Acta Otorrinolaringol Esp.

2019;70(5):290---300

www.elsevier.es/otorrino

SPECIAL ARTICLE

Update on consensus on diagnosis and treatment of

idiopathic sudden sensorineural hearing loss夽
Mayte Herrera,a,∗ José Ramón García Berrocal,b Ana García Arumí,c María José Lavilla,d
Guillermo Plazaa , Grupo de Trabajo de la Comisión de Audiología de la SEORL

a
Servicio de Otorrinolaringología, Hospital Universitario de Fuenlabrada, Universidad Rey Juan Carlos, Madrid, Spain
b
Servicio de Otorrinolaringología, Hospital Universitario Puerta de Hierro Majadahonda, Universidad Autónoma, Madrid, Spain
c
Servicio de Otorrinolaringología, Hospital Vall d’Hebron, Universidad Autónoma, Barcelona, Spain
d
Servicio de Otorrinolaringología, Hospital Clínico, Universidad de Zaragoza, Spain

Received 10 April 2018; accepted 18 April 2018

KEYWORDS Abstract
Idiopathic sudden Introduction: Idiopathic sudden sensorineural hearing loss (ISSNHL) is a sudden, unexplained
sensorineural hearing unilateral hearing loss.
loss; Objectives: To update the Spanish Consensus on the diagnosis, treatment and follow-up of
Acumetry; ISSNHL.
Audiometry; Material and methods: After a systematic review of the literature from 1966 to March 2018, on
Magnetic resonance; MESH terms ¨(acute or sudden) hearing loss or deafness¨, a third update was performed, including
Steroids; 1508 relevant papers.
Adverse effects Results: Regarding diagnosis, once ISSNHL is clinically suspected, the following diagnostic tests
are mandatory: otoscopy, acumetry, tonal audiometry, speech audiometry, and tympanometry,
to discount conductive causes. After clinical diagnosis has been established, and before treat-
ment is started, a full analysis should be performed. An MRI should then be requested, ideally
performed during the first 15 days after diagnosis, to discount specific causes and to help to
understand the physiopathological mechanisms in each case. Although treatment is very con-
troversial, due to its effect on quality of life after ISSNHL and the few rare adverse effects
associated with short-term steroid treatment, this consensus recommends that all patients
should be treated with steroids, orally and/or intratympanically, depending on each patient. In
the event of failure of systemic steroids, intratympanic rescue is also recommended. Follow-up
should be at day 7, and after 12 months.
Conclusion: By consensus, results after treatment should be reported as absolute dBs recovered
in pure tonal audiometry and as improvement in speech audiometry.
© 2018 Sociedad Española de Otorrinolaringologı́a y Cirugı́a de Cabeza y Cuello. Published by
Elsevier España, S.L.U. All rights reserved.

夽
Please cite this article as: Herrera M, Berrocal JRG, Arumí AG, Lavilla MJ, Plaza G. Actualización del consenso sobre el diagnóstico y
tratamiento de la sordera súbita idiopática. Acta Otorrinolaringol Esp. 2019;70:290---300.
∗ Corresponding author.

E-mail address: mariateresa.herrera@salud.madrid.org (M. Herrera).

2173-5735/© 2018 Sociedad Española de Otorrinolaringologı́a y Cirugı́a de Cabeza y Cuello. Published by Elsevier España, S.L.U. All rights
reserved.
Update on consensus on diagnosis and treatment of idiopathic sudden sensorineural hearing loss 291

PALABRAS CLAVE Actualización del consenso sobre el diagnóstico y tratamiento de la sordera súbita
Sordera súbita; idiopática
Acumetría;
Resumen
Audiometría;
Introducción: : La sordera súbita idiopática (SSI) es aquella hipoacusia neurosensorial de inicio
Resonancia
súbito de causa desconocida.
magnética;
Objetivos: : Actualización del consenso sobre el diagnóstico, tratamiento y seguimiento de la
Corticoides;
SSI.
Efectos secundarios
Material y métodos: Presentamos una tercera actualización del consenso de SSI, mediante
revisión sistemática de la literatura sobre la SSI desde 1966 hasta marzo de 2018, sobre los
términos MESH ¨(acute or sudden) hearing loss or deafness¨, incluyendo 1.508 artículos relevantes.
Resultados: En cuanto al diagnóstico, ante una sospecha clínica de SSI, las pruebas diagnósticas
que se consideran necesarias son: otoscopia, acumetría, audiometría tonal, audiometría verbal
y timpanograma para descartar causas transmisivas de sordera. Una vez hecho el diagnóstico
clínico de SSI, antes de comenzar el tratamiento, se solicitará una batería analítica, debiendo
completarse más tarde el estudio con RM de oído interno, idealmente en los primeros 15 días,
para descartar causas específicas de sordera súbita neurosensorial y para contribuir a elucidar
posibles mecanismos fisiopatológicos. A pesar de la controversia en cuanto el tratamiento de SSI
se recomienda, por los efectos en la calidad de vida de la SSI y los raros eventos indeseables con
tratamiento esteroide a corto plazo, que el tratamiento de la SSI esté basado fundamentalmente
en los corticoides, pudiendo utilizarse la vía oral y/o intratimpánica, en función del paciente.
En caso de fracaso de la vía sistémica, se recomienda usar corticoides intratimpánicos como
rescate. Respecto al seguimiento, se realizará un control a la semana del inicio del mismo, a 7
días y hasta 12 meses.
Conclusiones: Como consenso, el resultado de los tratamientos aplicados debería presentarse,
tanto en cantidad de dBs recuperados en el umbral auditivo tonal, como con parámetros de
audiometría verbal.
© 2018 Sociedad Española de Otorrinolaringologı́a y Cirugı́a de Cabeza y Cuello. Publicado por
Elsevier España, S.L.U. Todos los derechos reservados.

Introduction more recently, following the world congress in Paris in June

In recent years, following a first approach in 2008 in the 3rd
AMORL Congress, the need has been highlighted to develop
a consensus document on the diagnosis and treatment of
idiopathic sudden sensorineural hearing loss (ISSNHL). This
document was necessary because this is an entity that con-
stitutes a medical emergency, whose exact incidence is
unknown, its pathogenesis is theorised, it is of variable and
multifactorial outcome, and its treatment is controversial.
For this reason, a first version of this consensus was
published in 2011, which has been widely disseminated in
recent years.1 Under the auspices of the SEORL, 2 subse-
quent sessions have been held on ISSNHL in Madrid in 2014
and in Seville in 2015.2 In May 2016, a meeting of experts
was held in Toledo, backed by the Audiology Commission,
from which a second version of the consensus was obtained,
which was disseminated through an instruction course in
the national congress of 2016 in Seville. Over these years,
intense dissemination work has been carried out, not only
nationally----making ISSNHL a better-known entity among
Spanish specialists----but also in international forums.3
During this time, new consensuses have been published
on ISSNHL. Of note are the Mexican consensus of 2011,4 the
consensus of the American Academy of Otolaryngology in
2012,5 the review of the German guidelines of 2014,6 and,
2017, a new consensus on the treatment of ISSNHL, of inter-
national intent.7
Therefore, it now seems appropriate to present a new,
third, update of the national ISSNHL consensus, for dissemi-
nation via SEORL, with a view to making it national in scope
and application.
Methodology
With respect to methodology, this consensus group has con-
ducted a systematic review of the literature published from
1966 to 20 March 2018, using MESH terms ‘‘(acute or sudden)
hearing loss or deafness’’, and has already collected a total
of 6256 articles in Spanish, English, German and French,
which have been reviewed by the authors in relation to the
evidence for each suggestion made.
When the first edition of the consensus was published
in 2008, there were no clinical practice guidelines or con-
sensuses on ISSNHL, and few completed randomised studies
or meta-analyses. However, in recent years a number of
consensuses and guidelines have been published on the
subject.4---7
In fact, out of the total number of articles reviewed,
1504 were selected, whose abstracts were relevant. Of
292 M. Herrera et al.

20 dB or more, within less than 12 h, including hearing loss

6,256 articles identified in Medline on waking in the morning, which could improve throughout
until March 2018 the same day.1,9---12 Finally, ISSNHL is defined as sudden hear-
ing loss with no clear cause, which occurs in more than 2/3
of cases.1,5,11,12
The incidence of ISSNHL is unknown due to cases that
4.752 excluded due
to title or abstract not
1,504 included in the resolve spontaneously and have not required medical moni-
systematic review
being relevant toring. However, an incidence of between 2 and 20/100,000
inhabitants per year is estimated.1,5,13---18 It can occur at any
age, but predominates between the ages of 40 and 55.18
The risk factors remain uncertain. There is no pre-
18 meta-analyses dominance of sex. Some studies propose a diet poor
in vegetables, low folate levels, metabolic syndrome or
chronic otitis media as risk factors. Although the condi-
tion has been linked as a risk factor to subsequent cerebral
14 systematic vascular accident and myocardial infarction, there is dis-
reviews agreement on this.17,18 Ninety percent of patients present
with tinnitus and between 20% and 60% with associated dizzi-
ness. The latter is a symptom of poorer prognosis.1,5
The pathogenesis of the condition has not been con-
39 non –systematic firmed. Several phenomena have been postulated: viral
reviews cochleitis (herpes virus, influenza. . .), labyrinth membrane
breakdown, vascular membrane breakdown, vascular phe-
nomena and autoimmunity. The latter theory is perhaps the
most accepted.19 However, the association of these patients
41 randomised clinical with genes related to prothrombotic states such as MTHFR
trials
polymorphism, with increased fibrinogen and homocysteine
levels provides the basis for microvascular events in this
process.18,20,21
7 consensuses or
clinical guidelines
Diagnosis of idiopathic sudden sensorineural
hearing loss

Patients with suspected sudden idiopathic sensorineural

> 1,300 case series
hearing loss must be seen urgently to discount diseases
of transmissible, not sensorineural, origin and to confirm
whether it is sensorineural or perceptive hearing loss.1,5
Figure 1 Search strategy for the systematic literature review
on sudden deafness. Role of primary care

these, 18 meta-analyses, 14 systematic reviews, 39 non-
systematic reviews, 41 randomised clinical trials and 7
consensuses/clinical guidelines were included to prepare
the consensus. The rest of the studies are case series (Fig. 1).
In addition, this document seeks to collect and com-
bine the experiences of several hospitals and systematically
review the available scientific evidence to reach a common
consensus on this entity, for the benefit of patients with
ISSNHL.
Definition of idiopathic sudden sensorineural
hearing loss
Sudden sensorineural hearing loss is generally defined as sen-
sorineural hearing loss greater than 30 dB HL, in 3 or more
consecutive frequencies, over less than 72 h.1---7 It can be
bilateral in 3% of cases, or can, rarely, occur sequentially in
the contralateral ear.8
Incomplete sudden hearing loss is considered to cases
where fewer than 3 frequencies are affected, with losses of
Otoscopy and acumetry must be performed to rule out
conductive causes such as cerumen plug, foreign body,
seromucous otitis, cholesteatoma, etc, and a neurological
examination to discount, for example, a cerebral vascu-
lar accident in the region of the anteroinferior cerebellar
artery, which can cause sudden deafness. If a neurological
cause has been ruled out and sudden deafness is confirmed,
it must be referred urgently to the ENT specialist to confirm
the sensorineural cause.
The use of tuning forks is essential to rule out a communi-
cable disease.22---25 According to Browning et al.,26 compared
to tonal audiometry, the tuning fork at 256 Hz is superior to
that at 512 Hz. The Rinne test correctly identifies conduc-
tive hearing loss of 30dB in 95% of patients (n = 127), with
specificity greater than 90%. According to these authors, the
intensity comparison is more effective than the intensity
reduction method.
When the Weber test lateralises to the normal ear, it can
identify sensorineural hearing loss, but when centred it can
go unnoticed.27 On the other hand, the possibility must be
remembered of obtaining a false negative Rinne if there is
Update on consensus on diagnosis and treatment of idiopathic sudden sensorineural hearing loss
profound or total deafness, which can be a confounding fac-
tor. If in doubt, the patient should be referred to the ENT
specialist.
Role of the ENT specialist
• By means of clinical history-taking and appropriate oto-
logical examinations an identifiable cause for sudden
hearing loss can be ruled out.
• The audiological tests needed to diagnose ISSNHL are
liminar tonal audiometry (LTA), speech audiometry and
tympanogram.
• The audiological tests will be always performed before
initiating treatment and following the Spanish Association
of Audiology’s (AEDA) recommendations (Table 1).28,29
• Other audiological tests, such as auditory evoked poten-
tials, otoemissions, high- frequency audiometry, etc. are
optional: they can provide a recovery prognosis.
• Vestibular tests are optional: they can also provide a
recovery prognosis. Therefore VEMPS in the presence or
otherwise of dizziness imply a poorer prognosis,30 as well
as an altered VNG.31,32
Imaging tests
Brain magnetic resonance imaging (MRI) is recommended for
all cases of sudden deafness,1,5 it is advisable within the first
15 days following diagnosis.33 Its importance lies in ruling
out a definite cause of sudden deafness, and to help clarify
whether there is an underlying pathogenesis for the ISSNHL.
Traditionally, MRI studies on sudden deafness have sought
to diagnose some pathological conditions such as tumours of
the pontocerebellar angle, auditory canal and inner ear, as
well as demyelinating neurogenerative diseases and cere-
brovascular accidents as possible causes of non-idiopathic
sudden deafness.33---36
However this concept has changed. Attempts are cur-
rently being made to deepen the knowledge of the
pathophysiological mechanisms responsible for ISSNHL.
Therefore, it is recommended that an MRI should be per-
formed in the first 2---4 weeks, in order to demonstrate
changes in the composition of the fluids in the labyrinth. This
has already been described in a case from many years ago:
it was attributed to a blood-labyrinth barrier breakdown,
possibly due to the deposition of immune complexes or anti-
bodies against certain inner ear antigens.37 The refinement
of MRI (3T) and use of 3D-FLAIR (fluid-attenuated inversion-
recovery) sequences, that are part of the routine protocol
for the study of the brain, enable current MRI images to
establish different radiological patterns corresponding to
bleeding, increased protein concentration in the inner ear
or cochlear inflammation due to blood-labyrinth barrier
breakdown by showing a pre-contrast hyperintense signal
as a consequence of increased vascular permeability.38 This
hyperintense signal in the labyrinth in enhanced sequence
on pre-contrast T1 observed in cases of labyrinthine haem-
orrhage and cochlear inflammation contrasts with acoustic
neuromas, which are usually diagnosed by T2-enhanced
sequences on increasing their signal after the administration
of gadolinium.39 Likewise, a hyper-signal has been positively
related with pre-treatment hearing loss and dizziness in a
293
study that proposed 3 radiological patterns correlated with
mild haemorrhage, acute inflammation and the presence or
absence of blood-labyrinth barrier breakdown.40 When there
are diagnostic doubts, as in cases of sudden repetitive deaf-
ness, endolymphatic hydrops must be ruled out, which has
recently been demonstrated with intratympanic gadolinium
MRI in patients with Menière’s disease41 and immunomedi-
ated disease of the inner ear.42
Laboratory tests
These are intended to rule out any biochemical, metabolic,
haematological, infectious or autoimmune cause for sud-
den deafness, and to find factors that would contraindicate
corticosteroid treatment.1,4,5,43
Elemental biochemistry, fibrinogen, blood count, antinu-
clear antibodies, erythrocyte sedimentation rate, serology
for syphilis and immunophenotype are recommended.1 If the
geographic area is endemic, Lyme’s disease must be ruled
out.5
Given that the rest of the analytical tests have not
proved cost-effective, we do not recommend requesting
other laboratory tests if there is no reason for them to be
recommended in an individual case.44,45
Prognostic factors associated with idiopathic
sudden sensorineural hearing loss
The following data should be noted in the clinical his-
tory as factors of proven influence in the prognosis for the
disease:1,5,7,46---48
• Age.
• Delay until start of treatment (days).
• Associated symptoms: dizziness, tinnitus, etc.
• Association with other diseases, especially cardiovascular
diseases.
• Curve type: pantonal, upward (low frequencies), down-
ward (high frequencies), in cuvette (averages).49,50
• Degree of hearing loss at onset.
Recommended treatment for idiopathic
sudden sensorineural hearing loss
Primary treatment
All patients diagnosed with ISSNHL should be treated with
corticosteroids within 45 days of onset of the episode. In
late cases, treatment will be discussed on a case-by-case
basis.
Only in the case of severe deafness (ISSNHL > 70 dB), in
one ear or with associated intense dizziness, can intravenous
(IV) treatment with corticosteroids be offered for 5 days,
at megadoses of 500 mg/day, followed by the usual oral
regimen51---55 : patients should be closely monitored for the
possible onset of side effects.56
Although the literature varies greatly,2,57---63 this consen-
sus recommends offering one of these 3 corticosteroid
treatment alternatives (at the discretion of each specialist
and depending on the patient’s concomitant disease):
294
Table 1 Recommendations for audiological tests in the study of sudden deafness.
Recommendations for liminar tonal audiometry
Although some authors, such as AAOHNS,5 propose the use of the frequency 500 Hz to 3 kHz, because our language is
phonetically different to English, we recommend recording the frequencies .25-.5-1-2-4-8 kHz, taking the average of all of
them as the PTA, following the instructions of AEDA28
Recommendations for speech audiometry
Study speech comprehension by speech audiometry, determining SRT and Dmax, following the instructions of AEDA29
AEDA: Spanish Association of Audiology; LTA: liminar tonal audiometry; SA: speech audiometry; Dmax: maximum discrimination; PTA:
pure tone average; SRT: speech reception threshold.
• Oral treatment (OR).64---69
• Intratympanic treatment (IT).70---74
• Combined treatment (OR+IT).75---80
Despite the criticism of some authors of corticosteroids
in ISSNHL,64,65 many revisions and meta-analyses have
reviewed the effectiveness of IT treatment with corti-
costeroids in ISSNHL, both as a single treatment and in
combined regimens,76,77,81---90 primary as well as salvage.91---94
Salvage treatment
After assessment of treatment at 5 days, the following
options are recommended:
• After failure of oral treatment (OR), adding IT is recom-
mended.
• After failure of IT treatment, systemic or hyperbaric
chamber treatment can be added.
• After failure of OR + IT, IV or hyperbaric chamber treat-
ment are recommended.
Corticosteroid treatment regimens for
idiopathic sudden sensorineural hearing loss
Oral treatment
For 25 days, in downward tapering doses every 5 days, which
it is advised to complete regardless of the initial treatment
response. One of these options is recommended:
• Prednisone (1 mg/kg weight per day).
• Methylprednisolone (1 mg/kg weight per day).
• Deflazacort (1.5 mg/kg weight per day).
Intratympanic treatment
A weekly dose is recommended over 3 weeks. Likewise, it
is recommended that the 3 doses are completed, regardless
of initial treatment response.
Prior to IT, topical anaesthesia with phenol or lidocaine
on the tympanic membrane is recommended.
For the injection, use an abbocath needle N. 24---27 or
22---27 G lumbar puncture needle. The higher the G of the
needle, the greater the injection point collapse achieved,
avoiding the complication of perforating the tympanic mem-
brane and minimising reflux of the drug towards the external
M. Herrera et al.
auditory canal and, therefore, capitalising on its availability
in the middle ear and consequently, its subsequent concen-
tration in the fluids of the labyrinth.
Once the dilution has been prepared, the greatest
amount of the drug is injected that the tympanic cavity will
allow (although the average volume of the tympanic cavity
is 0.5 cc, .7---.8 cc can usually be injected).
Mixing the drug with lidocaine is not recommended, to
avoid reducing its concentration or impairing its effect,
although its use can reduce the pain associated with the
procedure.
The patient’s head should be kept in the otological posi-
tion (affected ear upwards) for 15---30 min, and the patient
should be told not to swallow, yawn or talk.95---100
The recommended regimens are:
• Methylprednisolone, 1 cc of a 40 mg vial diluted in 2 cc.
• Dexamethasone, 9 cc of an 8 mg/ml vial.
Some authors suggest increasing the dose of intratym-
panic dexamethasone to 24 mg/ml101 or the frequency of
injections to alternate days.102
Other treatments for idiopathic sudden
sensorineural hearing loss
Following other consensuses and guidelines and despite
some sporadic work where there might have been a
response, this consensus does not recommend the use of
antivirals, thrombolytics, vasodilators or N-acetylcysteine as
routine treatment for ISSNHL.
However, various randomised clinical trials versus
placebo have shown that hyperbaric oxygen can increase
perilymph oxygen tension and restore hearing in a signif-
icant number of patients with ISSNHL.103---111 Efficacy can
depend on many factors, including the degree of perme-
ability of the labyrinthine artery and the ability to increase
perilymph oxygen tension. The best results are found when
patients are treated within 14 days of symptom onset and
concomitantly with steroids (systemic or intratympanic).
The treatment includes 20 sessions of approximately half
an hour in patients with no contraindications. Therefore, as
in other consensuses, its use is recommended in patients
with ISSNHL if primary treatment with oral or intratympanic
corticosteroids has failed.
Regarding exploratory tympanotomy and sealing of a pos-
sible labyrinthine fistula, there is increasing literature on its
use in ISSNHL, especially in Germany, based on retrospec-
Update on consensus on diagnosis and treatment of idiopathic sudden sensorineural hearing loss 295

Table 2 Follow-up of idiopathic sudden sensorineural hearing loss.

The proposed check-up visits are:
䊉 At 5---7 days with LTA (if IT weekly, assess LTA at each visit)
䊉 At 30 days with LTA-SA
䊉 And at 12 months, with LTA-SA
䊉 At 12 months, it is advisable to confirm whether the diagnosis of ISSNHL is maintained, or whether a definite cause has
been found (Menière’s disease, neurinoma, autoimmune, etc.).
LTA: liminar tonal audiometry; SA: speech audiometry; IT: intratympanic; ISSNHL: idiopathic sudden sensorineural hearing loss.

Table 3 Criteria for response to treatment of idiopathic sudden sensorineural hearing loss.
䊉 Full recovery: achieving a final PTA less or equal to 10 dB HL of the previous threshold and an SRT at most 5%---10% poorer
than the unaffected ear.
䊉 Partial recovery: an improvement of more than 10 dB of the final PTA or improvement of more than 10% of the SRT, without
achieving full recovery.
䊉 No recovery: improvement of less than 10 dB in the final PTA.
PTA: pure tone average; SRT: speech reception threshold.

tive studies.112---119 The authors who defend this technique
recommend that it should be used early, within the first
5---10 days because its results improve.114,120 However, there
are only symptoms compatible with perilymphatic fistula
in 10% of ISSNHL cases, without a baseline traumatic or
anatomical cause.116 Furthermore, evidence of fistula during
exploratory tympanotomy is independent of the prior exis-
tence of symptoms compatible with a fistula or improvement
achieved after it has been sealed.116 On the other hand,
the coexistence of corticosteroid treatment in most of the
patients included in the studies makes it impossible to deter-
mine with certainty which treatment is responsible for the
improvement.119 Therefore, this consensus group only rec-
ommends this surgery when there is a clear clinical suspicion
of labyrinthine fistula.
Follow up of patients with idiopathic sudden
sensorineural hearing loss
Five to seven days following start of treatment, an LTA will
be performed to determine response and indicate the sal-
vage treatment in the event of failure.
At each follow-up visit, an LTA should be performed. It is
advisable to perform speech audiometry during the visit one
month following start of treatment and 12 months following
the episode of ISSNHL (Table 2).
Follow-up of patients diagnosed with ISSNHL must be
at 12 months at least to rule out recurrence121 and possi-
ble development of Menière’s disease.122,123 Furthermore,
in patients at vascular risk, neurological follow-up could be
advisable after ISSNHL.124,125
Assessment of response to treatment of
idiopathic sudden sensorineural hearing loss
This consensus proposes that hearing recovery after ISSNHL
should be defined according to the criteria of Stachler
et al.,5 including speech audiometry improvement, rather
than Siegle’s criteria.126
If there has been no audiometry prior to the episode of
ISSNHL, the unaffected ear will be used as the benchmark,
provided there has been no history of asymmetry in hearing
loss.
The possible responses gathered are defined in Table 3.
In addition, this consensus recommends providing results
of treatment in relation to the grades of initial hearing
loss and to the prognostic factors of unfavourable outcome,
which will enable the comparative results to be better anal-
ysed.
The effect on patients’ quality of life is another measure
to be considered in assessing ISSNHL.127,128
Hearing rehabilitation after failure of
treatment of idiopathic sudden sensorineural
hearing loss
When there has been no full response to the abovemen-
tioned treatments and the hearing loss is not sufficiently
alleviated with hearing aids appropriate to the hearing loss
and, therefore, hearing is not functional, the indication for
a hearing aid should be assessed on an individual basis:
In cases of bilateral severe-profound hearing loss sec-
ondary to sudden hearing loss (in both ears, or in the only
hearing ear), a cochlear implant (CI) would be indicated with
a view to alleviating the hearing loss and masking tinnitus.129
In a situation of asymmetric hearing loss when there is
sensorineural hearing loss that is severe-profound in one ear
and moderate-severe in the contralateral ear (41---89 dB HL)
placement of a CI would be indicated in the poorer hearing
ear and a hearing aid in the contralateral ear. This hearing
stimulation strategy is termed bimodal stimulation.130
In a condition of extreme asymmetry, i.e., sudden
profound unilateral hearing loss (with normal-hearing con-
tralateral ear or mild hearing loss no greater than 20 dB),
hearing rehabilitation options include the conventional
CROS (contralateral routing of signal) systems, bone-
anchored implants with CROS effect131---134 and, finally, a CI.
The latter option is possible since the brain can integrate
296
both acoustic and electrical information, even if one ear
hears well. The CROS systems and bone-anchored implants
do not provide true binaurality since the brain only receives
and processes hearing input from one side, however, binau-
rality can be restored using a CI and it has the consequent
benefits, among others, in sound source location and noise
discrimination.135
Furthermore, unilateral hearing loss can be associated
with tinnitus in the affected ear, which is sometimes
incapacitating. In adult patients, over the age of 18, if
tinnitus causes disability with a Tinnitus Handicap Inven-
tory score higher than 58, lasts for more than one year
and conventional treatments have failed, including tinnitus
retraining therapy, outcomes after CI have demonstrated
clinical improvement in the perception and tolerance of
tinnitus,136---139 and restored hearing.
In sudden profound unilateral hearing loss in children of
any age, since this is acquired hearing loss, the prognosis
of a CI in terms of speech discrimination and advantages
of binaurality is just as favourable as in adults, especially
if implantation takes place soon after the hearing loss,
given the good previous hearing experience of the affected
ear.140,141
Objectives for the 4th review of the idiopathic
sudden sensorineural hearing loss consensus
for 2020
Once there is greater knowledge of the disease in Spain,
the diagnostic and therapeutic protocol for ISSNHL must be
made more uniform throughout Spain.
To gain the collaboration of all professionals treating
patients with ISSNHL to form a national ISSNHL register, in
online format, which can be briefly completed and easily
sent to SEORL, via the Audiology Commission.
To notify the relevant outcomes from the register for
years to come to show the epidemiology of ISSNHL in Spain
and assessment of treatments.
Conflict of interests
The authors have no conflict of interests to declare. This
research study has received no specific grants from the pub-
lic or commercial sector or from not-for-profit entities.
References
1. Plaza G, Durio E, Herráiz C, Rivera T, García-Berrocal JR, Aso-
ciación Madrileña de ORL. Consenso sobre el diagnóstico y
tratamiento de la sordera súbita. Acta Otorrinolaringol Esp.
2011;62:144---57.
2. Amarillo E, Hernández-García E, Herrera M, García Berrocal
JR, García Arumí A, Durio E, et al. Sordera súbita: encuesta
nacional en España. Acta Otorrinolaringol Esp. 2016;67:59---65.
3. García-Berrocal JR. Current diagnosis and treatment of sudden
sensorineural hearing loss. Keynote lecture. 4th Congress of
European ORL-HNS. 7---11 October, 2017. Barcelona, Spain.
4. Alfaro MPM, Ascencio MM, Millan YK. Guía de Práctica Clínica:
Diagnóstico y tratamiento de la hipoacusia sensorineural
súbita idiopática. Centro Nacional de Excelencia Tecnológica
en Salud. Gobierno de México. IMSS-416-10. Disponible en:
M. Herrera et al.
http://www.cenetec.salud.gob.mx/descargas/gpc/Catalogo
Maestro/416 IMSS 10 hipoacusia sensorineural idio/EYR IMSS
416 10.PDF (consultado el 2-Noviembre-2014).
5. Stachler RJ, Chandrasekhar SS, Archer SM, Rosenfeld RM,
Schwartz SR, Barrs DM, et al. Clinical practice guideline: sud-
den hearing loss. Otolaryngol Head Neck Surg. 2012;146 3
Suppl:S1---35.
6. Arnold W, Brusis T, Canis M, Hesse G, Klemm E, Löhler J,
et al. Akuter idiopathischer sensorineuraler Hörverlust.
Hörsturz.Leitlinie der Dt. Ges. f. Hals-Nasen-OhrenHeilkunde,
Kopf- und Hals-Chirurgie. Disponible en: http://www.
awmf.org/uploads/tx szleitlinien/017-010l S1 Hoersturz
2014-02.pdf (consultado el 5-Diciembre-2015).
7. Marx M, Younes E, Chandrasekhar SS, Ito J, Plontke S, O’Leary
S, et al. International consensus (ICON) on treatment of sud-
den sensorineural hearing loss. Eur Ann Otorhinolaryngol Head
Neck Dis. 2018;135(1S):S23---8.
8. Fetterman BL, Luxford WM, Saunders JE. Sudden bilateral sen-
sorineural hearing loss. Laryngoscope. 1996;106:1347---50.
9. Hughes GB, Freedman MA, Haberkamp TJ, Guay ME. Sud-
den sensorineural hearing loss. Otolaryngol Clin North Am.
1996;29:393---405.
10. Mattox DE, Lyles CA. Idiopathic sudden sensorineural hearing
loss. Am J Otol. 1989;10:242---7.
11. Rauch SD. Clinical practice. Idiopathic sudden sensorineural
hearing loss. N Engl J Med. 2008;359:833---40.
12. Schreiber BE, Agrup C, Haskard DO, Luxon LM. Sudden sen-
sorineural hearing loss. Lancet. 2010;375:1203---11.
13. Byl F. Thirty-two cases of sudden profound hearing loss occur-
ring in 1973: incidence and prognostic findings. Trans Sect Oto-
laryngol Am Acad Ophthalmol Otolaryngol. 1975;80:298---305.
14. Alexander TH, Harris JP. Incidence of sudden sensorineural
hearing loss. Otol Neurotol. 2013;34:1586---9.
15. Kim SH, Kim SJ, Im H, Kim TH, Song JJ, Chae SW. A trend
in sudden sensorineural hearing loss: data from a population-
based study. Audiol Neurootol. 2018;22:311---6.
16. Kitoh R, Nishio SY, Ogawa K, Kanzaki S, Hato N, Sone M,
et al. Nationwide epidemiological survey of idiopathic sud-
den sensorineural hearing loss in Japan. Acta Otolaryngol.
2017;137(sup565):S8---16.
17. O’Malley MR, Haynes DS. Sudden hearing loss. Otolaryngol Clin
North Am. 2008;41:633---49.
18. Weber PC. In: Dechler DJ, Waltham MA, editors. Sudden sen-
sorineural hearing loss. 2017 [Fecha de consulta: Marzo 18,
2018].
19. García-Berrocal J, Ramírez-Camacho R. Sudden sensorineural
hearing loss: supporting the immunologic theory. Ann Otol Rhi-
nol Laryngol. 2002;111:989---97.
20. Sauvaget E, Kici S, Petelle B, Kania R, Chabriat H, Herman P,
et al. Vertebrobasilar occlusive disorders presenting as sudden
sensorineural hearing loss. Laryngoscope. 2004;114:327---32.
21. Kim HA, Lee H. Recent advances in understanding
audiovestibular loss of a vascular cause. J Stroke.
2017;19:61---6.
22. Stankiewicz JA, Mowry HJ. Clinical accuracy of tuning fork
tests. Laryngoscope. 1979;89:1956---63.
23. Burgess LP, Frankel SF, Lepore ML, Yim DW. Tuning fork screen-
ing for sudden hearing loss. Mil Med. 1988;153:456---8.
24. Bagai A, Thavendiranathan P, Detsky AS. Does this patient have
hearing impairment? JAMA. 2006;295:416---28.
25. Miltenburg DM. The validity of tuning fork tests in diagnosing
hearing loss. J Otolaryngol. 1994;23:254---9.
26. Browning GG, Swan IR, Chew KK. Clinical role of informal tests
of hearing. J Laryngol Otol. 1989;103:7---11.
27. Shuman AG, Li X, Halpin CF, et al. Tuning fork testing in sudden
sensorineural hearing loss. JAMA Intern Med. 2013;173:706.
28. AEDA. Normalización de las pruebas Audiológicas
(I): La audiometrıa tonal liminar [en línea]. Auditio.
Update on consensus on diagnosis and treatment of idiopathic sudden sensorineural hearing loss
2002;1(2):16---9. Disponible en: http://www.auditio.com/
revista/pdf/vol1/2/010201.pdf. (consultado el 20-Diciembre-
2015).
29. AEDA. Normalización de las pruebas audiológicas (II):
La audiometría verbal o logoaudiometríar [en linea].
Auditio. 2002;1(2):34---6. Disponible en: http://www.
auditio.com/revista/pdf/vol1/3/010301.pdf. (consultado el
20-Diciembre-2015).
30. Genestar Bosch EI. Tesis doctoral. Potenciales evocados
vestibulares miogénicos en la sordera brusca. Barcelona: Uni-
versidad Autónoma; 2011.
31. Oiticica J, Bittar RS, Castro CC, Grasel S, Pereira L, Bas-
tos S, et al. Contribution of audiovestibular tests to the
topographic diagnosis of sudden deafness. Int Arch Otorhino-
laryngol. 2013;17:305---14.
32. Yu H, Li H. Vestibular dysfunctions in sudden sensorineural
hearing loss: A systematic review and meta-analysis. Front
Neurol. 2018;9:45.
33. Conte G, Di Berardino F, Sina C, Zanetti D, Scola E, Gavagna C,
et al. MR imaging in sudden sensorineural hearing loss. Time
to talk. Am J Neuroradiol. 2017;38:1475---9.
34. Liao WH, Wu HM, Wu HY, Tu TY, Shiao AS, Castillo M, et al.
Revisiting the relationship of three-dimensional fluid attenua-
tion inversion recovery imaging and hearing outcomes in adults
with idiopathic unilateral sudden sensorineural hearing loss.
Eur J Radiol. 2016;85:2188---94.
35. Gimsing S. Vestibular schwannoma: when to look for it? J Laryn-
gol Otol. 2010;124:258---64.
36. Hentschel M, Scholte M, Steens S, Kunst H, Rovers M.
The diagnostic accuracy of non-imaging screening protocols
for vestibular schwannoma in patients with asymmetrical
hearing loss and/or unilateral audiovestibular dysfunction:
a diagnostic review and meta-analysis. Clin Otolaryngol.
2017;42:815---23.
37. García-Berrocal JR, Vargas JA, Vaquero-Ruano M, Ramón y
Cajal S, Ramírez-Camacho R. Coganś syndrome: an oculo-
audiovestibular disease. Postgrad Med J. 1999;75:262---4.
38. Tanigawa T, Tanaka H, Sato T, Nakao Y, Katahira N, Tsuchiya Y,
et al. 3D-FLAIR MRI findings in patients with low-tone sudden
deafness. Acta Otolaryngol. 2010;130:1324---8.
39. Cadoni G, Cianfoni A, Agostino S, Scipione S, Tartaglione T,
Galli J, et al. Magnetic resonance imaging findings in sudden
sensorineural hearing loss. J Otolaryngol. 2006;35:310---6.
40. Berrettini S, Seccia V, Fortunato S, Forli F, Bruschini L,
Piaggi P, et al. Analysis of the 3-dimensional fluid-attenuated
inversion-recovery (3D-FLAIR) sequence in idiopathic sudden
sensorineural hearing loss. JAMA Otolaryngol Head Neck Surg.
2013;139:456---64.
41. Tuñón Gómez M, Lobo Duro D, Brea Álvarez B, García Berrocal
JR. Diagnóstico de hidrops endolinfático mediante RM de 3T
tras administración de gadolinio intratimpánico. Radiología.
2017;59:159---65.
42. Lobo D, Tuñón M, Villareal I, Brea B, García-Berrocal JR.
Intratympanic gadolinium MRI can support the role of endolym-
phatic hydrops in immune-mediated inner ear disease. J
Laryngol Otol. 2018;132:554---9.
43. Lobo D, López FG, García-Berrocal JR, Ramírez-Camacho R.
Diagnostic tests for immunomediated hearing loss: a system-
atic review. J Laryngol Otol. 2008;122:564---73.
44. Wittig J, Wittekindt C, Kiehntopf M, Guntinas-Lichius O. Prog-
nostic impact of standard laboratory values on outcome in
patients with sudden sensorineural hearing loss. BMC Ear Nose
Throat Disord. 2014;14:6.
45. Fasano T, Pertinhez TA, Tribi L, Lasagni D, Pilia A, Vecchia L,
et al. Laboratory assessment of sudden sensorineural hearing
loss: a case-control study. Laryngoscope. 2017;127:2375---81.
46. Nakagawa T, Yamamoto M, Kumakawa K, Usami S, Hato N,
Tabuchi K, et al. Prognostic impact of salvage treatment on
297
hearing recovery in patients with sudden sensorineural hear-
ing loss refractory to systemic corticosteroids: a retrospective
observational study. Auris Nasus Larynx. 2016;43:489---94.
47. Okada M, Hato N, Nishio S, Kitoh R, Ogawa K, Kanzaki S,
et al. The effect of initial treatment on hearing prognosis
in idiopathic sudden sensorineural hearing loss: a nation-
wide survey in Japan. Acta Otolaryngol (Stockh). 2017;
137(sup565):S30---3.
48. Xie S, Qiang Q, Mei L, He C, Feng Y, Sun H, et al.
Multivariate analysis of prognostic factors for idiopathic
sudden sensorineural hearing loss treated with adjuvant
hyperbaric oxygen therapy. Eur Arch Otorhinolaryngol.
2018;275:47---51.
49. Tran Ba Huy P, Sauvaget E. Idiopathic sudden sensorineu-
ral hearing loss is not an otologic emergency. Otol Neurotol.
2005;26:896---902.
50. Choo OS, Yang SM, Park HY, Lee JB, Jang JH, Choi SJ,
et al. Differences in clinical characteristics and prognosis of
sudden low- and high-frequency hearing loss. Laryngoscope.
2017;127:1878---84.
51. Aoki D, Takegoshi H, Kikuchi S. Evaluation of super-high-dose
steroid therapy for sudden sensorineural hearing loss. Oto-
laryngol Head Neck Surg. 2006;134:783---7.
52. Egli Gallo D, Khojasteh E, Gloor M, Hegemann SC. Effec-
tiveness of systemic high-dosed dexamethasone therapy for
idiopathic sudden sensorineural hearing loss. Audiol Neurootol.
2013;18:161---70.
53. Ettekharian A, Amizadeh M. Pulse steroid therapy in idiopathic
sudden sensorineural hearing loss: a randomized controlled
clinical trial. Laryngoscope. 2016;126:150---5.
54. Kim MJ, Heo KW, Jeon PH, Lee JJ, Lee JH. Necessity of
admission to improve the hearing-recovery rate in idio-
pathic sudden sensorineural hearing loss. Acta Otolaryngol.
2018;138:357---62.
55. Lamm K. Sudden sensorineural hearing loss (including perilym-
phatic fistula). In: Anniko M, Bernal-Sprekelsen M, Bonkowsky
V, Bradley P, Iurato S, editors. Otorhinolaryngology, head and
neck surgery (European Manual ot Medicine). Berlín: Springer;
2010. p. 111---6.
56. García-Berrocal JR, Ramírez-Camacho R, Lobo D, Trinidad
A, Verdaguer JM. Adverse effects of glucocorticoid therapy
for inner ear disorders. ORL J Otorhinolaryngol Relat Spec.
2008;70:271---4.
57. Coelho DH, Thacker LR, Hsu DW. Variability in the management
of idiopathic sudden sensorineural hearing loss. Otolaryngol
Head Neck Surg. 2011;145:813---7.
58. Conlin AE, Parnes LS. Treatment of sudden sensorineural hear-
ing loss: II. A meta-analysis. Arch Otolaryngol Neck Surg.
2007;133:582---6.
59. O’Connell BP, Hunter JB, Haynes DS. Current concepts in the
management of idiopathic sudden sensorineural hearing loss.
Curr Opin Otolaryngol Head Neck Surg. 2016;24:413---9.
60. Diaz RC. lt is 2016: what is the best treatment for sudden sen-
sorineural hearing loss? Curr Opin Otolaryngol Head Neck Surg.
2016;24:402.
61. Jarvis SJ, Giangrande V, John G, Thornton AR. Management of
acute idiopathic sensorineural hearing loss: a survey ot UK ENT
consultants. Acta Otorhinolaryngol ltal. 2011;31:85---9.
62. Metrailer AM, Babu SE. Management of sudden sensorineu-
ral hearing loss. Curr Opin Otolaryngol Head Neck Surg.
2016;24:403---6.
63. Stobbs N, Goswamy J, Ramamurthy L. Our experience: how are
we managing sudden sensorineural hearing loss in the United
Kingdom? Clin Otolaryngol. 2014;39:385---8.
64. Hultcrantz E, Nosrati-Zarenoe R. Corticosteroid treatment of
idiopathic sudden sensorineural hearing loss: analysis of an RCT
and material drawn from the Swedish national database. Eur
Arch Otorhinolaryngol. 2015;272:3169---75.
298
65. Nosrati-Zarenoe R, Hultcrantz E. Corticosteroid treatment
of idiopathic sudden sensorineural hearing loss: random-
ized tripleblind placebo-controlled trial. Otol Neurotol.
2012;33:523---31.
66. Wilson WR, Byl FM, Laird N. The efficacy of steroids in the
treatment of idiopathic sudden hearing loss. A double-blind
clinical study. Arch Otolaryngol. 1980;106:772---6.
67. Wilson WR. Why treat sudden hearing loss. Am J Otol.
1984;5:481---3.
68. Chen CY, Halpin C, Rauch SD. Oral steroid treatment of sud-
den onset sensorineural hearing loss: a ten tear retrospective
analysis. Otol Neurotol. 2003;24:728---33.
69. Slattery WH, Fisher LM, Iqbal Z, Liu N. Oral steroid regimens
for idiopathic sudden sensorineural hearing loss. Otolaryngol
Head Neck Surg. 2005;132:5---10.
70. Barreto MA, Ledesma AL, de Oliveira CA, Bahmad F Jr.
lntratympanic corticosteroid for sudden hearing loss: does it
really work? Braz J Otorhinolaryngol. 2016;82:353---64.
71. Filipo R, Attanasio G, Russo FY, Cartocci G, Musacchio A, De
Cario A, et al. Oral versus short-term intratympanic pred-
nisolone therapy for idiopathic sudden hearing loss. Audiol
Neurootol. 2014;19:225---33.
72. Filipo R, Covelli E, Balsamo G, Attanasio G. Intratympanic
prednisolone therapy for sudden sensorineural hearing loss: A
new protocol. Acta Otolaryngol (Stockh). 2010;130:1209---13.
73. Hobson CE, Alexander TH, Harris JP. Primary treatment of
idiopathic sudden sensorineural hearing loss with intratym-
panic dexamethasone. Curr Opin Otolaryngol Head Neck Surg.
2016;24:407---12.
74. Rauch SD, Halpin CF, Antonelli PJ, Babu S, Carey J, Gantz B,
et al. Oral vs intratympanic corticosteroid therapy for idio-
pathic sudden sensorineural hearing loss: a randomized trial.
JAMA. 2011;305:2071---9.
75. Ashtiani MK, Firouzi F, Bastaninejad S, Dabiri S, Nasirmohtaram
S, Saeedi N, et al. Efficacy of systemic and intratympanic
corticosteroid combination therapy versus intratympanic or
systemic therapy in patients with idiopathic sudden sen-
sorineural hearing loss: a randomized controlled trial. Eur Arch
Otorhinolaryngol. 2018;275:89---97.
76. Gao Y, Liu D. Combined intratympanic and systemic use
of steroids for idiopathic sudden sensorineural hearing
loss: a meta-analysis. Eur Arch Otorhinolaryngol. 2016;273:
3699---711.
77. Han X, Yin X, Du X, Sun C. Combined intratympanic and sys-
temic use of steroids as a first-line treatment for sudden
sensorineural hearing loss: A meta-analysis of randomized,
controlled trials. Otol Neurotol. 2017;38:487---95.
78. Jung da J, Park JH, Jang JH, Lee KY. The efficacy of combina-
tion therapy for idiopathic sudden sensorineural hearing loss.
Laryngoscope. 2016;126:1871---6.
79. Tsounis M, Psillas G, Tsalighopoulos M, Vital V, Maroudias N,
Markou K. Systemic, intratympanic and combined adminis-
tration of steroids for sudden hearing loss. A prospective
randomized multicenter trial. Eur Arch Otorhinolaryngol.
2018;275:103---10.
80. Yoo MH, Lim WS, Park JH, Kwon JK, Lee T-H, An Y-H, et al.
Simultaneous versus sequential intratympanic steroid treat-
ment for severe-to-profound sudden sensorineural hearing
loss. Audiol Neurotol. 2016;21:399---405.
81. El Sabbagh NG, Sewitch MJ, Bezdjian A, Daniel SJ. Intratym-
panic dexamethasone in sudden sensorineural hearing loss: a
systematic review and meta-analysis: intratympanic dexam-
ethasone for SSNHL. Laryngoscope. 2017;127:1897---908.
82. Garavello W, Galluzzi F, Gaini RM, Zanetti D. Intratympanic
steroid treatment for sudden deafness: a meta-analysis of ran-
domized controlled trials. Otol Neurotol. 2012;33:724---9.
83. Lai D, Zhao F, Jalal N, Zheng Y. Intratympanic gluco-
corticosteroid therapy for idiopathic sudden hearing loss:
M. Herrera et al.
meta-analysis of randomized controlled trials. Medicine (Bal-
timore). 2017;96:e8955.
84. Lavigne P, Lavigne F, Saliba I. Intratympanic corticosteroids
injections: a systematic review of literature. Eur Arch Otorhi-
nolaryngol. 2016;273:2271---8.
85. Li H, Feng G, Wang H, Feng Y. Intratympanic steroid ther-
apy as a salvage treatment for sudden sensorineural hearing
loss after failure of conventional therapy: A meta-analysis of
randomized, controlled trials. Clin Ther. 2015;37:178---87.
86. Liebau A, Pogorzelski O, Salt AN, Plontke SK. Hearing changes
after intratympanically applied steroids for primary therapy
of sudden hearing loss: A meta-analysis using mathemati-
cal simulations of drug delivery protocols. Otol Neurotol.
2017;38:19---30.
87. Ng JH, Ho RCM, Cheong CSJ, Ng A, Yuen HW, Ngo RYS.
Intratympanic steroids as a salvage treatment for sudden
sensorineural hearing loss? A meta-analysis. Eur Arch Otorhi-
nolaryngol. 2015;272:2777---82.
88. Qiang Q, Wu X, Yang T, Yang C, Sun H. A comparison between
systemic and intratympanic steroid therapies as initial therapy
for idiopathic sudden sensorineural hearing loss: a meta-
analysis. Acta Otolaryngol. 2017;137:598---605.
89. Saenz-Piñones JC, Villarreal IM, García-Chilleron R, Trinidad
A, Ramirez-Camacho R, García-Berrocal JR. Intratympanic
methylprednisolone for sudden sensorineural hearing loss:
comprehensive reexamination of the model. J Otolaryngol ENT
Res. 2015;3:00067.
90. Swachia K, Sharma D, Singh J. Efficacy of oral vs. intratympanic
corticosteroids in sudden sensorineural hearing loss. J Basic
Clin Physiol Pharmacol. 2016;27:371---7.
91. Belhassen S, Saliba I. Intratympanic steroid injection as a
salvage treatment for sudden sensorineural hearing loss. J
Laryngol Otol. 2014;128:1044---9.
92. Dai Y, Lu L, Hou J, Yang X, Li H, Yang Y, et al. Intratympanic
methylprednisolone perfusion as a salvage treatment for pro-
found idiopathic sudden sensorineural hearing loss. J Laryngol
Otol. 2017;131:404---10.
93. Dallan I, Fortunato S, Casani AP, Bernardini E, Sellari-
Franceschini S, Berrettini S, et al. Long-term follow up
of sudden sensorineural hearing loss patients treated with
intratympanic steroids: audiological and quality of lite eval-
uation. J Laryngol Otol. 2014;12:669---73.
94. Dispenza F, De Stefano A, Costantino C, Marchese D, Riggio
F. Sudden sensorineural hearing loss: Results of intratym-
panic steroids as salvage treatment. Am J Otolaryngol.
2013;34:296---300.
95. Chandrasekhar SS, Rubinstein RY, Kwartler JA, Gatz M, Con-
nelly PE, Huang E, et al. Dexamethasone pharmacokinetics
in the inner ear: Comparison of route of administration
and use of facilitating agents. Otolaryngol Head Neck Surg.
2000;122:521---8.
96. Herraiz C, Miguel Aparicio J, Plaza G. Vía intratimpánica en
el tratamiento de patología de oído interno. Acta Otorrino-
laringológica Española. 2010;61:225---32.
97. Lee JJ, Jang JH, Choo OS, Lim HJ, Choung YH. Steroid
intracochlear distribution differs by administration method:
systemic versus intratympanic injection. Laryngoscope.
2018;128:189---94.
98. Parnes LS, Sun A-H, Freeman DJ. Corticosteroid pharmacoki-
netics in the inner ear fluids: an animal study followed by
clinical application. Laryngoscope. 1999;109(S91):1---17.
99. Silverstein H, Choo D, Rosenberg SI, Kuhn J, Seidman M, Stein
I. Intratympanic steroid treatment of inner ear disease and tin-
nitus (preliminary report). Ear Nose Throat J. 1996;75:468---71,
474, 476 passim.
100. Yang J, Wu H, Zhang P, Hou D-M, Chen J, Zhang S-G. The
pharmacokinetic profiles of dexamethasone and methylpred-
nisolone concentration in perilymph and plasma following
Update on consensus on diagnosis and treatment of idiopathic sudden sensorineural hearing loss
systemic and local administration. Acta Otolaryngol (Stockh).
2008;128:496---504.
101. Alexander TH, Harris JP, Nguyen QT, Vorasubin N. Dose effect
of intratympanic dexamethasone for idiopathic sudden sen-
sorineural hearing loss: 24 mg/ml is superior to 10 mg/mL.
Otol Neurotol. 2015;36:1321---7.
102. Suzuki H, Wakasugi T, Kitamura T, Koizumi H, Do BH, Ohbuchi
T. Comparison of 2 and 4 intratympanic steroid injections in
the treatment of idiopathic sudden sensorineural hearing loss.
Ann Otol Rhinol Laryngol. 2018;127:235---40.
103. Alimoglu Y, lnci E. Is hyperbaric oxygen therapy a salvage treat-
ment option for sudden sensorineural hearing loss? J Laryngol
Otol. 2016;130:943---7.
104. Karatop-Cesur I, Uzun G, Ozgok-Kangal K, Mutluoglu M, Yildiz
S. Early treatment response predicts outcome in patients
with idiopathic sudden sensorineural hearing loss treated
with hyperbaric oxygen therapy. Undersea Hyperb Med.
2016;43:781---6.
105. Ajduk J, Ries M, Trotic R, Marinac I, Vlatka K, Bedekovic V.
Hyperbaric oxygen therapy as salvage therapy for sudden sen-
sorineural hearing loss. J Int Adv Otol. 2017;13:61---4.
106. Chin CS, Lee TY, Wu MF. Adjunctive hyperbaric oxygen
treatment for idiopathic sudden sensorineural hearing loss.
Undersea Hyperb Med. 2017;44:63---71.
107. Hosokawa S, Sugiyama KI, Takahashi G, Hashimoto YI,
Hosokawa K, Takebayashi S, et al. Hyperbaric oxygen ther-
apy as adjuvant treatment for idiopathic sudden sensorineural
hearing loss after failure of systemic steroids. Audiol Neu-
rootol. 2017;22:9---14.
108. Hosokawa S, Sugiyama K, Takahashi G, Takebayashi S, Mineta
H. Prognostic factors for idiopathic sudden sensorineural
hearing loss treated with hyperbaric oxygen therapy and intra-
venous steroids. J Laryngol Otol. 2017;131:77---82.
109. Sherlock S, Thistlethwaite K, Khatun M, Perry C, Tabah A.
Hyperbaric oxygen therapy in the treatment of sudden sen-
sorineural hearing loss: a retrospective analysis of outcomes.
Diving Hyperb Med. 2016;46:160---5.
110. Bennett MH, Kertesz T, Perleth M, Yeung P, Lehm JP. Hyper-
baric oxygen for idiopathic sudden sensorineural hearing
loss and tinnitus. Cochrane Database Syst Rev. 2012;10(Oct
17):CD004739.
111. Xie S, Qiang Q, Mei L, He C, Feng Y, Sun H, et al. Multi-
variate analysis of prognostic factors for idiopathic sudden
sensorineural hearing loss treated with adjuvant hyperbaric
oxygen therapy. Eur Arch Otorhinolaryngol. 2018;275:47---51.
112. Hoch S, Vomhof T, Teymoortash A. Critical evaluation of round
window membrane sealing in the treatment of idiopathic
sudden unilateral hearing loss. Clin Exp Otorhinolaryngol.
2015;8:20---5.
113. Loader B, Atteneder C, Kaider A, Franz P. Tympanotomy with
sealing of the round window as surgical salvage option in sud-
den idiopathic sensorineural hearing loss. Acta Otolaryngol.
2013;133:1285---91.
114. Kampfner D, Anagiotos A, Luers JC, Hüttenbrink KB, Preuss
SF. Analysis of 101 patients with severe to profound sudden
unilateral hearing loss treated with explorative tympanotomy
and sealing of the round window membrane. Eur Arch Otorhi-
nolaryngol. 2014;271:2145---52.
115. Lee DH. Analysis of 60 patients after tympanotomy and sealing
of the round window membrane after acute unilateral sen-
sorineural hearing loss. Am J Otolaryngol. 2011;32:88---9.
116. Haubner F, Rohrmeier C, Koch C, Vielsmeier V, Strutz J, Klein-
jung T. Occurence of a round window membrane rupture in
patients with sudden sensorineural hearing loss. BMC Ear Nose
Throat Disord. 2012;12:14.
117. Loader B, Seemann R, Atteneder C, Sterrer E, Franz P,
Lill C. Sealing of the round and oval window niches
with triamcinolone-soaked fascia as salvage surgical ther-
299
apy in sudden sensorineural hearing loss. Acta Otolaryngol.
2017;137:923---7.
118. Prenzler NK, Schwab B, Kaplan DM, El-Saied S. The role of
explorative tympanotomy in patients with sudden sensorineu-
ral hearing loss with and without perilymphatic fistula. Am J
Otolaryngol. 2018;39:46---9.
119. Lou Z, Lou Z. Surgical indications or inclusion/exclusion
criteria of explorative tympanotomy on sudden sen-
sorineural hearing. Am J Otolaryngol. 2018;(Feb 1),
http://dx.doi.org/10.1016/j.amjoto.2018.01.015, pii:
S0196-0709(17)30973-0.
120. Plontke SK. Diagnostics and therapy of sudden hearing loss.
GMS Curr Top Otorhinolaryngol Head Neck Surg. 2018;16,
http://dx.doi.org/10.3205/cto000144. Doc05.
121. Wu CM, Lee KJ, Chang SL, Weng SF, Lin YS. Recurrence of
idiopathic sudden sensorineural hearing loss: a retrospective
cohort study. Otol Neurotol. 2014;35:1736---41.
122. Hornibrook J, Flook E, Greig S, Babbage M, Goh T, Coates M,
et al. MRI inner ear imaging and tone burst electrocochleog-
raphy in the diagnosis of Ménière’s disease. Otol Neurotol.
2015;36:1109---14.
123. Ciccone MM, Scicchitano P, Gesualdo M, Cortese F, Zito A,
Manca F, et al. Idiopathic sudden sensorineural hearing loss
and ménière syndrome: the role of cerebral venous drainage.
Clin Otolaryngol. 2018;43:230---9.
124. Kim JY, Hong JY, Kim DK. Association of sudden sensorineural
hearing loss with risk of cardiocerebrovascular disease: a study
using data from the Korea National Health Insurance Service.
JAMA Otolaryngol Head Neck Surg. 2018;144:129---35.
125. Chang TP, Wang Z, Winnick AA, Chuang HY, Urrutia VC, Carey
JP, et al. Sudden hearing loss with vertigo portends greater
stroke risk than sudden hearing loss or vertigo alone. J Stroke
Cerebrovasc Dis. 2018;27:472---8.
126. Siegel LG. The treatment of idiopathic sudden sensorineural
hearing loss. Otolaryngol Clin North Am. 1975;8:467---73.
127. Sano H, Okamoto M, Ohhashi K, Iwasaki S, Ogawa K. Quality of
life reported by patients with idiopathic sudden sensorineural
hearing loss. Otol Neurotol. 2013;34:36---40.
128. Harkónen K, Kivekas I, Rautiainen M, Kotti V, Vasama JP. Qual-
ity of life and hearing eight years after sudden sensorineural
hearing loss. Laryngoscope. 2017;127:927---31.
129. Carreño F, García V, Valverde J. Audífonos y generadores de
ruido. In: Manrique Rodríguez M, Marco Algarra J, editors.
Audiología. Ponencia oficial de la sociedad española de otorri-
nolaringología y patología cérvico-facial 2014. Madrid: CYAN,
Proyectos Editoriales, S.A.; 2014. p. 307---22.
130. Morera C, Manrique M, Ramos A, García-Ibáñez L, Cavalle
L, Huarte A, et al. Advantages of binaural hearing provided
through bimodal stimulation via a cochlear implant and a
conventional hearing aid: a 6-month comparative study. Acta
Otolaryngol. 2005;125:596---606.
131. Finbow J, Bance M, Aiken S, et al. A Comparison between wire-
less CROS and bone-anchored hearing devices for single-sided
deafness: a pilot study. Otol Neurotol. 2015;36:819---25.
132. Hol MK, Kunst SJ, Snik AF, Cremers CW. Pilot study on the effec-
tiveness of the conventional CROS, the transcranial CROS and
the BAHA transcranial CROS in adults with unilateral inner ear
deafness. Eur Arch Otorhinolaryngol. 2010;267:889---96.
133. Niparko JK, Cox KM, Lustig LR. Comparison of the bone
anchored hearing aid implantable hearing device with con-
tralateral routing of offside signal amplification in the
rehabilitation of unilateral deafness. Otol Neurotol. 2003;24:
73---8.
134. Stewart CM, Clark JH, Niparko JK. Bone-anchored devices in
single-sided deafness. Adv Otorhinolaryngol. 2011;71:92---102.
135. Arndt S, Aschendorff A, Laszig R, Beck R, Schild C, Kroeger
S, et al. Comparison of pseudobinaural hearing to real bin-
aural hearing rehabilitation after cochlear implantation in
300
patients with unilateral deafness and tinnitus. Otol Neurotol.
2010;32:39---47.
136. Van de Heyning P, Vermeire K, Diebl M, Nopp P, Anderson I,
De Ridder D. Incapacitating unilateral tinnitus in single-sided
deafness treated by cochlear implantation. Ann Otol Rhinol
Laryngol. 2008;117:645---52.
137. Kleinjung T, Steffens T, Strutz J, Langguth B. Curing tinnitus
with a Cochlear Implant in a patient with unilateral sudden
deafness: a case report. Cases J. 2009;2:7462.
138. Ramos A, Polo R, Masgoret E, Artiles O, Lisner I, Zabal-
los ML, et al. Implante coclear en pacientes con hipoacusia
súbita unilateral y acúfeno asociado. Acta Otorrinolaringol
Esp. 2012;63:15---20.
M. Herrera et al.
139. Tavora-Vieira D, Marino R, Krishnaswamy J, Kuthbutheen
J, Rajan GP. Cochlear implantation for unilateral deafness
with and without tinnitus: a case series. Laryngoscope.
2013;123:1251---5.
140. Sladen DP, Carlson ML, Dowling BP, Olund AP, Teece K, DeJong
MD, et al. Early outcomes after cochlear implantation for
adults and children with unilateral hearing loss. Laryngoscope.
2017;127:1683---8.
141. Blasco MA, Redleaf MI. Cochlear implantation in unilateral
sudden deafness improves tinnitus and speech comprehen-
sion: meta-analysis and systematic review. Otol Neurotol.
2014;35:1426---32.