Wakeel et al. / Journal of Science / Vol 4 / Issue 8 / 2014 / 538-540.

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RAMSAY HUNT SYNDROME (HERPES ZOSTER OTICUS) A RARE

CLINICAL ENTITY
Wakeel, Shahid Hassan*, Ajaz Shah and Irshad Ahmad
Department of Oral and Maxillofacial surgery, Govt. Dental College, Srinagar, India.

ABSTRACT
Herpes zoster oticus also known as Ramsay Hunt syndrome is a rare complication of herpes zoster in which
reactivation of latent varicella zoster virus infection in the geniculate ganglion causes otalgia, auricular vesicles, and
peripheral facial paralysis. Ramsay Hunt syndrome is rare in children and affects both sexes equally. Incidence and clinical
severity increases when host immunity is compromised. Because these symptoms do not always present at the onset, this
syndrome can be misdiagnosed. Although secondary to Bell's palsy in terms of the cause of acute atraumatic peripheral facial
paralysis, Ramsay Hunt syndrome, with incidence ranged from 0.3 to 18%, has a worse prognosis. Herpes zoster oticus
accounts for about 12% cases of facial palsy, which is usually unilateral and complete and full recovery occurs in only about
20% of untreated patients. The most advisable method to treat Ramsay Hunt syndrome is the combination therapy with
acyclovir and prednisone but still not promising, and several prerequisites are required for better results. We present a case of
32-year-old man suffering from Ramsay Hunt syndrome with grade V facial palsy treated effectively with rehabilitation
program, after the termination of the combination therapy of acyclovir and prednisone.

Key words: Geniculate ganglion, Facial palsy, Otalgia, Ramsay Hunt syndrome.

INTRODUCTION
Ramsay Hunt syndrome (RHS) is defined as an
acute peripheral facial neuropathy associated with
erythematous vesicular rash of the skin of the ear canal,
auricle (also termed herpes zoster oticus), and/or mucous
membrane of the oropharynx. RHS was first described in
1907 by James Ramsay Hunt in a patient who had otalgia
associated with cutaneous and mucosal rashes, which he
ascribed to the infection of geniculate ganglion by human
herpes virus 3 (i.e. varicella zoster virus [VZV]) [1,3,7].
This syndrome is also known as geniculate neuralgia or
nervus intermedius neuralgia. Primary infection of VZV,
also known as varicella or chickenpox, is a common
pediatric erythematous disease [3]. VZV remain latent in
neurons of cranial nerve and dorsal root ganglia,
subsequent reactivation can result in prodromal period of
severe pain followed by localized vesicular rash, known
as herpes zoster (HZ). Classic RHS usually present with
paroxysmal pain deep within the ear, often radiates
outward into the pinna and may be associated with a more
constant, diffuse and dull background pain. The onset of
pain usually precedes the rash by several hours and even
days. Vesiculation and ulceration of the external ear and
ipsilateral anterior twothirds of the tongue and soft palate
are common (as many as 80% of cases) [4]. The rash
might precede the onset of facial paresis/palsy; other
manifestations include vertigo and ipsilateral hearing loss
(CN VII), tinnitus, otalgia, headaches, dysarthria,
headaches, gait ataxia, fever, cervical adenopathy [5].
In severe cases of HZO, involvement of
vestibulocochlear nerve leads to sensorineural hearing
loss in 10% and vestibular symptoms in 40% patients.
Definitive treatment consists of antiviral therapy and
steroids. This article describes the case of RHS with grade
V facial palsy of House-Brackmann grading system
treated effectively with combination therapy of acyclovir
and prednisone, supported by rehabilitation program
CASE REPORT
We present a 50-year-old uncontrolled diabetic
female patient who reported with clusters of vesicles in
the skin over the left side of the face, and pain in left ear

Corresponding Author:- Dr. Shahid Hassan Email:- shahidfaciomax@yahoo.co.in

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for last 2 days following removal of carious left
mandibular third molar. Patient also complained of fever,
fatigue, myalgia, facial and ear pain, altered taste
perception. The vesicles were present unilaterally in the
facial skin along the left mandibular dermatome. External
auditory canal and tragus of right ear also showed similar
lesions fig1. Intraorally multiple ulcers of varying size
distributed over the left buccal mucosa. 24 to 36 h after
the onset of otalgia, patient developed facial weakness
along with vesicular eruptions on conchae and in external
auditory meatus of left side. On examination, there was
lower motor neuron facial palsy on left side which was
complete. Bells phenomenon was present on left side. A
neurologic examination revealed a weakness in the
marginal mandibular branch of the left facial nerve
(Figure 2). Loss of definition in the ipsilateral nasolabial
fold and weakness in the temporal branch of the facial
nerve was detected fig3. There were painful adherent
crusts and scabs in right conchae and external auditory
meatus fig4. Initially patient was given antibiotics and
treatment for post extraction pain and dry socket. The
Fig 1. External auditory canal and tragus of left ear also
showed similar lesions
Fig 3. Ipsilateral nasolabial fold and weakness in the
temporal branch of the facial nerve was detected
DISCUSSION AND CONCLUSION
RHS is estimated to account for 18% of facial
palsies in adults [2,4]. Early initiation of antiviral
treatment and adjuvant steroid therapy can prevent the
occurrence of facial paralysis. Poor prognostic factors
diagnosis was revised as RHS. Patient was prescribed
tablet acyclovir 800 mg five times a day for 5 days, tablet
paracetamol 500 mg sixth hourly for 3 days,
chlorhexidine mouthwash and topical anesthetics.
Steroids were not used as pt was having uncontrolled
diabeties. Patient was advised physiotherapy for facial
palsy and showed good recovery. The rehabilitation
program that the patient attended included transcutaneous
electrical nerve stimulation and facial neuromuscular
exercises. The facial exercise program was composed of
(1) relaxation of hyperactive muscles, (2) facial massage
exercises, (3) biofeedback training using a mirror to let
patient know facial movement, and (4) specific facial
exercises like smiling, grimacing, and whistling. The
patient was instructed to follow this exercise program for
two times a week, at least 60 min per visit and encouraged
him to do methodically the facial exercise himself in front
of a mirror at home. It took additional 3 weeks of
outpatient rehabilitation program for total remission of his
facial palsy to occur, in addition to the 1-week inpatient
rehabilitation.
Fig 2. Weakness in the marginal mandibular branch of
the left facial nerve
Fig 4. Crusts and scabs in right conchae and external
auditory meatus
for good functional recovery of facial nerve include
patient older than 50 years, complete facial paralysis and
lack of CN VII nerve excitability.4 Approximately 25%
of patients with RHS experience vestibular or cochlear
symptoms or both; apparently caused by the spread of
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inflammatory cells from geniculate ganglion to inner ear,
spiral or vestibular ganglion. In one-fourth of patient,
symptom also includes vertigo, nystagmus, tinnitus and
hearing loss. Sensorineural hearing loss occurs in only
approximately 6% of patients. Audiograms vary and
suggest primary cochlear involvement or cochlear nerve
damage [5-7]. In our case, there was no history of
deafness or vertigo. Examination revealed normal tuning
fork test and normal pure tone audiogram, thereby ruling
out involvement of auditory apparatus. There was no
feature suggestive of vestibular involvement which was
evident by the absence of vertigo and nystagmus. This
case was presented for its rarity and classical clinical
presentation. Similar to our case many of the patients with
HZ in the head and neck region undergo unnecessary
extraction/endodontic treatment for many teeth during the
prodromal period with no clinical lesions. The most
recommended therapy for RHS is the combination of
acyclovir and prednisone. RHS patients with different
levels of severity treated with acyclovir-prednisone
combination showed complete facial recovery, i.e., House
grade I, in 52% patients, no matter what their
pretreatment gradings were.

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