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Craniofacial Conditions: Microtia & Ear Deformities

Congenital (present at birth) ear anomalies may occur independently or within a variety of different syndromes.

Microtia is a malformation of one or both ears. The outer ear (pinna) may be small, malformed, low-set or completely absent with only skin tags to indicate any growth. Rarely (one in 25,000 live births), it may occur on both ears, known as bilateral microtia. Most of the time, it affects only one ear, known as unilateral microtia (one in 8,000 live births). Interestingly, it usually affects only the right side. Boys have microtia more often than girls.

Although we don’t yet understand the cause of microtia, it can be treated with great success.

In all cases, an exterior sound conduction device is critical for proper sound/speech formation. Often, it is possible for children without an exterior ear opening to hear after corrective surgery. Depending on the extent of the condition, ear reconstruction may be needed. Usually, this is a two-stage surgery (based on the Japanese technique of Dr. Safor Nagata) beginning at age 8-10 when the ears have reached about 90 percent of their adult size.