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The ear divides into three areas: the external, middle, and inner. Based on location, different malformations can present. A malformation is not only a change in appearance but also an alteration in function. External ear defects are common and occur in roughly 5% of the total population.[1] The most common malformations consist of combined external and middle ear deficits, called congenital aural atresia.[2] Microtia is a term used to describe the underdevelopment of the pinna, whereas anotia is a term used for an absence of pinna.

There are different types of inner ear anomalies, and they currently get placed into categories numbered 1 through 7. These categories primarily have their basis on defects in the modiolus and the scala vestibule. Because there are several anomalies, there are many associated hypothesized pathophysiologies. Some, in particular, include high cerebrospinal fluid pressure, defective endosteum, large quantities of endolymph, and decreased cochlear vascular supply.[11]