Microtia is a malformation of the outer ear that is congenital. In this case, the outer ear is not fully formed. Sometimes the ear canal is only very small or completely absent. Reconstruction of the ear and surgery to improve hearing are possible treatments.
What is microtia?
The malformation of the outer ear is congenital. Due to incomplete development of the ear, minor deformities may occur or the ear canal may be absent altogether. The term microtia is derived from the translation “small ear.” A cartilage plate completely separates the outer ear from the inner ear, which subsequently results in a significant limitation of hearing. Microtia can occur either on only one side of the head or on both sides. In many cases, underdevelopment of the lower jaw occurs simultaneously with microtia. microtia is one of the largest manifestations of congenital ear defects. Microtia is classified according to four stages:
- Grade 1 describes a minimal malformation in which most of the ear has normal anatomy.
- Grade 2 has visually normal auricular formation, especially in the lower part. However, the ear canal may be different, smaller or completely closed.
- In grade 3, the ear has a shape similar to the peanut and has no ear canal.
- In grade 4, the outer ear and the auditory canal are completely absent.
Causes
The causes of microtia is probably not entirely clear. Both genetic factors and environmental influences could play a role in this congenital malformation. However, genetics is likely to be actually responsible in only five percent of patients. Vascular disorders that occurred during fetal development are also possible, but this is unlikely to be clearly proven. Possible causes of microtia are likely to be related to the consumption of coffee, alcohol or drug abuse during pregnancy – especially in the first trimester. Medications could also be possible triggers for this malformation. The frequency of occurrence of microtia is more common among certain peoples such as Asians or inhabitants from the Andes. The odds of having a child born with microtia are 1 in 6000 and 12,000 births, respectively.
Symptoms, complaints, and signs
Microtia is found on both sides in ten percent of affected individuals, called bilateral microtia. The incidence of this congenital anomaly is usually found more often on the right side of the head and usually forms on only one side. The main complaint is hearing loss due to the absence of a normal ear canal, eardrum and ossicles. Affected individuals with microtia still hear some sounds, but not through the ear canal. In microtia, the pinna is missing, and sometimes an earlobe with a remnant of the pinna is present.
Diagnosis and course of the disease
Human genetics should be consulted for clarification in cases of microtia occurring more frequently in families. Early clarification by specialists such as otolaryngologists, pediatricians, and phoniatrists and pediatric audiologists is also recommended. Possible negative influences during early pregnancy should be clarified. From the age of 10, a computer tomography can provide more precise information about the middle ear structures. Already in early childhood a diagnosis of the malformation is recommended. Suitable methods for clarification here are regular hearing tests and computed tomography to detect middle ear defects.
Complications
Due to microtia, patients primarily experience hearing difficulties. Due to the defective formation of the auricle, this may result in limited hearing or complete deafness. However, the patient’s life expectancy is not reduced or limited by this complaint. Young people or small children in particular can develop severe psychological complaints or depression due to the microtia. Those affected can only hear certain sounds and suffer from significant limitations in their daily lives. In children, microtia can also lead to developmental disorders, so that development is slowed down, resulting in consequential damage in adulthood. In some cases, microtia can also lead to balance disorders.The symptoms of the disease can be relatively well limited and solved with the help of implants and hearing aids. There are no particular complications or discomforts. However, parents need to strengthen their children’s self-esteem to avoid inferiority complexes. Hearing complaints can be further reduced by surgical intervention. Again, there are no special complications.
When should you go to the doctor?
A severe microtia is usually diagnosed immediately after birth or in the first months of the infant’s life. In principle, this malformation of the ear is not dangerous unless it is part of a syndrome. In the majority of cases this is not the case. Nevertheless, it would be advisable to consult the pediatrician or a specialist. Depending on the severity and form of the microtia, it can significantly limit the child’s hearing. If left untreated, it can result in speech deficits and subsequent damage in adulthood. For this reason, it is advisable to have a hearing test performed by an ENT specialist as early as possible. A doctor should definitely be consulted if there are also balance disorders. Surgical correction of microtia and insertion of a bone conduction hearing aid can be performed from the age of four or five at the earliest, as these procedures require a certain level of physical maturity. However, provided that only a mild form of microtia is present and the child is not limited by it, there is no need for medical treatment. In some cases, the affected person may develop psychological complaints such as inferiority complexes or depression due to microtia, especially in childhood and adolescence. In this case, it is recommended to seek therapeutic help.
Treatment and therapy
Due to the hearing loss associated with microtia, treatment by pediatric audiologists and otolaryngologists is helpful. The existing hearing loss must be continuously clarified and treated accordingly. With a so-called otoplasty, the missing auricle can be reconstructed from the body’s own tissue. The normal hearing ear also needs consistent control. If a conductive or sensorineural hearing loss occurs, action is required. In the case of bilateral malformation due to microtia, the child can be fitted with a bone conduction hearing aid. Surgery to correct the malformation should not be performed until the child is four to five years old at the earliest, in order to have the necessary physical maturity for this procedure. Alternatively, in the case of microtia, an implant can be placed to avoid the invasive procedure by surgery. Since microtia is not as obvious as defects in the face, the psychological burden is not necessarily great. However, reconstruction of the ear is an improvement in the quality of life of those affected and also a boost to self-esteem. In addition, the reduction of the congenital deficit of microtia is associated with an increase in security in the social environment. Since the hearing ability is almost always limited in microtia, ENT surgery is usually necessary or recommended to improve hearing. Before the ear is built up, surgery of the middle ear must be performed, because if the procedure is reversed, possible scars could interfere with the ear reconstruction. The operation is performed in several partial steps. After about a year, the feeling in the newly built up auricle returns in affected persons. Hearing gain after surgery to improve hearing ranges from 20 to 40 decibels.
Outlook and prognosis
As a congenital malformation, microtia has no prospect of spontaneous recovery or improvement. However, the prognosis regarding possible improvement in auricular malformation is usually very good. Several factors are relevant for the exact prognosis – for example, whether both ears are affected and the inner and middle ear are completely intact. If, for example, only the auricles as such are malformed, small-scale interventions from the field of plastic surgery can at least improve hearing enormously. Further adaptation and reconstruction of a normal ear is often possible. However, possible developmental disorders that occur as a result of impaired hearing carry the risk of greater limitations. These should be addressed early for the best chance of compensation.It should also be noted that the perceived quality of life for those affected is greatly improved by rapid action. In many cases, the limited hearing is not the only burden for those affected, but also the dissatisfaction with their own face and head due to the deformation. If microtia also occurs more frequently in families, a genetic component can be assumed. This can be investigated in case of doubt. Findings about this are relevant with regard to further family planning and a certainty that has taken place. No real prevention of auricular malformation can be promised at present either, but certainty about the risk for one’s own child can be very helpful.
Prevention
To prevent microtia, special care should be taken, especially during the first trimester of pregnancy, to ensure that pregnant women do not consume alcoholic beverages, smoke, or use drugs. Possibly, the consumption of medications should also be clarified with the attending physician to rule out any risk.
Follow-up care
Existing hearing loss can lead to various complaints and complications in affected individuals that may require ongoing follow-up care. Although these complaints do not reduce life expectancy, they can have a very negative impact on the patient’s quality of life and lead to significant limitations in everyday life. Therefore, an examination by a physician should take place at the first signs and symptoms. Those affected are sometimes tense due to the limited hearing ability and not infrequently suffer from psychological upsets. Sensitive conversations with friends and family help to alleviate the mental suffering. It is also useful to make the social environment aware of the existing disease in order to prevent prejudices or misunderstandings. Sometimes this can lead to inferiority complexes or lowered self-esteem in those affected if the illness persists and restricts the affected person’s everyday life. Especially in stressful situations, the symptoms can intensify, so that the affected person can no longer concentrate properly. Therefore, specifically addressing fellow patients is an essential element of aftercare in order to master dealing with the disease in the long term.
What you can do yourself
Affected individuals usually suffer not only from hearing loss, but also from the aesthetic impairment associated with the missing or incompletely formed pinna. In families where a malformation of the auricle is common, a hearing test should be carried out on very young children in order to detect any disorders at an early stage. Undetected hearing impairment can lead to developmental delays that, in severe cases, continue to affect affected individuals even as adults. Deficits in hearing can usually be compensated for initially by hearing aids. Later, correction of the impairment is usually also possible through surgery on the middle ear. Plastic surgical reconstruction of the auricle is recommended only afterwards. However, surgical interventions on the ear are usually not performed in childhood. Children suffering from microtia, which is accompanied by a clearly visible malformation of the outer ear, are therefore often teased in kindergarten and elementary school. Parents should pay attention to whether their child also suffers psychologically from the disorder and take countermeasures in time. Simple remedies often help, for example a hairstyle that covers the ears so that the malformation is not constantly visible in everyday life. Insofar as children suffer emotionally from the aesthetic impairment, a child psychologist should be consulted.
