An auricular malformation is characterized by abnormalities in the shape of the auricle. It often does not represent a disease value as in the case of protruding ears. However, severe auricular malformations may be an accompanying symptom of a syndrome with other physical malformations.
What is an auricular malformation?
The term auricular malformation includes both mild abnormalities of the auricle, such as protruding ears, and severe malformations, in which the auricles may even be completely absent. Often, the abnormalities are not associated with hearing loss and in such cases do not have any pathological value. This is especially true for the so-called sail ears (protruding ears). In other cases, the malformations are more serious and sometimes associated with other physical malformations. A malformation with reduction of the ears is called microtia. High-grade microtia is very rare and occurs in 100 to 150 newborns per year in Germany. In most cases, there is an isolated auricular malformation. In approximately 20 to 30 percent of cases, it is a symptom of an underlying syndrome that is either genetic or caused by bleeding during pregnancy. Overall, auricular malformations are classified into three degrees of severity from first-degree dysplasia to third-degree dysplasia.
Causes
There are many causes of auricular malformations. Often, this abnormality does not represent a disease at all as in the case of protruding ears. In these cases, it is only an external feature, which, however, deviates from the norm by definition only in the social context. Only the psychological stress of being teased and ridiculed can cause those affected to have a surgical correction. Other deformities are more serious, especially when microtia are involved. In the majority of cases, the malformation is isolated and the exact cause is unknown. Sometimes a familial clustering is registered. Then a hereditary auricular malformation can be assumed. As has been established, the malformation is passed on to the offspring with varying penetrance. An autosomal dominant mode of inheritance is present. The different expression of the characteristics can have environmental but also congenital causes. In about 30 percent of cases, the auricular malformation occurs together with other malformations. Here, either diseases during pregnancy or genetic causes play a role. Auricular malformation occurs particularly in connection with two syndromes. One is the Goldenhar syndrome and the other is the Franceschetti syndrome. In Goldenhar syndrome, there is a unilateral malformation of one side of the face with auricular malformation, displaced chin to the affected side, malformed or missing eye, limited facial expression and hearing problems. The cause of this condition in the fetus is thought to be hemorrhage in the tissues that serve as the ears or jaws. Franceschetti syndrome, in turn, is a genetic disorder that also has other malformations.
Symptoms, complaints, and signs
Auricular malformations manifest themselves in a variety of deviations of the auricle. Often, it is only a matter of protruding ears (sail ears). We speak of sail ears when the angle from the auricle to the base of the ear is more than 30 percent. In extreme cases it can be up to 90 percent. Singe ears, however, do not have any disease value. However, they are considered an aesthetic problem. Those affected do not suffer from hearing disorders. However, due to teasing and ridicule, this misalignment of the ears often leads to psychological problems and social isolation. More serious, however, are the microtia. These are ear deformities with abnormally small ears. Hearing disorders usually only occur when both ears are affected by a microtia. Normal development of speech could be observed in unilaterally affected individuals. However, this is not true for bilateral auricular malformations. When auricular malformations occur as part of a syndrome, other parts of the body are also affected by malformations.
Diagnosis and course of the disease
Diagnosis of auricular malformations is mainly concerned with determining whether hearing disorders also occur. Furthermore, the causes of microtia are sought.Thus, the family history often provides evidence for a hereditary disorder. Thereby, the probability of its occurrence in further children can be estimated.
Complications
As a rule, auricular malformation does not result in any particular complications or a serious course of the disease. The disease itself does not have a negative impact on the health of the affected person, so there are usually no limitations in everyday life or in the life of the patient. However, most affected individuals suffer from teasing or bullying due to the auricular deformity. Children in particular may experience these factors, often becoming aggressive or irritable. Psychological complaints, moods or depression can also make themselves felt and additionally reduce the quality of life of the affected person. In most cases, an auricular malformation occurs together with other malformations on the body. The further course depends strongly on the cause of the disease. As a rule, the auricular malformation does not cause hearing difficulties or other restrictions in the life of the affected person. An auricular malformation can be corrected by surgery. This does not lead to complications either. The treatment itself is also not mandatory. The life expectancy of the affected person is usually unaffected by the auricular malformation.
When should you go to the doctor?
In a large number of cases, a doctor does not need to be consulted for an auricular malformation. For most of those affected, it is an optical blemish that has no disease value. If there are no other complaints, there is usually no need to consult a doctor. If there are limitations of the hearing ability or if there is pain as well as disturbances of the balance, a doctor is needed. There are changes or further diseases of the ear that need to be examined and clarified. Headaches, dizziness, gait unsteadiness or abnormalities of the skin appearance should also be presented to a doctor. If psychological disorders occur due to the auricular malformation, there is a need for action. In case of emotional or mental irregularities, a doctor should be consulted. Peculiarities of behavior, depressed mood or withdrawal from social life should be discussed with a doctor. Consultation with a physician is advised for vegetative problems, stress, or lowered mood.
Treatment and therapy
For first-degree dysplasia, noninvasive treatment should begin in newborns. The infant’s ear cartilage is still malleable during the first days of life. It can be well shaped with an auricle former in the period from the fifth to the seventh day of life, so that the malformation is corrected. If treatment begins later, success will not be as good due to the increasing stiffness of the auricles. Invasive surgical methods for ear correction can begin from the fourth to fifth year of the child’s life. The material used is the body’s own cartilage or plastic. Cartilage material from the ribs has proven best. In most cases, the surgical procedure consists of three steps, each of which is performed three months apart. In the first step, the malformed ear cartilage is removed and the rib cartilage is harvested. After preparation of the rudimentary skin, the auricular framework is formed from the rib cartilage and implanted. In a second step, the auricle is lifted off and an ear fold is formed. In a third operation, further corrections are made. However, sometimes this step is no longer necessary.
Outlook and prognosis
The prognosis for auricular malformations has not been very good. Unless hearing was impaired or extremely diminished, auricular malformation was considered a cosmetic problem at best. It was often covered up by longer hair. The only alternative was a plastic auricle prosthesis placed over the malformed auricle. In addition to audiological rehabilitation, however, cosmetic surgery has also contributed to successful improvement of the appearance in more recent times. The health insurance companies cover the costs for this if the affected person suffers from severe inferiority complexes and depression because of it. This is often the case with adolescents, for example.In the future, the outlook for patients with auricular malformations could be significantly improved. Chinese scientists have transplanted auricles into some affected children. The skin and cartilage tissue for the hearing organ were grown from the body’s own cells in the laboratory. The new auricles were then produced by 3D printer, and implanted in place of the malformed auricle. The risk of rejection was minimized by using the body’s own cells. However, it remains to be seen whether the surgical results, published in a medical journal with photographic evidence, will convince Western scientists. Most auricular malformations are already congenital. So they hit especially children and teenagers hard. Only a few auricular malformations occur in adults, for example, due to accidents or disfiguring diseases.
Prevention
Prevention from auricular malformation is not possible. Only a quick noninvasive intervention in the first days of life of an affected newborn can still correct the malformation in mild cases.
Follow-up
In most cases, the affected person has only a few and also limited measures of direct aftercare available to him or her in the case of an auricular malformation. Therefore, the affected person should ideally see a physician at a very early stage to prevent further occurrence of complications or discomfort. There can be no independent healing, so a doctor should be contacted at the first signs and symptoms. If affected individuals wish to have children, genetic testing and counseling is recommended to prevent the recurrence of the auricular malformation in the children. In many cases, this malformation can be alleviated by a minor surgical procedure. There are no particular complications or discomforts. After the surgery, regular check-ups by a doctor are still very useful. Since the auricular malformation can lead to severe psychological discomfort or depression, especially in children, they are in some cases dependent on the psychological support of their own family. In this case, the disease itself does not reduce the patient’s life expectancy, and no further aftercare measures are available.
This is what you can do yourself
In most cases, an auricular malformation does not have any disease value. It is an optical blemish to which the affected person is exposed. In everyday life, it can therefore be teasing, which in severe cases lead to psychological problems in the patient. In order to prevent this development, possibilities should be perceived which trigger an improvement of the well-being and are thus perceived as helpful for the affected person. With the help of various methods or techniques, the auricular malformation can be hidden. Wearing headgear or creating different hairstyles can hide the conspicuousness on the head well. By this measure, the ear malformation remains hidden and thus unnoticed by many fellow men. Comments, remarks or unpleasant looks of other people are reduced in this way. In dealing with people from the social environment, the affected person needs a healthy as well as stable self-confidence. The comments of others should not have any influence on the patient’s mental state. To promote this, relatives and partners help by building a sense of achievement and encouraging the personality of the affected person. In conversations, it should be conveyed that a person’s worth does not depend on their appearance. The focus should be on the abilities and competencies of the person affected, so that a strengthening of the inner forces takes place.