iCliniq logo

Ask a Doctor Online Now

HomeHealth articlescongenital abnormalitiesHow Is Congenital Atretic Ear Reconstruction Done?

Congenital Atretic Ear Reconstruction: Indication, Prognosis, and More

Verified dataVerified data

3 min read


Congenital aural atresia is a common condition in which the ear canal does not develop normally. Scroll down to read more.

Medically reviewed by

Dr. Arpit Varshney

Published At February 2, 2023
Reviewed AtApril 25, 2023

What Is Congenital Aural Atresia?

Aural atresia is a condition in which children are born with an underdeveloped ear canal and middle ear structures. This can result in conductive hearing loss in newborn babies. This condition can also be acquired during their lifetime, which is known as acquired aural atresia, which can occur due to an inflammatory process following a trauma or surgery. The human ear can be divided into three parts: the external ear, the middle ear, and the inner ear. The external ear, which is also known as the outer ear, consists of the auricle and the external auditory canal. The external ear channels the soundwaves to the eardrum and the middle ear.

The condition is caused by failure to integrate the structures during embryonic development, which leads to malformation of the external ear and conductive hearing loss. This condition can occur along with another congenital condition known as microtia. Microtia is another congenital condition in which the pinna does not form correctly.

What Causes Congenital Aural Atresia?

The exact cause of congenital aural atresia is unknown. The condition results from disturbances during the development of the embryo at around the sixth week of gestation. This disturbance will affect the development of the first pharyngeal cleft, which ultimately forms the external auditory canal. This disruption to the development mostly occurs randomly but is seen in association with several syndromes such as Goldenhar (a rare congenital condition characterized by abnormal development of the eye, ear, and spine), Treacher Collins (a rare congenital condition with distinctive abnormalities of the head and the face), and Crouzon syndrome (a genetic disorder characterized by premature fusion of skull bones).

How Common Is Congenital Aural Atresia?

Congenital aural atresia is not a very common condition. It is seen in one in every 10,000 to 20,000 live births. Congenital aural atresia can affect both ears, but unilateral occurrence is more common than bilateral. Even though unilateral, the condition has more predilection towards the right ear than the left ear. Even though the children with this condition have normal hearing in the unaffected ears, it is seen that they have delayed language development.

What Are the Complications Of Congenital Aural Atresia?

The complications seen in congenital aural atresia are:

  • Delayed language development.

  • Severe learning disabilities.

  • External ear cholesteatomas.

The complications following the surgery of the aural atresia correction include:

  • Lateralization of the tympanic membrane graft.

  • Restenosis of the canal.

  • Temporomandibular joint dysfunction.

  • Facial nerve paralysis.

  • Taste disturbance.

  • Sensorineural hearing loss.

  • Conductive hearing loss, infection.

  • Vertigo.

How Is Congenital Aural Atresia Treated or Managed?

The treatment for congenital aural atresia will depend upon factors like hearing status, cosmetic desires, and the feasibility of hearing restoration. Children with bilateral aural atresia will have severe hearing loss, in which children require early bone conduction hearing aids. Whereas in the case of children with unilateral aural atresia, they will have only some amount of hearing loss, they should be closely observed by the teachers and speech therapists to ensure a normal progression of speech and language. They should undergo routine checkups every 6 to 12 months to check for hearing in the unaffected ear.

  • Medical Therapy- Other than doing the surgery, this condition can be treated by using amplification devices such as a bone conduction hearing aid with a band worn around the head or normal conventional hearing aid if the auricle structure supports the aid.

  • Reconstruction Surgery- The surgery to correct the aural atresia is done following the multistage autologous rib graft reconstructive surgery for microtia. This surgery will also depend upon the age of the child so that the child cooperates with the surgery, postoperative dressing changes, packing removal, and ear canal cleaning to ensure a good result. The reconstruction surgery is done under general anesthesia. The surgery is done by making a postauricular incision into the temporalis fascia. Several grafts with fascia and skin are placed so as to correct the defective affected structure of the ear anatomically.

What Is the Prognosis for Congenital Aural Atresia?

The prognosis for congenital aural atresia will depend upon the severity of the condition and the associated abnormalities. In most cases, they have a favorable prognosis. The most important factors in having a good prognosis are the selection of the patient for the surgery and the surgical technique. Surgery is not recommended for patients with unilateral aural atresia. It is difficult to achieve hearing back to normal following the surgery. The patient will be able to wear a conventional hearing aid in the new canal so as to bring the hearing threshold back to the normal range.


Congenital aural atresia is a condition present in children from the time of birth and affects the development of the external ear canal. Patients with congenital aural atresia can also have congenital microtia. These children can have hearing disabilities and learning disabilities. The parents and the teachers of these children need to take extra care and observe them during their growing age so as to look for their problems.

Frequently Asked Questions


Is It Possible to Treat Atresia With Surgery?

Yes, surgery is an option for treating atresia. Surgery is recommended in situations like:
- Surgery is performed to create an ear canal for hearing restoration.
- Replacement of one, two, or all three of the middle ear bones.
- To remove abnormal tissue from the ear.


Can Atresia Lead To Hearing Loss?

Aural atresia is considered an acquired or congenital condition. The congenital aural atresia is seen in newborns with an underdeveloped ear canal and middle ear structures. This can result in conductive hearing loss in newborn babies. 


How to Differentiate Between Microtia and Atresia?

Yes, there is a difference between microtia and atresia. 
- Microtia - It is a congenital deformity affecting the outer ear.
- Atresia - It is also known as aural atresia. It is defined as the absence or an underdeveloped external auditory ear canal. 
- Microtia affects the outer ear, and it is mostly associated with middle ear deformities and hearing loss.


How Many Grades Does Atresia Have?

There are 4 grades of atresia:
- Grade I: A small ear with a shape much like a normal ear often accompanies a small but narrow ear canal.
- Grade II: A partial ear with an absent ear canal.
- Grade III: Absence of most of the external part of the ear with a small "peanut" shaped remaining ear lobule and an absent ear canal and ear drum. 
- Grade IV: Total absence of the ear or anotia.


What Is the Classification of Atresia of the Ear?

Atresia is the lack of a fully developed ear canal, eardrum, middle ear space, and ear bones. Atresia is classified into 2 types:
Unilateral Atresia:
- In unilateral atresia, only one ear is affected by the condition, while the other ear typically develops normally.
- This type of atresia can lead to hearing loss in the affected ear, as sound cannot travel through the underdeveloped or blocked ear canal to reach the middle and inner ear structures.
- Individuals with unilateral atresia often rely on their unaffected ears for hearing and may experience challenges with sound localization and understanding speech in noisy environments.
Bilateral Atresia:
- Bilateral atresia refers to the presence of atresia in both ears, meaning both ear canals and associated structures are underdeveloped or absent.
- People with bilateral atresia often face more profound hearing difficulties, as they lack a functional pathway for sound to enter the ear and reach the inner ear structures responsible for processing sound signals.
- Treatment options for bilateral atresia may include surgical interventions to reconstruct the ear canals and improve hearing or the use of bone-conduction hearing devices to bypass the ear canal and directly stimulate the inner ear.


What Is the Comparison Between Atresia and Stenosis?

It is reposted that some children are born without an external ear canal. This condition is called aural atresia. In other cases, if a child is born with a narrowed ear canal, the eardrums can still be viewed, but the canal is narrower than normal. This condition is called ear canal stenosis.


What Are the Death Rates of Atresia?

The death rates of atresia conditions vary widely depending on the specific type and severity of the atresia, as well as timely medical intervention. With advancements in medical and surgical techniques, survival rates have generally improved over the years for many atresia-related conditions. However, untreated or severe cases of atresia can still pose a significant risk to life, especially in the absence of timely medical intervention. Therefore, it is crucial to diagnose and manage atresia conditions promptly to optimize outcomes and reduce mortality. Specific survival rates can only be determined on a case-by-case basis.


What Are the Risk Factors Associated With Atresia?

The following are the risk factors associated with atresia.
- Abnormal pregnancy history.
- Genital system infection.
- Teratogenic drugs.
- Folic acid supplementation.
- Paternal chronic conditions history of exposure to indoor decoration.
- Paternal occupational exposure to noise.
- Maternal acute respiratory infection.


What Is the Hearing Investigation for Atresia?

The most commonly used investigation is the audiogram, X-rays, and computed tomography (CT scan). A complete audiogram is the best suitable for investigation, and it involves testing certain parameters:
- Bone Conduction.
- Air Conduction.
- Immittance Testing.
- Speech Discrimination Testing.


Can a Person Have Atresia Without Microtia?

Atresia is defined as the absence or underdevelopment of the ear or ear canal or structures of the middle ear. Microtia is almost always accompanied by atresia because the outer ear and the middle ear develop around the same time as fetal development. 


At What Age Does Atresia Seen?

Congenital aural atresia is referred to as the absence or an underdeveloped ear or ear canal. It may be seen during the first few days in a newborn, and acquired atresia can occur at any age of a person.


How to Treat Atresia?

Treatment options for atresia include:
- Surgery to build a new ear using the rib cartilage.
- Surgery to implant an ear made of special plastic.
- Wearing a removable (prosthetic) ear.
- Leaving the ear as it is.


What Are the Clinical Features of Atresia in Newborns?

The following are the main clinical features of atresia in newborns.
- Trapped skin in the small ear canal.
- Slowed speech and language development.
Source Article IclonSourcesSource Article Arrow
Dr. Arpit Varshney
Dr. Arpit Varshney

General Medicine


congenital abnormalities
Community Banner Mobile
By subscribing, I agree to iCliniq's Terms & Privacy Policy.

Source Article ArrowMost popular articles

Ask your health query to a doctor online

General Medicine

*guaranteed answer within 4 hours

Disclaimer: No content published on this website is intended to be a substitute for professional medical diagnosis, advice or treatment by a trained physician. Seek advice from your physician or other qualified healthcare providers with questions you may have regarding your symptoms and medical condition for a complete medical diagnosis. Do not delay or disregard seeking professional medical advice because of something you have read on this website. Read our Editorial Process to know how we create content for health articles and queries.

This website uses cookies to ensure you get the best experience on our website. iCliniq privacy policy