Kawasaki disease: always straight to the heart?

Abstract

Kawasaki disease is an important differential diagnosis when evaluating prolonged fever in childhood. Although the aetiology of this vasculitic disease process remains obscure, prompt recognition and treatment significantly reduces vascular complications such as coronary artery aneurysms. Patients presenting with atypical or incomplete diagnostic features remain a challenge. Here the authors describe the atypical features of Kawasaki disease that led to profound acute hearing loss in a 6-year-old boy with complex congenital heart disease. Coronary changes were not seen on early follow-up, but the patient required bilateral hearing aids. Considering Kawasaki disease early, even in the possibly confusing context of complex congenital heart disease and atypical symptomatology, is crucial to avoid long-term sequelae.

Case report: Cochlear implantation was effective for progressive bilateral severe hearing loss associated with Kawasaki disease

“…Development of severe hearing loss also seems to be rare in SNHL associated with KD ( 10 – 12 ). Etiologically, SNHL association with KD occurring in acute or subacute phases and often being reversible is perhaps related to administration of aspirin.…”

“…Progression over a long term seems to be rare and results in underestimation of its association with KD. Although SNHL associated with KD is usually mild hearing loss ( 5 , 7 , 8 ), there are a few reports of patients that developed severe hearing loss ( 10 – 12 ). Most cases of KD occur in children less than five years of age ( 6 ), so poor prognosis of bilateral SNHL associated with KD can cause delays in cognitive and speech development ( 13 ).…”

Sensorineural hearing loss in patients with Kawasaki disease

“…Since then, studies on the association have been conducted, but interpretations of the outcomes have varied 20) . Novo et al 5) , Clausen et al 11) , and Silva et al 18) reported cases of profound bilateral sensorineural hearing loss in patients with Kawasaki disease, despite the use of steroids for treatment, their hearing function was not improved. They reported that the sensorineural hearing loss might be caused by acute inflammatory reaction in the blood vessels of the middle ear, and their clinical manifestations were not same as those caused by aspirin ototoxicity 5 18) .…”

“…In this way, sensorineural hearing loss can develop due to the inflammatory reaction in the acute phase of Kawasaki disease, or from the side effects of the high-dose aspirin. In previous studies, various cases of sensorineural hearing loss duration, directionality, and severity have been reported in Kawasaki disease 4 5 6 7 8 9 10 11) . According to Magalhaes et al 8) , and Alves et al 10) , in Kawasaki disease, the patients with permanent sensorineural hearing loss showed anemia and thrombocytosis significantly more often, and had a longer duration of the increase in the erythrocyte sedimentation rate than patients with temporary hearing loss.…”