What is a congenital ear anomaly?

Congenital means dating from birth.  Congenital anomalies are of two major categories, malformations and deformations.  Strictly speaking, malformations are anomalies in which parts of the ear are missing or malformed.  Deformations are conditions in which all or most of the ear is present, it is just folded over, has additional pleats and creases, or is prominent.

It has long been taught that many ear deformities resolve and no treatment is necessary.  Recent evidence concludes that this is not the case.  In a study from China involving over 1500 patients, the incidence of ear deformities was 58% and only one-third of these resolved at 30 days.  Another study from Japan noted that, although some of these deformities resolve, many do not and it is recommended, therefore, that all deformities be treated.

Normal Ear Anatomy

For purposes of discussion with your doctor and to help you better understand your child’s deformity, it may be helpful to visualize a normal ear and what terminology doctors use to describe its various parts. 


Normal Ear Examples

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Untreated Ear Deformities in Older Children, Adolescents, and Adults


Types of Ear Deformities

Prominent Ears

Ears that “stick out” from the head and do not have a fold just inside the rim of the ear.                                                              

 

Helical Rim Deformity

Ears in which the outer rim is folded, irregular, or pleated.  An infinite variety of such irregularities may be seen.

 

 

Combination, Complex, or Mixed Deformities

Ears in which two or more components of deformity are seen.

Stahl's Deformity

Ears where there is a third fold near the upper part that flattens out the outer rim and may make the ear look pointed.            

 

Cup, Lop, or Lidded Ears

Ears in which the upper rim (helical rim) is folded over.                                                

 

 

Cryptotia

Ears in which the anterior part of the helical rim is buried under the skin.  If one gently pulls the ear backward the normal cartilage shape is seen.

 
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Constricted Ears

Strictly speaking constricted ears are malformations as they are
deficient of skin and cartilage. Many however, have folding and
pleating and irregularities (deformations) that can be corrected with ear molding.

 

 

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