What is anotia

Anotia is characterized by total absence of the ear, and it is exceptionally rare. This condition can affect one or both ears, though it is more common to only have one missing ear. Anotia is also associated with conductive hearing loss, which occurs when sound waves do not travel well through the ear and sound is not conducted efficiently from the outer ear canal to the eardrum. Otologic assessment of middle and internal ear structures will determine the best options to restore (bring back) hearing.

Causes

The cause of anotia is unknown. Up to 40 percent of patients may have an associated syndrome such as Treacher Collins or Goldenhar syndrome. Some recent findings suggest that women who have diabetes before they become pregnant and those with a diet lower in carbohydrates and folic acid during pregnancy may be at an increased risk of having a baby with anotia or microtia (underdeveloped external ears). Anotia usually occurs during the first few weeks of pregnancy when the baby is still developing.

Evaluation and diagnosis

Anotia is typically diagnosed at birth with a physical examination. Prenatal ultrasounds may assist with early diagnosis.

If your child’s external ears did not develop normally, your doctor may also want to test for other problems that may have happened at the same time during prenatal development. The external ear begins to develop around the 5th week of gestation, which is the same time kidneys develop. If your child is diagnosed with anotia, a renal ultrasound may be recommended to rule out any kidney abnormalities.

Treatment

Anotia is best treated by a multidisciplinary team of specialists who are experienced in treating this condition. This may include a plastic surgeon for external ear reconstruction, an otolaryngologist for inner ear and hearing treatment, and a speech pathologist for speech treatment.

Standard treatment for a missing ear is total ear reconstruction. In most cases of anotia, your child’s doctor will use a piece of your child’s own rib cartilage to create a framework to reconstruct the outer ear. Alternatively, a pre-constructed framework made from plastic (porous polyethylene) may be used instead of the rib cartilage in select cases.

The treatment of anotia is very similar to the treatment of microtia (an underdeveloped external ear). The main difference is that if your child’s ear is completely missing, she will also need to have an earlobe constructed. The same framework of rib cartilage used to reconstruct the ear will be used to construct an earlobe in patients with anotia.

Ear reconstruction procedures are generally performed between 6 and 10 years of age to allow for adequate growth of the child’s rib cartilage. The procedure may be performed earlier (as early as 3 years of age) if using the prefabricated framework.

If middle ear development is affected with resultant hearing loss, a Bone Anchored Hearing Aid (BAHA) device may be implemented. The BAHA is surgically implanted onto the skull. Sound waves are conducted through the skull bone, which allows for some hearing. An ear canal may also be surgically created to assist in optimal hearing. This is usually done after reconstruction of the external ear.

Long-term outlook

If an isolated condition, children with anotia can lead normal lives. In cases where anotia is associated with a genetic syndrome, your child should receive coordinated care from specialists with experience in treating specific issues related to the syndrome.

Because of the visible differences between themselves and other children, your child may experience issues with self-esteem. Patient and parent support groups may provide comfort and helpful resources for families with children with congenital anomalies of the head and neck. At The Children’s Hospital of Philadelphia, our dedicated psychologists and social workers can connect you and your child with the supportive resources you may need.