PRISMA 2020 checklist.

<div><p>Context</p><p>External Ear Malformations (EEM) continue to be a common malformation seen in the pediatric patient population. This study aims to further elucidate the correlation between EEM and cardiac and renal anomalies.</p><p>Objective</p><p>A systematic review and meta-analysis to study the incidence of cardiac and renal anomalies associated with syndromic and isolated (EEM).</p><p>Data sources</p><p>The literature search spanned multiple databases, including Google Scholar, PubMed, Scopus, Web of Science, and MEDLINE.</p><p>Study selection</p><p>Studies must be focused on EEM and cardiac and/or renal anomalies. Only articles written in English were included.</p><p>Data extraction</p><p>General study characteristics, number of EEM patients, number of cardiac and renal anomalies and whether cases were syndromic were extracted from the studies.</p><p>Results</p><p>Of 1,058 initial studies, 33 were included for meta-analyses. Mean JBI score for all included studies was 92.06%, indicating acceptable study quality. Interrater reliability was high, with a Cohen kappa score for all studies of 0.94. The resulting pooled prevalence of cardiac abnormalities was 20% [95% CI:13–28%], while renal abnormalities were 13% [95% CI: 7–20%]. The most common anomalies were VSD (3.725%) and renal agenesis (2.04%). The presence of syndrome data across studies was not a significant modifier of prevalence rates.</p><p>Limitations</p><p>Primary limitation is due to heterogeneity in individual study methodology and reporting standards.</p><p>Conclusions</p><p>These results highlight a higher prevalence of cardiac-related conditions than renal anomalies in patients with both syndromic and non-syndromic EEM in the included studies, underscoring the need for thorough clinical evaluations.</p></div>