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Case Report  47

Bilateral sudden sensorineural hearing loss in Kawasaki

disease
Hossam Sanyelbhaa Talaata, Abdullah M. Jamosc, Ahmed Thabet Kamal Eldinb,
Mohamed Samer S. Abdelaald
a
Department of ENT, Audiology Unit, Menoufiya Kawasaki disease is an acute self-limited vasculitis and is commonly seen in children. This
University, Shebin El Kom, Menoiufia, case report is a rare complication of Kawasaki disease. A 5-year-old boy had persistent
b
Department of ENT, Alazhar University,
fever, associated with bilateral nonexudative conjunctivitis, cervical lymphadenopathy, and
Cairo, Egypt, cDepartment of Speech and
Hearing, University of Jordan, Amman, Jordan, maculopapular erythema on his trunk. The patient was diagnosed as Kawasaki disease and
d
Department of Radiology, Hadiclinic Hospital, was treated by intravenous immunoglobulins and aspirin. However, 3 days after recovery of
Kuwait the symptoms, he developed bilateral severe sensorineural hearing loss. Corticosteroid therapy
Correspondence to Hossam Sanyelbhaa Talaat, of 40 mg/day was immediately administrated for 15 days; no improvement was noticed in the
MD, ENT Department, Hadiclinic Hospital, child’s hearing as evidenced by serial audiometry follow-up.
PO Box 44630, Code 32061, Kuwait
Tel: +965 667 53572;
e-mail: sanyelbhaa@yahoo.com Keywords:
corticosteroids, sudden hearing loss, vasculitis
Received 15 February 2014
Accepted 1 March 2014
AAAA 01:47–49
Advanced Arab Academy © 2014 Advanced Arab Academy of Audiovestibulogy
of Audiovestibulogy 2014, 01:47–49 2314-8667

bilateral conjunctivitis. The patient developed arthritis

Introduction and rationale
Kawasaki disease was first described in 1967 by
Tomisaku Kawasaki. Kawasaki disease is a self-limited
acute vasculitis seen in children, and it predominantly
affects the small and medium-sized vessels including
coronary arteries [1]. Kawasaki disease has replaced
acute rheumatic fever as the leading cause of acquired
heart disease among children in developed countries.
The etiology of Kawasaki disease remains unknown,
although an infectious agent is strongly suspected on the
basis of clinical and epidemiologic features. A genetic
predisposition is also likely, because the incidence rate
varies among the ethnic groups. The annual incidence
of Kawasaki disease in children of Japanese descent is
about 150 per 100 000 children younger than 5 years;
this is 10–15 times more than the annual incidence in
the USA [1].
Although hearing involvement is extremely rare
in Kawasaki disease, 15 patients with sudden
sensorineural hearing loss (SNHL) associated with
Kawasaki disease were reported; eight of them were
bilateral [2–4]. In the current case report, we aim to
show a rare complication of Kawasaki disease resulting
in SNHL and the outcome of corticosteroid therapy.
Case report
A 5-year-old Filipino male child living in Kuwait was
presented with a marked diminution of hearing of 2 days
duration. Before 12 days, the patient had persistent
fever (39°C) associated with maculopapillary erythema
on the trunk, cervical lymphadenitis, and nonexudative
2314-8667 © 2014 Advanced Arab Academy of Audiovestibulogy
on his ankles, edema, and desquamation of the hands
and feet.
Laboratory examinations were performed 4 days after the
onset of the fever, revealing erythrocyte sedimentation
rate of 48 mm/h; C-reactive protein of 28 mg/l; Anti-
Streptolysin O titre (ASO) of 54 IU; albumin of
4.1 g/l; and negative serological tests for toxoplasmosis,
mononucleosis, and cytomegalovirus. Echocardiographic
and ECG abnormalities were not observed. The
diagnosis of Kawasaki disease was established according
to the American Heart Association (AHA) criteria:
fever for at least 5 days and four or more of the five
major clinical features (i.e. conjunctival infection, cervical
lymphadenopathy, oral mucosal changes, polymorphous
rash, and swelling or redness of the extremities) and
exclusion of alternative diagnoses [1]. The patient was
conventionally treated with intravenous g-globulin
(2 g/kg/day) and aspirin (100 mg/kg/day up to 14 days
after disease onset). The clinical symptoms resolved
3 days after the treatment was implemented.
The child was referred to the audiology clinic about
10 days after the onset of the fever because of marked
hearing deterioration reported by the patient’s family.
Pure tone audiometry revealed bilateral severe SNHL
and poor speech discrimination. Speech discrimination
was judged through observation of Filipino continuous
discourse delivered by native person at most
comfortable level. Immittancemetry showed bilateral
normal middle ear pressure with absent acoustic
reflex. Both auditory brainstem-evoked response and
distortion product otoacoustic emissions were absent
in both ears. Computed tomography for the temporal
DOI: 10.4103/2314-8667.137566
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48  Advanced Arab Academy of Audiovestibulogy  2014, Vol 1 No 1
bone and MRI revealed no abnormalities. High-
dose corticosteroid therapy was applied, 2 mg/kg/day
(40 mg/day) for 15 days, then the dose was gradually
reduced to 20 mg/day for another 2 weeks. However,
no improvement in hearing was detected in either ear.
Discussion
Several serious complications are related to Kawasaki
disease, and they are related to cardiac involvement.
Such complications include coronary artery aneurysms
or ectasia, and they develop in ∼15–25% of untreated
children, possibly leading to ischemic heart disease or
sudden death [5]. Sudden SNHL is a rare complication
of Kawasaki disease; only 14 cases of sudden hearing
loss were reported since the disorder was described in
1967 until 2002 [2]. However, the actual prevalence
of hearing loss in Kawasaki disease might be
underestimated; many cases of hearing loss due to
Kawasaki disease may have been overlooked as they
were of mild degree, unilateral, or even transient [6].
However, Kawasaki disease is traditionally treated with
high doses of aspirin, which might be responsible for
the incidence of some cases of hearing loss, especially
for patients who develop transient and mild hearing
loss with onset after initiating aspirin therapy.
Kawasaki disease is more common in children below
5  years of age and in those with Asian ethnicity as
reported in this patient. The current patient was
diagnosed with Kawasaki disease on the basis of
the AHA [1]. Auditory brainstem-evoked response
and distortion product otoacoustic emissions were
performed to confirm the presence of hearing loss
and the cochlear affection by the pathological process,
in addition to eliminate the possibility of auditory
neuropathy spectrum disorders. No congenital
malformations or neurological abnormalities were
detected by imaging.
The pathophysiological mechanism that causes SNHL
is possibly through impairment of cochlear vessels or
vasa nervorum, which results in the involvement of
the auditory nerve [1]. Sundel et al. [6] suggested
that hearing involvement could be anticipated in
patients showing fever with a tendency of higher
temperature, longer duration, and also associated with
laboratory markers indicating more severe systemic
inflammation (e.g. higher white blood cell counts).
Although the role of corticosteroids is controversial
in Kawasaki disease [1], it has been traditionally
used for the treatment of hearing loss complicating
Kawasaki disease [2,3,5]. The commonly used daily
dose of corticosteroids in the treatment of sudden
hearing loss is 2 mg/kg/day with a maximum dose of
60 mg/day. Recovery is expected within 2 weeks [7].
This treatment protocol was followed to treat this
patient; then, the dose was reduced after 2 weeks
to avoid any hazardous effect of corticosteroids on
the endangered coronary vessels [1]. A few studies
have reported partial [1] or complete [2] recovery
of hearing after administration of corticosteroids;
however, no improvement was recorded in the patient
presented in the present case.
The discrepancies of the results of corticosteroids therapy
in patients with sudden SNHL complicating Kawasaki
disease indicate the need for another line of treatment
in addition to corticosteroids. Lee and colleagues
used low-dose oral immunosuppressive, methotrexate,
for treatment of 17 children with Kawasaki disease,
showing resistance to the standard intravenous
immunoglobulin therapy — 10 mg/m2 body surface
area once weekly without folate supplementation.
Administration of methotrexate was continued
in all patients until C-reactive protein levels were
normalized. The patients treated with methotrexate
experienced fast resolution of fever and rapid
improvement in inflammation markers, without
showing adverse effects [8]. Furthermore, methotrexate
has been used for treatment of autoimmune inner ear
disorders with reported success in both auditory and
vestibular symptoms [9]. The authors suggest adding
low-dose methotrexate to the therapeutic regimen for
patients with SNHL who would show unsatisfactory
response to corticosteroids. Although such suggestion
needs controlled clinical trial to verify its validity,
this may be hindered by the rarity of incidence of
sudden SNHL with Kawasaki disease. Although the
concomitant administration of corticosteroids and
methotrexate for treatment of autoimmune disorders
is common in clinical practice [9], close monitoring
for signs and symptoms of bone marrow suppression
and nephrotoxicity is advisable during concomitant
administration [10].
Acknowledgements
Conflicts of interest
There are no conflicts of interest.
References
 1 Freeman A, Shulman S. Kawasaki disease: summary of the American
Heart Association Guidelines. Am Fam Physician 2006; 74:1141–1148.
 2 Da Silva C, Roscoe I, Fernandes K, Novaes R, Lázari C. Sensorineural
hearing loss associated to Kawasaki disease. J Pediatr (Rio J) 2002;
78:71–74.
 3 Sundel R, Newburger J, McGill T, Cleveland S, Miller M. Sensorineural hearing
loss associated with Kawasaki disease. J Pediatr 1990; 117:371–377.
 4 Novo A, Pinto S, Prior A, Alvares S, Soares T, Guedes M. Kawasaki
disease and sensorineural hearing loss: an (un)expected complication.
Eur J Pediatr 2012; 171:851–854.
[Downloaded free from http://www.aaj.eg.net on Monday, January 26, 2015, IP: 200.8.191.56]  ||  Click here to download free Android application for this journal
 5 Newburger J, Takahashi M, Gerber MA, Gewitz MH, Tani LY. Diagnosis,
treatment, and long-term management of Kawasaki disease: a statement for
health professionals from the Committee on Rheumatic Fever, Endocarditis,
and Kawasaki Disease, Council on Cardiovascular Disease in the Young,
American Heart Association. Pediatrics 2004; 114:1708–1733.
 6 Sundel R, Cleveland S, A Bieser, Newburger J, McGill T, Baker A.
Audiologic profiles of children with Kawasaki disease. Am J Otol 1992;
13:512–515.
 7 Slattery W, Fisher L, Iqbal Z, Liu N. Oral steroid regimens for idiopathic sudden
sensorineural hearing loss. Otolaryngol Head Neck Surg 2005; 132:5–10.
Sudden SNHL in Kawasaki Talaat et al.  49
 8 Lee T, Kim K, Chun J, Kim D. Low-dose methotrexate therapy for
intravenous immunoglobulin-resistant Kawasaki disease. Yonsei Med J
2008; 49:714–718.
 9 Matteson E, Tirzaman O, Facer G, Fabry D, Kasperbauer J, Beatty C,
McDonald T. Use of methotrexate for autoimmune hearing loss. Ann Otol
Rhinol Laryngol 2000; 109:710–714.
10 Jover JA, Hernández-García C, Morado IC, Vargas E, Bañares A,
Fernández-Gutiérrez B. Combined treatment of giant-cell arteritis with
methotrexate and prednisone. A randomized, double-blind, placebo-
controlled trial. Ann Intern Med 2001; 134:106–114.