Ear Anomalies Program

Types of Ear Anomalies and Deformities

When we describe ear anomalies, we will often place them into two different categories, deformities and malformations. With deformities, the ear may be misshapen or deformed, but all of the key elements of the ear are present. With malformations, the ear may be smaller, missing key elements, contain excess tissue, or present with a combination of the three. The best treatment option typically depends on the type of anomaly.

Deformities

Prominent Ear Deformity (Protruding Ear)

• This deformity is best characterized as protruding or prominent ears with loss of normal ear definition. The standard treatment for this is surgical, known as an otoplasty or ear pinning. If this is recognized early on, ear molding can often treat this deformity. For older patients (typically no younger than five years of age), surgical intervention is necessary. This includes shaping the cartilage of the ear and setting (or pinning) the ears back so they are less prominent.

Constricted Ear Deformity (Lop or Cup Ear)

• This deformity is best characterized as a folding of the rim of the ear. There are varying degrees of severity, and the treatment options are often guided by such. If this is recognized early on, ear molding can treat this deformity. For older patients (typically no younger than five years of age) or for those with a more severe form of this ear, surgical intervention is necessary. This includes shaping the cartilage of the affected ear and or using cartilage and other tissue from the body to establish the desired shape of the ear.

Cryptotia Deformity (Buried Ear)

• This deformity is best characterized as a buried ear, usually affecting the upper third of the ear. While the upper portion of the ear may look absent, it is actually buried under the skin of the scalp at or within the hairline. Mild forms of this deformity can sometimes be treated with ear molding; however, most forms will require surgery. For older patients (typically no younger than five years of age) or for those with a more severe form of this ear, surgical intervention is necessary. This often includes release of the ear from the overlying skin and skin grafting.

Stahl’s Ear Deformity

• This deformity is best described as an extra cartilage fold in the upper part of the ear. It can sometimes be associated with a prominent point along the upper half of the ear (i.e. Spock’s ear deformity). This is sometimes associated with prominent ears as well. If this is recognized early on, ear molding can often treat this deformity. For older patients (typically no younger than five years of age), surgical intervention is necessary. This includes shaping the cartilage of the ear or removing the extra fold.

Malformations

Microtia and Anotia Malformations

• Microtia is best characterized as a small, partially absent, or completely absent outer ear. Microtia can be associated with a very small (stenotic) or absent ear canal and inner ear abnormalities. When the outer ear and canal are completely absent, this is termed anotia—this particular condition is very rare. Microtia and anotia can affect one or both sides, though only having one side affected is more common.

Ear Tag and Pit Malformations (Accessory Tragus, Accessory Auricle)

• This malformation often presents as an extra piece of skin with or without cartilage in front of the ear. These extra or accessory auricles may be associated with a syndrome and or kidney abnormalities. Surgery is required to remove these extra pieces of tissue.

Earlobe Malformations

• Clefting or duplication of the earlobe can occur in isolation or as part of a syndrome and can be associated with microtia. Like most malformations, surgery is required to correct the issue. Surgery involves making an incision, or few incisions, or rearrange the tissue and restore earlobe anatomy. If a duplication of the earlobe is present, removal of one accessory earlobe may be required.

Ear Keloids

• Keloids are severe and exaggerated scars that can occur after trauma or from an ear piercing. Surgical management is often necessary and involves excision with other means of scar prevention and management, such as compression therapy, silicone application, localized steroid therapy, and directed massage therapy.

Traumatic and Acquired Ear Deformities

• A variety of ear deformities can occur because of trauma or due to cancer. Reconstruction often uses the same principles as described above for common deformities and malformations. Common deformities treated include split ear lobes related to earring trauma, ear defects related to cancer, cauliflower ear (hematoma auris) related to direct and repetitive trauma from contact sports, ear burns, and ear avulsion injuries (outer ear being traumatically severed or removed, such as from a dog bite or car accident).

To determine which treatment option may be best for your child, please contact the CHOC Ear Anomalies Program for more information.

Why is it important to treat ear anomalies?

The term “ear anomaly” refers to any differences in the shape, size, location, or presence of the outer ear. It is estimated that ear anomalies of the outer ear and canal occur in as many as 1 out of 2000 births every year, making them a relatively common birth-related difference. There are many different types of ear anomalies and severity of the anomaly can vary from child to child. While such ear differences are not life-threatening, the consequences of having a small, misshapen, or “different” ear in a child can negatively impact their psychosocial health. At CHOC, this important aspect of care is taken into consideration when discussing treatment options for children with ear differences. Sometimes ear anomalies are associated with other health issues, such as kidney function. CHOC has access to leading experts in pediatric nephrology, urology, and many other highly specialized providers.

What treatment options are available for ear anomalies?

When a child is born with an ear anomaly, prompt evaluation by a specialist is necessary. The type of ear anomaly a child has will often dictate the best treatment option. There are two categories of treatment: Non-surgical ear molding and surgical reconstruction.

Non-Surgical Ear Molding

When an ear is misshapen (and there are no missing elements), non-surgical ear molding may be the best treatment option—this is the case for most ear deformities. This process works by slowly shaping the cartilage the child has into a more anatomic position.

For ear molding to work successful, it is necessary that the child be seen relatively soon after birth to determine if ear molding is an option. There is a small window of opportunity for ear molding to be effective, and that window typically closes by a few months of age. After this period, success with ear molding decreases. Common ear deformities treated with non-surgical ear molding include a misshapen rim, prominent ears, and constricted ears. At CHOC, we use the EarWell system and other means of non-surgical ear molding to achieve the best ear shape possible.

Surgical Reconstruction of the Ear

When the ear is underdeveloped or misshapen with missing elements, surgical intervention may be the best treatment option—this is typically the case for most malformations of the ear. The type of malformation will often dictate the best treatment option. This can range from simple excision of accessory ear tags to costal (rib) cartilage- or porous polyethelene (i.e. Medpor)-based reconstruction of a missing ear.

Autologous costal cartilage-based reconstruction is a typically multistage process. This is the most common approach to ear reconstruction and uses the patient’s own tissue to reconstruct or build the missing outer ear. This involves making an incision along the chest to access a portion of the costal cartilage, followed by sculpting the cartilage into the framework of an ear.

The new ear construct is then placed into position under the skin on the head to approximate the initial position of the new ear. Several months later, the ear is “elevated” into its final position to give it a more natural look. The result is a natural looking ear constructed from the patient’s own tissue. This surgery is typically done when the patient is between eight and ten years of age.

Porous polyethelene-based reconstruction is a one-stage process, reserved for special circumstances, that uses a combination of grafts and tissue flaps to reconstruct the ear.

This procedure does not require as much time as costal cartilage reconstruction and the result can look quite natural. However, the artificial implant will not grow or integrate as it is a foreign material. Reconstruction can be done earlier than eight years of age because it does not rely on the child’s costal cartilage to be of sufficient size; however, the implant will have to be slightly larger to account for future growth of the unaffected ear.

Current Research

The Center for Tissue Engineering, a highly productive laboratory within the Department of Plastic Surgery at UCI, is exploring various ways to create cartilage from cells (chondrogenesis). The ultimate goal is to create an “ear” from a patient’s own tissue that can be used as a framework for reconstruction. This is one of many exciting and innovative projects the Center for Tissue Engineering within the Department of Plastic Surgery is working on to better the lives of our patients.