Year : 2014 | Volume
: 1 | Issue : 1 | Page : 47--49
Bilateral sudden sensorineural hearing loss in Kawasaki disease
Hossam Sanyelbhaa Talaat1, Abdullah M Jamos2, Ahmed Thabet Kamal Eldin3, Mohamed Samer S Abdelaal4,
1 Department of ENT, Audiology Unit, Menoufiya University, Shebin El Kom, Menoiufia, Egypt
2 Department of ENT, Alazhar University, Cairo, Egypt
3 Department of Speech and Hearing, University of Jordan, Amman, Jordan, Kuwait
4 Department of Radiology, Hadiclinic Hospital, Kuwait
Hossam Sanyelbhaa Talaat
ENT Department, Hadiclinic Hospital, PO Box 44630, Code 32061, Kuwait
Kawasaki disease is an acute self-limited vasculitis and is commonly seen in children. This case report is a rare complication of Kawasaki disease. A 5-year-old boy had persistent fever, associated with bilateral nonexudative conjunctivitis, cervical lymphadenopathy, and maculopapular erythema on his trunk. The patient was diagnosed as Kawasaki disease and was treated by intravenous immunoglobulins and aspirin. However, 3 days after recovery of the symptoms, he developed bilateral severe sensorineural hearing loss. Corticosteroid therapy of 40 mg/day was immediately administrated for 15 days; no improvement was noticed in the child«SQ»s hearing as evidenced by serial audiometry follow-up.
|How to cite this article:|
Talaat HS, Jamos AM, Kamal Eldin AT, Abdelaal MS. Bilateral sudden sensorineural hearing loss in Kawasaki disease.Adv Arab Acad Audio-Vestibul J 2014;1:47-49
|How to cite this URL:|
Talaat HS, Jamos AM, Kamal Eldin AT, Abdelaal MS. Bilateral sudden sensorineural hearing loss in Kawasaki disease. Adv Arab Acad Audio-Vestibul J [serial online] 2014 [cited 2020 Dec 2 ];1:47-49
Available from: http://www.aaj.eg.net/text.asp?2014/1/1/47/137566
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 . 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 .
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.
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 bilateral conjunctivitis. The patient developed arthritis 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 . The patient was conventionally treated with intravenous γ-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 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.
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 . 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 . 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 . 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 . 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 . Sundel et al.  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 , 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 . 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 . A few studies have reported partial  or complete  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/m 2 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 . Furthermore, methotrexate has been used for treatment of autoimmune inner ear disorders with reported success in both auditory and vestibular symptoms . 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 , close monitoring for signs and symptoms of bone marrow suppression and nephrotoxicity is advisable during concomitant administration .
|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.|
|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.|
|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.|