Otoplasty Blogs

The ears develop during the first 4 weeks of fetal gestation.  If that process does not occur completely, then various forms of underdeveloped ears may be present at birth. One of these conditions is called anotia and reconstructive ear surgery (the most major form of otoplasty) can help.

What is Anotia?

Anotia is the most extreme form of microtia. Microtia literally means “little ear” and can be just that - a little ear - or it can refer to almost complete absence of the ear with the presence of only a nubbin of tissue where the ear would normally be. Anotia refers to the complete absence of any ear remnant at all. Microtia is quite common but anotia is extremely rare; there is almost always some evidence of an ear. These conditions most commonly affect one ear (90% of the time) and much less commonly affect both ears (10% of the time). When the outer, visible portion of the ear is underdeveloped, the ear canal and eardrum are usually absent and the middle ear is also usually underdeveloped, resulting in hearing loss on that side. 

The ears develop during the first 4 weeks of fetal gestation.  If that process does not occur completely, then various forms of underdeveloped ears may be present at birth. One of these conditions is called anotia and reconstructive ear surgery (the most major form of otoplasty) can help.

What is Anotia?

Anotia is the most extreme form of microtia. Microtia literally means “little ear” and can be just that - a little ear - or it can refer to almost complete absence of the ear with the presence of only a nubbin of tissue where the ear would normally be. Anotia refers to the complete absence of any ear remnant at all. Microtia is quite common but anotia is extremely rare; there is almost always some evidence of an ear. These conditions most commonly affect one ear (90% of the time) and much less commonly affect both ears (10% of the time). When the outer, visible portion of the ear is underdeveloped, the ear canal and eardrum are usually absent and the middle ear is also usually underdeveloped, resulting in hearing loss on that side. 

What are the causes?

To this date, there is no known cause of microtia or anotia. Sometimes, microtia (including anotia) may occur in association with a syndrome such as:

Hemifacial Microsomia

This is a congenital condition where one side of the face (including the ears, facial bones, facial nerve, and soft tissue) is underdeveloped. Like microtia, this usually affects only one side of the face but can sometimes affect both. 

Goldenhar Syndrome

This is a syndrome that has all the components of Hemifacial Microsomia but is also associated with benign tumors on the eye called dermoids and abnormalities of the bones in the neck. 

Treacher Collins Syndrome

This is a condition that involves underdevelopment of both sides of the face with a distinctive pattern including underdevelopment of the cheekbones and lower eyelids, with a characteristic facial appearance. Once Treacher Collins syndrome is in the family, then 50% of that patient’s children will be affected. 

How is anotia or microtia treated?

These conditions are treated by a multidisciplinary team of experts that includes a plastic surgeon for the reconstruction of the external ear or auricle. Dr. Thorne has over 30 years of experience in this type of Pediatric ear reconstruction. 

For those patients who are missing all or most of an ear, surgical reconstruction is best performed at about 10 years of age. Older techniques involved surgical correction at a younger age but these have largely been replaced. The techniques for ear reconstruction involve using either a framework of cartilage taken from the patient’s own ribs or a framework of artificial material (polyethylene). The cartilage technique has fewer complications but is more difficult that the technique that uses artificial material. Dr. Thorne makes use of otoplasty and ear reconstruction techniques that will best suit the unique nature of the particular patient.

If you would like to learn more about microtia and anotia and the surgical techniques involved in reconstructive otoplasty, schedule an appointment with us. Dr. Thorne is Chairman of the Department of Plastic Surgery at Lenox Hill Hospital in New York City.