Presentation at italian congress of ENT doctors in Rome 2022 regarding SSNHL in pediatric patients

1. “Idiopathic Sudden Sensorineural Hearing Loss in
Children: A Systematic Review and Meta-analysis”
Authors:
Leonardo Franz, MD1, Chiara Gallo, MD2, Frosolini Andrea, MD1, Gino
Marioni, MD1, Cosimo de Filippis, MD2, and Andrea Lovato, MD2
Affiliation:
1) Università degli Studi di Padova, Department of Neuroscience,
U.O.C. di Audiologia e Foniatria
2) Università degli Studi di Padova, Department of Neuroscience,
U.O.C. di Otorinolaringoiatria
Contact:
andrea.frosolini@studenti.unipd.it
I have no potential conflict of interest to report

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Background
Main differences with Adult SSNHL
Epidemiology
• Its incidence was estimated to be 10-20 fold
lower than in adults.
• It has been reported that 6.6% of patients with
SSNHL were <18 years of age, 3.5% <14
years, and only 1.2% <9 years.
Etiology
• As opposed to that in adults, SSNHL in
children was often associated with laboratory
findings suggesting a definite etiology
Idiopathic sudden sensorineural hearing loss
(ISSNHL) is uncommon in children, and its
treatment and outcome are debated. We
aimed to critically review evidence in the
literature about treatment options and
functional outcomes of pediatric ISSNHL.
Additionally, we performed a meta- analysis
of the results of combined
systemic-intratympanic steroid therapy versus
solely systemic treatment.
> 3 frequencies
> 30 dB
2
Chung JH, Cho SH, Jeong JH, et al. Multivariate analysis of prognostic factors for
idiopathic sudden sensorineural hearing loss in children. Laryngoscope. 2015

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Background
Causes:
• Infections: cytomegalovirus, rubella virus, herpes
simplex virus
• Microcirculatory disorders: platelet alterations,
high serum homocysteine, hypercholesterolemia,
coagulation disorders
• Autoimmune disorders: anti-P0 antibodies.
• A significant portion of pediatric cases remained
unexplained.
Treatment:
based on systemic and local injecting steroids
(intratympanic or retroauricular), peripheral
vasodilators, hyperbaric oxygen, or carbogen
inhalation
There is not wide agreement on the most
appropriate treatment.
To date, only 2 review articles have
attempted to provide a
comprehensive picture of the
clinical, prognostic, and therapeutic
challenges associated with SSNHL
in children.
1) Wood JW et al. SSNHL in children management and outcomes: a meta-analysis. Laryngoscope. 2020;
2) Ovet G et al. Sudden pediatric hearing loss: comparing the results of combined treatment (intratympanic
dexamethasone and systemic steroids) with systemic steroid treatment alone. Otol Neurotol. 2016

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Objective
The main goal of the present investigation was to
critically review the literature about pediatric ISSNHL,
discussing the efficacy of the described treatment
options and the functional hearing outcomes.
A secondary goal was to compare, through a
meta-analysis approach, the results of combined
systemic-intratympanic steroid therapy VS solely systemic
steroid treatment.
Meta-
analysis
Systemic +
Intratympanic
Steroid
Systemic
Steroid

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Material and Methods
• We searched PubMed, Scopus, and Google
Scholar electronic databases for investigations on
pediatric ISSNHL.
• The date of last search was June 15, 2020.
• The search terms used in all combinations were as
follows: ‘‘sudden’’ AND ‘‘hearing loss’’ OR
‘‘sensorineural hearing loss,’’ ‘‘sudden hearing loss,’’
or ‘‘sudden deafness,’’ plus the following
specifications ‘‘children,’’ ‘‘childhood,’’ or ‘‘pediatric.’’
● The ‘‘Related articles’’
option on the PubMed
homepage was also
considered.
● No restriction about
publication date was
applied.
● The full texts of the
publications identified were
screened for original data,
and the related references
were retrieved and checked
manually for other relevant
studies.

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Inclusion and Exclusion Criteria
(1) articles were original reports; (2) reports were published in
the English language; (3) age was between 2 and 19 years;
and (4) studies included only patients who experienced
ISSNHL.
Single case reports, editorials, and reviews were excluded, as
well as investigations in which all patients had identifiable
etiologies (eg, infectious, autoimmune, malformation, or
traumatic).
Data Extraction
Descriptive data of patients, treatments, outcomes, and
possible prognostic factors were extracted and recorded for
every included study.
In case of studies reporting information on non-idiopathic
cases, only data about patients with idiopathic sudden hearing
loss were used for the meta- analysis.

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Statistical Analysis
The overall effect size and 95% CI were estimated.
Heterogeneity was assessed by using the I2 statistic.
We chose to use a Bayesian hierarchical (random
effects) model, which accounted for between-study
variations in odds ratios (ORs).
Forest plots were produced to display the OR and
95% CI for the outcome pooled in our meta-analysis.
The ‘‘meta’’ suite of Stata version 16.0 (StataCorp) was used.
A meta-analysis approach was
performed to:
1) compare the effect of
combined
systemic-intratympanic
steroid therapy VS solely
systemic steroid therapy
2) estimate the pooled
overall recovery rate of
studies that reported
comparable hearing
outcomes.

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Quantitative results
The pooled overall recovery rate
was 67.91% (95% CI,
58.34%-77.48%), using a random
effects model. Large and significant
heterogeneity (80%).
The pooled OR for the concomitant
use of intratympanic steroid, as
compared with systemic steroid alone,
was 0.90 (95% CI, 0.36-2.27) based
on a random effects model, ruling out
any significant difference between
these treatment options.
Nonsignificant small to moderate
heterogeneity (36.23%).

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13. Discussion
Results of the present meta-analysis did not
support greater effectiveness of the combined
treatment when compared with the solely
systemic one.
No formal recommendations about
therapeutic management of pediatric ISSHL
can be provided.
However, a personalized choice of the possible
therapeutic strategies, tailored to the specific
clinical situation, may be considered in
managing such an uncommon condition in
children and adolescents.
The optimal management
of ISSHL is still a matter of
debate.
Intratympanic steroid
injection is currently
considered as salvage
treatment (Grade B
recommendation).
Chandrasekhar SS et al. Clinical Practice Guideline: Sudden Hearing Loss.
Otolaryngol Head Neck Surg. 2019

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Discussion
Another insight from this review and meta-analysis regarded
the overall recovery rate in the pediatric population.
Cumulatively among all cases with significant hearing
improvement, an overall recovery rate of 67.91% (95% CI,
58.34%-77.48%) was found after data were pooled from all the
considered studies, with rates ranging from 27.27% to 92.81%.
The overall pooled rate appeared to be similar to that of
the adult population.
Intratympanic steroid administration
could allow higher drug concentrations
within the cochlea, reducing systemic
spread and therefore metabolic side
effects, nonetheless is an invasive
procedure.
Currently, there is a lack of agreement
about how to perform tympanic
injection in children: local anesthesia?
sedation? via a transtympanic
ventilation tube?
Adverse events, including otalgia,
persistent tympanic membrane
perforation, vertigo, dysgeusia, and
infections.
Dedhia K, Chi DH. Pediatric sudden sensorineural hearing loss: etiology, diagnosis and treatment in
20 children. Int J Pediatr Otorhinolaryngol. 2016
We also provided a comprehensive summary of known
prognostic factors.
Early onset of steroid therapy, older age, tinnitus, and
mild hearing loss seemed to be predictive of better
outcome. However, a relevant predictor of poor improvement
appeared to be profound hearing loss. Sex was not
significantly associated with occurrence risk and hearing
prognosis.

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Limits
However, the characteristics of available case series
intrinsically affect the strength of the evidence provided by a
review or meta-analysis about this topic.
In fact, comparing functional results of studies about ISSNHL
treatment in children was difficult due to the following reasons:
(1) the incidence of this disease was much lower in children
than in adults, and as a consequence the populations included
in the available studies were limited;
(2) the design of the studies was always retrospective;
(3) no randomized clinical trials were available;
(4) there were no standardized treatment options, and the
described therapeutic regimens varied among and within
studies; and
(5) there was variability in the final outcome definition.
Strengths
The main strength of this
investigation is the fact that
only data about ISSNHL in
children were considered.
The homogeneity in case
definition contributed to support
the confidence around the
findings of this review on
pediatric ISSNHL.

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Conclusions
Take Home Messages
• Pediatric idiopathic SSNHL is a rare
but challenging condition
• Prognostic factor and recovering
rate are similar with adult population
• There are no evidence that supported
greater effectiveness of a combination
therapy than systemic steroid
administration only.
Few reports on pediatric ISSNHL
treatment were available. The lack of
PCR and the heterogeneity of the
methodological characteristics of the
available studies made it difficult to
achieve conclusive data supporting any
specific treatment option.
All the available studies reported the use
of systemic steroids, variably combined
with other therapies (eg, intratympanic
steroids, vasodilators). However, no
evidence supported greater
effectiveness of a combination therapy
than systemic steroid administration only.
Further prospective clinical trials are
necessary to provide information about
the effectiveness of different treatment
options in pediatric ISSNHL.

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Contact: Dr. Andrea Frosolini
(andrea.frosolini@studenti.unipd.it)
Thanks for the attention!
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