Congenital absence of (ear) auricle

This code signifies the complete congenital absence of the external ear, also known as anotia. This is a rare birth defect where the auricle, or pinna, fails to develop during gestation, resulting in no visible external ear structure. It can occur unilaterally or bilaterally and may be associated with other craniofacial anomalies.

Use this code for patients diagnosed with anotia, confirmed by physical examination at birth or early childhood. Documentation should clearly state the complete absence of the auricle, distinguishing it from microtia (underdeveloped ear). This code is appropriate for initial diagnosis and subsequent encounters related to the condition, such as for surgical planning or prosthetic fitting.

• Differentiate from microtia (Q16.1), which involves an underdeveloped but present auricle.
• Code laterality (left, right, bilateral) if specified in the documentation using appropriate seventh characters or secondary codes.
• Consider additional codes for associated congenital anomalies or syndromes, such as hemifacial microsomia, if documented.