ABSTRACT

Background and objectives: Pediatric sudden sensorineural hearing loss (SSNHL) is uncommon,
and the current guidelines for its management refer to adults. Our objective was to review cases of
SSNHL in children and examine their etiologies, management, and outcome. Methods: We
performed a retrospective chart review of all children under the age of 18 years treated for SSNHL
between January 2003 and September 2014. Data recorded included age, gender, symptoms, onset
of hearing loss, audiometric results, diagnostic studies, treatment, and outcome. Results: Nineteen
children were included. Mean age was 14 years (range 7–18 years). Male: female ratio was 9:10.
Degree of hearing loss varied from mild to profound across the tested frequencies. Most common
accompanying symptom was tinnitus. Serologic tests demonstrated recent Epstein–Barr virus
infection in one patient and previous cytomegalovirus infection in six patients. Imaging studies
included computed tomography scan (n = 3) and/or magnetic resonance imaging (n = 12). All
imaging studies did 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 finding 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 benefit these
children. Further studies are required to investigate the etiologies and establish guidelines for the
management of SSNHL in children.

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 majorproblem 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 specific 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.

2. METHODS
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 defined 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 defined 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 defined 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.

RESULTS

A total of 19 children were included. Mean age was 14 years (range 7–18 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 a
family history of hearing loss. Physical examination was normal 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 first
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
0–40 days) (median 6 days after the hearing loss was noticed by the patient). Table 1
summarizes the demographic data and clinical findings. 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 significantly elevated in only one of them (10.52, normal range:
<0.06 mg/L). Serologic tests results included cytomegalovirus (CMV) IgG in six (32%),
Epstein–Barr 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 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
findings 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
Meniere’s 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 difficult 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 workup
for viral infection in children should be performed on an individual basis according to the
history and clinical findings. Current guidelines for the treatment of SSNHL recommend
systemic steroids as primary treatment and IT steroid administration 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 specified [11]. Our literature review identified 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 efficacy of IT treatment
specifically 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 first series exclusively describing children with SSNHL who received IT steroid
salvage therapy. Although the numbers are small, it seems that IT steroids can benefit
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 significantly higher and the ‘‘no improvement’’ rate significantly
lower in children than in adults. However, the overall recovery rates were similar in
children and adults. In both populations, hearing recovery was significantly higher in
patients with mild hearing loss compared to profound hearing loss, but in children with
moderate hearing loss, the rate of 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
benefit 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 beneficial effect of IT treatment. SSNHL may be difficult 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 reflected 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
findings. Most children in this study had hearing improvement. It was shown that IT
steroid treatment can benefit these children. Further research is required to establish
standardization of diagnosis and management pathways.

Funding

No external funding was secured for this study.

Conflict of interest

None.

Acknowledgment

We would like to thank the Department of Audiology at Assaf Harofeh Medical Center
for their contribution.