What is Anotia?

Anotia is a very rare congenital anomaly in which the external part of the ear is entirely absent. It is difficult to determine the incidence of anotia, because this birth defect is often bundled in with microtia, in which part of the ear is missing, for the purpose of keeping statistics. There are no obvious risk factors for anotia, and sometimes it may occur as part of a congenital condition or syndrome, in which a variety of other anomalies may present themselves as well.

In true anotia, where the auricle of the ear is missing, the patient is usually diagnosed very soon after birth. A doctor may recommend follow up exams to check the baby's hearing, because anotia is sometimes associated with hearing damage. Surgery to reconstruct the ear is recommended between the ages of five and seven, after the child has had a chance to mature slightly, allowing the reconstructive plastic surgeon who performs the surgery to get a close match to the existing ear.

A series of surgeries are needed for reconstruction of the ear. The surgeon takes grafts of cartilage from the ribs to build up the body of the ear, and grafts of skin to cover the ear. In a series of surgeries, the grafts will be encouraged to grow and sculpted by the surgeon to take on a realistic appearance. Once the surgeries are complete, the patient should have a very realistic replacement for the missing ear.

Anotia and microtia both are caused by problems with the development of the ear in the fetal state. Signals get mixed, growth fails to happen, and the ear remains absent instead of developing, or does not develop normally, leading to microtia. This can be related to environmental exposure, as for example in the cases of babies born to mothers who took thalidomide during pregnancy, and sometimes a genetic condition can lead to anotia. In other cases, there are no clear causes, and something simply went awry during the development of the baby.

When selecting a surgeon to reconstruct the missing or malformed ear, it is a good idea to seek out a surgeon who has experience with patients who have anotia or microtia. Patients may also find it helpful to seek treatment at a hospital which specializes in caring for children with congenital conditions. A medical team can evaluate the child for any other signs of problems, providing prompt intervention while also addressing the missing ear.