Definition of Microtia in English

Microtia is a congenital malformation of the auricle. The outer ear is not completely formed. Sometimes the ear canal is very small or missing entirely. Ear reconstruction and surgery to improve hearing are possible treatments.

What is a microtia?

The malformation of the outer ear is congenital. The incomplete development of the ear can lead to minor malformations or the auditory canal can be missing entirely. The term microtia is derived from the translation “small ear”. A cartilage plate completely separates the outer ear from the inner ear, which subsequently leads to a significant impairment of hearing.┬áSee AbbreviationFinder for abbreviations related to Microtia.

Microtia can occur on just one side of the head or on both sides. In many cases, underdevelopment of the mandible occurs simultaneously with microtia. Microtia is one of the largest manifestations of congenital ear defects. Microtia is divided into four stages:

  • Grade 1 describes a minimal malformation in which most of the ear has normal anatomy.
  • Grade 2 shows an optically normal pinna formation, especially in the lower part. However, the ear canal may be different, smaller, or completely closed.
  • At grade 3, the ear is peanut-shaped and has no ear canal.
  • In grade 4, the outer ear and ear canal are completely absent.

Causes

The causes of a 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 responsible for only five percent of patients. It is also possible that there are vascular disorders that occurred during the development of the fetus, but this has not been 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 of pregnancy. Drugs could also be possible triggers for this malformation. The incidence of microtia is found to be more common among certain peoples, such as Asians or Andean people. The probability of a child being born with microtia is 1 in 6000 or 12,000 births.

Symptoms, Ailments & Signs

Microtia is found in ten percent of those affected on both sides, the so-called bilateral microtia. The frequency of this congenital anomaly is usually found more often on the right side of the head and usually only develops on one side. The main complaints are hearing loss, since there is no normal auditory canal, eardrum and auditory ossicles. Affected people with microtia still hear a few sounds, but not through the ear canal. In microtia, the pinna is missing, sometimes there is an earlobe with a remnant of the pinna.

Diagnosis & disease progression

Human genetics should be consulted to clarify microtia that occurs more frequently in families. Early clarification by specialists such as ENT doctors, paediatricians, phoniatrists and pediatric audiologists is also recommended. Possible negative influences during early pregnancy should be clarified.

From the age of 10, computed tomography can provide more detailed information about the middle ear structures. A diagnosis of the malformation is recommended in early childhood. Suitable methods for clarification are regular hearing tests and computer tomography to detect middle ear defects.

Complications

Microtia primarily causes hearing problems in the patient. The incorrect formation of the auricle can lead to hearing impairment or complete deafness. However, the patient’s life expectancy is not reduced or restricted by this complaint. Young people or small children in particular can develop severe mental health problems or depression as a result of microtia.

Those affected can only hear certain sounds and suffer from significant limitations in their everyday lives. In children, microtia can also lead to developmental disorders, so that this is slowed down and consequential damage occurs in adulthood. In some cases, microtia can also lead to balance problems.

The symptoms of the disease can be limited and resolved relatively well with the help of implants and hearing aids. There are no particular complications or discomfort. However, parents must strengthen their children’s self-esteem so that inferiority complexes do not develop. Surgical intervention can further reduce the hearing problems. Here, too, there are no particular complications.

When should you go to the doctor?

Serious microtia is usually detected immediately after birth or in the first few months of the infant’s life. In principle, this malformation of the ear is not dangerous unless it is part of a syndrome. This is mostly not the case. Nevertheless, it would be advisable to consult the pediatrician or a specialist. Depending on the severity and shape of the microtia, this can significantly limit the child’s hearing. If left untreated, this can result in language deficits and consequential damage in adulthood. For this reason, it is advisable to have a hearing test carried out by an ENT doctor as early as possible . A doctor should definitely be consulted if there are also balance disorders.

Surgical correction of the microtia and the insertion of a bone conduction hearing aid can be carried out at the earliest from the age of four or five, since these interventions require a certain level of physical maturity. If only a mild form of microtia is present and the child is not affected by it, no medical treatment is required. In some cases, those affected can develop psychological symptoms such as inferiority complexes or depression due to microtia, especially in childhood and adolescence. In this case, it is advisable to seek the help of a therapist.

Treatment & Therapy

Due to the impairment of hearing associated with microtia, treatment by pediatric audiologists and ENT specialists is helpful. The existing hearing loss must be continuously clarified and treated accordingly. With a so-called otoplasty, the missing auricle can be reproduced from the body’s own tissue. The normal hearing ear also requires consistent monitoring. If conductive or sensorineural hearing loss occurs, there is a need for action.

In the case of a bilateral malformation due to microtia, the child can be fitted with a bone conduction hearing aid. An operation to correct the deformity should be carried out at the earliest at the age of four to five years in order to have the necessary physical maturity for this operation. Alternatively, an implant can be used for microtia to avoid the invasive procedure through surgery. Since microtia is not as clearly visible as defects on the face, the psychological burden is not necessarily great.

However, an ear reconstruction improves the quality of life of those affected and also boosts their self-esteem. In addition, reducing the congenital deficit of microtia is associated with an increase in security in the social environment. Since the ability to hear is almost always limited in microtia, an ENT surgery operation to improve hearing is usually necessary or recommended.

An operation on the middle ear must be carried out before the ear can be reconstructed, as possible scars could impede the ear reconstruction if the procedure is reversed. The operation takes place in several sub-steps. After about a year, the affected person regains the feeling in the newly built auricle. The hearing gain after an operation to improve hearing is between 20 and 40 decibels.

Outlook & Forecast

As a congenital malformation, microtia has no chance of spontaneous healing or improvement. However, the prognosis with regard to a possible improvement in the auricle 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 are malformed as such, small-scale interventions in the field of plastic surgery can at least improve hearing enormously. Further adaptation and reconstruction of an ordinary ear is often possible.

Possible developmental disorders that occur as a result of limited hearing, however, harbor the risk of greater limitations. These should be addressed early for the best chance of compensation. It should also be noted that the quality of life perceived by those affected is greatly improved by quick action. In many cases, it is not the limited hearing that is the sole 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. If in doubt, this can be investigated. Findings about this are relevant with regard to further family planning and a certainty that has taken place. There is currently no real promise of preventing ear malformations, but certainty about the risk for one’s own child can be very helpful.

Prevention

To prevent microtia, especially in the first trimester of pregnancy, special care should be taken to ensure that pregnant women do not consume alcoholic beverages, smoke or consume drugs. The consumption of medication should possibly also be clarified with the doctor treating you in order to rule out any risk.

Aftercare

An existing hearing loss can lead to various symptoms and complications in those affected, which can make ongoing follow-up care necessary. Although these symptoms do not reduce life expectancy, they can have a very negative effect on the patient’s quality of life and lead to significant restrictions in everyday life. Therefore, an examination by a doctor should take place at the first signs and symptoms.

Those affected are sometimes tense due to the limited hearing ability and often suffer from mental disorders. Empathetic conversations with friends and family help reduce emotional suffering. It is also useful to draw the social environment’s attention to the existing disease in order to prevent prejudice or misunderstandings.

Sometimes it can lead to inferiority complexes or reduced self-esteem in those affected if this disease persists and restricts the everyday life of the person affected. The symptoms can worsen, especially in stressful situations, so that the person concerned can no longer concentrate properly. Therefore, the targeted addressing of fellow human beings is an essential element of aftercare in order to master dealing with the disease in the long term.

You can do that yourself

Those affected usually not only suffer from the hearing loss, but also from the aesthetic impairment associated with the missing or incompletely formed pinna.

In families in which auricle malformations are widespread, a hearing test should be carried out on very young children in order to detect any disorders at an early stage. Undetected hearing damage can lead to a delay in development, which in severe cases still affects those affected even as adults. Hearing deficits can usually be compensated initially with hearing aids. Later, the impairment can usually be corrected by surgical intervention on the middle ear. A plastic surgical reconstruction of the auricle is only recommended afterwards.

However, surgical interventions on the ear are usually not carried out in childhood. Children who suffer 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 is also suffering from the disorder mentally and take countermeasures in good time. Simple remedies often help, for example a hairstyle that covers the ears so that the deformity is not constantly visible in everyday life. If children suffer severely from the aesthetic impairment, a child psychologist should be consulted.