Ear Deformities | The Children's Hospital of Philadelphia

Plastic and Reconstructive Surgery

Ear Deformities

Protruding Ears

Ears that stick out more than 2 cm from the side of the head are considered to be prominent or protruding. In most people this is due to lack of formation of the antihelical fold, which makes the helix stick out. Additionally, most people with protruding ears have a deep concha, which pushes the entire ear away from the side of the head.

Treatment Approach

There are both non-surgical and surgical options for treating protruding ears.

Ear molding

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As early as 1984 it was realized that the cartilage of newborn ears is softer than normal due to maternal estrogen, and deformed ears can be molded into a normal shape during infancy. After several weeks to months, the cartilage stiffens and maintains its new shape.

This technique has been applied to a variety of congenital ear deformities, including protruding ears, with excellent results. If ear molding is started early enough (ideally in the first week of life), the expense and morbidity of an operation can often be avoided. Results are less successful if treatment is delayed longer than 3 weeks as maternal estrogen levels fall to normal by 6 weeks of age.

Ear molding is a treatment option that is available to our patients if they are referred to us early enough while their ears are still soft. Using a combination of a commercially available ear molding system and orthodontic molding materials, non-operative correction of protruding ears is possible.

Otoplasty

For ears that are already protruding and too stiff for molding, surgical correction is an option. It may be preferable to wait until about age 6, when ears are almost fully grown. This coincides with the age when children typically begin to receive teasing from their peers, which provides additional incentive for them to undergo surgery. The operation is usually performed through an incision behind the ears. A combination of cartilage scoring and suturing is used to create an antihelical fold. Additional sutures on the back of the conchal cartilage bring the entire ear closer to the side of the head. Although a general anesthetic is needed, the operation is done on an outpatient basis.

Parents should be aware that insurance companies consider otoplasty to be a cosmetic operation, and therefore they will often not cover the fees associated with this procedure. 
 

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Constricted Ears

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This term applies to a variety of ear deformities where the helical rim is either folded over (also called lop ear), wrinkled, or tight. Newborns with constricted ear deformities may respond to non-operative molding while their ear cartilage is still soft (see ear molding for protruding ears).

Once the cartilage has stiffened, operative correction is necessary to correct the deformity. Depending upon the degree of distortion, it may be possible to work with the ear cartilage already present to achieve a better contour. In more severe cases of constricted ears, a rib cartilage graft may be necessary to replace wrinkled native helical cartilage. If there is a tight band of skin that pulls the helix down, it may be necessary to add additional skin and cartilage to the native ear to match the other side. Some patients have bilateral constricted ears, in which case a decision can be made whether to repair both simultaneously, or in stages.

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Microtia

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Ears are like flower buds that blossom in utero. If ears fail to fully form and are smaller than normal, patients are diagnosed with an ear deformity called microtia.

  • Microtia occurs in 1 out of every 6,000 births
  • Occurs twice as often in males
  • Most often appears on the right side of the head (twice as often as the left)
  • Up to 20 percent of patients with microtia have it on both sides

Microtia can occur all by itself, or it may occur with syndromes such a hemifacial microsomia, where the entire half of the face is smaller, or with Treacher-Collins syndrome, a hereditary craniofacial abnormality. Children with microtia should have a kidney ultrasound to rule out possible deformities, since the urinary system forms at the same time as the ear during prenatal development.

Patients with microtia will also undergo an evaluation by the Division of Otolaryngology, also known as Ear, Nose and Throat (ENT). This is important to assess hearing status, as there may be varying degrees of middle ear development. Your child’s team of physicians will make recommendations regarding the need for hearing aids or surgical options to restore hearing.

Most patients with microtia have an earlobe, but they may lack any other recognizable ear elements. Some will have a conchal cavity and some may have an ear canal.

Treatment Approach

The timing of ear reconstruction depends upon the surgeon and the method of ear reconstruction. There are several methods and techniques currently used.

  • The majority of surgeons use rib cartilage for the ear framework and perform the surgical procedure when the patient is at least 6 years of age, when the opposite ear is close to adult size and the ribs will provide enough material to carve a framework of adequate size.
  • Another technique (also known as the Nagata method) that is increasing in popularity requires additional cartilage, and children should be closer to 10 years of age to ensure adequate donor cartilage. The cartilage framework is usually placed under the skin and merged with the pre-existing earlobe.
  • There is a less popular technique that uses an artificial polyethylene framework, a flap of tissue from the side of the head that lies just below the scalp, and a skin graft. No donor rib cartilage is needed, which avoids the discomfort of rib harvest and allows the operation to be done at an earlier age, but concerns about potential framework exposure and contamination have led most surgeons to prefer the time-tested cartilage framework.

Microtia reconstruction is a staged operation that also requires later separation of the ear from the side of the head and placement of a skin graft. Additional stages are often desirable to add additional definition to the final ear shape.

It is also important for parents to realize that reconstructed ears are never as detailed and delicate as normal ears. The goal is to create an ear shape that is realistic enough that it does not attract attention to itself.

Artificial ears attached by adhesives or bone implants are rarely suitable for children, as the prostheses are delicate and expensive. They tend to be better suited for adults with acquired ear loss, as they lead a less active lifestyle and are less likely to damage the prostheses.

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Cryptotia

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Cryptotia refers to an ear cartilage framework that is partially buried beneath the skin on the side of the head. The upper portion of the ear is hidden, and there may be some malformations of the cartilage of the upper ear (scapha and superior and inferior crura).

Ear molding may provide sufficient correction to avoid the need for surgery, and unlike other congenital ear deformities, molding may be successful well beyond the neonatal period. There are reports of successful non-operative correction even up to 5 years of age, possibly because these ears have a normal cartilage framework and require mostly skin expansion.

Surgical correction of cryptotia may require the use of local skin flaps or skin grafts to elevate the ear framework from the side of the head. Although a general anesthetic is needed, the operation is done on an outpatient basis.

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Anotia

Anotia is characterized by total absence of the ear, and it is exceptionally rare. Up to 40 percent of patients may have an associated syndrome. Otologic assessment of middle and internal ear structures will determine the best options to restore hearing.

Options for total ear reconstruction are similar to microtia, with the difference being the need to create a framework that includes the earlobe. Patients with microtia have an earlobe that requires repositioning, but not total reconstruction. The external ear begins to develop around the 5th week of gestation, which is the same time kidneys develop; therefore a renal ultrasound is advisable to rule out any kidney abnormalities.

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Stahl’s ear

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A Stahl’s ear deformity consists of a pointy ear shape and an extra cartilage fold (crus) in the scapha portion of the ear. It is sometimes also called a Spock’s ear, in reference to Leonard Nimoy’s character in the Star Trek television show and movies.

Ear molding in the neonate may correct this deformity and avoid the need for surgery. In children, surgical correction is necessary to correct the deformity and involves reshaping and suturing the cartilage to try to reverse the pointed shape. Although a general anesthetic is needed, the operation is done on an outpatient basis.

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Ear tags

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Also known as an accessory tragus or a branchial cleft remnant, ear tags are usually located in front of the ear or on the cheek, and consist of skin and cartilage. Many tags have a narrow stalk (a pedunculated tag), and some pediatricians may tie a string around the tag to intentionally “strangle” it.

Because most tags have cartilage within the stalk, it is preferable to make a small incision around the base of the tag, and transect the cartilage stalk a couple of millimeters below the surface of the skin to avoid a small cartilage bump. Although children will have a small scar, this is the preferable treatment of a pedunculated tag. Fortunately, excision is curative, and these tags do not grow back after surgery.

Infants with a simple pedunculated tag may be candidates for excision during their visit to the doctor’s office, using a local anesthetic. If multiple tags or complex-shaped tags exist, removal under a general anesthetic is preferable. Infants should be about 6 months of age in order to decrease the risk of apnea following general anesthesia.

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Earlobe deformities

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Congenital earlobe deformities come in a variety of shapes, including earlobes with clefts, duplicate earlobes, and earlobes with skin tags.

Surgical options include merging segments of the lobule into a single earlobe, removal of duplicate lobules, local skin flaps to add more tissue to the lobule, and removal of skin tags.

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Traumatic ear deformities

Traumatic lacerations, tears, and bite injuries are most often treated in an emergency room setting, and then followed in the office to ensure adequate healing and ear contour.

Debridement of unhealthy tissue, irrigation of the wound, and careful closure of the skin and torn cartilage will usually result in a satisfactory shape. Completely avulsed ear parts or total ear amputation may require a range of complex treatments that can include debridement and closure of the residual wound, skin grafting to cover any exposed cartilage, removal of the skin from the detached part and temporary cartilage “banking” elsewhere in the subcutaneous layer of the body to be used later, and attempted replantation using microsurgical techniques to restore circulation to the severed part.

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Split earlobes

Most split earlobes occur gradually due to large or heavy earrings. The skin heals as the hole slowly enlarges. Repair requires excision of the defect to freshen up the edges followed by careful suture repair. Complete and near-complete splits require a pie-wedge excision. Small splits do not always require a complete cut through the earlobe.

Some surgeons will try to save the pierce hole, while others will completely repair the ear and re-pierce it after a couple months. Repair is usually done under local anesthesia in the office. Patients should understand that a repair always leaves a permanent scar.

Intentionally dilated earlobe holes using progressively larger earrings are challenging to reconstruct. Repair involves excision of the edges of the defect and careful rearrangement of the residual earlobe tissue to form a more conventional contour. This always leaves permanent surgical scars.

A split earlobe from an earring that has suddenly pulled through can be repaired immediately because the edges are still raw. For example, this may occur with a mother whose baby has grabbed her earring.

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Cauliflower ears

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Normally ear cartilage is covered by a thin adherent layer called the perichondrium, which makes cartilage. Traumatic sheering forces can cause the perichondrium to separate from the underlying cartilage, resulting in bleeding (hematoma) or formation of a chronic fluid pocket (seroma).

If the perichondrium remains separated, it will begin to form abnormal cartilage on top of the normal cartilage, resulting in bulky misshapen ears. Ear hematomas and seromas should be drained immediately and compressed to permit the perichondrium to re-adhere to the cartilage.

Longstanding cauliflower ear deformities require operative removal of the abnormal cartilage and postoperative compression, drain placement, and/or quilting sutures to optimize the result. The deformity is commonly seen in wrestlers, and it may be advisable to postpone operative correction until the wrestling season or the patient’s wrestling career is over.

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Ear keloids

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Ear keloids are caused by excessive scar tissue formation after minor trauma, most commonly after ear piercing. They can occur in earlobes as well as from pierces that pass through skin and cartilage higher along the ear. It is unclear why keloids occur more commonly in people with darker skin pigmentation.

Treatment Approach

Keloids are notoriously difficult to treat, as surgical excision may lead to recurrent keloid scar formation, sometimes even larger than the original keloid scar.

Small keloids may respond to a series of steroid injections, which can be done in the office.

Depending upon the age of the child and the size of the keloid, a surgical excision may be performed in the office procedure room using local anesthesia, or in the operating room under a general anesthetic. Large keloids are generally excised, as long as patients and their parents accept the risk that the keloids may recur.

To decrease the risk of recurrence, the surgeon may elect to inject the edges with steroid medication prior to suture closure, and patients may also benefit from the use of pressure earrings after the incisions are no longer tender. Additional steroid injections may be indicated if the scars begin to thicken in the months following surgery.

Three days of post-surgical radiation may also decrease the risk of ear keloid scars. Candidates for radiation are referred to the Radiation Oncology Department at the Hospital of the University of Pennsylvania to discuss the risks and benefits of short term radiation exposure.

Most recently, the use of liquid nitrogen to freeze ear keloids has been reported to have a high rate of success and low rate of recurrence. This technique was first trialed at the Hospital of the University of Pennsylvania in 2013. Families should speak with their plastic surgeon if they are interested in this potential treatment option.

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Ear hemangiomas

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Hemangiomas are the most common benign tumor of infancy, and they can occur anywhere on the body, including the external ear and the salivary gland in front of the ear (parotid gland). Ear hemangiomas will commonly cause distortion of the ear and can result in permanent external ear deformities. Parotid hemangiomas can compress the external ear canal and stretch the cheek skin in front of the ear.

Most hemangiomas appear within the first couple weeks of life and slowly enlarge during the first year. They stop growing when infants are about 6-12 months of age and then slowly shrink over an average of 5 years.

Treatment Approach

During the growth period, patients with ear hemangiomas may be candidates for laser therapy if the hemangioma is relatively small and flat. Because the laser penetrates only about a millimeter into the skin, thicker and deeper hemangiomas are less responsive to laser treatments.

Topical or oral beta blockers, such as Timolol gel and Propranolol, have largely replaced steroid therapy to suppress hemangioma growth. Patients with significant ear and facial hemangiomas are referred to CHOP’s Division of Dermatology for oral beta blocker therapy. Patients undergoing beta blocker therapy will be admitted to the hospital for monitoring during the first several days of treatment where they will be monitored for potential side effects, including low heart rate, low blood pressure and low blood sugar.

Residual ear deformities after hemangioma regression usually involve the excision of excess residual skin and fatty scar tissue. Residual red or purple pigmentation can be treated with laser therapy. 

 

Reviewed by: David W. Low, MD
March 2013

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