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SYSTEMATIC REVIEW article

Front. Pediatr.

Sec. Pediatric Otolaryngology

Volume 13 - 2025 | doi: 10.3389/fped.2025.1520200

A Systematic Review of Congenital External Ear Anomalies and their Associated Factors

Provisionally accepted
  • Autonomous University of Chihuahua, Chihuahua, Mexico

The final, formatted version of the article will be published soon.

    Objective. External ear anomalies may lead to conductive hearing loss with significant childhood disability, psychological distress, anxiety, social avoidance, and behavioral problems. The aim of this study is to compile and review published literature on the frequency of isolated and non-isolated external ear anomalies, their associated factors, and associated malformations/deformations in nonisolated cases. Methods. We conducted a systematic review of PubMed, Google Scholar, and Science Direct searching for any type of article (excluding reviews and meta-analyses) reporting isolated and non-isolated external ear anomalies in humans. Two authors extracted the information according to the main variables of interest according to PICO criteria. Details of studied population and main findings were also obtained (malformation type, unilateral or bilateral malformations and associated factors). Results. Twenty-six studies met eligibility criteria to be included in this review. Anotia/microtia was the most reported malformation, more frequently found in males, mostly unilateral; being the right ear the most affected, and more frequent in Hispanic population. Associated factors for external ear anomalies included parental age, maternal education, multiple pregnancies, high maternal body mass index and diabetes, pregnancy, and perinatal complications (low birth weight, prematurity, threatened abortion, etc.), twining, and chemical/drug exposure. The most reported malformations and syndromes associated with congenital external ear defects included: skull/face anomalies, cleft lip/palate, congenital heart defects, musculoskeletal malformations of skull, face and jaw, Treacher-Collins, OAVS (oculo-auriculo-vertebral spectrum), and trisomy 18, 13 and 21. Conclusion. Congenital external ear anomalies can occur isolated or associated with other malformations or syndromes. Environmental, socioeconomic, and cultural factors may explain the variation across populations for congenital external ear anomalies. Depending on their type and severity, they can lead to speech impediments and childhood disability, particularly in bilateral cases, highlighting the relevance of early detection and repair to avoid childhood disability.

    Keywords: Auricle, Ear anomalies, Congenital malformations, External ear, review

    Received: 30 Oct 2024; Accepted: 24 Mar 2025.

    Copyright: © 2025 Acosta-Rodríguez, Reza-López, Aguilar-Torres, Hinojos- Gallardo and Chávez-Corral. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Dora Virginia Chávez-Corral, Autonomous University of Chihuahua, Chihuahua, Mexico

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.

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