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Congenital Ear Anomolies in Infants

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.

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Following the first week of life, only 30% of these anomolies will improve spontaneously, while the majority will remain for the lifetime of the infant.

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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

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What Causes Ear Anomolies?

Congenital ear anomalies are birth defects that affect the shape and position of the ear. These conditions can involve the soft cartilage around the ear along with other structures that affect both the function and appearance of the ear. These anomalies can also cause significant social impairment

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Any abnormal development can cause complications to a person’s ears.  In some cases the problem may be more cosmetic, while in other cases it could cause problems in the ear, nose, and throat of the person and affect normal development. Some of these problems can be attributed to abnormal positioning during development inside the uterus, or a problem that occurs during birth.

 

It is estimated that as many as 30 percent of children are born with some type of congenital ear deformity, but it’s not something that many parents or medical professionals discuss regularly. Fortunately there are several treatment options for a baby born with most types of ear deformities.

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 Types of Ear Anomolies

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Prominent Ears

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

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

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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Helical Rim Deformity

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

Cup, Lop, or Lidded Ears

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

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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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Combination, Complex, or Mixed Deformities

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

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