Understanding your child's ear canal.

Aural Atresia

Hearing that your child was born without an open ear canal raises urgent questions. Will they hear? Will they talk on time? Is something else going on? This page explains what aural atresia is, how it’s found and measured, and what to watch for as your child grows.

 

For an overview of all microtia and atresia treatment, see Microtia & Aural Atresia Care.

What Aural Atresia Is?

The ear canal is the passage that carries sound from the outer ear to the eardrum. In aural atresia, that passage didn’t form, or formed only partly. It’s one of a family of related conditions:

  • Atresia: The canal is closed completely, by bone, soft tissue or both.
  • Stenosis: The canal exists but is very narrow, usually under about 4 mm across.
  • Acquired atresia: The canal closes later in life after infection, injury, or ear surgery. This page focuses on atresia present from birth.

Because sound can’t pass through, the result is conductive hearing loss on that side. Behind the closed canal, the eardrum and middle ear bones may be small, joined together or missing. The inner ear usually formed normally.

How Common It Is &
How It Happens

  • Congenital aural atresia affects roughly 1 in 10,000 to 20,000 babies.
  • It’s more common in boys and usually affects one side, often the right.
  • Most children with atresia also have microtia, but a child can have a closed or narrow canal with a normal-looking outer ear.

The outer ear, ear canal and middle ear all form early in pregnancy, from the same small cluster of tissue that also shapes the jaw and parts of the face. For a while, the developing canal is filled with a plug of cells. That plug normally clears in the later months of pregnancy and leaves an open passage. When this process is interrupted, the canal stays closed or narrow.

This is why atresia so often appears together with microtia, and sometimes with differences in the jaw or face. In most children no single cause is found, and it isn’t linked to anything parents did or didn’t do.

Conditions Sometimes Linked with Atresia

Most children have atresia on its own or with microtia only. In some, it’s part of a wider pattern, such as:

  • Craniofacial (hemifacial) microsomia, including Goldenhar syndrome
  • Treacher Collins syndrome
  • Crouzon syndrome
  • Less often, others such as Pierre Robin sequence, Klippel-Feil syndrome or DiGeorge syndrome.

 

When other features are present, a genetics consultation can clarify the diagnosis and what it means for future children. Some of these conditions also affect how well canal surgery is likely to work, which is why we look at the whole child, not just the ear.

What To Watch For

At birthNo visible ear canal opening, or a very small one
An outer ear that is small or differently shaped
A newborn hearing screen that doesn’t pass on one or both sides
As your child learns to talk

Children with hearing in one ear usually develop speech normally, but keep an eye on these milestones and mention any concerns at your next visit:

By 12 months: not yet pointing, waving or using other gestures
By 18 months: relying mostly on gestures rather than sounds, rarely copying sounds, or not following simple spoken requests
By 2 years: repeating words without starting to use language on their own

Children with atresia in both ears need hearing support from the first weeks of life, so speech can develop on time.

If the canal is narrow rather than closed

A narrow canal can trap skin and wax behind it. Over time this can form a cholesteatoma, a growth of trapped skin that slowly damages surrounding bone. Call us if your child has:

  • Discharge or an unpleasant smell from the ear
  • Ear pain or a feeling of pressure
  • Dizziness or ringing in the ear
  • Any new weakness in the face

Children with stenosis need regular checks even if they hear well.

How Atresia is Diagnosed

Hearing is checked in stages, from the first days of life to a CT scan at around age 2½.”

WhenWhat happens
First days to first monthsNewborn hearing screening, then an auditory brainstem response (ABR) test. This measures hearing in each ear while the baby sleeps.
From about 6 monthsBehavioral hearing tests: an audiologist watches how your child responds to sounds and speech.
Around age 2½A temporal bone CT scan maps the canal, middle ear, and inner ear. It’s done earlier if a narrow canal raises concern about cholesteatoma.
Any time, if neededGenetic evaluation when other facial or body features are present.

Reading the CT:
the 10-point Score

Surgeons often use the Jahrsdorfer scale to judge whether a new canal is likely to give useful hearing. Each structure that’s present and well formed earns points:

What the score means: generally, 7 or above suggests a good chance of strong hearing after canal surgery, 6 is borderline, and 5 or below usually points toward a hearing device instead. The score guides the decision but doesn’t make it on its own.

Structure on CTPoints
Stapes (the smallest hearing bone)2
Oval window open1
Middle ear space present1
Facial nerve in a normal position1
Malleus and incus present1
 Mastoid air cells developed1
 Incus joined to the stapes1
 Round window present1
 Outer ear appearance 1
 Total possible 10

Outlook

Most children with atresia grow up to hear, speak and learn well, especially when hearing is checked early and supported where needed. Hearing in the affected ear may not become completely normal, but treatment often brings it close. Regular follow-up makes sure any change is caught early, especially in children with narrow canals.

More Questions About Atresia?

Our FAQ covers diagnosis, hearing, surgery, recovery, and travel.

Yes. Some children have a closed or narrow canal with a normal-looking outer ear. They still need the same hearing tests and imaging.

Not necessarily. A narrow canal may let some sound through, but it can trap skin and lead to cholesteatoma, so it needs regular monitoring.

Usually not. Balance is controlled by the inner ear, which typically forms normally in atresia.

No. A closed canal doesn’t cause pain on its own. New pain, discharge or odor in a narrow canal should be checked promptly.

Yes. Hearing evaluation, canal surgery and hearing devices are all options at any age.

 

Exploring Treatment?

Once the CT shows what’s behind the closed canal, there’s almost always a way to bring sound through. Dr. Nazarian and Dr. Reinisch will help you choose the path that fits your child’s anatomy, age and daily life. Every option is explained at your consultation, including what to expect for hearing, recovery and long-term care.

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