Overview

This site has been developed to foster an understanding of Microtia and other ear deformities to provide information on the treatments available for these ear conditions.

The Microtia-Congenital Ear Deformity Institute evaluates and operates hundreds of children born with a congenital ear deformity known as microtia/atresia.

Classifications of Microtia:

Microtia is divided into separate classifications (the classic microtia is considered Grade III).

Anotia

Anotia technically means no ear. This ear deformity, the most severe form of microtia may at times be called anotia.

Grade 1 Microtia

The ear is smaller than normal although most of the features of a normal ear such as a well-defined lobule, helix and anti-helix are present. This can occur with or without an external auditory canal.

Grade 2 Microtia

The normal features of the ear are missing. There is still a lobule and a remnant of helix and antihelix.

Grade 3 Microtia

"The Classic Microtia": The ear consists of a vertical skin appendage with a malformed lobule (earlobe) on the lower end. There is usually firm tissue at the upper end which is made up of a disorganized cartilaginous vestige. The lower end is usually a piece of lobular tissue which will be the future earlobe when reconstructed. Usually there is no external auditory canal (atresia).

Incidence and possible causes of Microtia

Microtia occurs more commonly in males and on the right side (unilateral). Approximately 10% may occur on both sides (bilateral).

Microtia may occur as an isolated deformity although it typically presents as part of a spectrum of other defects, either minor or major. Most patients with the most severe form of microtia also lack an external auditory canal, also known as "atresia". Microtia is known to occur as part of a spectrum of syndromes, especially hemifacial microsomia, Goldenhar syndrome and Treacher-Collins syndrome.

Although causes of microtia and other ear deformities are difficult to define, some theories include a decreased blood supply to the developing ear in-utero. Others have theorized certain medication taken by the mother during pregnancy such as Accutane, Thalidomide and retinoic acid. Environmental factors have also been questioned.

Bilateral Microtia/Atresia

Children born with bilateral microtia/atresia (both sides affected) are managed differently than those with unilateral (one-sided) microtia/atresia. These children require bone-conduction hearing aids within the first few months of life. Of course, hearing examinations should be performed first. Although these surgeries used to be started as early as 4 years of age in the past, the preference is now to start the external ear surgeries at about 6 to 7 years of age (depending on the size of the child).

Because these children are dependent on bone conduction hearing aids, the goal of starting earlier is to at least finish one ear. If the child is a surgical candidate, the canal may be drilled soon after the ear is reconstructed with the eventual goal of obtaining adequate hearing without the use of hearing aids. The bone-anchored hearing aid or BAHA has gained much more popularity over ear canal surgery as a means to improve the hearing.

It is ideal to start the external ear reconstructions BEFORE the middle ear surgery. Once an attempt is made to open the canal prior to the external ear reconstruction, the elasticity of the "virgin" skin as well as the circulation is compromised.