Ramsay Hunt Syndrome

Nyilo Purnami

, Cita Rosita Sigit Prakoeswa

, Damayanti Tinduh

Department of Otolaryngology, Faculty of Medicine, Universitas Airlangga, Dr. Soetomo General Hospital, Surabaya,

Indonesia

Department of Dermatology Venereology, Faculty of Medicine, Universitas Airlangga- Dr.Soetomo General Hospital,

Surabaya, Indonesia

Department of Medical Rehabilitation, Faculty of Medicine, Universitas Airlangga Dr.Soetomo General Hospital,

Surabaya, Indonesia

Keywords: Ramsay Hunt Syndrome, Varicella Zoster Virus, Audiogram Examination, Audiology.

Abstract: Background: Ramsay Hunt syndrome (RHS) is a rare disease that caused by a varicella zoster virus (VZV)

infection who common in the geniculate ganglion. RHS causes severe facial nerve damage compared to

Bell's palsy but there is an important condition to be evaluated i.e., damage to the vestibulocochlear nerve.

Objectives: To reports the cases that include diagnostic, therapeutic, and prognosis of RHS patients.

Methods: To observed the case reports from patients admitted to the hospital until patient returns. Case: A

29-year-old man with vertigo symptoms, a mouth congealed, perceived skin efflorescence of the right arm,

and hearing loss. Audiogram examination was obtained sensory neural hearing loss desktra with masking

bone conduction of 60 dB. Electromyography amplitude results in 70% of frontal muscles, 57% of

orbicularis oculi muscle, 40% of nasalis muscle, and 43% of the orbicularis oris. The patient received

corticosteroid therapy and performed facial massage. The patient improved after treatment for 2 months

with mild facial paralysis and hearing function within normal limits. Conclusions: The RHS patient that

observed by audiological examination needs to performed in order to prevent the progression of a threshold

reduction.

1 INTRODUCTION

Varicella-zoster virus (VZV) causes chickenpox is a

characteristic disease that characterized by skin

lesions distributed and mostly occurs in childhood

(John and Canaday, 2017). Several cases of VZV

infection were found in the ganglion geniculate. The

condition is commonly known as Ramsay Hunt

syndrome (RHS). Typical manifestations of RHS

consist of triad herpes eruption symptoms in the

outer ear, facial nerve paralysis, and ear pain (Chen

et al., 2016). The condition is caused by the

reactivation of VZV that had previously been

infected because of the decrease in immune system

(Park et al., 2015; John and Canaday, 2017).

RHS is a relatively rare case, this disease was

lasts for a short time and self-limited. Furthermore,

RHS causes a disturbance in the facial nerve (John

and Canaday, 2017; Kuya et al., 2017) and

vestibulocochlear nerves (Psillas et al., 2012). RHS

has a poor prognosis with 10-30% of patients have

residual symptoms (Kayakurt et al., 2014; Kuya et

al., 2017). Thus, a fixed diagnosis is required

through some tests. Audiological examination is

important to determine the condition of the

vestibulocochlearis nerves (Psillas et al., 2012) and

electromyography (EMG) examination was to

evaluate the facial nerve conditions (Xing et al.,

2013). The goal is to determine therapy and evaluate

the prognosis of RHS patients.

2 CASE

The subject of this study was a man (29 years) that

experienced right ear pain since a week ago, vertigo,

and decreased hearing. In the external aureus was

obtained in bulls and crusts, while in the external

acoustic meatus that has no secretions were found,

but the facial palsy was found. The patient had a

history of otorea at age 5 and recovered a year later.

Patient working in the fish storage area with room

Purnami, N., Prakoeswa, C. and Tinduh, D.

Ramsay Hunt Syndrome.

DOI: 10.5220/0008157903810384

In Proceedings of the 23rd Regional Conference of Dermatology (RCD 2018), pages 381-384

ISBN: 978-989-758-494-7

381

temperature below 20

C since 3 months and un-

denied of having chickenpox.

In the initial symptoms was appear in 10 days

ago before the patient had to go to a general

practitioner and was diagnosed with strep throat.

The patient also had control twice to the general

practitioner however it did not improve. Then the

patient went to Otolaryngology unit at Dr. Soetomo

General Hospital Surabaya, Indonesia. The initial

condition of the patient comes are shown figure 1.

Figure 1: The external auric conditions.

Examination of the right auricle was obtained a

picture of vesicles that partially dried and covered in

crust around the front concha. Meatus acoustic

externus was obtained the hyperemia and cicatrik.

While, Weber test was obtained by conduction of

sinusra and examination of audiogram obtained

sensory neural hearing loss desktra with masking

bone conduction of 60 dB. On examination of

tympanometry was obtained a results within normal

limits. Then, patients were diagnosed with ramsay

hunt syndrome and received dexamethasone therapy

1 mg per 8 hours and dicloxacillin 250mg per 8

hours.

Figure 2: Graph examination of facial muscles.

The results of EMG examination were obtained a

ratio of 70% frontalis amplitude, 57% orbicularis

oculi, nasalis 40%, and oris 43% orbicularis.

Electrodiagnosis was showed a reversible prognosis

and patients were given the facial massage. The

results of examination of the facial muscles are

shown in Figure 2.

The second week of the patient was eximined by

weber test 3 times that leads to the left and 2 in the

middle. Audiogram examination of the light-

conductive dexterous deafness was dexogram with

the masking of the left bone conduction of desktra

70 dB at a frequency of 4000 Hz. Tympanometry

within normal limits and positive left and left

stapedius reflexes. The patients also complained

uncomfortably in the eyes and performed Schirmer

tests (Table 1). Then, the patients also given the

dexamethasone therapy 1 mg per 12 hours and

added neurotrophic up to week six that showed good

prognosis.

3 DISCUSSION

The VZV infection of the geniculate ganglion is

usually called RHS. The condition is a rare case, so

it is often overlooked in diagnosis and therapy.

Detailed clinical examination was obtained a typical

set of symptoms of herpes eruption in the ear, ear

pain, and facial nerve paralysis that easily found in

early stage to prevent complications (Chen et al.,

2016; Kuya et al., 2017).

Herpes Zoster infection could occur due to viral

reactivation after previous varicella infections (Kuya

et al., 2017). However, in this case was denied that

the patient ever getting infected by Herpes Zoster

before. One possible factor triggering reactivation of

viral infections was extreme room temperature

because the patient works in an air-conditioned room

with temperatures below 200 C. These conditions

possibility lower the individual's immune system if it

was exposed continuously (Park et al., 2015; John

and Canaday, 2017).

The condition of oblique mouth and stiffness on

the right face is caused by an infection that attacks

the nerves vii (facial nerves) and decreased hearing

(The vestibulocochlear nerve). The condition was

complained by RHS patients by 84.6% (Zainine et

al., 2012) and if not managed properly it possibility

causes a persistent symptoms as much as 12%

(Kayakurt et al., 2014). The identified herpes

eruption is the usual stage of crustation that

occurring at 1-2 weeks after infection (Avci and

Ertam, 2014). Thus, the Tzanck smear cytological

examination was not carried out with such

considerations (Riahi et al., 2014).

01234

Frontalis

Orbicularis

Oculi

Corrugulator

Nasalis

Zigomatikus

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Table 1: The results of Schirmer test.

Wee

II III IV

Schirmer

test

Different >

50%

Different >

50%

Different

< 25%

The results of the audiogram examination was

obtained that sensory neural hearing loss desktra in

the first week and re-examination on the second

week that obtained a deaf conduction dexter sinistra.

This neurological disorder was caused by

inflammation of the geniculate ganglion thus

suppressing the cranial nerve of the nerves VIII

(Godani et al., 2013; Kuya et al., 2017). The

improvement was caused by a decrease in

compression of nerves VIII. The EMG examination

of the amplitude illness/healthy were ranged from

40-70%, that estimated the lower the amplitude ratio

than the muscle contractures then more visible and

the prognosis was getting worse (Xing et al., 2013).

Antiviral (acyclovir) was not given with the

consideration of patients who coming a week after

VZV infection and did not show any

immunocompromised symptoms. Also, thus given

dexamethasone at a dose of 3 times 1 mg (Avci and

Ertam, 2014; Chen et al., 2016; Kuya et al., 2017).

The treatment was given for 2 weeks and gradually

decreased the dose and the patient also gets face

massage. Massage proved capable of blood

circulation, so the cells more rapid regeneration and

expected facial paralysis can be minimized (Field,

2014). The second week the patient were

complained of blurred vision and examination, eye

condition within normal limits.

Postherpetic neuralgia complications were a

common complication by 10-30% (Kayakurt et al.,

2014; Kuya et al., 2017). It is necessary to take

preventive steps in the case of RHS which is the

determination of accurate diagnosis and follow

prognosis by using EMG. The prognosis in this case

was good which shows an amplitude ratio above

25% (Xing et al., 2013).

4 CONCLUSION

RHS is a rare occurrence with a characteristic triad

of symptoms. In this case, the patients come to

health services a week after the appearance of

paralysis facial and hearing loss. Patients receive

corticosteroid, neurotrophic and facial massage

therapy without antiviral therapy. A few weeks after

the therapy the patient has improves their facial

muscles and audiology functions. As a preventive

measure to prevent the occurrence of RHS

complications required audiological diagnosis and

facial paralysis.

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