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Atresia of the ear canal

Complete or partial absence of the ear canal. It often occurs in conjunction with microtia — the underdevelopment of the external ear.
Despite the fact that atresia is an extremely rare congenital pathology, children who need help may number in the hundreds.

Anatomy of the human ear

 Anatomy of the human ear
Anatomy of the human ear
The ear consists of three parts: outer, middle, inner. The outer ear, which includes the pinna and the ear canal, accumulates sounds coming from the external environment and directs them to the tympanic membrane. The eardrum is directly connected to the chain of the auditory bones of the middle ear, to which it transmits the incoming sound. Next, the sound for processing is sent to the inner ear. Well hearing person recognizes the sound volume from 0 to 10 dB.

What happens in atresia of the ear canal?

Atresia translated from the medical language means absence. In this case, there is no ear canal, partially or completely. The sound coming from the outside environment has no chance to reach the bone structures of the middle ear. The ear doesn’t take part in the process of listening.

In some cases, atresia of the ear canal is combined with microtia (underdevelopment of the ear) or various anomalies of the middle ear (size reduction of the tympanic cavity, deformation of the ear bones)

What are the causes of atresia of the ear canal?

The exact causes of intrauterine underdevelopment of the ear canal are not fully known. However, a connection has been stated with a small weight of the child at birth, prenatal trauma, intoxication, infections.


Statistics of the disease


  • atresia of the ear canal occurs in 1 out of 10 000−20 000

  • unilateral occurs 3−6 times more often than bilateral

  • right ear > left ear

  • in family anamnesis 14% patients

  • in 75% of cases, atresia is accompanied by underdevelopment of the ear (microtia)

  • if the atresia of the ear canal is not accompanied by microtia, the identification occurs on average at the age of 2.5 years

What happens with the hearing?

We can hear sounds in two ways:

  1. By air. When we talk with friends, in the classroom, when we move in an urban environment, the sounds are captured by the ear, go into the ear canal and reach the bones of the middle ear.

  2. By bone. Remember the feeling when you can definitely say that the train has gone under the ground. You also heard it, not by the ear, but by the bone.

We can find loads of examples for the first way. However, it is hard to find the examples for the second one. The main way of getting the sound is by air. People with atresia of ear canal do not have such possibility.


What does it look like in testing of hearing?

Under normal conditions a person hears sounds with a volume of 0−10 DB, whereas with atresia of the ear canal only from 50 DB.

Move the slider on the image to see the difference in the hearing threshold.

Classification of atresia of the ear canal by Schuknecht




  • Type A: atresia only of the cartilaginous part of the ear canal

  • Type B: partial atresia with narrowing of the bone and cartilaginous parts of the ear canal

  • Type C: complete atresia with the absence of the bone part of the canal, malformation of the bone system of the middle ear, absence of the ear drum

  • Type D: the same as type C, but with no pneumatization of the temporal bone

Treatment

A well-known BAHA treatment is based on preserved bone conduction in the diseased ear. However, it is not so effective as surgical treatment, which repairs completely the way in which sounds reach the middle and inner ear. Dr.Ashesh Bhumkar simultaneously repairs the ear canal, the pinna in case of deformation and reconstructs the bones of the middle ear in their absence or inability to perform their functions properly.
Ashesh Bhumkar
ENT surgeon, Mumbai, India

What do we need the binaural hearing for?

Sound localization

When the sound goes at different angles, the brain measures the time difference which the sound needs to reach each ear. For instance, the sound from the right registers in the right ear before it reaches the left ear. In the same way that the vision loss in one eye eliminates depth perception, the loss of hearing in one ear eliminates the equivalent of hearing, which is sound localization. A person’s deficiency of unilateral atresia and microtia is getting obvious on the playground or in the park: when the child hears his name, he will be forced to scan the environment with his vision to find the source of the sound.

Binaural summation

Our brain combines inputs from both ears to create a basic perception better than separate signals.

Hearing in noise

Every day we have to listen to the noise in various forms and choose the information needed. Without the full functioning of two ears, the child will have hearing problems even in a small amount of background noise.

Sometimes binaural people who hear with two ears cannot distinguish speech from noise. It is called auditory neuropathy. If a person has hearing impairment, it develops into functional auditory neuropathy.

Skills mastering

It has been proved that children with partial hearing loss develop worse than their peers, have a lower verbal IQ, learn worse.

Cognitive load

Understanding speech in a noisy environment is often hard for people with normal hearing. The louder the background noise is, the harder it is for a person to complete the task. Children with hearing impairment often experience a "cognitive load", trying to catch the necessary information from the flow of noise.

Why do we need two ears or why is surgery needed if there is 50% of hearing?

Even in the last century, doctors believed that if one ear hears, then nothing can prevent the development of the functions of speech and language, but the latest research has been argued against this belief. Unilateral hearing loss among children creates a number of functional problems in development. For instance, a child with partial hearing impairment behaves quite well at home in a calm environment, but the same child may have great difficulties in a noisy classroom. Even if teachers, parents and experts believe that today a child with hearing impairment does not experience this problem, do not forget that the unrecoverable unilateral hearing loss can have a significant influence on the health and full person’s functioning in the near future.

The arguments supporting the importance of binaural hearing are definitely clear. Objective evidence show that children with unilateral hearing loss have serious problems in a noisy environment; they have an incorrect speech development. The brain system, which provides hearing and speech functions, develops in the first 5−10 years of life. This fact forces parents to decide on the hearing repair in childhood at an early age. It often happens that if the hearing is not developed in the first years of life, the brain cannot fully develop later, which limits the child in the future. These handicaps may be evident in primary school. With unilateral hearing loss, the child can hear, but only in a very quiet environment. In a noisy class, child’s hearing and productivity may be reduced by almost 50%. Children with unilateral hearing loss are 10 times more likely to have problems with behavior than their binaural peers.

Impex LIFE target

Our target: to return the child full binaural hearing as soon as possible.

Doctors used to be taught that unilateral deafness did not require treatment, but studies have argued against it. Treatment is not just necessary — it is obligatory to give a chance to a person to have a normal productive life.

All situations are different, and they should be discussed individually by professional highly experienced doctors in the field of atresia and microtia.

What does Impex LIFE offer

We offer highly qualified treatment by the best doctors in the world in the field of atresia and microtia. Our team of doctors is created to achieve the best results for the patient. Specialized medical consultations are carried out in Moscow based modern clinics equipped with high-tech equipment. Consultations are held under the supervision of Professor Ashesh Bhumkar (India). He is assisted by experienced surgeons from India and Russia, anesthesiologists, nurses.
Natalia Chuchueva

Assistant Professor, Department of ear, nose and throat disease, Sechenov University, Moscow.
ENT-specialist, health centre "Barviha" Presidential Affairs.

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