International Journal of Clinical Case Reports 2015, Vol.5, No.

12, 1-5
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Research Report Open Access

Herpes Zoster Oticus in a 12 Year Old Child and Review of Literature - A Case
Report
Menon Narayanankutty Sunilkumar , Narendran Gayathrivarma, Vadakut Krishnan Parvathy
Amala Institute of Medical Sciences, Amala Nagar, Kerala, India
Corresponding author email: sunilsree99@gmail.com
International Journal of Clinical Case Reports, 2015, Vol.5, No.12 doi: 10.5376/ijccr.2015.05.0012
Received: 4 Dec., 2014
Accepted: 4 Jan., 2015
Published: 28 Feb., 2015
Copyright 2015 Sunilkumar et al., This is an open access article published under the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Preferred citation for this article:
Sunilkumar et al., 2015, Herpes Zoster Oticus in a 12 year old child and review of literature - A case report, International Journal of Clinical Case Reports,
Vol.5, No. 12 1-3 (doi: 10.5376/ijccr.2015.05.0012)

Abstract The enigma of Ramsay Hunt syndrome is that it is an association of an acute peripheral facial neuropathy and herpetic
vesicular rash of the skin of the ear canal, auricle (Herpes Zoster Oticus) and/or mucous membrane of the mouth. Paediatricians see
many children with vesiculous eruptions over the face and the body. A combination of otalgia and cutaneous / mucosal eruptions is
not very uncommon in these children. Facial palsy can result if a proper history of a varicella zoster infection is not identified,
diagnosed with delay and if appropriate measures not taken in such a child. In this case study, we report 12 year-old boy who
presented with Herpes Zoster Oticus.
Keywords Ramsay Hunt Syndrome; Herpes Zoster Oticus; Varicella-Zoster Virus; Facial Nerve Palsy; Acyclovir

Introduction order to provide the adequate nursing care and to start

James Ramsay Hunt, an eminent American
neurologist in 1907, first described a syndrome of
otalgia associated with coetaneous and mucosal rashes
in his patient (Louis and Williams, 2003). He
postulated that it is a rare complication of latent
infection of the geniculate ganglion by human
herpesvirus 3 ie Varicella-Zoster Virus (VZV) (Hunt,
1907). This infection gives rise to vesiculation and
ulceration of the external ear and ipsilateral anterior
two thirds of the tongue and soft palate, as well as
ipsilateral facial neuropathy (in 7th cranial nerve).
RHS is HZO, or auricular herpes zoster with acute
facial palsy and is also known as geniculate neuralgia
or nervus intermedius neuralgia. It is a self-limiting
disease and the morbidity mainly results from facial
weakness (Hunt, 1907; Louis and Williams, 2003).
RHS has been reported in a case of varicella infection
in utero and presentation in infancy at 3 months age
and also in an 82 year old woman (Aframian, 1999;
Balatsouras et al., 2007). The incidence of RHS in
paediatric age group below 10 years of age is about
3/100.000 and so should be considered as a
differential diagnose in any child with atraumatic
facial palsy (Bleicher et al., 1996). The importance of
being aware of the syndrome is very crucial, both in
1
treatment with antiviral agents immediately to prevent
RHS with facial nerve palsy. In this case study, we
present 12 year-old boy who presented with HZO.
Case Report
A 12 year old boy was admitted with pain in the right
side of the face, toothache, right ear ache of 5 days
duration. Vesicles on the right side of face appeared on
the previous day evening. He had tingling sensation in
the right side of face with pain along with giddiness,
vomiting and severe headaches. He was the second
sibling of a non-consanguineous couple, had normal
milestones of development and was immunized to
date. But he has not received vaccination against
varicella. Previously, he had history of chicken pox at
the age of 7 years. On general physical examination,
he was febrile, 1000F, Respiratory rate 28/minute,
Heart rate 86/minute, Blood pressure 110/78 mm Hg
and very lethargic. He had cervical lymphadenopathy
and did not have any pallor, clubbing and jaundice.
The skin lesions were tender, grouped vesicular
lesions over the right side of the face and lips,
dermatomal in distribution, with similar grouped
vesicles noticed over the right pinna (Figure1). Some
of the lesions gradually were seen to be secondarily
infected and features of mild cellulitis started. His oral
cavity showed gingivitis, large ulcers over the lateral
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aspect of the tongue. Central nervous system and urine culture sensitivity results were normal
examination revealed only pain in the area of the (Table 1).
vesicles on the right side of the face, there was
excessive tears from the right eye, pain and tingling Patient was given symptomatic treatment for his fever
sensation in the right side of the face along the and pain with paracetamol and treated with Acyclovir
vesicular lesions, mild difficulty in clenching the teeth (400 mg tablet, 1 tablets 6th hourly), co-amoxyclav
and fortunately no other severe features of facial nerve intravenously (30 mg /kg/dose) 3 times/day along with
palsy such as facial asymmetry and the House-Brackmann metrogyl intravenously (7.5 mg/kg/dose) in 3
facial nerve grading system was found to be grade 1 (ie times/day for treating secondary infection of the
normal facial function in all areas). There was no dysarthria, vesicles and the cellulitis, silver sulfadiazine ointment
gait ataxia or hearing loss. He was conscious and oriented local application over the lesions, proper hydration as
signs of meningeal irritation were absent. Systemic
he was very much lethargic for the first 3 days.
examination was normal.
Routine eye care was given like artificial tear drops
Dermatologist evaluation was done for the child. and antibiotic eye drops. His skin lesions resolved in
Tzanck smear showed giant cells. Ear Nose and one week. Patient was discharged after full treatment
Throat specialists evaluation revealed tympanic of 7 days with acyclovir. He was followed-up and is
membranes intact in both ears and the right ear lobe doing well for the last 4 months.
was erythematic appearance, vesicles extending to
external auditory canal and positional vertigo ruled
out. Ophthalmology evaluation showed congested
right eye and there were no vesicles in the eye and
cornea appeared clear. Diagnosis of HZO and
prevention of the RHS was made based on the history
of varicella infection in the past and the clinical
presentation now. Without undue delay on the same
day itself appropriate treatment was started.
Laboratory investigations such as Hemogram, urine
routine, serum electrolytes, Serum Glutamic Pyruvate
Figure 1-Child on admission with the characteristic herpetic
Transaminase (SGPT), peripheral blood smear, blood
blisters in the right side of the face-Herpetic Zoster Oticus
Table 1 Laboratory investigations
Sl Investigations Biological reference values
1 Hematological Hb:13.7 g/dl, Hb: 12-14 g/dl,
PCV:33.7%, PCV: 30-42 %,
Total count:7600/ul, Total count:4000-10000/ul, neutrophils:40-55 %,
neutrophils:50 %, Lymphocytes: 28-48%,
Lymphocytes:45%, Eosinophils:2%, Eosinophils: 1-5%,
Monocytes: 3 %, Monocytes: 3-6%,
Basophils: 0%, Basophils: 0-1%,
ESR: 20mm/hr, ESR: 22 mm/hr,
Platelets;285000/ul Platelets; 150000-400000/ul
2 Urine routine Albumin: nil,
sugar-:nil,
pus cells:1-2/hpf, epithelial cells:+/hpf,
bile salt & bile pigment: negative
3 Serum electrolytes, Serum sodium 137 mmol/L, Serum sodium 135-145 mmol/L
Potassium: 4.3 mmol/L, Potassium: 3.5-4.5 mmol/L,
Bicarbonate: 25 mmol/L, Bicarbonate: 24-28 mmol/L
Chloride: 102 mmol/L Chloride: 96-105 mmol/L
4 Serum Glutamic Serum SGPT: 35U/L Serum SGPT < 45U/L
Pyruvate
Transaminase
(SGPT)

2
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Discussion
The reactivation of VZV (chickenpox) in the geniculate
ganglion of the 7th cranial nerve resulting in ipsilateral facial
paralysis/paresis along with the vesicular herpetic rash
(HZO) and signs such as lethargy, otalgia, tinnitus, hearing
loss, nausea, vomiting, vertigo and nystagmus is known as
the classic RHS or type 2 RHS (Hunt, 1907; Louis
and Williams, 2003). James Ramsay Hunt is also
credited with identifying two other neurological
syndromes such as RH cerebellar syndrome or the
Type 1RHS having an association of seizures,
cognitive impairment, myoclonus and progressive
ataxia and the RHS Type 3, a neuropathy of the deep
palmar branch of the ulnar nerve (Hunt, 1907).
VZV infection initially causes the acute chickenpox which
generally occurs in children and young adults as seen in our
case 5 years ago and resolves fast. The VZV then remains
latent in the nerve cell bodies, the cells of the dorsal
root, cranial nerve or autonomic ganglia without causing
any symptoms and later on causes herpes zoster or
shingles (Hunt, 1907; Bleicher et al., 1996). To cause RHS,
VZV breaks out of nerve cell bodies and travel down
nerve axons from one or more ganglia along nerves of
an affected segment and infect the correspond-
ing dermatome causing a painful rash along with the
facial palsy. It can affect all ages, including children
especially in the age group 6-15 years (Bleicher et al.,
1996; Furuta et al., 2005). RHS is distinctive from
herpes zoster in that there is a motor component. In
RHS, first the visceral efferent motor fibres from the
motor nucleus of the 7th cranial nerve that leave the
brain stem are affected as they pass through the
geniculate ganglion, impairing motor supply of the
facial nerve. Also visceral afferent taste fibres from
the anterior two thirds of the tongue going to the
nucleus of the solitary tract via the nervus intermedius
can be affected by local inflammation as they pass the
geniculate ganglion. Thirdly decreased lacrimation
may result from involvement of these visceral efferent
parasympathetic fibres to the lacrimal and salivary
glands from the superior salivatory nucleus, again via
nervus intermedius as they branch at the level of the
geniculate ganglion. Fourth important factor is that the
cell bodies of the neurons from Spinal nucleus of 5th
cranial nerve receiving general somatic afferent fibres
from the geniculate zone of the ear via the chorda
tympani lie in the geniculate ganglia and are the site of
3
viral reactivation in classic RHS, causing vesicular
eruptions in geniculate zones (Cavoy, 2013).
The diagnosis of Ramsay Hunt syndrome is usually
made without difficulty when the clinical
characteristics are present (Hato et al., 2000). The
clinical presenting feature is often pain deep within
the ear which is paroxysmal at first but, after a day or
two, the pain often radiates outward into the pinna and
then becomes constant. The patient also complains of
rash or blisters in the distribution of the nervus
intermedius which may be on anterior two thirds of
the tongue, soft palate, external auditory canal, pinna
(HZO) associated with secondary infection and
cellulitis as in our case reported. A lower motor
neuron pattern of ipsilateral face drop or weakness
may be obvious. There may be hyperacusis on that
side due to paralysis of the stapedius and tensor
tympani .The other presenting features include vertigo
and ipsilateral hearing loss, tinnitus facial RHS causes
210% of acute peripheral facial paralysis (PFP) cases
(Hato et al., 2000; Yawn et al., 2007). The diagnosis
RHS can be missed if there is no rash. This condition
with only acute PFP is termed zoster sine herpete
and tends to be misdiagnosed as Bell's palsy (Mori et
al., 2002). The House-Brackmann scale is most
commonly used to quantify the degree of facial
muscle weakness (Gilchrist, 2009). The child in the
case study was grade 1 (ie normal facial function in all
areas) according to this scale.
Differential diagnosis of RHS if present includes
Bell's palsy as it is the most common PFP in children
(24-70%) (Kansu and Yilmaz, 2012). But the rash is
the characteristic diagnostic feature to differentiate
with RHS. 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) and carcinoma of the nasopharynx. simple
otitis (external, media) (Aframian, 1999; Kim and
Bhimani, 2008).
Regarding diagnosis virological studies are available
but usually the diagnosis of RHS/HZO in children is
clinical and a good medical history. VZV isolation in
conventional cell culture is considered the definite
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diagnostic test .It has a specificity of 100% but is not
always feasible. Tzanck test was done in our case and
has significance to identify the etiology
(Durdu et al., 2008). VZV antigen detection by direct
immunofluorescence assay has a sensitivity of 90%
and specificity 99% (Coffin and Hodinka, 1995). In
our case a tzanck smear and timely clinical diagnosis
was made and treatment started.
Antiviral agents are effective in reducing the severity
and duration of acute herpes zoster and HZO perse
when given within 72 hours of rash onset. Moderate
pain relief can be achieved with anticonvulsants,
tricyclic antidepressants, opioids, and topical
treatment modalities such as lidocaine-containing
patches and capsaicin cream (Galluzzi, 2007).
Corticosteroids and oral acyclovir are commonly used
in the treatment of RHS. Steroids reduce the
inflammation of the cranial nerves and help alleviate
the pain and neurologic symptoms Retrospective
studies have shown earlier administration of steroids
along with antivirals within 3 days of symptom onset
have 75% rate of full recovery whereas only 30% if
combined therapy is started 7 days after onset of
symptoms. The typical combined therapy involves a
7- to 10-day course of famciclovir (500 mg, 3 times
daily) or acyclovir (60-80 mg/Kg/day, 8th to 6th
hourly), along with oral prednisone (60 mg daily for 3
to 5 days) (Murakami et al., 1997). Surgical
decompression of the facial nerve has no role in this
syndrome (Muecke and Amedee, 1993).
In our case, there was mild difficulty in eating but no
evident facial droop. He was started on only acyclovir
on day 1 itself for 7 days and there was no facial palsy.
Antibiotics were given to treat the secondary infection.
He was very closely monitored with all supportive
care, and eye/corneal care. He did not develop facial
palsy and steroids were not prescribed. The zoster
(shingles) vaccine is considered the most effective
way to reduce incidence of herpes zoster/HZO and
postherpetic neuralgia, and to reduce severity of any
outbreak (Muecke and Amedee, 1993; Galluzzi,
2007).
The prognosis for HZO/RHS in children is better than
that in adults (Hato et al., 2000). Advanced facial
paralysis, audiovestibular findings and starting
treatment late result in a bad prognosis (Kansu and
4
Yilmaz, 2012). Hearing loss usually recovers well.
Age of the patient and other co-existing illness has
poor prognostic features (Yeo et al., 2007).
The prognosis of the facial paralysis in HZO/RHS is
worse than that in Bell's palsy, and only 10% of
complete facial paralysis in RHS recovers completely
(Muecke and Amedee, 1993; Yeo et al., 2006).
HZO caused by VZV reactivation can result in the rare
RHS in children. So a child with HZO is vulnerable to
progress to RHS, if not diagnosed immediately and
timely treatment started. Physician education is vital
in detecting HZO/RHS at very early stage as it can be
a rare cause of facial palsy in young children and
thereby prevent the associated morbidity.
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