Case Report

Ramsay Hunt syndrome in a patient with human

immunodeficiency virus infection
Mrinal Gupta
Treatwell Skin Centre, Canal Road, Jammu, Jammu and Kashmir, India

Address for correspondence:

Dr. Mrinal Gupta, Sudhaa Skin Centre, 35‑A, Lane No‑7, Tawi Vihar, Sidhra, Jammu ‑ 180 019, Jammu and Kashmir, India.
E‑mail: drmrinalgupta@yahoo.com

Abstract
Ramsay Hunt syndrome (RHS) is a rare, severe complication of varicella‑zoster virus reactivation in the
geniculate ganglion, characterized by hearing loss, pain, and vesicles in the ear or mouth along with ipsilateral
facial palsy. Although it is rare, it is more commonly found with immunodeficiency conditions. We report
a case of a 35‑year‑old human immunodeficiency virus positive male, having CD4+ count of 336/µl, who
presented with RHS and had vertigo, painful vesicular eruptions on the right ear and unilateral sensorineural
hearing loss. He was treated immediately with valacyclovir and prednisolone, and the complete recovery was
achieved at 6 months after the onset.

Key words: Facialpalsy, geniculateganglion, Ramsay Hunt syndrome, varicella zoster

INTRODUCTION CASE REPORT

Ramsay Hunt syndrome (RHS), also called herpes
zoster oticus, is a rare, severe complication of
varicella‑zoster virus (VZV) reactivation in the
geniculate ganglion. The classic triad consists of
otalgia, vesicles in the auditory canal and ipsilateral
facial paralysis. [1] It is the second most common
cause of atraumatic peripheral facial paralysis.[2] In
mild cases, it may or may not be associated with
any neurological signs, but in its severest form,
it is associated with sensorineural hearing loss,
disturbance of vestibular function and even viral
encephalitis. Without treatment, full recovery of the
facial paralysis occurs in only about 20% of cases;
the prognosis is better if treatment is started within
72  h.[3,4] We report a case of RHS in a 35‑year‑old
human immunodeficiency virus (HIV) positive male,
having CD4+  count of 336/µl, who was treated
immediately with valacyclovir and prednisolone
leading to a complete recovery.
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A 35‑year‑old male, a known case of HIV infection
for the last 4 years, presented to us with complaints
of right earache and pain over the right side of
the face for the last 9 days which was followed by
painful erythematous eruption over the right ear
and inability to close the right eye. The patient took
some oral analgesics and a topical antibiotic from
a local practitioner for the same, but there was no
relief in symptoms. The patient also gave a history
of tinnitus and vertigo, but there was no history of
ear discharge, nasal discharge, headache, seizure,
or weakness of the limbs. The patient was HIV
positive with CD4+  count of 336/µl and had been on
antiretroviral therapy with zidovudine, lamivudine,
and nevirapine for the last 2 years.
On examination, the right ear had a few vesicles
along with crusted erosions on the concha of the
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DOI: How to cite this article: Gupta M. Ramsay Hunt syndrome in a

10.4103/0253-7184.203439 patient with human immunodeficiency virus infection. Indian J Sex
Transm Dis 2017;38:89-91.

© 2017 Indian Journal of Sexually Transmitted Diseases and AIDS | Published by Wolters Kluwer - Medknow 89
 Gupta: Ramsay Hunt syndrome
right pinna [Figure 1]. There was no discharge,
and the tympanic membrane was intact. Bell’s
phenomenon was present on the right side along
with loss of nasolabial fold [Figure 2].
The patient was diagnosed as a case of RHS and
started on tablet valacyclovir 1 g three times a day
for 7  days, tapering dose of tablet prednisolone,
acetaminophen, physiotherapy for facial nerve palsy
and ocular care. After 10 days, the vesicles and
crusting over the right ear had cleared, and there
was mild improvement in Bell’s palsy and vertigo.
The patient was advised to continue physiotherapy
and was advised regular follow‑up. After 6 months
of physiotherapy, the facial nerve function had
improved considerably, and the patient was able to
completely close his eyes, and there was no facial
asymmetry.
DISCUSSION
The Center for Disease Control estimates that 32%
of people in the USA will have herpes zoster (HZ)
during their lifetime with the risk increasing with
the progression of age. [5] RHS has a much lower
incidence, presenting in only 0.2% of all HZ cases.[6]
The risk of developing HZ depends on the decline
in cell‑mediated immunity. Herpes zoster is one
of the AIDS‑defining illnesses. Other factors are
old age, an immunosuppressive therapy used in
malignancies, transplantation, and autoimmune
disorders. HIV‑infected patients have twenty times
more risk of developing HZ than their age‑matched
seronegative persons and about 30% of HIV persons
develop zoster at least once within 12 years after the
diagnosis of HIV.[7]
HZ is also a common manifestation in immune
reconstitution inflammatory syndrome (IRIS) and
Figure 1: Herpetic lesions in the right ear
90 Indian Journal of Sexually Transmitted Diseases and AIDS Volume 38, Issue 1, January-June 2017
usually manifests after 2–3 months of initiation of
highly active antiretroviral therapy (HAART), but
late onset HZ has also been reported even after
3 years of HAART initiation.[8] Our case may also be
a manifestation of IRIS as the patient had been on
HAART for the last 2 years.
RHS is diagnosed clinically and is based on
unilateral facial weakness plus vesicular lesions in
the ipsilateral ear, hard palate, or anterior two‑third
of the tongue. Facial weakness is identified by
facial drooping, a widened palpebral fissure, and
decreased smile on the affected side. Otalgia or
vertigo completes the triad picture of RHS.[1]
The use of CSF analysis or MRI adds no additional
diagnostic value. The gold standard for diagnosing
VZV reactivation is polymerase chain reaction of
skin, saliva, or middle ear fluid samples but this is
rarely done clinically.
Treatment of RHS follows the general treatment
goal of VZV infection. In the normal host, the
primary goal of treatment is to reduce acute pain
and postherpetic neuralgia. Several controlled
studies verified that high‑dose oral acyclovir
of valacyclovir within the first 72  h speeds
resolution of the lesions and seems to reduce
the risk of prolonged pain. [9] For the severely
immunocompromised host, intravenous acyclovir
has been shown to prevent disease progression. For
mildly to moderately immunocompromised persons,
however, oral valacyclovir or famciclovir might be
acceptable alternatives to intravenous treatment.
The management of IRIS associated HZ is similar
and monosegmental zoster can be treated with oral
acyclovir, valacyclovir, or famciclovir for 7–10  days
but complicated, multisegmental or facial zoster
Figure 2: Facial nerve palsy with Bell’s phenomenon and loss of
nasolabial fold
 Gupta: Ramsay Hunt syndrome
should be treated intravenously for 10–14 days. As
our patient had a monosegmental zoster, we treated
him with oral valacyclovir for 7  days with good
response.
RCTs have shown that adjunct corticosteroids
resulted in quicker healing of the rash and decreased
incidence and severity of pain during HZ. Yet, there
was no reduction in the incidence or severity of
postherpetic neuralgia in these studies.[10] Steroids
reduce the inflammation in the facial canal. Early
administration of acyclovir‑prednisone has proved
to reduce nerve degeneration by nerve excitability
testing. Hearing recovery is also better in these
patients.[4] Apart from these, eye patches, taping the
eye closed, artificial tears and oral analgesics are also
advised to prevent ocular complications.
If untreated, the course of RHS can be complicated
with ocular complaints like corneal ulcers if eyelid
closure is impaired, permanent facial paralysis,
postherpetic neuralgia, or long‑term ipsilateral
hearing loss. The prognosis of IRIS associated HZ
is similar and tends to resolve completely without
complications with treatment, but chronic courses
with ulcerating forms and involvement of other
organs may be seen as may be pneumonia or central
nervous system involvement.
RHS is a rare disease that can present with vague
symptoms and atypical presentations. A high index
of suspicion and close follow‑up is essential as early
intervention with antivirals and corticosteroids has
shown to significantly improve outcomes in these
patients.
Declaration of patient consent
The authors certify that they have obtained all
appropriate patient consent forms. In the form the
patient(s) has/have given his/her/their consent for his/
her/their images and other clinical information to be
Indian Journal of Sexually Transmitted Diseases and AIDS Volume 38, Issue 1, January-June 2017 91
reported in the journal. The patients understand that
their names and initials will not be published and
due efforts will be made to conceal their identity,
but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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