International Journal of Pediatric Otorhinolaryngology 82 (2016) 3437

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International Journal of Pediatric Otorhinolaryngology

journal homepage: www.elsevier.com/locate/ijporl

Sudden sensorineural hearing loss in children:

Etiology, management, and outcome
Jacob Pitaro *, Avital Bechor-Fellner, Haim Gavriel, Tal Marom, Ephraim Eviatar
Department of Otolaryngology-Head and Neck Surgery, Assaf Harofeh Medical Center, Afliated to the Sackler Faculty of Medicine, Tel Aviv University,
Zerin 70300, Tel-Aviv, Israel

A R T I C L E I N F O A B S T R A C T

Article history: Background and objectives: Pediatric sudden sensorineural hearing loss (SSNHL) is uncommon, and the
Received 21 September 2015 current guidelines for its management refer to adults. Our objective was to review cases of SSNHL in
Received in revised form 23 December 2015 children and examine their etiologies, management, and outcome.
Accepted 24 December 2015
Methods: We performed a retrospective chart review of all children under the age of 18 years treated for
Available online 5 January 2016
SSNHL between January 2003 and September 2014. Data recorded included age, gender, symptoms,
onset of hearing loss, audiometric results, diagnostic studies, treatment, and outcome.
Keywords:
Results: Nineteen children were included. Mean age was 14 years (range 718 years). Male: female ratio
Hearing loss
was 9:10. Degree of hearing loss varied from mild to profound across the tested frequencies. Most
Sensorineural
Child common accompanying symptom was tinnitus. Serologic tests demonstrated recent EpsteinBarr virus
Steroids infection in one patient and previous cytomegalovirus infection in six patients. Imaging studies included
Injection computed tomography scan (n = 3) and/or magnetic resonance imaging (n = 12). All imaging studies did
Intratympanic not demonstrate any pathology. Treatment included systemic steroids in 19 (100%) children and
intratympanic steroids in eight (42%). Hearing completely improved in three (16%) children, partially
improved in nine (47%), and there was no improvement in six (32%). One child was lost to follow-up.
Conclusions: Viral infection was a common nding in children with SSNHL and no pathological changes
were demonstrated on imaging studies. In most patients (63%), hearing improvement was observed.
Intratympanic steroid injection can benet these children. Further studies are required to investigate the
etiologies and establish guidelines for the management of SSNHL in children.
2016 Elsevier Ireland Ltd. All rights reserved.

1. Introduction
Although much has been published on sudden sensorineural
hearing loss (SSNHL) in adults, data on the pediatric population is
limited. Studies have shown that the incidence of SSNHL can range
between 10.7 and 27 per 100,000 persons per year [1,2]. It has been
shown that the incidence increases with age, with eight per
100,000 under the age of 18 years and 70 per 100,000 over 65 years
[1]. SSNHL is considered to be an otologic emergency, and therefore
early treatment is necessary in order to avoid permanent hearing
loss. This is of special importance in children since hearing loss at
an early age may affect speech and language development, as well
as academic and social performance [3]. An additional major
* Corresponding author at: Department of Otolaryngology-Head and Neck
Surgery, Assaf Harofeh Medical Center, Zerin 70300, Tel-Aviv, Israel.
Tel.: +972 8 9779417; fax: +972 8 9779421.
E-mail address: kobypi@hotmail.com (J. Pitaro).
problem is that the youngest patients are not able to report on their
hearing loss, which may go undetected during the acute stage
when treatment is most effective. The degree of hearing loss can
have an effect on the rate of recovery [4].
In adults, most cases of SSNHL are idiopathic [5]. In children, on
the contrary, the percentage of idiopathic cases is unknown due to
the small size of the available series. The current guidelines for the
treatment of SSNHL are aimed at adult patients and include
systemic steroids as primary treatment and intratympanic (IT)
steroid administration when there is a contraindication or no
improvement following systemic treatment [6]. There are reports
on systemic treatment with steroids in children, but there is a lack
of data on IT steroid administration in this age group. There is a
need for more data in order to establish specic management
pathways in children.
The objective of this study was to review cases of pediatric
SSNHL treated at our department in order to examine their
etiology, management, and outcome.

http://dx.doi.org/10.1016/j.ijporl.2015.12.022
0165-5876/ 2016 Elsevier Ireland Ltd. All rights reserved.
 J. Pitaro et al. / International Journal of Pediatric Otorhinolaryngology 82 (2016) 3437 35

2. Methods a family history of hearing loss. Physical examination was normal

The study was approved by the Institutional Review Board of
Assaf Harofeh Medical Center. We performed a retrospective chart
review of all patients under the age of 18 years who were treated
for SSNHL at the Department of Otolaryngology-Head and Neck
Surgery, Assaf Harofeh Medical Center between January 2003 and
September 2014. SSNHL is dened as a unilateral or bilateral
sensorineural hearing loss of 30 decibels (dB) affecting at least
three consecutive frequencies, and occurring over a maximum
period of 72 h [6]. Patients were admitted and a complete head and
neck examination was done including an otoscopic examination
using a microscope. Pure tone audiometry was performed at
frequencies between 0.250 and 8 kHz. Normal hearing is dened at
20 dB. The treatment protocol for SSNHL included oral predni-
sone 1 mg/kg/day, or intravenous (IV) hydrocortisone 1 mg/kg/day
divided into three doses. Both treatments were administered for a
minimum of 7 days. Whenever there was no improvement
following systemic therapy, or a contraindication for steroid
treatment, IT treatment was offered. Intratympanic steroid
injection of dexamethasone 1 mg every 12 h for 7 days was
performed via a ventilation tube inserted into the tympanic
membrane. Following IT injection, patients were positioned on the
non-affected side for 30 min. Complete improvement of hearing
was dened as a hearing level the same as the non-affected ear,
partial hearing recovery as an improvement of more than 10 dB in
at least one frequency, and no improvement when there was no
change in the audiogram following treatment.
Data recorded included age, gender, symptoms other than
hearing loss (ear fullness sensation, tinnitus, otalgia, and vertigo),
onset of hearing loss, audiometric results, serologic tests, imaging
studies, type and length of treatment, and outcome.
3. Results
A total of 19 children were included. Mean age was 14 years
(range 718 years). The male to female ratio was 9:10. The right
ear was affected in 9 (47%) children, the left ear in 9 (47%), and
both ears in one (5%) child. All subjects were healthy and none had
in all except one child, who had a previous history of bilateral
serous otitis media (not the patient who presented with bilateral
SSNHL). At least two pure-tone audiograms were performed. The
rst audiogram was done on hospital admission, before initiation
of treatment, and another one, at the end of the systemic
treatment prior to discharge. Children who were treated with IT
injection had another audiogram at the end of the IT treatment.
The degree of hearing loss varied between mild and profound
across frequencies. Sixteen patients (84%) reported other symp-
toms in addition to hearing loss, most commonly tinnitus. One
patient presented with diplopia and herpetic lip lesion, but
serology for the herpes simplex virus was negative. All patients
reported an abrupt onset of hearing loss. The mean timing
between seeking medical help and initiation of treatment was 9
days (range 040 days) (median 6 days after the hearing loss was
noticed by the patient). Table 1 summarizes the demographic data
and clinical ndings.
Initial treatment included oral steroids in 10 (53%) patients and
IV steroids in 9 (47%). Systemic treatment lasted between 5 and 14
days (median = 7). In two cases, the caregivers decided to stop this
treatment due to fear of side effects. Intratympanic steroids were
administered in eight (42%) children and lasted between 5 and 9
days (mean = 7). One patient refused to continue IT treatment
after 5 days. There were no adverse effects following either
systemic or IT treatment. Oral antibiotics were given in two
patients. Imaging studies of the temporal bones included non-
contrast CT scan in three patients and MRI in 12 patients. None of
the imaging studies demonstrate any pathology. CRP was
obtained in 11/20 patients, and was signicantly elevated in
only one of them (10.52, normal range: <0.06 mg/L). Serologic
tests results included cytomegalovirus (CMV) IgG in six (32%),
EpsteinBarr virus (EBV) IgM in one, EBV EBNA IgG in seven (37%),
and herpes simplex IgG in three (16%). Following treatment,
tinnitus improved in seven (37%) children, vertigo was reported in
one and otalgia and fullness sensation were not reported. Hearing
completely improved in three (16%) patients, partially improved
in nine (47%), and there was no improvement in six (32%). There
was no worsening of hearing following treatment. One patient

Table 1
Clinical ndings in 19 children with sudden sensorineural hearing loss.

No. Sex Age (years) Side Day of the treatment Other symptoms Frequencies (kHz) Steroids CT MRI Viral titer Outcome
started

1 F 16 R 0 Fullness Tinnitus Pancochlear IV, IT N EBV IgM P

2 F 15 L 4 0.25, 0.5, 1, 6, 8 IV N I
3 M 11 L 4 Tinnitus Otalgia Vertigo 1, 2, 3, 4, 6, 8 Oral, IT N P
4 F 15 R 0 Tinnitus Pancochlear IV, IT N CMV IgG NI
5 M 8 L 6 Otalgia 4, 6, 8 IV, IT N CMV IgG P
6 F 15 L 30 Tinnitus Otalgia Pancochlear IV, IT NA CMV IgG P
7 F 11 R 7 Tinnitus Pancochlear IV N CMV IgG I
8 F 16 L 6 Tinnitus Vertigo Pancochlear Oral N CMV IgG P
9 F 7 L 40 Fullness 0.5, 1, 2, 4, 6, 8 Oral NA NI
10 F 18 R 6 Fullness Tinnitus 4, 6, 8 Oral N P
11 M 17 R 7 Tinnitus Vertigo 2, 3, 4, 6, 8 Oral N N P
12 M 18 R 6 Tinnitus Pancochlear IV N P
13 M 14 R, L 7 Tinnitus 1.5, 2, 3, 4, 6, 8 Oral NA NA
14 M 14 R 2 Tinnitus 2, 3, 4, 8 Oral N NA NI

Clinical ndings in 19 children with sudden sensorineural hearing loss.

No. Sex Age (years) Side Day of the treatment Other symptoms Frequencies (kHz) Steroids CT MRI Viral titer Outcome
started

15 M 11 L 8 0.25, 0.5, 0.75 Oral NA I

16 M 16 R 4 Fullness Tinnitus Pancochlear IV, IT N P
17 F 13 R 3 Tinnitus 0.25, 0.5, 4, 6, 8 Oral, IT NA NI
18 M 14 L 0 3, 2 Oral N NA NI
19 F 10 L 30 Fullness Tinnitus Otalgia Pancochlear IV, IT N CMV IgG NI

R, right; L, left; IV, intravenous, IT, intratympanic; EBV, EpsteinBarr virus; CMV, cytomegalovirus; N, normal; NI, no improvement; P, partial improvement; I, improvement.
36 J. Pitaro et al. / International Journal of Pediatric Otorhinolaryngology 82 (2016) 3437
Fig. 1. Mean pure-tone hearing level in the involved ears before and after treatment across mid, low, and high frequencies.
was lost to follow-up. Fig. 1 shows the mean hearing level before
and after treatment across the low, mid, and high frequencies.
4. Discussion
In the present study, most cases of the cases of pediatric SSNHL
were idiopathic. Similar ndings have been reported in studies on
adults. A systematic review on the etiologies of SSNHL in adult
patients showed that in 71% of cases, the etiology was idiopathic
[5]. Possible etiologies of SSNHL in adults include infection,
otologic disease such as Menieres disease and autoimmune inner-
ear disease, trauma, vascular/hematologic incidence, and neoplas-
tic disease [5]. Cardiovascular causes associated with smoking,
alcohol, hyperlipidemia, and vascular degeneration are less
relevant in the pediatric population [4,7]. In children, etiologies
of SSNHL include congenital CMV and EBV infection, enlarged
vestibular aqueduct, Mondini dysplasia, syndrome of common
cavity, ototoxicity, trauma, noise induced, meningitis and parotitis
[7,8]. However, due to the scarcity of pediatric series, it is difcult
to conclude the real contribution of each etiology. Tarshish et al. [7]
reported that only two out of 20 pediatric patients (10%) with
SSNHL were idiopathic cases. On the contrary, in the present study
no etiology was found in 74% of the subjects. Therefore, the data
from the present study is similar to that found in studies on adult
patients.
Congenital CMV infection is a known etiology of hearing loss
[9]. Children with an asymptomatic CMV infection may present
with acute or progressive hearing loss [8]. Fowler et al. [9] showed
that about 18% of asymptomatic patients with congenital CMV
infection can present with delayed-onset sensorineural hearing
loss. In the present study, six (32%) patients were found to have
CMV IgG. However, no data was available to clarify whether these
patients had a congenital CMV infection and since imaging studies
were normal, it was not possible to directly relate the hearing loss
to a viral infection.
A recent EBV infection may also cause SSNHL. Shian et al. [10]
presented two cases of sensorineural hearing loss following EBV
infection and a review of the literature. They found that most cases
of EBV-related SSNHL occurred during the convalescent phase of
the infection. However, some occurred without clinical symptoms
of infectious mononucleosis. Only 15% of the patients with an EBV
infection completely recovered their hearing. In the present study,
one child had positive EBV IgM, pointing to a recent infection. This
child had partial hearing improvement. No routine screening for
viral causes is warranted in adults [5], but in our opinion, a work-
up for viral infection in children should be performed on an
individual basis according to the history and clinical ndings.
Current guidelines for the treatment of SSNHL recommend
systemic steroids as primary treatment and IT steroid administra-
tion as second line or salvage therapy [6]. Although the guidelines
are aimed at patients over 18 years of age, it is clear from the
literature that children with SSNHL are treated with steroids as
well. A systematic review on IT steroids for SSNHL demonstrated
that salvage treatment following systemic steroid treatment
failure offers a potential for some degree of additional hearing
recovery. However, the age of the patients in the included studies
was not specied [11]. Our literature review identied two series
on IT salvage treatment for SSNHL that included children.
However, there was no separation between children and adults
and therefore it was not possible to conclude on the efcacy of IT
treatment specically in children [12,13]. In the present study,
eight (42%) children were treated with IT steroids as salvage
treatment. Five (62%) of these children had partial hearing
improvement following the treatment and three did not improve.
To our knowledge, this is the rst series exclusively describing
children with SSNHL who received IT steroid salvage therapy.
Although the numbers are small, it seems that IT steroids can
benet children with SSNHL.
The rate of recovery from SSNHL in children varies between
studies. Chen et al. [14] presented a series of 14 patients under the
age of 18 years treated with IV prednisolone. The rate of complete
recovery was 57%, whereas the partial recovery rate was 36%.
Tarshish et al. [7] presented a series of 20 children with SSNHL. In
this study, 10% had complete recovery and another 10% had some
improvement. In both studies, patients received systemic steroids
and no IT treatment was given. In the present study, hearing
completely improved in 16%, partially improved in 47%, and there
was no improvement in 32%. Therefore, more studies are needed to
elaborate on the rate of recovery from SSNHL in children.
Na et al. [4] compared a group of 87 children to 707 adults with
SSNHL. The rates of complete recovery and no improvement of
hearing in children were 54% and 17.3% respectively. Interestingly,
the complete recovery rate was signicantly higher and the no
improvement rate signicantly lower in children than in adults.
However, the overall recovery rates were similar in children and
adults. In both populations, hearing recovery was signicantly
higher in patients with mild hearing loss compared to profound
hearing loss, but in children with moderate hearing loss, the rate of
 J. Pitaro et al. / International Journal of Pediatric Otorhinolaryngology 82 (2016) 3437
recovery was lower compared to adults, whereas in children with
profound hearing loss, the rate of recovery was higher compared to
adults, showing that age is associated with a poorer prognosis.
Interestingly, treatment in this study included oral prednisolone, a
high-protein low-salt diet, peripheral vasodilators, and bed rest
[4].
In the present study, we found that there was an overall
improvement of about 20 dB following steroidal treatment, which
indicates that steroidal treatment can benet children with SSNHL.
The fact that there was hearing improvement in 62% of the patients
in the group treated with systemic steroids as well as additional IT
steroids, supports the benecial effect of IT treatment.
SSNHL may be difcult to diagnose in children, especially at a
young age, therefore a high index of suspicion is required. Hearing
loss at an early age may affect speech and language development,
and academic and social performance. The low incidence of
pediatric SSNHL is reected in the small number of series
published on this topic. It is still not clear which work-up and
treatment would be most appropriate for this population. The role
of viral infection is still not clear, but it appears that in children
CMV may play a role in SSNHL. As in adults, IT steroids should be
considered as secondary treatment following systemic steroid
treatment.
The present study is limited by its retrospective nature and
inherent biases, and the small number of subjects. It does,
however, provide further data on this subject.
5. Conclusions
The present study shows that the etiology of SSNHL in children
is most commonly idiopathic. A careful work-up should include
viral etiologies based on history and clinical ndings. Most
children in this study had hearing improvement. It was shown
that IT steroid treatment can benet these children. Further
research is required to establish standardization of diagnosis and
management pathways.
Funding
No external funding was secured for this study.
37
Conict of interest
None.
Acknowledgment
We would like to thank the Department of Audiology at Assaf
Harofeh Medical Center for their contribution.
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