*4.3.3. Macrotia*

Macrotia refers to an oversized or enlarged but well-developed auricle without any other malformations of the ear (**Figure 7**). The most exaggerated portion of the ear is the scaphoid fossa. The condition is usually bilateral and symmetric.

Generally, it has an autosomal dominant pattern of transmission and an unknown pathogenesis [42].

Macrotia is commonly associated with the following syndromes:


• Variant of De Lange type 2 syndrome [44]: characterized by macrotia associated with severe microcephaly, mild mental retardation, muscular hypotonia and dysmorphic faces (flat profile, mild ptosis, short nose with a large tip and anteverted nares, narrow mouth, retrognathism).
