Case Report

Sudden Deafness

Presentator : dr. Heribertus Diwyacitra Aribawa

Moderator : dr. Siswanto Sastrowijoto, Sp.T.H.T.K.L(K), M.H

Otorhinolaryngology and Head Neck Surgery Departements

Medical Faculty of Gadjah Mada University / DR. Sardjito Hospital
Yogyakarta
2017

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INTRODUCTION documented theory, both anatomically-
pathologically, with post-mortem findings
Sudden deafness is a frightening
related to infection by type 1 herpes simplex
symptom that often prompts an urgent or
virus, and by the frequent history of upper
emergent visit to a physician.. Since its first
respiratory tract catarrh or its higher
description in 1944, the incidence of sudden
incidence at certain times of the year.
deafness (SD) has been increasing steadily
However, no specific serological profiles or
over the years, and currently represents
response to common antiviral treatment such
1.2% of the cases at ENT Emergency Units.
as acyclovir have been reported. Theory of
Looking at the literature on its
alteration in inner ear microcirculation.
epidemiology, its impact can be established
Some studies have found a higher incidence
in 5-20 cases per 100,000 inhabitants per
of SD in patients with cardiovascular risk,
year. Recently, it is reported an even higher
especially with mitral prolapse or
incidence, of up to 160 cases per 100,000
antiphospholipid syndrome. Sudden hearing
inhabitants per year, especially in cases
loss with classic symptoms immediately
where there are national registers of SD, as
after rapid reduction of blood pressure in
in Japan.1
malignant hypertension provided strong
Sudden deafness (SD) is considered evidence that cochlear hypoperfusion was a
as sensorineural or perceptual hearing loss possible cause of sudden deafness.3,4
cases with a sudden onset, within 72 hours,
On the other hand, SD has been
with a loss of over 30 dB, in at least 3
reported after transient ischemic events in
consecutive frequencies of tonal audiometry,
the inner ear, such as during general
without other prior otological history. It is
anesthesia, or confirmed after an episode of
needed to differentiate CHL from SNHL for
intralabyrinthine haemorrhage, objectified
defining potential treatments and prognosis.
by MRI imaging. It has also been
These 2 common causes of hearing loss can
reported that the frequency of stroke is
be diagnosed by a combination of history,
greater in the 5 years after having suffered
physical examination, tuning fork tests, and
SD. These alterations in the microcirculation
audiometry.2
of the inner ear have been the basis of
There are several theories about the treatments such as administration of low
cause of SD. Viral theory is the most molecular weight heparins, plasmapheresis

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for the cleaning of plasma LDL, the use of consultations, in addition to confirming the
inhaled carbogen or hyperbaric oxygen normal otoscopy and sensorineural
chambers, with mixed results. Theory of acoumetry, it is necessary to carry out a
immune-mediated disease is supported by tonal and verbal audiometry and a
pathological studies, spontaneous recoveries tympanogram. Tonal audiometry will
and the response to treatment with steroids. determine the pure tone audiometry (PTA),
However, in some patients with SD, there is taking the average dB threshold at
no evidence of impaired immunity and the frequencies 0.25, 0.5, 1, 2, 4 and 8 kHz as
clinical evolution is not always compatible PTA, which must be greater than 30 dB in
with an autoimmune case. Moreover, it is bone conduction to confirm the diagnosis of
common for SD cases initially classified as SD. It is advisable to supplement the
idiopathic to be diagnosed as a specific diagnostic process with: Stapedial reflex, to
autoimmune disorder with the passage of rule out cochlear recruitment and Ménière’s
time. Conversely, although it would not be disease. Series of analyses whose extraction
an idiopathic SD, the “theory of cochlear should be performed prior to treatment,
membrane rupture” has been described, due including at least the following parameters:
to a possible perilymph fistula, appearing in blood count, erythrocyte sedimentation rate
connection with physical exercise, (ESR), luetic serology (VDRL and FTAabs)
barotrauma or a Valsalva manoeuvre. Most and antinuclear antibodies (ANA),
SSNHL cases (almost 80%), however, fit according to prior systematic reviews. MRI
into the idiopathic category and their cause of the inner ear to exclude retrocochlear
remains unknown. 5,6 pathology or show intracochlear
haemorrhage.1
In primary healthcare and ENT
emergency services, the two tests required Treatment of SD is very
are otoscopy and acoumetry: Otoscopy controversial, due to the absence of solid
should be normal in both ears; however, the evidence to clearly endorse any of the
finding of a wax plug does not exclude a options raised. The doses employed are also
possible SD. The plug should be removed very variable. Traditional general measures
and test whether hearing becomes normal. such as bed rest or diets with a restricted salt
Acoumetry (tuning forks) will give us a intake have not demonstrated effectiveness,
sensorineural pattern. In outpatient ENT so hospital admission to maintain bed rest is

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debatable. For this reason, there is no immune response, improvement of
agreement on the need for a first phase of decreased microvascular circulation,
hospital treatment for 4 to 7 days, followed mineralocorticoid effects, or decrease in
by outpatient treatment. With regard to endolymphatic pressure are the effects of the
antiviral therapy, although antiviral agents steroids. 1,7,8
should theoretically have a positive effect on
If the diagnosis was possible within
SD, randomized clinical trials conducted by
30 days of onset of symptoms, the treatment
Stokross et al in 1998,Tucci et al in 2002
should be with oral steroids for 1 month,
and Westerlaken et al in 2003 were unable
with 3 main options : 1) Prednisone 1 mg/kg
to find statistically-significant differences
bw/day, in decreasing doses every 5 days. 2)
between antiviral agents and placebo. Other
Methylprednisolone, 1 mg/kg bw/day, in
widely used treatments, on the basis of
decreasing doses every 5 days. 3)
vascular aetiology, such as vasodilators,
Deflazacort, a similar decreasing pattern, 1.5
carbogen or hyperbaric oxygen have been
mg/kg bw/day, in decreasing doses every 5
reviewed recently in a meta-analysis and by
days.. If SD is severe (> 70 dB), in a single
Cochrane. However, there were no findings
ear or with severe associated vertigo
of significant effectiveness in SD.
(suspected vestibular neuritis), treatment
Consequently, having reviewed the
should be offered with intravenous
literature, once SD has been diagnosed, this
corticosteroids for 7 days, in a day hospital
consensus group advises the following
regime or through hospital admission, with a
therapeutic scheme, based mainly on
dose of 500 mg methylprednisolone per day,
systemic corticosteroids. 21-amino
slowly passing to one dose in serum in 30
glucocorticoid steroids, such as
minutes. Subsequently, the oral regime
methylprednisolone, are commonly
described above should be reintroduced. If
prescribed to treat this condition, but their
there is contraindication for the use of
usage is associated with potential side
systemic corticosteroids, or if there is no
effects. The specific action of steroids in the
response to treatment with systemic
cochlea is uncertain but their use has been
corticosteroids, oral or intravenous, after 7
based on their ability to decrease
days of its establishment, a rescue treatment
inflammation and oedema. Steroids have
with intratympanic corticosteroids should be
many effects in the inner ear. Supression of
offered with 1 dose weekly for 3 weeks in

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outpatient ENT consultation : 1)
Methylprednisolone, 0.9 cc of a 40 mg vial,
mixed with 1% lidocaine at 0.1 ml. 2)
Dexamethasone, 0.9 cc of an 8 mg vial. In
cases of suspected vascular aetiology
(known cardiovascular risk factors), it may
be associated with vasodilators, such as
nimodipine, intravenously (5-15 cc in 500
ml of saline solution, passing slowly, every
8 hours), or such as oral trimetazidine
(especially if the patient is already in
treatment for hypertension, 1 comp/8 h for 1
month), and then refer the patient to the
Internal Medicine Department for evaluation
and possible antiplatelet therapy. If the
diagnosis is late, between 30 days and 90
days after the onset of symptoms, treatment
should be with oral corticosteroids for 1
month, following the previous scheme. If the
diagnosis is very late, more than 90 days
from the onset of symptoms, treatment
should be discussed individually.1
The natural history of SD is variable,
since its causes are multiple. Some patients
recover completely without medical
intervention usually during the first three
days (spontaneous recover) and generally do
not see a doctor. Others improve slowly over
a period of 1 to 2 weeks, with reports of
improvement or spontaneous recovery in up
to 65% of cases in the most classical series
5
of the 70s and 80s. However, most patients
do not recover their hearing with treatment,
and up to 10% of patients experience a
worsening of their hearing over time, despite
the introduction of adequate treatment.8
CASE REPORT
A 53 years old woman came to the
ENT clinic Dr. Sardjito after referral from
RSUD Wirosaban with chief complaint
hearing loss of the right ear since 10 days
ago after she woke up in the morning. The
hearing loss came suddenly and no
improvement of hearing. There was no
complaint or disease of the ear before. The
hearing loss was accompanied with buzzing
that came continuously. She sometimes felt
dizzy for a while but she still could do her
routine. There is no history of discharge and
pain of the ear. She felt no fullness of the
ear. She lived not in noisy environment.
History of the previous illness : there is no
history with same complaint, diabetes
mellitus (-), hypertension (-)
On physical examination found that
the general condition was good, compos
mentis, blood pressure 110/70 mmHg, pulse
82 x/ minute, respiratory rate 18 x/minute,
temperature 36,70 C. From ENT examination
: palpation of auris dextra : tragus pain (-),
auris sinistra tragus pain(-), otoscopy auris
dextra : discharge (-), hyperemic (-),
oedema (-), tympanic membrane intact, cone
of light (+). Otoscopy auris sinistra :
discharge (-), hyperemic (-), oedema (-),
tympanic membrane intact, cone of light (+).
Anterior rhinoscopy : hyperemic turbinate (-
), discharge (-), mass (-), septum deviation
(-). Oropharyng examination : hyperemic (-),
granulation (-), tonsil T1-T1. Indirect
laryngoscopy within normal limit.
Tuning fork examination : Rinne test
right ear difficult to evaluate left ear (+),
Weber lateralization to the left, Scwabach
diminished on the right ear and equal with
examiner on the left ear. Conclusion : auris
dextra sensorineural hearing
Audiometry examination : severe
sensorineural hearing loss auris dextra and
mild conductive hearing loss auris sinistra
Based on the anamnesis and physical
examination the patient was diagnosed with
auris dextra sudden deafness
This pasien was treated
metilprednisolone 16 mg 1-1-0 for the first 3
days, tapering off 8 mg 1-1-0 for the second
3 days and betahistin maleate 3 x 8 mg. The
patient was suggested to control to ENT
clinic 1 week after. On the control after one
week treatment the patient still complained
about the hearing loss and buzzing, but the
dizziness reduced. Otoscopy examination
both ear was still within normal limit. The
therapy metilprednisolone 8 mg 1-1-0 and
betahistine maleate continued. The patient
suggested to control after 1 week. After the
next control the patient still felt the hearing
loss but there was improvement in buzzing
and dizziness. Otoscopy examination was
within normal limit, and audiometry
examination was held. Audiometry
examination : severe sensoryneural hearing
loss auris dextra and normal hearing auris
sinistra. Metilprednisolone 8 mg 1-1-0 for
the first 3 days and 4 mg for the second
three days continued and the patient was
suggested to control after a week
loss.
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.
The following hearing recovery criteria
based on average hearing threshold at 0,25,
with
0,5 ,1 ,2 ,4 and 8 kHz : A) No improvement:
improvement < 15 dB. B) Slight
improvement: improvement > 15 dB, but
final PTA > 45dB. C) Partial improvement:
improvement > 15 dB, but final PTA
between 25-45 dB. D) Full recovery: until
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obtaining PTA < 25 dB or independently of treatment protocols and recovery definitions
dB gained.1 may be responsible for the lack of
consensus. According to Edizer et al, the
The most commonly accepted factors
spontaneous recovery rate of sudden
of poor prognosis in SD in the literature are
deafness ranges between 32% and 65% and
the following: 1) Advanced age of the
most commonly occurs within the first two
patient. 2) Cardiovascular risk factors
weeks after the onset of hearing loss.
(arterial hypertension, etc.). Exposure to
Sudden deafness occurs across a wide range
noise trauma. 3) Intensity of the initial loss:
of ages; however, it is more common
the greater the degree of loss, the worse the
between 30 and 50 years of age. More
prognosis for recovery of hearing function.
importantly, older age (>60 years) was
4) Type of audiometric curve: cophosis or
shown to have a negative prognostic impact
high frequency deafness cases have a lower
with 55.9% of the cases in this age group
percentage of recovery. 5) Healthy ear
having no recovery. Although some
auditory function: when the contralateral ear
previous reports have shown that older age
suffers a different pathology, the SD of the
is related to negative prognosis, others have
affected ear has a poorer prognosis for
found no correlation between age and
recovery. 6) Associated symptoms: the
prognosis. By contrast, younger patients
presence of vestibular symptoms represents
were reported to have a better prognosis.
a greater involvement of the entire labyrinth,
The coexistence of vestibular symptoms has
and has a worse prognosis. 7) Promptly
been considered as a negative prognostic
initiated treatment: the sooner the process is
factor in many previous studies. The
treated, the greater the chances of recovery.
involvement of the superior vestibular
8) Speed of onset of clinical improvement:
pathway and extensive damage of the inner
the earlier the onset of the improvement of
ear may be responsible for the appearance of
clinical symptoms, the better the functional
vertigo in sudden deafness. The type of the
outcome of the SD.1
audiogram curve has also been investigated
Many factors have been reported to as a prognostic factor in many previous
have a prognostic impact on sudden studies. Downward sloping (greater hearing
deafness. Unfortunately, no consensus has loss at high frequencies) has been reported
been achieved on these factors. Different as a negative prognostic factor, whereas

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upward sloping (low frequency hearing loss)
has been related to a better prognosis. The
correlation between the degree of initial
hearing loss and recovery has been well
established. In the present study, patients
presenting profound hearing loss had the
highest rate of no recovery (45.9%)
compared with a complete recovery rate of
6.6%. The hearing outcomes of patients with
or without tinnitus did not significantly
differ (p=0.253). Tinnitus seems
influencing the prognosis. On the other
hand, tinnitus has been reported as a
prognostic factor correlated with better
recovery rates in some studies. It is assumed
that presence of tinnitus after cochlear injury
would indicate that hair cells remain viable
The impact of the presence of comorbidities
(hypertension and diabetes mellitus) on the
outcome is not well known. These systemic
diseases have been considered as poor
prognostic factors in some studies, whereas
they were shown to be unrelated to the
outcome in some other studies. In our study,
the only notable correlation was found
between hypertension and prognosis, where
we found that patients with hypertension
have a higher ratio of non-recovery
compared to those without hypertension.
Additional treatment modalities, including
Hyperbaric Oxygen, antivirals,
vasodilators, have also been extensively
studied with conflicting results. The time
between the onset of hearing loss and of
treatment appears to be an important
prognostic factor in this study. The effects
of the causative factor may become
permanent with time. The beginning
treatment 10 days after the onset of hearing
loss is related to a worse prognosis. A
delayed onset of treatment is reported to be
not a negative prognostic factor in many
previous reports. Many previous studies
have found that the subjects seen earlier
after onset of hearing loss show greater
improvement, reflecting either the benefit of
early treatment or the natural recovery rate.
Fetterman et al. suggested that if therapy
with one or more treatment types was
started within 1 month of sudden hearing
loss, there was a 50% rate of improvement
,25% if started later than 1 month.
According to several reports, advanced age,
hypertension, diabetes, and hyperlipidemia
are poor prognostic factors, suggesting that
microvascular dysfunction in the cochlea
gives rise to poor outcomes. 8,9,10
In this patient, the prognostic may be
not good because of the level of hearing loss
(severe SNHL) and the etiology remains
unknown. The patient is in advanced age
and
and it is accompanied with vertigo.
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SUMMARY
It is reported a 53 years old woman
diagnosed with auris dextra sudden
deafness. The patient was treated with oral
steroid tapering off. The patient was
suggested to control every week to evaluate
the hearing loss. On the second control the
patient still complained the hearing loss and
there was no improvement in the audiometry
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