Colesteatoma do Canal Auditivo Externo: Caso Atípico de Apresentação Bilateral

INTRODUCTION: Cholesteatoma of the external auditory canal is rare and often asymptomatic despite the insidious bone destruction that it causes in the external auditory canal and surrounding structures. Reports of bilateral disease are episodic and usually idiopathic. Keratosis obturans is the main differential diagnosis.CLINICAL CASE: A 13-year-old child diagnosed with bilateral cholesteatoma of the external auditory canal, associated with mastoid invasion and erosion of the ossicular chain in the left ear. The patient presented with bilateral otalgia, sporadic otorrhea and progressive hearing loss on the left ear. After several surgical procedures to eradicate the disease, the patient remains under annual surveillance without relapse after 4 years.CONCLUSION: This case emphasizes the need for early recognition of this uncommon entity that can occur with extensive and even irreversible lesions. Surgical treatment is curative but regular surveillance is mandatory.