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Microtia

Definition

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

Causes

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, a condition that involves the underdevelopment of the lower eyelids, cheekbones and lower jaw, may also have microtia.

Hearing status

When meeting an infant with microtia for the first time, the greatest source of parental anxiety that needs to be addressed is the issue of hearing loss. It is important that the plastic surgeon recognizes this and takes the time to discuss the matter carefully before proceeding to a dialogue about reconstruction. It may be helpful to point out to the parents that the outer and middle ear derive embryologically from different tissue (first and second branchial arches) than does the inner ear (from ectodermal sensory placodes). The parents should understand that the visibly anomalous portion of the ear does not necessarily reflect aberrant inner ear structures (inner ear abnormalities occur in approximately 10% of microtic ears, and are usually minor). Patients with monaural hearing are at risk for delayed speech development and poor school performance but may be followed closely without a hearing aid.

Baha Implant

It is a surgically implantable system for treatment of hearing loss. BAHA stands for Bone Anchoring Hearing Aid.

It has three parts:

  • A sound processor
  • An external abutment – Transmits sound vibrations
  • A titanium implant- Sets up vibrations within the skull and inner ear

A Baha implant enhances natural bone transmission as a pathway for sound to travel to the inner ear, bypassing the external auditory canal and middle ear.

Macrostomia

This form of facial clefting (Tessier No. 7) reflects an error in fusion between the embryologic maxillary and mandibular processes. This presents along a spectrum of severity from a subtle transverse cheek crease, or mild lateral oral commissure displacement, to a complete cleft with commissure displacement extending far into the involved cheek. Infants with significant macrostomia may have some difficulty with feeding from non-cleft feeders because of problems with oral seal. Older children with macrostomia may suffer from oral salivary incontinence.

Treatment

Dr. Tahiri has extensive experience treating microtia. He works closely with other specialists, including Ear, Nose and Throat physicians when hearing aid placement or canal reconstruction 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. After having performed multiple techniques for ear reconstructions, Dr. Tahiri decided to only perform Medpore Ear Reconstruction; technique that was popularized by his colleague and founder of the surgery, Dr. John Reinisch.

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.

Dr. Youssef Tahiri is a highly respected pediatric craniofacial surgeon who was hand selected by Dr. John Reinisch to partner and continue his practice of Microtia reconstruction. Dr. John Reinisch is the founder of the Medpore ear reconstruction technique and is considered the worldwide leader in Medpore ear reconstruction.

Dr. Tahiri embraces the most innovative, cutting-edge technique available in craniofacial and microtia surgery.