What is Aural Atresia

Aural Atresia is an underdeveloped external ear canal, eardrum and middle ear bones. It is often associated with microtia, a condition in which the outer ear is small, malformed or missing. Those with aural atresia most often have abnormalities of the outer and middle ear, but a normally functioning inner ear (cochlea) and hearing nerve. This condition results in a CONDUCTIVE hearing loss.

Types of Aural Atresia

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Totally absent ear canal (most common)

Complete Aural Atresia

A canal is present but it is very small with conductive hearing loss

Aural Stenosis

Aural atresia usually involves only one ear (unilateral atresia) and occurs in 1 in 10,000 to 20,000 births. Bilateral atresia, when both ears are affected, is much more rare. Children with aural atresia and one normal hearing ear may develop normal speech but have trouble knowing where sound comes from, referred to as “sound localization.” Regular appointments with an audiologist are important to confirm the child's hearing isn’t changing in either ear.

Babies with bilateral atresia have significant hearing loss. Doctors recommend newborns use hearing aids to help develop speech and language.

How Hearing Works

Learn how sound is normally processed through the outer, middle and inner ear through this animated video.

Video courtesy of MED-EL

Children with aural atresia have a conductive hearing loss with abnormal air conduction, but often have normal bone conduction. An audiogram is used to diagnose the type and severity of hearing loss. Learn about audiograms in this video.

Video courtesy of MED-EL

Treatment Options for Aural Atresia

Bone conduction hearing devices

Commonly known as BAHAs (Bone Anchored Hearing Aids) or BAHS (Bone Anchored Hearing Systems), these devices bypass the abnormal outer and middle ear by sending sound through the skull bone directly to the functioning inner ear.

The BAHA can be worn on a softband, an adhesive sticker, a titanium abutment or magnetically. The softband or adhesive form can be worn starting in infancy; the titanium abutment and magnet can be surgically implanted when the skull is of proper thickness (approximately 6 years old). These four videos explain how each system works.

Oticon Ponto (abutment)

Cochlear Connect (abutment)

Med-el Adhear (sticker)

Cochlear Attract (magnet)

                 

Combining BAHA & PIER in one surgery

During PIER surgery, Dr. Lewin uses a scarless technique to surgically place the titanium implant into the skull. This eliminates the need for an additional surgery with general anesthesia after the microtia ear reconstruction. The type of surgical implant (abutment or magnet), brand of processor, and processor accessories are all chosen by the patient and their local audiologist.

Dr. Lewin can surgically implant the following options 

hearing-loss_small1

Cochlear abutment

hearing-loss_small2

Cochlear 

magnet

hearing-loss_small3

Oticon Medical abutment

Atresia Repair (Canal Surgery)

Atresia Repair (canal surgery) attempts to surgically restore hearing by creating a skin-lined ear canal with an “eardrum” that allows sound waves to be transmitted to the middle and inner ear. This surgery is only performed by an Otologist specializing in atresia repair. Dr. Lewin does NOT perform this type of surgery. Atresia repair can be done as early as 3-6 years old, depending on the specialist. An audiogram and a high resolution CT scan are needed to determine if your child is a good candidate for atresia repair.

 

The appearance of the canal after atresia repair before PIER surgery.

As with any surgery, there are multiple risks of atresia repair including:

  • Infection
  • Canal “closing” (stenosis)
  • “Eardrum” separation (requires surgery to correct)
  • Facial nerve injury/facial paralysis
  • Hearing nerve injury
  • Permanent hearing loss
  • Loss of the initial hearing improvement over time
  • Failure of the atresia repair, requiring surgery to close the canal

It is important for parents to know that about 50% of children with atresia should NOT have a surgical canal because the anatomy is too abnormal to achieve successful hearing.  Even those that do have favorable anatomy may have initial hearing improvement after atresia repair, but then may lose some or all of the hearing gained due to the unpredictable nature of the healing process. Sadly, some children who have atresia repair can actually have complications from the surgery that worsen their hearing. So having atresia repair is not a decision to be taken lightly. Extensive research into this procedure and multiple consultations with experienced atresia surgeons are recommended due to the varying opinions among different doctors. Since the results of atresia repair are surgeon-dependent and somewhat unpredictable over time, a BAHA may still be required to achieve satisfactory hearing. This is especially true for bilateral atresia patients. After atresia repair, the surgical canal will require lifelong regular cleanings by an ENT in order to keep the canal functioning properly. For those interested in atresia repair, it must be completed 4 months BEFORE the Porous Implant Ear Reconstruction surgery with Dr. Lewin.