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Microtia & Atresia

Microtia and Atresia : A Deeper Understanding

Microtia: Microtia is a congenital deformity that influences the outer ear (pinna) and occurs when the ear doesn’t fully develop during the first trimester of pregnancy. Microtia ears can vary in appearance, often characterized by their smaller size, a peanut-shaped structure, the presence of a small nub or lobe, or even complete absence at birth. The term “Microtia” is derived from Latin, where “micro” and “otia” combine to mean “little ear.” It can affect one ear (unilateral) or both ears (bilateral) and is relatively rare, occurring in approximately 1 out of 6,000 to 12,000 births, with a slight predilection for the right ear. It’s important to note that Microtia is often accompanied by Atresia, further complicating the auditory challenges faced by those affected.

Atresia (Aural Atresia)

Aural atresia is characterized by the absence or closure of the external auditory ear canal and may also involve malformations of the middle ear bones, such as the incus, stapes, and malleus. This condition can lead to the narrowing of the ear canal, a condition known as canal stenosis. The term “Atresia” hails from Latin, signifying the absence of an opening. Aural atresia typically affects one ear (unilateral atresia) and occurs in about 1 in 10,000 to 20,000 births. Bilateral atresia, where both ears are affected, is much less common. In children with aural atresia and one normal hearing ear, there may be challenges in sound localization, making it important to maintain regular appointments with an audiologist to monitor hearing. For babies with bilateral atresia, significant hearing loss is experienced, often necessitating the use of hearing aids from a young age to facilitate the development of speech and language skills.

Microtia Grading:

The manifestation of Microtia varies from child to child, with some born having a slightly smaller ear while others may experience a more severe form where the ear is entirely absent (anotia). To describe the different presentations of Microtia, a commonly used grading system is employed, which ranges from Grade 1 to Grade 4, depending on the severity of the condition.

Causes of Microtia:

In most instances, the exact cause of Microtia remains elusive. It is believed to result from a complex interplay of environmental, genetic, and other factors. It’s essential to emphasize that parents should not carry the burden of guilt for their child’s condition, as Microtia is not caused by anything a mother did during pregnancy. While it’s rare, Microtia can be attributed to single gene abnormalities or exposure to a medication called isotretinoin (Accutane®) during pregnancy. Additionally, the CDC has recently noted an increased risk of Microtia in mothers who had pre-existing diabetes before becoming pregnant.

Syndromes Associated with Microtia:

Although Microtia is often observed as an isolated condition, it can sometimes coincide with a syndrome. Syndromes associated with Microtia may also impact various other aspects of a child’s health, including the kidneys, heart, eyes, craniofacial bones, and skeletal system. In such cases, these children are typically under the care of a Craniofacial Team, a multidisciplinary group of healthcare professionals who collaborate to address the unique needs of these individuals.

Effects of Microtia

Parents often face challenging emotions and uncertainties when their child has Microtia, including:

- Guilt Perception: A common feeling of guilt, with parents sometimes assuming they must have done something wrong during pregnancy.
- Frustration with Lack of Information: Frustration arising from a lack of support and information about the condition.
- Worries about School: Concerns about how their children will handle the challenges, especially when it’s time for them to attend school.

For Children with Microtia, these effects can manifest as:

Self-Consciousness: A sense of self-consciousness due to the appearance of their ear.
Teasing and Taunting: Becoming targets of cruel taunting from peers.
Unwanted Attention: Receiving unwelcome stares from other children and even adults who may not be familiar with the condition.
Hearing Difficulties: Difficulty in hearing, which can impact their speech development.
Academic Performance: Struggles in school, affecting academic performance.
Emotional Impact: Lowered self-esteem and other emotional challenges due to the condition.

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