Microtia

Microtia is diagnosed when a child is born with small, abnormally-shaped or completely absent, outer ear on one or both sides of the head. Most children with this condition have a normally-formed hearing structures within the skull but lack all or part of the structure that forms the outer ear. In some cases, hearing is compromised. Riley Hospital for Children at Indiana University Health is the only center in Indiana performing all available techniques for hearing and ear reconstruction. Our experienced physicians offer the most advanced treatments for Microtia.

What is Microtia?

Microtia literally means small ear. Children with this rare condition have a small, abnormally-shaped or absent external ear. Most children have this condition only on one side (unilateral), however it can occur on both sides (bilateral). Typically microtia only involves the outer ear, but can also cause difficulty hearing and determining the direction of sound.

In some cases, a child with microtia will have no hearing in the affected ear, due to the absence of the ear canal. This is called aural atresia. A child with microtia and aural atresia in both ears will be significantly hard of hearing and require a hearing aid. Children with aural atresia typically also have incomplete formation of the small middle ear bones and may lack an eardrum.

What causes Microtia?

Microtia is found in children at birth and is usually an isolated condition. In some children, microtia occurs along with other facial abnormalities, such as hemifacial microsomia, which involves the under development of one side of the face, including the ear. In very rare cases, hemifacial microsomia can occur on both sides of the face at once.

Children with Treacher Collins syndrome [link to page], a condition that involves the underdevelopment of the lower eyelids, cheekbones and lower jaw, may also have microtia.

How is Microtia treated?

Riley Hospital for Children at IU Health has extensive experience treating microtia, and is the only hospital in Indiana performing all available techniques for hearing and ear reconstruction. Our experienced physicians offer the most advanced treatments for microtia, working closely with other specialists, including Ear, Nose and Throat [link] physicians when hearing aid placement is necessary. Because microtia may occur in conjunction with other craniofacial conditions that require reconstruction, our coordinated care means your child receives the comprehensive treatment necessary to accomplish the best outcomes.

A series of surgeries is typically required to completely correct microtia. The structure of the outer ear is built up, using cartilage from the patient’s ribcage to begin building the framework for the outer ear. Most children do not undergo surgery to treat microtia until their ear has stopped significantly growing, usually around six to eight years old.

Prior to surgery, patients should undergo a complete hearing evaluation. If it is determined their hearing is compromised, a hearing aid will be recommended. Surgery for reconstruction of the middle ear can take place only after the outer ear has been reconstructed, and is usually only performed on children with hearing loss in both ears. This is done because children with microtia and hearing loss in only one ear will typically hear at normal levels, with the exception of hearing sounds in “stereo”.

In addition to hearing and language screenings, a high resolution, 3D CT scan of the temporal bones is recommended. This test is performed to rule out a benign tumor of the middle ear, known as a cholesteatoma, which is more common in children with aural atresia.

Additional information about Microtia

It is important there is sufficient hearing during a child’s early years in order to ensure normal speech development. Two types of hearing tests help determine if a child will need a hearing aid:

  • BAER testing (Brain stem Auditory Evoked Response testing)—is used for infants and small children
  • Reliable behavioral testing—is used when a child is old enough to cooperate and understand directions.

Patients with bilateral aural atresia, meaning they have hearing loss in both ears, receive bone conduction hearing aids within the first few months of life. In addition, speech therapy will be recommended as soon as a child begins to talk. Older children may receive a bone-anchored hearing aid (BAHA), which works by directly stimulating the inner ear through the bone.
 

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