Introduction

About 1 in 6000 people in the UK are thought to be born with microtia.¹ Moreover, it is estimated that, on average, microtia occurs in 2 out of every 10,000 births worldwide. The word "microtia" comes from Greek and means a small ear. Microtia happens when the outer or middle ear does not develop properly from birth; it is a congenital defect. It can affect one or both ears.² It may happen alone or be connected to other ears or face issues or a specific genetic problem. Microtia can be mild, with a small ear problem, or severe, with no ear at all. Due to its complicated nature and the associated long-term problems, multidisciplinary care is needed.³

This article will discuss the common problems people with microtia face in their day-to-day lives and the available resources.

Causes of microtia

The exact cause of microtia remains unclear, but there are a lot of risk factors that can boost the chances, such as:^4,5,6

• Cold-like syndrome during pregnancy:  Having the flu during pregnancy can lead to more problems
• Gestational diabetes
• Smoking during pregnancy
• Drinking alcohol during pregnancy
• Essential hypertension and using antihypertensive medication during pregnancy
• Chronic disease during pregnancy like any autoimmune conditions
• Overuse of medications
• Genetics: Family history suggests microtia can be inherited
• Folic acid supplementation during pregnancy has been shown to help protect against microtia.

Microtia grading

Doctors use a classification system for the evaluation of microtia. The severity of the condition can be classified into four types:⁶ 

• Type 1: Small external ear but all parts have little deformities
• Type 2: The outer ear is partially developed and small
• Type 3: There are some ear structures, but there are no ear parts formed
• Type 4: Anotia (total absence of external ear)

Physical challenges

People with microtia may have some degree of hearing loss in the affected ear, especially if there are problems with the development of the middle ear or ear canal. 

The types of hearing impairment in microtia

Different types of hearing problems can be connected to microtia. 3 types of hearing loss that can occur are as follows:⁷

• Conductive hearing loss: When sound waves cannot be effectively transmitted from the outer to the inner ear due to structural abnormalities. Small ears often have no opening or are too narrow. The inside of the ear can also be deformed, such as the small bones known as ossicles
• Sensorineural Hearing Loss: Microtia can be linked to inner ear anomalies which are less common. It can affect the sensory or neural pathways of hearing
• Mixed Hearing Loss: A mix of both conductive and sensorineural components. It happens because of problems in the middle and inner ear at the same time

Symptoms of microtia

Symptoms vary among individuals depending on the severity, the grading and the side. In some cases, there is no difficulty with hearing but, there are common presentations, such as:⁷

• Reduced ability to hear soft sounds causes difficulty understanding speech, particularly in noisy environments in daily life. It is difficult for families and children who are born with the condition, particularly in their early years. Hearing is necessary for speech development
• Hearing and distinguishing between different sounds can also be tough
• People with microtia on one side may struggle to pinpoint where sounds come from (spatial awareness)
• Associated structural defects: Weakness in the nerve supply of the face, varying from mild to severe, up to 15%. It is rare to find other general defects such as heart issues, urological problems, cleft palate or lip
• Oculoauriculovertebral dysplasia, or Goldenhar Syndrome, and Treacher-Collins Syndrome are two head and face abnormalities that are sometimes associated with microtia⁸
• The inner ear, which sends impulses and information to the brain, has a role in balance and place orientation. Microtia-related inner ear underdevelopment can cause problems related to balance that need medical attention

Psychological and emotional challenges 

There are multiple studies that aim to provide the appropriate support and highlight the struggles and challenges faced by children, their families and adults. These include:⁹

• Body Image and Self-Esteem

How we think and feel about our physical appearance is known as our body image. While the way we view and regard ourselves is referred to as our self-esteem. It resembles our inbuilt sense of value. Our self-esteem usually increases when we feel good about our physical appearance.¹⁰

Children with microtia may have some degree of low self-esteem and increased anxiety. The same situation is found in adults; some stated they used their hairstyle to hide their microtia.¹¹

• Social Isolation

This is a significant issue, with anxiety and the degree of hearing loss playing an important role in its occurrence. Individuals with the condition, no matter how old they are, might avoid being with others. They might have bad times with friends or have trouble talking, and that can cause being picked on or being seen as different.^1,11

• Depression

The period of childhood and teenage is one of the most difficult times when the body and hormonal changes could be a factor for depression. It is more apparent in individuals with any physical defect.¹²

• Familial burden

The parents experience difficult times, and they experience a wide range of emotions, from shock to grief. They are concerned about their child's academic performance, social acceptance, and future. As a result, they become extremely protective, which may restrict their child's freedom and social prospects.¹³

Educational and occupational struggles

Educational and occupational aspects in individuals with this disorder may have more trouble than others. 

Educational issue

Hearing impairment has an impact on different stages in their life, starting from childhood, and passing through to their social and academic life. In other words, we can say that they could have delayed speech.¹⁴

Children with hearing impairments learn words far slower. They may have trouble hearing some sounds, such as "sh," "s," "t," "f," and "k," and as a result, they have trouble pronouncing them. This has an impact on their speech patterns and may make it hard for them to be understood.¹⁴

They also may struggle to understand complex sentences. Consequently, the speech disorder represents a burden on the child during his or her academic life. 

Occupational barriers

Effective hearing and communication skills are essential for several jobs like teaching, public speaking, and customer service. Also, the frequent medical appointments could disrupt work commitments. The presence of adaptive technologies would make a difference in their workplace with the awareness of their needs.¹⁵

Medical and surgical challenges

Any condition that requires long-term medical attention has some sort of difficult adaptation. It affects the way of life either: 

• Availability of appointments and matching with the daily commitments
• The continuity of the care provided to the patients with high standards
• The need for effective communication between the multidisciplinary team regarding the referrals and treatment
• The education in regards to the available options: surgical, non-surgical, the speech therapist, and the psychological support needed
• The normalisation after the surgery¹⁶

The treatment choices

Treatment options for microtia aim to address physical appearance, help with hearing and provide the appropriate psychological support. There are options as follows:

Surgical option¹⁷

• Prosthetic ear: It is a way in which a designed prosthetic ear is attached with special glue. It is non-invasive, but some people can find it uncomfortable
• Autologous Rib Reconstruction: It is an invasive surgery involving 2 steps. Firstly, the surgeon removes the rib and tries to shape it to resemble the ear. Secondly, after an average of 6 months, the removed rib is implanted. It shows a good result, as there is no chance to be rejected, as it is the patient's own body. At the same time, it carries the risk of infection and pain
• MedPor graft surgical reconstruction: Instead of using the rib, this type of surgery uses a synthetic porous polyethene framework. It has the advantage of requiring less surgical intervention and thus reducing the risk of infection. The major complication, though uncommon, is the rejection of synthetic material

Non-surgical option¹⁸

• Headband bone conduction devices:   It is good for those who do not want surgery or are waiting. It's a headband that sends sound through the skull to the ear. The downside is it needs pressing on the skull, which can cause sores, headaches, and sometimes cause skin issues
• Spectacle bone conduction hearing aids: The sound transmits in the same way. It is a combined advantage for patients to correct their eyesight if needed

Coping strategies and psychological support

Early intervention is beneficial, like speech therapy, reconstructive surgery, hearing devices and a supported school/work environment with continuous follow-ups. The need for combined support by family, friends and other individuals to provide a safe and compliant environment is crucial.

They benefit from getting help early, like speech therapy, reconstructive surgery, hearing devices and a supported school and work environment with continuous follow-up. The need for combined support by the family, friends, society and school to provide a safe and compliant environment to be engaged in the right way.¹⁹

Summary

Microtia is a condition affecting the pinna (outer ear). It poses specific challenges that require a combination of medical care and strong family involvement. This deformity can influence not only the ear's physical appearance but also an individual's hearing ability, potentially impacting their daily life and overall well-being. Effective management typically involves a multidisciplinary team providing solutions such as reconstructive surgery, hearing aids, or bone conduction devices to address hearing issues. Emotional support is also critical, as coping with microtia may influence self-confidence and social interactions. Families play a crucial role by offering emotional and practical support, helping their loved ones adapt to the condition, and ensuring adherence to prescribed treatments. Consistent follow-ups and clear communication between families and healthcare providers are vital for achieving positive outcomes.

FAQs

1. Can ear problems affect mental health?
   • Yes
2. Is microtia considered a disability?
   • Microtia itself is not a disability, the problem is associated with hearing loss.
3. Does microtia affect balance?
   • Not always, according to the grade.