Auricle malformation is characterized by anomalies in the shape of the auricle. It often does not represent a disease value, as is the case with protruding ears. However, severe pinna malformations can also be an accompanying symptom of a syndrome with other physical malformations.
What is an auricular malformation?
The term auricle malformation includes both slight anomalies of the auricle, such as protruding ears, and severe deformities, in which the auricles can even be missing completely. The deviations are often not associated with hearing loss and in such cases have no pathological significance. This applies in particular to the so-called protruding ears. In other cases, the deformities are more serious and sometimes associated with other physical deformities. See acronymmonster for Definition of Desbuquois Syndrome in English.
A malformation with a reduction in the size of the ears is referred to as microtia. A high-grade microtia is very rare and occurs in Germany in 100 to 150 newborns per year. There is usually an isolated auricle malformation. In about 20 to 30 percent of cases, it is a symptom of an underlying syndrome, which is either genetic or caused by bleeding during pregnancy. All in all, auricle malformations are divided into three degrees of severity from first-degree dysplasia to third-degree dysplasia.
The causes of auricle malformations are varied. This deviation often does not represent a disease at all, as is the case with protruding ears. In these cases, it is only an external feature, which, however, only deviates from the norm in the social context by definition. Only the psychological stress of being teased and ridiculed can prompt those affected to have surgical correction performed. Other malformations are more serious, especially when it comes to microtia.
In the majority of cases, it is an isolated malformation whose exact cause is not known. Familial clustering is sometimes recorded. Then a hereditary auricular malformation can be assumed. As has been established, the malformation is passed on to the offspring with variable penetrance. There is an autosomal dominant inheritance. The different expression of the characteristics can have environmental but also plant-related causes. In about 30 percent of cases, the auricle malformation occurs together with other malformations.
Either diseases during pregnancy or genetic causes play a role here. Auricle malformation occurs particularly in connection with two syndromes. One is Goldenhar Syndrome and the other is Franceschetti Syndrome. Goldenhar syndrome is a unilateral malformation of one half of the face with auricular malformation, a shifted chin to the affected side, a malformed or missing eye, restricted facial expressions and hearing problems. The cause of this disease in the fetus is suspected to be bleeding in the tissues that serve as the ear or jaw rudiments. Franceschetti syndrome is a genetic disease that also has other malformations.
Symptoms, Ailments & Signs
Auricle malformations are expressed in a variety of deviations of the auricle. Often it is just protruding ears (big ears). We speak of jug 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. However, the protruding ears have no disease value. However, they are considered an aesthetic problem. Those affected do not suffer from hearing disorders.
However, due to teasing and ridicule, this misaligned ear often leads to psychological problems and social isolation. However, the microtia are more serious. These are ear deformities with abnormally small ears. Hearing problems usually only occur when both ears are affected by microtia. Normal spoken language development was found in unilaterally affected individuals. However, this does not apply to bilateral auricle malformations. If the auricle malformations occur as part of a syndrome, other parts of the body are also affected by malformations.
Diagnosis & course of disease
When diagnosing auricular malformations, the main concern is to determine whether hearing disorders also occur. Furthermore, the causes of microtia are sought. The family history often provides clues for a hereditary disorder. The probability of their occurrence in other children can be estimated.
As a rule, the auricle malformation does not lead to any particular complications and does not lead to a serious course of the disease. The disease itself has no negative impact on the health of those affected, so that there are usually no restrictions in everyday life or in the patient’s life. However, most of those affected suffer from teasing or bullying due to the auricle malformation.
These factors can occur, especially in children, so that they are often aggressive or irritable. Psychological complaints, moods or depression can also make themselves felt and additionally reduce the quality of life of those affected. In most cases, an auricle malformation occurs together with other malformations of the body. The further course depends heavily on the cause of the disease.
As a rule, the auricle malformation does not lead to hearing problems or other restrictions in the life of the affected person. An auricular malformation can be corrected by surgery. There are no complications here either. The treatment itself is not mandatory. The life expectancy of those affected is usually unaffected by the auricle malformation.
When should you go to the doctor?
In a large number of cases, a doctor does not have to be consulted in the case of an auricle malformation. For most of those affected, it is an optical defect that has no disease value. If there are no other symptoms, there is usually no need to consult a doctor. If you have hearing impairments or if you experience pain or balance problems, you need to see a doctor. There are changes or other diseases of the ear that need to be examined and clarified.
Headaches, dizziness, unsteady gait or abnormalities of the complexion should also be presented to a doctor. If mental disorders occur as a result of the auricle malformation, there is a need for action. In the case of emotional or psychological irregularities, a doctor should be consulted. Peculiarities of behavior, a depressive mood or a withdrawal from social life should be discussed with a doctor. In the case of vegetative problems, stress or a low mood, it is advisable to consult a doctor.
Treatment & Therapy
Bei einer Dysplasie ersten Grades sollte eine nichtinvasive Behandlung bereits bei Neugeborenen beginnen. Der Ohrknorpel des Säuglings ist in den ersten Lebenstagen noch formbar. Er kann mit einem Ohrmuschelformer in der Zeit vom fünften bis zum siebten Lebenstag gut geformt werden, sodass die Fehlbildung korrigiert wird. Wenn die Behandlung später beginnt, werden aufgrund der zunehmenden Steifigkeit der Ohrmuscheln keine so guten Erfolge mehr erzielt.
Invasive surgical methods for correction of the auricle can begin when the child is four or five years old. The body’s own cartilage or plastic is used as the material. Cartilage material from the ribs has proven to be the best. The surgical procedure usually consists of three steps, which are carried out at intervals of three months. In a first step, the malformed ear cartilage is removed and the costal cartilage removed.
After preparation of the rudimentary skin, the auricle framework is formed from the costal cartilage and implanted. In a second step, the auricle is lifted and an ear fold is formed. In a third operation, further corrections are made. However, sometimes this step is no longer necessary.
Outlook & Forecast
The prognosis for auricle malformations has not been very good so far. Unless the ability to hear was impaired or severely impaired, a malformation of the pinnae was at best a cosmetic problem. It was often covered by longer hair. The only alternative to this was a plastic prosthetic ear that was placed over the malformed ear.
In addition to audiological rehabilitation, cosmetic operations have recently also contributed to the successful improvement of optics. The health insurance companies cover the costs if the person concerned suffers from severe inferiority complexes and depression . This is often the case with young people, for example.
In the future, the prospects for patients with auricle malformations could improve significantly. Chinese scientists have transplanted ear pinnae to some affected children. The skin and cartilage tissue for the hearing organ were grown in the laboratory from the body’s own cells. The new auricles were then produced using a 3D printer and implanted in place of the malformed auricle. The risk of rejection could be minimized by the body’s own cells.
It remains to be seen whether the surgical results published in a medical journal with photographic evidence will also convince western scientists. Most ear malformations are congenital. They hit children and young people especially hard. Only a few auricle malformations occur in adults, for example as a result of accidents or disfiguring diseases.
It is not possible to prevent an auricular malformation. In mild cases, the deformity can only be corrected with a quick, non-invasive procedure in the first few days of an affected newborn’s life.
In most cases, those affected with an auricle malformation only have a few and only limited measures of direct aftercare available. Therefore, ideally, the person affected should consult a doctor very early on in order to prevent further complications or symptoms from occurring. It cannot heal on its own, so a doctor should be contacted as soon as the first signs and symptoms appear.
If those affected have a desire to have children, a genetic examination and counseling is recommended in order to prevent the recurrence of the auricle malformation in the children. In many cases, this malformation can be alleviated with a minor surgical procedure. There are no particular complications or discomfort.
After the procedure, regular check-ups by a doctor are very useful. Since the auricle malformation can lead to severe psychological problems or depression, especially in children, they are dependent on the psychological support of their own families in some cases. The disease itself does not reduce the patient’s life expectancy, and no further follow-up measures are available.
You can do that yourself
In most cases, an auricular malformation is not considered to be pathological. It is an optical flaw that the person concerned sees himself exposed to. In everyday life, it can therefore lead to teasing, which in severe cases can lead to psychological problems in the patient. In order to prevent this development, opportunities should be taken that trigger an improvement in well-being and are therefore perceived as helpful for those affected.
The auricle malformation can be hidden using various methods or techniques. Wearing headgear or creating different hairstyles can hide the conspicuousness of the head well. As a result of this measure, the malformation of the ear remains hidden from many people and thus goes unnoticed. Comments, remarks or unpleasant looks from other people are reduced in this way.
When dealing with people from the social environment, the affected person needs a healthy and stable self-confidence. The statements of others should not affect the mental state of the patient. Relatives and partners help to promote this by creating a sense of achievement and promoting the personality of the person concerned. In conversations it should be conveyed that the value of a person does not depend on their appearance. The focus should be on the skills and competencies of the person concerned, so that the inner strength is strengthened.