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Ponto Pro has opened up a whole new world of sounds for me

Diagnosed with microtia/atresia and anotia at birth, Justin was a toddler before his bilateral hearing loss was treated. He had begun speaking a few words, but his parents realised that he was not responding to everyday sounds. A hearing check confirmed his hearing loss. Over the next few years, Justin underwent several reconstructions of his left middle ear. When the reconstructions failed to separate the delicate middle ear bones, Justin was fitted for one of the original bone conduction solutions.

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 A Cut Above — Two Devices Are Teamed to Simplify, Quicken and Improve Ear Reconstruction

Because there is no properly shaped ear canal or ear drum to capture and relay sound waves, a child with microtia or anotia usually has partial deafness as well. Now, thanks to two novel tools developed by a Johns Hopkins Medicine resident and a former resident, the traditionally difficult surgery to create a replacement ear from a patient’s rib cartilage may soon be done faster, more simply and accurately, without wasting valuable tissue, and with the ability to “custom fit” the new organ for each patient.

Articles of Interest

A Child With Microtia & Atresia

Microtia is a term used to describe malformation of the pinna (external part of the ear). Microtia varies in severity from quite minor changes (such as the ear being smaller than expected) to 'classic microtia' where the pinna is missing and is associated with absence of the external auditory meatus (ear canal).

Diagnosing and Treating Microtia

Microtia is noted on visual inspection at birth as an obvious hypoplastic external ear deformity. A variable extent of middle ear pathology, with associated conductive hearing loss, is expected.

Ear anomalies in the newborn

Microtia-anotia is a rare condition, reported to occur in approximately 1.5:10,000 deliveries 1 (although it may be more common in some populations). About two-thirds of cases are isolated malformations, but it may occur in association with other malformations, and with specific syndromes in 10% of cases such as Treacher-Collins syndrome and Goldenhar (Hemifacial Microsomia) syndrome. Sporadic microtia is most likely to be unilateral; syndromic forms or those associated with other major malformations are more commonly bilateral.

Microtia and anotia

Microtia and anotia are medical terms used to describe the under or no development of the outer ear or pinna. When a baby is born with this disorder and especially if not picked up with antenatal ultrasound it is undoubtedly a major disappointment and stressor for the family. This reaction by the parents, is understandably but in the majority of cases not as serious as contemplated. In the first place it has to be understood that this condition can occur in 0,7 – 2,3 out of 10 000 life births. When your baby is born with this condition it is important to note that in over 99% of cases it is nothing that you as the mother did wrong!

Microtia and atresia: When ears don't develop fully

Microtia usually occurs with atresia, also known as aural atresia, a condition in which the auditory ear canal is either underdeveloped, absent or closed. Microtia and atresia almost always affect hearing and generally require ongoing medical care from a pediatric ear-nose-throat (ENT) specialist as well as an audiologist. It's more common in males than females.

Microtia-Anotia: A Global Review of Prevalence Rates

We observed marked variation in the prevalence of microtia-anotia across surveillance programs and within countries. These results must be interpreted cautiously as this variability may be explained mainly by differences in surveillance methods.

Position Statement: Treatment for Microtia and Anotia

The American Academy of Otolaryngology—Head and Neck Surgery (“AAO-HNS”) recognizes microtia and anotia as congential birth defects. These conditions are associated with appreciable psychological and functional ramifications if left untreated. Reconstructive surgery is appropriate as a primary treatment in both children and adults. Therefore, microtia and anotia shall be considered reconstructive surgery to restore a missing or significantly deformed body part visibly present under normal circumstances.

The epidemiology of anotia and microtia

Anotia and microtia are equally often associated with other malformations and show other similar epidemiological characteristics. In unilateral cases, the right side is more frequently malformed than the left side, especially when the ear malformation is isolated.

Understanding Atresia, Microtia, and the Baha System

Regarding microtia, there are several different modes of treatment. With microtia, we are mainly dealing with cosmetics of the outer ear. Prosthetics work best with anotia, but we are going to look at some cases later where that can be achieved by other means.

What Can We Do About Microtia and Atresia?

There are four different grades of microtia, the most common involving aural atresia, the absence of or abnormally narrowed closure of the ear canal and eardrum.

Resources

Ear Community

Ear Community is a 501(c)(3) nonprofit organization that offers a warm and supportive community for individuals who were born with Microtia (missing or underdeveloped ears) and Atresia (absent or underdeveloped ear canals resulting in hearing loss).

Little Baby Face Foundation

Our mission is to transform the lives of children born with debilitating facial deformities through world-class reconstructive surgery.

Microtia and Atresia Support Group

The Microtia & Atresia Support Group is for individuals born w/Microtia & Atresia & is part of a nonprofit organization called Ear Community.

International Institute for Microtia Repair

Microtia causes children to be born with a small, misshapen, or missing ear. Because the condition is so uncommon, only a few doctors have the necessary skill and knowledge to treat it successfully. The International Institute for Microtia Repair was founded by Dr. Youssef Tahiri and Dr. Reinisch, world leaders of Medpor Ear Reconstruction, to help children all over the world with this ear deforming condition.

Lifespan

Microtia is a condition in which the external ear is smaller than normal size and not fully formed. This condition can range from a mild deformity that is not very noticeable, to a major defect called anotia where the external ear is completely missing. The child may have a narrow or missing ear canal, which can affect their ability to hear. Atresia is the absence of underdevelopment of the ear canal and middle ear structures. Atresia is often present with microtia because the outer and middle ear develop at the same time in the fetus. Most children will only have one ear affected, but it is possible for the condition to occur for both ears.

CDC

Anotia and microtia are birth defects of a baby’s ear. Anotia happens when the external ear (the part of the ear that can be seen) is missing completely. Microtia happens when the external ear is small and not formed properly.