Acute mastoiditis complicated by sigmoid sinus thrombosis in congenital aural atresia

Congenital aural atresia occurs approximately once in every 20,000 live births, although the incidence of otitis media in aural atretic children should not differ from the rest of the population, detection is difficult. Unless severe, the infection usually goes unnoticed, especially with the use of antibiotics. A two-year-old male with congenital aural atresia presented with fever and facial nerve paresis. As his illness progressed, he developed mastoiditis with subperiosteal abscess and sigmoid sinus thrombosis. Literature review showed this to be the first reported case of sigmoid sinus thrombosis in congenital aural atresia. Diagnosis and management are presented.