of Chagas disease), based on the presence of vesicular rash by the lip.[hearinghealthmatters.org]
  • Subsequent reactivation of latent VZV can result in localized vesicular rash, known as herpes zoster.[medifitbiologicals.com]
  • On Otological examination, we noticed multiple vesicular rashes on the left Pinna, Pre-auricular and Post-auricular region and the external auditory canal. The tympanic membrane on the left side was congested.[medresearch.in]
Skin Patch
  • Other treatments may be given to people who have prolonged residual pain (called postherpetic neuralgia) such as medicated skin patches, anticonvulsants, or tricyclic antidepressants.[msdmanuals.com]
Eczema
  • […] most striking is the appearance of clean linear erosions in skin creases, with the appearance of a knife cut. [ 8 ] Herpetic sycosis Herpetic sycosis is a recurrent or initial herpes simplex infection affecting primarily the hair follicle. [ 9 ] :369 Eczema[enacademic.com]
  • One of the most striking is the appearance of clean linear erosions in skin creases, with the appearance of a knife cut. [15] Herpetic sycosis is a recurrent or initial herpes simplex infection affecting primarily the hair follicles. [16] :369 Eczema[ipfs.io]
Tinnitus
  • Other symptoms may include hearing loss, vertigo (abnormal sensation of movement), and tinnitus (abnormal sounds). Taste loss in the tongue and dry mouth and eyes may also occur.[ninds.nih.gov]
  • This may occasionally be associated with tinnitus, vertigo, deafness, severe otalgia, and inflammation of the pinna.[mesh.kib.ki.se]
  • The clinical presentation comprises of a typical rash in the form of erupting vesicles in the external auditory canal, the eardrum, pinna, and cavum conchae, whereas otalgia (ear pain that is described as severe), tinnitus, vertigo, and even hearing impairment[symptoma.com]
  • […] facial palsy (VZV may overlap CN 7 & 8) Vesicles on external ear or in ear canal Combined Ear Pain and Vertigo If Dx secure & pt If no telltale findings as above, it mimics Acute Labyrinthitis Sudden onset vertigo, often nausea/vomiting Hearing loss, tinnitus[diagnosisdude.com]
Otalgia
  • Common presenting symptoms include cutaneous auricular vesicles, severe otalgia, inflammation of the pinna, and occasionally unilateral sudden facial paralysis.[ncbi.nlm.nih.gov]
  • Case Report A 65-year-old male presented with severe otalgia in the right ear continuous for 5 days. He complained of otorrhea, hoarseness and difficulties in swallowing after the onset of otalgia. He denied hearing loss or vertigo initially.[ejao.org]
  • The clinical presentation comprises of a typical rash in the form of erupting vesicles in the external auditory canal, the eardrum, pinna, and cavum conchae, whereas otalgia (ear pain that is described as severe), tinnitus, vertigo, and even hearing impairment[symptoma.com]
  • Contemporary clinical dentistry 2010 DOI: 10.4103/0976-237X.68588 Herpes zoster oticus also known as Ramsay Hunt syndrome is a rare complication of herpes zoster in which reactivation of latent varicella zoster virus infection in the geniculate ganglion causes otalgia[semanticscholar.org]
Hearing Impairment
  • Hearing impairment is more severe in patients with vertigo than in those without vertigo in both the high and low frequency ranges, even though the degree of hearing impairment is not significantly different between patients with and without facial palsy[ncbi.nlm.nih.gov]
  • The clinical presentation comprises of a typical rash in the form of erupting vesicles in the external auditory canal, the eardrum, pinna, and cavum conchae, whereas otalgia (ear pain that is described as severe), tinnitus, vertigo, and even hearing impairment[symptoma.com]
  • […] of hearing impairment was not significantly different between patients with and without facial palsy.[journals.lww.com]
  • impairment, etc, is called as Ramsay Hunt syndrome or Herpes Zoster Oticus.[kjorl.org]
Lesion of the Pinna
  • In 1907, Ramsay Hunt described facial nerve palsy associated with ipsilateral hearing loss and vesicular lesions on the pinna or in the auditory canal. 1 Facial weakness with associated vesicles establishes the diagnosis of Ramsay Hunt syndrome. 3 The[jamanetwork.com]
Referred Otalgia
  • Coatesworth, Referred otalgia: a structured approach to diagnosis and treatment, International Journal of Clinical Practice, 61, 6, (1015-1021), (2007). Imran Rahman and S.[doi.org]
Vertigo
  • Most patients with vertigo also had hearing loss (28 of 30), and patients without hearing loss did not have vertigo (19 of 21).[ncbi.nlm.nih.gov]
  • Vertigo may be treated with the drug diazepam Prognosis Generally, the prognosis of herpes zoster oticus is good. However, in some cases, hearing loss may be permanent. Vertigo may last for days or weeks.[ninds.nih.gov]
  • […] onset vertigo, often nausea/vomiting Hearing loss, tinnitus Need to R/O Stroke (see Acute Labyrinthitis ) See posting Vertigo – 2.[diagnosisdude.com]
Neuralgia
  • ., Principles of Neurology, 6th ed, p757) Svenska synonymer Herpes inom huvud-halsområdet — Ramsey Hunt artikulärt syndrom Engelska synonymer Ramsay Hunt Auricular Syndrome — Neuralgia, Geniculate — Geniculate Neuralgia — Geniculate NeuralgiasNeuralgias[mesh.kib.ki.se]
  • Geniculate Neuralgias Herpes Zoster Auricularis Herpes Zoster Cephalicus Herpes Zoster, Geniculate Herpetic Geniculate Ganglionitides Herpetic Geniculate Ganglionitis Neuralgia, Geniculate Neuralgias, Geniculate Ramsay Hunt Auricular Syndrome Ramsay[decs.bvs.br]
  • , Geniculate; Geniculate Herpes Zoster; Syndrome, Ramsay Hunt; Neuralgias, Geniculate; Herpetic Geniculate Ganglionitides; Herpes Zoster, Geniculate; Geniculate Neuralgias; Geniculate Neuralgia; Geniculate Ganglionitis, Herpetic; Geniculate Ganglionitides[reference.md]
  • , and nervus intermedius neuralgia.[acronymfinder.com]
  • Nervus intermedius neuralgia Overview Herpes zoster oticus is a common complication of shingles , an infection caused by the varicella-zoster virus (which is the virus that also causes chickenpox ).[rarediseases.org]
Dizziness
  • Key Words: Herpes zoster oticus, Vestibular nerve, Vertigo, Dizziness Keywords: Herpes zoster oticus ; Vestibular nerve ; Vertigo ; Dizziness[e-rvs.org]
  • However, when we found the aspect of dizziness is a different kind from peripheral vertigo, we thought the dizziness was derived from ocular nervous system lesions that can also be involved by HZO2.[cyberleninka.org]
  • If you are experiencing dizziness associated with herpes zoster oticus, physicians typically prescribe the drug diazepam. Your doctor can determine which treatment is right for you.[sharecare.com]
  • Dizziness, Hearing Loss, and Tinnitus. F.A. Davis Company. 1998. pages 150-151.[meducator3.net]
  • This is characterized by blisters around the opening of the ear and is often associated with hearing loss and/or dizziness. In addition to corticosteroids, anti-herpes viral drugs are usually prescribed.[nyee.edu]
Cranial Nerve Involvement
  • In neurologic examination there was revealed 6th, 7th, 8th, 9th, and 10th cranial nerve involvement on the left.[tjn.org.tr]
  • History Meanings of "multiple cranial nerve involvement caused by herpes zoster oticus" in Turkish English Dictionary : 1 result(s) Category English Turkish Medical 1 Medical multiple cranial nerve involvement caused by herpes zoster oticus herpes zoster[tureng.com]
  • Sun WL in 2011 reported another case with V VII, VII, IX and X cranial nerve involvement. Sugita-Kitajima A in 2009 also reported a case with VII, VIII, IX and X cranial nerve involvement.[medresearch.in]
  • Herpes zoster cranial neuropathy (or polyneuropathy) without skin lesions, subcategorized by the cranial nerves involved. Group 3.[medlink.com]
  • Herpes zoster infection of the larynx accompanied by RHS with cranial polyneuropathy is extremely rare, with only few reported cases in the literature. 2, 3, 4, 5) In our case, the patient presented with multiple cranial nerve involvements without facial[kjorl.org]
Lower Motor Neurone Lesion
  • It presents classically with severe otalgia, a vesicular rash in the Concha or on the Pinna of the affected ear in association with a lower motor neurone lesion of the homolateral facial nerve.[ncbi.nlm.nih.gov]
  • It has variable presentation which may include a lower motor neuron lesion of the facial nerve, deafness, vertigo, and pain. TRIAD of Ramsay Hunt syndrome type II Ipsilateral Facial Paralysis Ear Pain Vesicles in the auditory canal and auricle.[slideshare.net]

Workup

With a thorough clinical workup, herpes zoster oticus can be easily diagnosed. Firstly, a detailed patient history should assess the presenting complaints, their duration, as well as severity, but the role of a properly conducted physical examination is perhaps of essential importance. A full inspection of the auditory canal and the face is sufficient to identify vesicular rash that is highly suggestive of a VZV infection. Furthermore, if facial paralysis is suspected, a neurological examination including the assessment of cranial nerves will detect features of facial nerve palsy [2]. Weakness of the muscles of facial expression supplied by the facial nerve will manifest as a drooping lower lid, incomplete eye closure, reduced movement of the lip and the forehead, and facial asymmetry [2]. Thus, a combination of clinical findings is usually enough to make the diagnosis of herpes zoster oticus or Ramsay Hunt syndrome, but in some patients, the findings may be nonspecific and microbiological studies should be performed in those cases. Detection of high antibody titers is a useful initial method, but testing of samples obtained from swabs or ear fluid by polymerase chain reaction (PCR) is a highly recommended diagnostic tool that carries a high rate of success [1] [6] [7].

Pseudomonas
  • Clinical Neurosciences, University of Cambridge, Cambridge, UK Correspondence to Harriet A Cunniffe, harriet.cunniffe{at}gmail.com Abstract This is a case of an 85-year-old woman whom was admitted with otalgia and an abducens nerve palsy alongside a Pseudomonas[casereports.bmj.com]

Treatment

  • Delay of treatment may result in permanent facial nerve paralysis.[en.wikipedia.org]
  • OBJECTIVES: To determine the effectiveness of antiviral agents in the treatment of adult patients with Ramsay Hunt syndrome (HZO with facial palsy).[ncbi.nlm.nih.gov]
  • Treatment Some cases of herpes zoster oticus do not require treatment. When treatment is needed, medications such as antiviral drugs or corticosteroids may be prescribed.[ninds.nih.gov]

Prognosis

  • We found that the results of measuring tear production, submandibular salivary flow, the response to maximal stimulation, and evoked electromyography gave us sufficient information to group these patients according to prognosis--either unfavorable or[ncbi.nlm.nih.gov]
  • Vertigo may be treated with the drug diazepam Prognosis Generally, the prognosis of herpes zoster oticus is good. However, in some cases, hearing loss may be permanent. Vertigo may last for days or weeks.[ninds.nih.gov]

Etiology

  • 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 ETIOLOGY Individuals with decreased cell-mediated immunity resulting from carcinoma, radiation therapy[slideshare.net]
  • If there is any question about viral etiology, vesicular scrapings may be collected for direct immunofluorescence or for viral cultures, and MRI is done to exclude other diagnoses.[msdmanuals.com]

Epidemiology

  • Serologic and epidemiologic data strongly suggest that VZV represents the reactivation of a latent virus rather than reinfection.[otologytextbook.net]
  • Although Pavan-Langston, Deborah MD, FACS Neurology December 01, 1995 The global epidemiology of herpes zoster ...Varicella- zoster virus (VZV) is a ubiquitous, highly neurotropic, exclusively human α-herpesvirus.[aan.com]
  • Epidemiology References: [1] [2] Epidemiological data refers to the US, unless otherwise specified.[amboss.com]
  • Epidemiology Herpes zoster is seen as a disease of older people (most commonly over 60 years old). [ 1 ] However, it can affect all ages, although is rare in children.[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • Molecular and Systemic 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 zoster oticus.[link.springer.com]
  • VZV infection or reactivation involving the geniculate ganglion of CN VII within the temporal bone is the main pathophysiological mechanism of Ramsay Hunt syndrome.[medifitbiologicals.com]
  • Pathophysiology [ edit ] Ramsay Hunt syndrome type 2 refers to shingles of the geniculate ganglion. After initial infection, varicella zoster virus lies dormant in nerve cells in the body, where it is kept in check by the immune system.[en.wikipedia.org]

Prevention

  • [ edit ] Shingles is prevented by immunizing against the causal virus, varicella zoster, for example through Zostavax, a stronger version of chickenpox vaccine.[en.wikipedia.org]
  • Prevention There is no known way to prevent Ramsay Hunt syndrome, but treating it with medicine soon after symptoms develop can improve recovery. References Brant JA, Ruckenstein MJ. Infections of the external ear.[nyee.edu]
  • Early administration ( 72 h) of acyclovir showed an increased rate of facial nerve function recovery and prevented further nerve degeneration.[emedicine.medscape.com]

References

Article

  1. Gondivkar S, Parikh V, Parikh R. Herpes zoster oticus: A rare clinical entity. Contemp Clin Dent. 2010;1(2):127-129.
  2. Wagner G, Klinge H, Sachse MM. Ramsay Hunt syndrome. J Dtsch Dermatol Ges. 2012;10(4):238-244.
  3. Yawn BP, Saddier P, Wollan PC, St Sauver JL, Kurland MJ, Sy LS. A population-based study of the incidence and complication rates of herpes zoster before zoster vaccine introduction. Mayo Clin Proc. 2007;82(11):1341-1349.
  4. Sampathkumar P, Drage LA, Martin DP. Herpes Zoster (Shingles) and Postherpetic Neuralgia. Mayo Clin Proc. 2009;84(3):274-280.
  5. Kim CH, Choi H, Shin JE. Characteristics of hearing loss in patients with herpes zoster oticus. Lin. Y-S, ed. Medicine (Baltimore). 2016;95(46):e5438.
  6. Gilden D, Cohrs RJ, Mahalingam R, Nagel MA. Neurological Disease Produced by Varicella Zoster Virus Reactivation Without Rash. Curr Top Microbiol Immunol. 2010;342:243-253.
  7. Espy MJ, Teo R, Ross TK, et al. Diagnosis of Varicella-Zoster Virus Infections in the Clinical Laboratory by LightCycler PCR. J Clin Microbiol. 2000;38(9):3187-3189.

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Last updated: 2019-07-11 21:03