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Fig 1.

PRISMA flow chart showing inclusion and exclusion criteria.

The diagram illustrates the process of study selection for this systematic review. Initially, 1058 studies were identified from various databases. In our post-screening and eligibility assessment, 779 studies were excluded for reasons such as wrong outcomes, wrong comparators, or inappropriate study design. Ultimately, 33 studies met inclusion criteria.

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Table 1.

Comprehensive overview of research studies.

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Table 1 Expand

Fig 2.

Prevalence diagram of EEM subtypes in this patient cohort.

Microtia, including anotia, accounted for 88.8% of patients. Accessory tragus accounted for 10.1% of patients, while preauricular pit/sinus and other unspecified malformations accounted for 1.5% and 1.4% of patients, respectively. Note, a subset of patients presented with more than one malformation.

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Fig 3.

Cardiac and renal anomalies in EEM patients.

Raw prevalence of cardiac and renal anomalies in patients with External Ear Malformations (EEM) as reported across 20 studies for cardiac anomalies (A) and 26 for renal anomalies (B).

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Fig 4.

Pooled prevalence of cardiac abnormalities.

Forest plot showing pooled prevalence of cardiac abnormalities among EEM patients from 20 studies. An overall pooled prevalence was 20% [95% CI:13–28%] was observed.

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Fig 5.

Cardiac subgroup prevalence analysis: Microtia vs Other EEM.

Subgroup Analysis showing Pooled Prevalence of Cardiac Abnormalities in Microtia vs Other EEM. (Top) Pooled prevalence of cardiac abnormalities in microtia from 10 studies. (Bottom) Pooled prevalence of cardiac abnormalities in other EEM from 10 studies.

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Fig 6.

Pooled prevalence of renal abnormalities.

Forest plot showing pooled prevalence of renal abnormalities among EEM patients from 26 studies. An overall prevalence of 13% [95% CI: 7–20%] between EEM and renal abnormalities was observed.

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Fig 7.

Renal subgroup prevalence analysis: Microtia vs Other EEM.

Subgroup Analysis showing Pooled Prevalence of Renal Abnormalities in Microtia vs Other EEM. (Top) Pooled prevalence of renal abnormalities in microtia from 7 studies. (Bottom) Pooled prevalence of renal abnormalities in other EEM from 19 studies.

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