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MICROTIA

What is Microtia?

Development of the external ear (auricle) begins during the 5th week of gestation. Six hillocks from the first and second pharyngeal arches are responsible for the intricate anatomy and three-dimensional topography of the external ear. Underdevelopment (microtia) or absence (anotia) of the external ear comes in many different forms and has aesthetic and functional implications.

Aural atresia is the absence of the ear canal, and therefore the ability to hear. The majority of cases (~90%) of microtia are unilateral (one-sided) and the hearing in the unaffected ear is normal. Adequate hearing is important for the development of speech. Children with unilateral hearing loss are able to develop speech normally. However, their ability to localize sounds is dimished due to the lack of stereo sound.

Epidemiology Of Microtia

Microtia has an incidence of 1 in 7,000 to 1 in 8,000 live births. It affects males more than females and is more likely to affect the right ear. Tha majority of cases of microtia occur in isolation. However, some cases are associated with other congenital malformations or syndromes. The most common of these is Craniofacial Microsomia (formerly known as Hemifacial Microsomia) in which the bones and soft tissues of the face are underdeveloped as well.

Types Of Microtia

There are many classification systems for microtia which grade ears either based on the anatomy that is underdeveloped (Tanzer, Marx, Nagata) or on the type of incision and reconstruction that is required (Firmin). One commonly used system divides microtia into lobule type, concha type, small concha type, or anotia. Regardless of which system is used to describe microtia, the important considerations are the amount of native cartilage present, the quality of the overlying skin envelope including any previous scars, and the location of the hairline.

New York Microtia Procedures

The first, and most important, issue to address in the child with microtia is hearing ability. An otoacoustic emissions test (OAE) is routinely performed shortly after birth as part of the newborn screening. Pure tone audiometry and speech recognition tests require cooperation on the part of the child, while ABR (auditory brain response) or BAER (brain stem evoked potential) testing monitors the brain’s response to noises.

In the majority of patients, microtia and aural atresia is unilateral and the hearing on the unaffected side is normal. Patients with unilateral aural atresia should have an annual hearing testing, and this may need to be more frequent in the presence of middle ear infections (otitis media).

Whether or not to perform middle ear reconstruction in children with unilateral aural atresia is debatable. A conversation of the risks and benefits is something families should have with their plastic surgeon and otolaryngologist.

In children with bilateral aural atresia, it is important to be evaluated for a hearing aid at an early age. Bone conduction hearing aids (on an elastic band) are typically fitted within the first few months of life. This can be converted to a more permanent bone-anchored hearing aid (BAHA) later on. Evaluation by an otolaryngologist can determine if a child with aural atresia is a candidate for middle ear reconstruction. A specialized CT scan is typically needed to evaluate the anatomy of the middle and inner ear.

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NEW YORK EAR RECONSTRUCTION

Timing of the reconstruction of the external ear and middle ear (if applicable) needs to be coordinated by the plastic surgeon and otolaryngologist. Typically, the auricle is constructed around age 8-10, depending on the type of reconstruction.

External ear reconstruction can be performed using the patient’s own cartilage (autologous) or with synthetic material (alloplastic). At New York Group for Plastic Surgery, we are able to offer both types of microtia repairs, as our craniofacial surgeons have trained with pioneers in each of these reconstructions.

Autologous ear reconstruction involves using rib cartilage to create an ear “framework.” This type of reconstruction is performed in two to four stages, depending on the specific technique used. It involves harvesting rib cartilage at the synchondrosis, or confluence of cartilage of the rib cage. Using specialized carving instruments, the building blocks of the ear are sculpted and assembled into the framework.

It is then inserted into the skin envelope and drains are placed to provide suction and ensure proper redraping of the skin over the cartilage. The second stage of this technique involves elevating the framework to give it adequate projection and placing a skin graft behind the ear.

Alloplastic ear reconstruction using Medpor® implants involves placing a detailed, “off-the-shelf” ear framework underneath the skin envelope and then covering this with a skin graft. This technique also requires a second stage to elevate the ear to its proper projection.

Which technique is right for your child is a discussion each parent should have with their plastic surgeon. For each of our New York ear reconstruction patients, the specific risks, benefits, and alternatives will be explained in detail and all questions answered.

GET STARTED WITH MICROTIA SURGERY

Do you want to learn more about New York microtia treatment options? Contact one of our doctors online to meet with them at the New York Group for Plastic Surgery. You can also give us a call at 888-664-8381 to reach our New York City office or 914-366-6139 to reach our Tarrytown office.