Congenital Disorders of the Ear

Also known as: Congenital ear problems, congenital ear deformities, congenital ear anomalies, congenital ear malformations

What are congenital disorders of the ear?

Any problem with the development of ear that occurs while the fetus is still in the uterus is known as a congenital disorder of the ear.

There are three main parts of the ear; outer, middle and inner ear. Protruding or prominent ear or a small outer ear (ear microtia) are the two commonest ear deformities seen. Congenital ear problems occur more often in boys, more frequently on the right side (rather than on both sides) with a higher incidence in Japanese and Navajo Indian populations. Often there are middle ear problems associated with outer ear disorders. Usually the inner ear is unaffected as it has a separate development in the uterus. As other parts of the baby’s body (face, jaw, teeth and kidney) develop at the same time as the ears, these should be evaluated carefully.

What causes congenital disorders of the ear? 

The cause/s of congenital disorders of the ear is/are not entirely clear. Lack of blood supply to the fetus, exposure to certain medicines or toxins, viral infections, and/or  genetic mutations have all been implicated.

What are the symptoms of congenital disorders of the ear? 

Congenital ear disorders are usually visible at birth and appearance varies depending on the type of deformity. They include a range of ear deformities, facial deformities, hearing loss, crooked teeth, ear infections, ear tags, and other types of ear anomalies.

What are congenital disorders of the ear care options? 

All infants should have a hearing evaluation early. Some may not require any treatment if hearing is normal. Potential treatments include ear moldings, reconstructive surgery to restore the shape of the ear, hearing aids (and/or cochlear implants), and early speech evaluation and therapy.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: March 20, 2019 04:08 PM