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].
Entire Body System
- 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]
On hospital day three, she developed fever; antibiotics were switched to ampicillin and cefepime. [afmr.org]
Call your doctor at once if you have: fever, swollen glands, sore throat, flu symptoms; breathing problems; or severe or painful skin rash. [drugs.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]
- Malaise
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]
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]
Gastrointestinal
- 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 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]
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]
J Ramsay Hunt, who described various clinical presentations of facial paralysis and rash, also recognised other frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus. [ncbi.nlm.nih.gov]
Skin
- 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]
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]
- 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]
Ears
- Tinnitus
Other symptoms may include hearing loss, vertigo (abnormal sensation of movement), and tinnitus (abnormal sounds). [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]
- 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]
- 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]
Neurologic
- 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
(From Adams et al., 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 [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]
Benign Paroxysmal Positional Vertigo is intense dizziness. Read about Benign Paroxysmal Positional Vertigo [evidence.nhs.uk]
In the presented cases, VZV infection was complicated byfacial paralysis and dizziness, partial hearing loss and erysipelas in onepatient. [journaldatabase.info]
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]
- 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]
The syndrome is most commonly associated with VIIth cranial nerve palsy but may involve other cranial nerves as well, as seen in the present case where V, VII, VIII, IX and X cranial nerve were involved. [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]
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].
Treatment
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]
Prognosis
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]
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]
Etiology
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]
Epidemiology
Epidemiology References:[1][2] Epidemiological data refers to the US, unless otherwise specified. [amboss.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]
Methods: We discuss the epidemiology, diagnosis and treatment of RHS using a case report of a cancer patient who presented with disseminated VZV reactivation, including RHS. [tandfonline.com]
Serologic and epidemiologic data strongly suggest that VZV represents the reactivation of a latent virus rather than reinfection. [otologytextbook.net]
Pathophysiology
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]
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]
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]
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
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]
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]
Early administration (< 72 h) of acyclovir showed an increased rate of facial nerve function recovery and prevented further nerve degeneration. [emedicine.medscape.com]
Fortunately, there is another vaccine, Zostavax, which is helpful in preventing viral reactivation. Consequently, shingles and Ramsay Hunt syndrome can be either prevented or their symptoms reduced if the vaccine is administered. [medicinenet.com]
References
- Gondivkar S, Parikh V, Parikh R. Herpes zoster oticus: A rare clinical entity. Contemp Clin Dent. 2010;1(2):127-129.
- Wagner G, Klinge H, Sachse MM. Ramsay Hunt syndrome. J Dtsch Dermatol Ges. 2012;10(4):238-244.
- 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.
- Sampathkumar P, Drage LA, Martin DP. Herpes Zoster (Shingles) and Postherpetic Neuralgia. Mayo Clin Proc. 2009;84(3):274-280.
- 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.
- 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.
- 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.