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Journal of Dentistry Indonesia

Volume 30 Number 1 Article 8

4-28-2023

Ramsay Hunt Syndrome with Oral Findings: A Rare Case


İlknur Eninanç
Department of Oral and Maxillofacial, Faculty of Dentistry, Sivas Cumhuriyet University, Sivas, Turkey,
i.eninanc2@gmail.com

Büşra Şahin
Department of Oral and Maxillofacial, Faculty of Dentistry, Sivas Cumhuriyet University, Sivas, Turkey,
bbusrashn@outlook.com

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Recommended Citation
Eninanç, İ., & Şahin, B. Ramsay Hunt Syndrome with Oral Findings: A Rare Case. J Dent Indones.
2023;30(1): 62-66

This Case Report is brought to you for free and open access by the Faculty of Dentistry at UI Scholars Hub. It has
been accepted for inclusion in Journal of Dentistry Indonesia by an authorized editor of UI Scholars Hub.
Ramsay Hunt Syndrome with Oral Findings: A Rare Case

Cover Page Footnote


CONFLICT OF INTEREST None declared.

This case report is available in Journal of Dentistry Indonesia: https://scholarhub.ui.ac.id/jdi/vol30/iss1/8


Journal of Dentistry Indonesia 2023, Vol. 30, No. 1, 62-66
doi: 10.14693/jdi.v30i1.1367

CASE REPORT

Ramsay Hunt Syndrome with Oral Findings: A Rare Case

İlknur Eninanç*, Büşra Şahin


Department of Oral and Maxillofacial, Faculty of Dentistry, Sivas Cumhuriyet University, Sivas, Turkey
*Correspondence e-mail to: i.eninanc2@gmail.com

ABSTRACT
Ramsay Hunt syndrome (RHS) is a disease that is caused by the varicella-zoster virus and is characterized by
severe ear pain, auricular vesicular eruptions, and peripheral facial paralysis. Objective: The aim of this case
report is to provide information about the clinical findings and treatment process of RHS, which is a rare case and
may have oral findings and stress the importance of early diagnosis. Case Report: A 60-year-old male patient
had previously consulted an otolaryngologist and a family physician with complaints of vesicular eruptions in
the right ear auricle and on the mandible. The patient in whom a diagnosis could not be established presented to
the Department of Oral and Maxillofacial Radiology after exacerbated lesions. White plaque-like and ruptured
vesicular lesions were observed in the intraoral examination. All vesicular lesions were on one side of the face,
and the patient was referred to the dermatology clinic with the diagnosis of RHS. Facial paralysis fully recovered
in a short time after early diagnosis and treatment. It should be kept in mind that there may also be oral findings
in RHS, and a patient’s intraoral and extraoral examination findings should be evaluated together. Conclusion:
Early diagnosis and treatment are highly important in preventing complications such as permanent facial paralysis,
vestibulocochlear dysfunction, and hearing loss

Key words: facial paralysis, Ramsay Hunt syndrome, varicella-zoster virus, vesicular eruptions
How to cite this article: Eninanç İ, Şahin B. Ramsay Hunt Syndrome with oral findings: A rare case. J Dent
Indones. 2023;30(1):62-66

INTRODUCTION

Ramsay Hunt syndrome is a disease, which was immune system over 60 years of age.7,8 Patients with
defined by James Ramsay Hunt in 1907 and is RHS have weakened functions, such as wrinkling their
characterized by severe otalgia, vesicular eruptions forehead, raising their eyebrows, and closing their eyes,
around the ear, peripheral facial paralysis, and frequent because of the weakening of the facial motor muscles
vestibulocochlear dysfunction.1,2 This disease is also on the affected side.9 When patients are requested
known as herpes zoster oticus, and the varicella- to laugh or show their teeth, these movements are
zoster virus, a member of the herpes virus family, impossible on the affected side, and the face moves
is responsible for it. Ramsay Hunt syndrome (RHS) toward the healthy side.9 Oral lesions are in the regions
emerges with facial nerve involvement of the varicella- of trigeminal nerve involvement and mobile areas such
zoster virus by 1% ,3 and only 8% of these cases have as the buccal mucosa. The lesions progress toward
been stated to have vesicles both around the ears and the midline, and the overlying skin is also involved.
inside the mouth.4 Pain and vesicular eruptions occur Sometimes it can lead to the devitalization of teeth and
along the nerve that is affected by the reactivation related bone necrosis.10
of the virus.3 Chronic diseases that reduce immune
resistance, diabetes mellitus, hypertension, and the use With this case report, it is aimed to provide information
of immunosuppressive drugs cause the reactivation of about the clinical findings and treatment process of
the virus.5,6 It can be seen at any age, but its incidence RHS, which may also produce oral findings, and stress
increases over 60 years of age. This increase has been the importance of early diagnosis and treatment in
reported to be proportional to the increase in the preventing permanent damage.
incidence of the deficiency associated with the cellular

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CASE REPORT

A 60-year-old male patient with diabetes mellitus


presented to the Oral and Maxillofacial Radiology
Department due to lesions in his mouth and vesicular
eruptions on the skin tissue of his lower jaw. It was
learned from his anamnesis that the patient had
presented to the Department of Ear-Nose-Throat
(ENT) with a complaint of ear pain 4 days before and
that an ENT specialist had prescribed ciprofloxacin-
derived antibiotics (ciprof loxacin hydrochloride 1
g – 3 times a day 2 tablets) and corticosteroid ear
drops (dexamethasone 1 g – 3 times a day 1 tablet) Figure 1. Vesicular eruptions in the patient’s ear auricle and
upon diagnosing an external auditory canal infection. on his lower jaw.
After using drugs for two days, lesions appeared in the
patient’s mouth and on his face. Then, the patient was
referred to the dentist by the family physician, and the
general dental practitioner prescribed oral antiseptic
mouthwash (chlorhexidine gluconate) and analgesic.
The patient, whose complaints did not disappear and
whose lesions gradually intensified, presented to our
department as a last resort.

From the patient’s detailed anamnesis, it was learned


that he had pain in the ear and eye. In the patient’s
Figure 2. Lesions in the patient’s oral mucosa and on
extraoral examination, vesicular eruptions and local
his tongue.
crusts were seen in the right ear auricle, preauricular
region, external auditory canal and zygomatic region
in the right half of his head and on the right side of his
lower jaw (Figure 1).

In the intraoral examination of the patient with poor


oral hygiene, white plaque-like lesions on the right
lateral of the tongue and edema in the tongue papillae
were observed. There were ruptured vesicular lesions
in the right sublingual region, alveolar mucosa of the
lower jaw and the lower lip mucosa, and significant
edema on the right side of the lower lip. Lesions of the
Figure 3. Crusts that occurred in the ear, face, and on the
entire mucosa and skin of the examined patient were
right side of the lower jaw on the fourth day of treatment.
unilateral (Figure 2). Apart from the ear, all lesions
covering the right side of the mandible and the intraoral
region were asymptomatic. Oral valacyclovir (1g - 3x1) therapy was started
and administered for 7 days. First, his previous
The patient, who could perform eye movements and corticosteroid dose (dexamethasone 1 g - 3 times a
mouth opening and closing functions completely, had day 1 tablet) was increased, and then vitamins B1,
limitations in his tongue movements. The patient did B6, B12 (1g – 1 time a day 1 tablet) and non-steroidal
not have any complaints such as nausea, vomiting, and anti-inflammatory drugs were added to his treatment.
dizziness and did not use diabetes drugs. The patient Moreover, vesicular eruptions decreased after four
had no such family history, but he was a smoker. days, and crusts started to occur in the patient to whom
insulin therapy started to be administered (Figure 3).
The patient was referred to the Dermatology Clinic
with the preliminary diagnosis of RHS and then was The patient was discharged after one week, and facial
hospitalized in the Dermatology Clinic on the same day. paralysis emerged on the 12th day. The patient, who
In the laboratory findings, the patient‘s fasting blood had mild asymmetry at the lower lip corner on the
sugar (161 mg/dL) and white blood cell (WBC - 11.76 right side and limited tongue movements, also had
109/L) and C-Reactive protein (CRP- 111.36 mg/L) difficulty and pain in moving the right eyelid. The
values from hemogram tests were high, whereas his patient could fully close his eyes. He was assessed
sedimentation rate was normal (2 mm/h). His HIV, as grade 2 according to the House-Brackmann facial
HBV, and HCV serology tests were negative. paralysis grading system.11 In his examinations, HSV

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around the ear. Twelve days after the onset of eruptions


around the ear, facial paralysis occurred on the same
side, although valacyclovir was used. The occurrence of
paralysis after vesicular eruptions is a good prognostic
sign, and the antiviral drug use probably delayed facial
paralysis and reduced the House-Brackmann grade of
facial paralysis.

In patients with RHS, erythematous eruptions are


rarely observed in the ipsilateral anterior two-thirds of
the tongue or palate. The chorda tympani is a sensory
branch of the facial nerve related to taste in the anterior
two-thirds of the tongue.

This nerve anastomoses with the lingual and maxillary


nerves that enable the distribution of Ramsay Hunt
lesions. A damaged chorda tympani leads to taste
impairment in the anterior two-thirds of the affected
half of the tongue and reduction in tear, nose, and
Figure 4. Facial paralysis that developed 12 days after salivary secretions. Patients with oral lesions report
vesicular eruptions in the ear.
a burning sense or numbness in the relevant mucosal
areas.13 In this case, in which intraoral findings
were rarely observed, the patient did not have taste
IgG and VZV (varicella-zoster virus) IgM antibodies impairment and numbness. There were edemas in the
were positive (Figure 4). tongue papillae on the right tongue lateral, and the
patient’s tongue and alveolar mucosal surface were
The patient was again hospitalized in the Department covered with a plaque-like white layer. Ruptured
of ENT, and the IV administration of corticosteroid vesicular lesions were observed in the sublingual
(methylprednisolone 40 mg - 1 time a day 2 tablets) region, the lower lip mucosa, and the alveolar mucosa.
and antibiotic (sulbactam 1gr - 4 times a day 1 tablet), The patient had a significant edema on the right side
vitamins B1, B6, B12, and eye drops (0.5%/15 ml) with of the lower lip. These oral findings show that dentists
a moisturizing effect were started. The patient was should also have information about patients with RHS.
discharged again after seven days, and his crusts and
peripheral facial paralysis disappeared completely at In addition to vesicular lesions observed in RHS,
his next-month follow-up. which are helpful for establishing a diagnosis, various
neurological disorders such as tinnitus, hearing
loss, nausea and vomiting, vertigo, and nystagmus
DISCUSSION may also emerge.1,13 The factors that worsen the
prognosis of the disease include stress, chemotherapy,
In RHS, the time of vesicular eruptions is important in immunodeficiency, malnutrition, infection, diabetes,
prognostic terms. According to the retrospective study hypertension, and advanced age.14 In this case, it is
conducted by Coulson et al. on 101 patients,4 the first thought that the reduced immunity of the patient who
symptom was ear pain in 55% of patients, whereas did not use diabetes drugs prepared the ground for
vesicles and facial paralysis occurred 2-3 days later. secondary infection, with the effect of advanced age.
In 2% of patients, vesicles appeared first. Twenty-three In addition to the antiviral therapy administered to the
percent first had facial paralysis. Furthermore, vesicles patient, the patient‘s blood sugar was also taken under
were observed on the auricle in 86% of patients, inside control during the treatment. Moreover, except for
the mouth in 7%, and both around the ear and inside vesicular lesions, the patient had pain in the eye only
the mouth in 8%.10 when he had otalgia and facial paralysis. However, the
patient did not experience hearing loss and vertigo.
Another study stated that 46.5% of the vesicles in the This situation can be explained by the fact that the 8th
ear occurred together with facial paralysis, 19.3% cranial nerve and vestibulocochlear system were not
before paralysis, and 34.2% after paralysis. The affected owing to early treatment.
eruption of vesicles before paralysis was reported to
affect the prognosis of facial paralysis positively.12 In Bell‘s paralysis is the most frequent cause of facial
the same study, the sense of taste and lacrimation were paralysis and is an acute-onset, idiopathic and short-
reported to be impaired.12 term paralysis. RHS is the second most frequent cause
of acute peripheral facial paralysis following Bell‘s
In this case, who first had ear pain, vesicular lesions paralysis.15 Facial paralysis in RHS is more severe
inside the mouth emerged a few days after the eruptions compared to Bell‘s paralysis, and the recovery rate is

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lower. Of patients, 71% recover from Bell‘s paralysis antiviral and steroid therapies as soon as possible in
fully. In RHS, on the other hand, only 21% of patients elderly patients and patients with a chronic disease such
with complete loss of facial functions recover fully. In as diabetes will prevent permanent facial paralysis,
patients with RHS, this rate was 25% for mild sequelae vestibulocochlear dysfunction, and hearing loss.
and 26% for moderate sequelae.15 It is crucial to start
antiviral agents such as acyclovir, valacyclovir, and
famciclovir, particularly in the first 72 hours, in terms CONFLICT OF INTEREST
of the efficiency of the treatment.16,17 Along with
these drugs, non-steroidal anti-inflammatory drugs, None declared.
corticosteroids or tricyclic antidepressants can be used
to relieve herpetic neuralgia.18 In the patient diagnosed
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(Received May 22, 2022; Accepted February 7, 2023)

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