CASE REPORT

SUDDEN DEAFNESS

Presentator : dr. Pradhana Fajar Wicaksana

Moderator : dr. Agus Surono, M.Sc, PhD, Sp.THT-KL

Otorhinolaryngology Head & Neck Department

Faculty of Medicine Universitas Gadjah Mada/ RSUP Dr. Sardjito

Yogyakarta

2017

0
INTRODUCTION
sudden sensorineural hearing loss or
sudden deafness ( SD ) is an unexplained
unilateral hearing loss with onset over a
period of less than 72 hours, without other
known otological diseases.
By Consensus on diagnosis and treatment
of sudden hearing loss in madrid, SD is
considered as sensorineural or perceptual
hearing loss cases with a sudden onset,
within 72 hours, with a loss of over 30 dB,
in at least 3 consecutive frequencies of
tonal audiometry, without other prior
otological history.1
Sudden sensorineural hearing loss affects 5
to 20 per 100.000 population, with about
4000 new cases per year in the United
States.1,2
Clinicians should distinguish sensorineural
hearing loss (SNHL) from conductive
hearing loss (CHL) in a patient presenting
with sudden hearing loss.1
The distinction between SSNHL and other
causes of SHL is one that should be made
by the initial treating health care provider,
so that early diagnosis and management
can be instituted.2
There are many theories that can cause this
condition, including blood disorders,
1
immune disorders, perilymphatic fistulas
and viral infection. 1,2
viruses can cause sudden hearing loss either
as an acute infection or as delayed onset with
the latent form of the virus being possibly
reactivated. Viruses implicated include
herpes zoster oticus and herpes simplex type
1.3
In the present study, Idiopatic sudden
sensory neural hearing loss (ISSNHL)
laterality was significantly associated with
diabetes and cardiovascular disease,
especially mitral valve disorder and
antiphospolipid syndrome. In another hand
the incident of trancient ischemic attack of
iner ears due to general anesthetic procedure
have been reported.1,4
Two major proposed etiologies for ISSNHL
development are vascular (vertebrovascular
ischemia) and inflammatory theories. Hino-
josa and Kohut showed temporal bone
histopathology in ISSNHL, with a loss of
sensory epithelium of the cochlear and the
vestibular labyrinth, which the authors
suggested to be an involvement of the inner
ear infarction.4
Theory of immune-mediated disease, This
theory is supported by pathological studies,
spontaneous recoveries and the response to
treatment with steroids. However, in some
patients with SD, there is no evidence of
impaired immunity and the clinical
evolution is not always compatible with an
autoimmune case. Moreover, it is common
for SD cases initially classified as
idiopathic to be diagnosed as a specific
autoimmune disorder with the passage of
time.1
conversely, although it would not be an
idiopathic SD, the “theory of cochlear
membrane rupture” has been described,
due to a possible perilymph fistula,
appearing in connection with physical
exercise, barotrauma or a Valsalva
manoeuvre. This theory could justify the
spontaneous recovery of some patients. 1,2
Faced with a clinical suspicion of SD, and
before considering a possible treatment,
the diagnostic tests required are tunning
folk and otoscopy examination. Tuning
forks will give us a sensorineural pattern:
positive Rinne in the diseased ear and
Weber lateralised to the healthy ear, with
addition of tympanometry allowing us to
rule out causes of SD due to middle ear
diseases: otitis media with effusion, etc.
with a transmission pattern (negative Rinne
in diseased ear and Weber towards the
diseased ear).However, in cases of severe
SD, , there can be a false negative Rinne
(the patient does not hear the tuning fork at
all). 1,2
2
Tonal audiometry will determine the pure
tone audiometry (PTA), taking the average
dB threshold at frequencies 0.25, 0.5, 1, 2, 4
and 8 kHz as PTA, which must be greater
than 30 dB at 3 consecutive frequencies in
the diagnosis of SD. 1,2
Audiometry is mandatory for definitively
diagnosing SSNHL because it distinguishes
CHL from SNHL and establishes frequency-
specific hearing thresholds. Varying criteria
have been used in the literature to diagnose
SSNHL, but a hearing loss ≥30 dB at 3
consecutive frequencies is the definition
adopted by the NIDCD and the definition
used in most RCT. 1,2
Series of analyses, whose extraction should
be performed prior to treatment, including at
least the following parameters: blood count,
erythrocyte sedimentation rate (ESR), luetic
serology (VDRL and FTAabs) and
antinuclear antibodies (ANA), according to
prior systematic reviews. It may also be
useful to request immune phenotype by
CD4+ and CD8+ lymphocyte
subpopulations, and the CD45RO+ and
CD45RA+ isoforms. MRI of the inner ear
with gadolinium to exclude retrocochlear
pathology or show intracochlear
haemorrhage. 1,2
Treatment of SD is very controversial, due
to the absence of solid evidence to clearly
endorse any of the options raised. The
doses employed are also very variable.
Traditional general measures such as bed
rest or diets with a restricted salt intake
have not demonstrated effectiveness, so
hospital admission to maintain bed rest is
debatable. For this reason, there is no
agreement on the need for a first phase of
hospital treatment for 4 to 7 days,followed
by outpatient treatment. Despite the excess
of existing literature on SD, there are only
a few randomized double-blind controlled
trials, which have been reviewed by
Cochrane 104 and summarized in a meta-
analysis. The most outstanding include the
classic work of Wilson et al, who compared
oral steroids versus placebo in 1980, laying
the foundation for their use. However, the
group of Cinamon et al found no
significant effect with steroids. 1
The specific action of steroids in the
cochlea is uncertain but their use has been
based on their ability to decrease
inflammation and oedema. Steroids have
many effects in the inner ear. Supression
of immune response, improvement of
decreased microvascular circulation,
mineralocorticoid effects, or decrease in
endolymphatic pressure are the effects of
the steroids. 1
3
If the diagnosis was possible within 30 days
of onset of symptoms, the treatment should
be with oral steroids for 1 month, with 3
main options: Prednisone (Prednison
Alonga®, Dacortin®), 1 mg / kgbw / day,
dosage tappering off every 5 days.
Methylprednisolone (Urbason®), 1 mg / kg
bb / day, in decreasing doses every 5 days.
Deflazacort (Dezacor®, Zamene®), 1,5 mg
/ kg bw / hari, similar decreasing dosage in
every 5 days. 1,5
if SD is severe (> 70 dB), in a single ear or
with severe associated vertigo (suspected
vestibular neuritis), treatment should be
offered with intravenous corticosteroids for
7 days, in a day hospital regime or through
hospital admission, with a dose of 500 mg
methylprednisolone per day, slowly passing
to one dose in serum in 30 minutes.1,5
Moreover, possible side effects of short-
term,high and medium-dose glucocorti-
costeroid therapy include risk of metabolic
complications, such as glucose intolerance
and diabetes mellitus, hypertension,
increased intraocular pressure and
glaucoma, psychotropic effects, risk of
hypothalamic-pituitary-adrenal-axis
suppression, gastrointestinal bleeding, bone
loss, avascular necrosis of the femoral or
humeral head and potential infections.6
Pharmacokinetic studies in animals and
humans have shown that only high doses of
systemic glucocorticoids will result in
detectable drug levels in the inner ear
perilymph and that substances applied to
the round window membrane lead to
significantly higher drug levels in the inner
ear fluids compared to systemic
application.6
Antiviral therapy, although antiviral agents
should theoretically have a positive effect
on SD, randomized clinical trials
conducted by Stokross et al in 1998,Tucci
et al in 2002 and Westerlaken et al in 2003
were unable to find statistically-significant
differences between antiviral agents and
placebo.1
Idiopathic sudden sensorineural hearing
loss (ISSHL) is common and often results
in permanent hearing loss. It therefore has
a high impact on the well-being of those
affected. Tinnitus (abnormal persistent
noises or ringing in the ear) is similarly
common and often accompanies the
hearing loss. Although the cause of these
complaints is not clear, they may be related
to a lack of oxygen secondary to a vascular
problem not yet identified. Hyperbaric
oxygen therapy (HBOT) involves
breathing pure oxygen in a specially
designed chamber and it is sometimes used
4
as a treatment to increase the supply of
oxygen to the ear and brain in an attempt to
reduce the severity of hearing loss and
tinnitus.7
At the time of ISSHL onset, the following
variables correlated with a worse prognosis:
dizziness, profound hearing loss, change in
contralateral ear hearing, and a delayed start
of treatment. Presence of tinnitus at ISSHL
onset correlated with a better prognosis..8,9
CASE REPORT
A 58 years-old male adult (MR
01.83.04.50) came to Sardjito hospital
with chief complain sudden hearing loss
in left ear since 1 weeks. He
complained ringing sensation and then
followed by hearing loss. He denied any
dizziness, vertigo, pain, ear fulness,
itchyness, paralysis, and discharge from
the ear. he also denied any nose and
throat problems. In the past History of
illness : Patient suffered diabetic
mellitus for 4 years and having
medication of its disease. In 2012,
patient had stroke ischaemic attack.
On physical examination found that the
general condition was good, compos
mentis, blood pressure 130/70 mmHg, pulse
82 x/ minute, respiratory rate 18 x/minute,
temperature 36,70 C. From ENT
examination we found normal external
auditory canal. Both tympanic membrane
was intact and we could see cone of light.
Rhinoscopy examination, anteriorly and
posteriorly, showed normal result.
Oropharyngeal examination within
normal limit. Indirect laryngoscopy also
within normal limit.
We performed tuning fork examination.
Rinne test positif on the left ear, negative
result on the right ear. Weber test showed
right lateralitation, Schwabach test
showed shortening in the left ear and
equal perception with examiner in right
ear. Pure tone audiometry showed AD
mild SNHL and AS SNHL Profound or
severe sensory neural hearing loss. OAE
examination showed refer to both ears.
Diagnosis of sudden deafness was made
and He was treated methylprednison 8mg
1-0-1, Patient was advised to come again
one week after.
When we followed up the next one
week, from anamnesis we found the
hearing loss complaint was still the
same,but ringing sensasation or tinnitus
5
is better than before. and the patient still
denied dizziness, vertigo, pain, ear
fulness, itchyness, paralysis, or
discharge from the ear. He also denied
any nose and throat problems. From
physical examination, we also found
normal external auditory canal. Both
tympanic membrane was intact and we
could see cone of light. Rhinoscopy
examination, anteriorly and posteriorly,
showed normal result. Oropharyngeal
examination within normal limit.
Indirect laryngoscopy also within
normal limit. Tuning fork test also still
showed same result, AS SNHL and AD
SNHL. The problem which I kindly
would to discuss is the prognosis.
DISCUSSION
The diagnose sudden deafness on this case
based on anamnese, physical and supporting
examination. There is a hearing decrease in a
last week that came suddenly followed by
tinnitus.
Stachler et al says, Sudden hearing loss is
defined as a rapid onset,occurring over a 72-
hour period, of a subjective sensation of
hearing impairment in one or both ears. 2
Moreover, the prevalence of tinnitus
correlates with the severity and frequency
characteristics of the hearing loss. Manche
SK mentioned that Sudden hearing loss more
40 dB shows the values of significant
tinnitus.10
Increased level of insulin resistance and some
drugs used for the treatment of hypertension
worsen the resistance and can favor tinnitus
onset. In the present study, tinnitus was
observed in 30.1% of patients with diabetes
mellitus, 38.8% in hypertension and 40%
with hypothyroidism. Medications such as
salicylates, aminoglycoside antibiotics,
quinine, or cisplatin used to treat otological
and nasopharyngeal diseases can cause
damage to the cochlea and trigger or enhance
tinnitus.10
From ENT examination we found normal
external auditory canal. Both tympanic
membrane was intact and we could see cone
of light.
Otoscopy should be normal in both ears;
however, the finding of cerumen impaction
does not exclude a possible SD. The cerumen
should be removed and test whether hearing
becomes normal. Tuning forks will give us a
sensorineural pattern.1
6
On supporting examination, tuning fork
shows result Auris Sinistra sensory neural
hearing loss. Pure tone audiometry showed
AD mild SNHL and AS SNHL Profound. On
oto acoustic emission (OAE) result AD refer
AS refer.
According to Guilermo et al, Acoumetry
(tuning forks) will give us a sensorineural
pattern: positive Rinne in the diseased ear
and Weber lateralised to the healthy ear
allowing us to rule out causes of SD due to
middle ear diseases.1
Auditory brainstem response (ABR) and
otoacoustic emissions ( OAEs) testing may
provide additional information regarding the
integrity of the auditory system. The presence
of measurable OAEs indicates preservation
of some outer hair cell function. The ABR
reflects function of the retrocochlear neural
pathways. The ABR and OAE results also
may assist in diagnosing a functional hearing
loss.11
The specific action of steroids in the cochlea
is uncertain but their use has been based on
their ability to decrease inflammation and
oedema. Steroids have many effects in the
inner ear. Supression of immune response,
improvement of decreased microvascular
circulation, mineralocorticoid effects, or
decrease in endolymphatic pressure are the
effects of the steroids. 1
At the time of ISSHL onset, the following
variables correlated with a worse prognosis:
1. Dizziness 2. Profound hearing loss
3. change in contralateral ear hearing 4.a
delayed start of treatment. 4. vertigo is a
factor for worse prognosis. 5. Age. Presence
of tinnitus at ISSHL onset correlated With a
better prognosis.8 ,9
In this patient, the prognostic may be not
good because of the level of hearing loss
(profound SNHL) and the etiology remains
unknown. The patient is in advanced age and
it is accompanied with Metabolic and
cardiovascular disease.
Summary
A 58 years-old male adult was reported with
sudden deafness, complaining sudden
hearing loss in left ear since 1 weeks. We
treated the patient with methylprednison 8mg
1-0-1, and did follow up one week after.
Daftar Pustaka
1. Guillermo Plaza, Enrique Durio,
Carlos Herráiz. Consensus on
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Esp. 2011;62(2):144−157
2. Robert J. Stachler, MD1, Sujana S.
Chandrasekhar, MD2. Clinical
Practice Guideline: Sudden Hearing
Loss. American Academy of
Otolaryngology—Head and Neck
Surgery Foundation 2012
3. Awad Z, Huins C, Pothier DD.
Antivirals for idiopathic sudden
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4. Chulho Kim, Jong-Hee Sohn, Min Uk Jang.
Ischemia as a potential etiologic factor in
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Analysis of posterior circulation
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144-151
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S. Steroids for idiopathic sudden
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and tinnitus. The Cochrane
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8. Eduardo Amaro Bogaz, André Souza
de Albuquerque Maranhão.
Variables with prognostic value in
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10. Santoshi Kumari Manche, Jangala
Madhavi. Association of tinnitus and
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in a South Indian population. Braz J
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sensory hearing loss. In :Bailey,
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