Sensorineural Hearing Loss Associated with Kawasaki Disease

Kawasaki disease (KD) is an acute systemic vasculitis of children characterized by fever, rash, conjunctival hyperemia, oropharyngeal erythema, edema and erythema of the hands and feet, and cervical adenopathy [1]. A variety of other features are also characteristics of this syndrome, including coronary artery aneurysms, urethritis, anterior uveitis, mild hepatobiliary dysfunction, and gallbladder hydrops. Coronary artery abnormalities, including aneurysms and ectasia, occur in approximately 20% of untreated patients [2]. Neurologic involvement is rare. Cranial nerve palsy, especially involving the seventh nerve, has been reported [3], as well as hemiparesis caused by cerebral thrombosis and infarction, and convulsions. Although about 30% of patients with acute KD in the United States have been reported to suffer mild sensorineural hearing loss (SNHL) [4], only a few such cases have been reported in Japan. On the other hand, in both countries, a few cases of severe or profound SNHL in children who were in the acute phase of KD have been documented [5].