Atresia: A Closer Look at This Rare Ear Condition

Atresia is a rare but important condition that affects the ear, typically present from birth. At High Peak Hearing, we believe in raising awareness and offering support for individuals living with hearing differences — and understanding conditions like atresia is an essential part of that mission.

What is Atresia?

Atresia refers to the absence or closure of the external ear canal. This can occur in one or both ears and is often accompanied by microtia — a condition where the outer ear (pinna) is underdeveloped. Together, these conditions can significantly impact hearing, especially if the ear canal is completely closed.

There are different levels of severity:

• Type I (mild): Narrow ear canal but with some function.

• Type II (moderate): A partially developed canal and middle ear.

• Type III (severe): Complete absence of the canal and underdeveloped middle ear.

• Type IV: Atresia with no middle ear or hearing structures.

What Causes Atresia?

Atresia typically occurs during early fetal development and is considered a congenital condition. The exact cause isn’t always known, but it’s sometimes associated with genetic syndromes or other craniofacial abnormalities. It affects about 1 in every 10,000 to 20,000 births and is more common in males, often occurring on the right side.

How Does Atresia Affect Hearing?

When the ear canal is absent or blocked, sound cannot reach the eardrum and middle ear. This leads to a conductive hearing loss — where the issue lies in transmitting sound rather than interpreting it.

In many cases, the inner ear (cochlea) is intact and functional, so the hearing loss is not sensorineural. This is why many people with atresia can benefit from bone conduction hearing solutions like:

• Bone Anchored Hearing Aids (BAHAs)

• Softband bone conduction devices for young children

• Surgical reconstruction, in some cases, to open the ear canal or build new hearing pathways

• CROS hearing aids in some cases

How is Atresia Managed?

Assessment begins with a comprehensive hearing test and imaging (such as CT scans) to evaluate the middle and inner ear structures. From there, management options may include:

• Audiological support and amplification using bone conduction devices

• ENT consultation to assess surgical options

• Speech and language therapy if speech development is delayed

• Ongoing hearing monitoring and educational support, particularly in school settings