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]

• Nausea

  […] 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]

• 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]

• Lagophthalmos

  Surgical Treatments If lagophthalmos persists and corneal damage is not amenable to medical management, patients could seek surgical treatments such as tarsorrhaphy, gold weight implantation, or lateral tarsal strip. [eyewiki.aao.org]

• 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]

• Eczema

  […] en Herpes viral infections (herpes simplex dermatitis [ eczema herpeticum ], herpes simplex [ cold sores ], Kaposi s varicelliform eruption en Pharmaceutical preparations with antiviral and/or antimicrobial properties for use in the treatment of herpetic [glosbe.com]

  Eczema herpectium is a generalized eruption caused by herpes infection in children suffering from eczema. Vagbhata Visarpa mainly straps from the affected area with wide spread ulceration. [drchethansblog.blogspot.com]

  […] 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]

• 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]

  Other treatments may be given to people who have prolonged residual pain (called postherpetic neuralgia) such as medicated skin patches, antiseizure drugs, or tricyclic antidepressants. [merckmanuals.com]

• 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]

  Other symptoms may include hearing loss, vertigo (abnormal sensation of movement), tinnitus (abnormal sounds), taste loss in the tongue, dry mouth, and dry eyes. [sharecare.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]

• 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]

  In this case, the patient presented with isolated vestibular dysfunctions without facial palsy and hearing impairment, and these findings were confirmed by electrophysiologic studies including video head impulse testing. [koreamed.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]

  […] of hearing impairment was not significantly different between patients with and without facial palsy. [journals.lww.com]

• Benign Paroxysmal Positional Vertigo

  Benign Paroxysmal Positional Vertigo is intense dizziness. Read about Benign Paroxysmal Positional Vertigo [evidence.nhs.uk]

  paroxysmal positional vertigo secondary to inner ear disease. 61 Lee NH...Kim SM 20723780 2010 35 Herpes zoster oticus: A rare clinical entity. 61 Gondivkar S...Parikh R 22114399 2010 36 The management of peripheral facial nerve palsy: "paresis" versus [malacards.org]

  Paroxysmal Positional Vertigo Persistent idiopathic facial pain Prompt diagnosis and initiation of steroids and antiviral are the current recommendations. [eyewiki.aao.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]

• 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]

  Benign Paroxysmal Positional Vertigo is intense dizziness. Read about Benign Paroxysmal Positional Vertigo [evidence.nhs.uk]

  Keywords: Herpes zoster oticus; Vertigo; Hearing loss [e-rvs.org]

• 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 Neuralgias — Neuralgias [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]

  Additionally, it has been found to decrease the incidence of postherpetic neuralgia. [clinmedjournals.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]

  Cardiology (Cardiovascular Disease) If you have herpes zoster oticus, you may develop any of the following symptoms: severe ear pain a rash on the skin around the ears, mouth, face, or neck, or on your scalp paralyzed nerves in the face hearing loss dizziness [sharecare.com]

  Benign Paroxysmal Positional Vertigo is intense dizziness. Read about Benign Paroxysmal Positional Vertigo [evidence.nhs.uk]

• 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]

• Hyperesthesia

  In the second patient, left-sided facial palsy was preceded by acute pharyngalgia and pain of the left ear with skin hyperesthesia and vesicles in the left external auditory canal. [termedia.pl]

  It is the most frequent complication, observed in 9-45% of all cases. [4] Most people report a deep burning or aching pain, paresthesia, dysesthesia, hyperesthesia, or electric shock–like pains. [emedicine.medscape.com]

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].

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]

[…] of recovery decreasing as treatment was delayed. [en.wikipedia.org]

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]

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 type 2 is caused by reactivation of varicella zoster virus in the geniculate ganglion. Risk Factors Immunocompromised state, lack of varicella vaccination. [eyewiki.aao.org]

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]

English: BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications CN congenital DI diagnosis DG diagnostic imaging DH diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology EH ethnology ET etiology [decs.bvs.br]

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. [merckmanuals.com]

English: 92 Allowable Qualifiers English: BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications CN congenital DI diagnosis DG diagnostic imaging DH diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology [decs.bvs.br]

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]

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]

Pathophysiology Reactivation of varicella-zoster virus involving the geniculate ganglion is the main cause of Ramsay Hunt Syndrome Type 2. There are three theories on the pathophysiology of the polyneuropathy nature of Ramsay Hunt Syndrome. [eyewiki.aao.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]

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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.