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To review systematically the literature for the current criteria and strategies used to diagnose acute mastoiditis in children.A comprehensive literature search was performed in PubMed, Embase, and the Cochrane Library of studies on the diagnosis of acute mastoiditis in children published between January 1980 and September 2007. The study type and setting, diagnostic criteria for acute mastoiditis, disease-specific history, presenting otologic and systemic signs and symptoms, diagnostic procedures, and final diagnosis were identified.The initial search resulted in 1,057 articles. The inclusion criteria were met in 65; 44 were retrospective case series, and 21 were case reports. These studies included 2,109 children with a median age of 32 months (range, 0 mo-18 yr). Only 26 of 65 articles reported the criteria upon which the diagnosis of acute mastoiditis in children was based. The criteria most frequently used were the clinical signs of postauricular swelling, erythema, tenderness, and protrusion of the auricle. The most frequently used imaging modality was computed tomographic (CT) scanning (reported in 39 of 65 studies, performed in 68% of patients). The most frequently used laboratory test was white blood count (100% of patients in 45 of 65 studies). In 63 studies, the result of culturing from the otomastoid was reported: Streptococcus pneumoniae was the most frequently isolated bacterium.Acute mastoiditis in children is an entity that is poorly evaluated and understood in the modern literature. There is a lack of consensus regarding the criteria and strategies for diagnosing acute mastoiditis in the pediatric population. It is crucial that such criteria are established, and consensus is achieved so that prognostic and controlled studies can be initiated to identify risk factors and establish the most effective management of this condition in children.