Congenital Bilateral Microtia-Anotia: A Clinical Challenge


Congenital Bilateral Microtia-Anotia: A Clinical Challenge


Dr Sumita Mehta1*, Senior Specialist & In-Charge, Dr Anshul Grover1, Specialist, Dr Avdesh Mehta 2, ENT Consultant, Dr Deepak Chugh3, CMO & Head of Department, Paediatrics

1Dept. of Obstetrics & Gynecology, Babu Jagjivanram Memorial Hospital, Delhi, India. 2Medhealers Clinic, Delhi, India. 3Dept. of Pediatrics, Babu Jagjivanram Memorial Hospital, Delhi, India.


Microtia- Anotia (M-A) is a rare congenital malformation of the external ear structure commonly associated with atresia or stenosis of the ear canal. Its prevalence is more common in Asians and Hispanics, is generally unilateral and is seen more commonly in males. It can either be an isolated defect or be a part of specific pattern of anomalies involving other organs. It has been documented as a component of either a teratogenic embryopathy (isotretinoin, thalidomide or alcohol) or single gene disorders like Treacher Collins and as a part of a pattern of multiple defects like Goldenhar, Nager and CHARGE syndrome. We report a case of a newborn with bilateral microtia-anotia and facial palsy, who on further evaluation was found to have congenital heart disease also. The triad of anotia, facial paralysis and cardiac anomaly is rare and has been first described by Pearl in 1984.Thus, the obviously visible ear malformation at birth should be evaluated in depth to rule out other underlying malformations.


Keywords: microtia, anotia, Pearl syndrome, Goldenhar syndrome, lower motor neuron facial palsy, Treacher Collins syndrome, Nager syndrome, CHARGE syndrome


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How to cite this article:

Sumita Mehta, Anshul Grover, Avdesh Mehta, Deepak Chugh. Congenital Bilateral Microtia-Anotia: A Clinical Challenge. International Journal of Case Reports, 2021; 5:221. DOI: 10.28933/ijcr-2021-05-2506


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