Microtia is a congenital condition characterised by underdevelopment or absence of the external ear (pinna). It can range from a smaller-than-average ear to a complete absence of the external ear. Microtia can occur unilaterally (affecting one ear) or bilaterally (affecting both ears). The severity of microtia varies among individuals, and it can occur as an isolated condition or as part of a syndrome involving multiple body systems. Microtia can impact hearing, and individuals with this condition often require medical evaluation and intervention, such as hearing aids or reconstructive surgery, to address the ear's functional and cosmetic aspects.

Here is a case study that sheds light on successful surgery, giving patients more than the desired results.

Case Study- Ear Reconstruction

Dr Aditya Kanoi (Consultant, Plastic, & Microvascular Surgery) NSH, Howrah

This 17-year-old young man was born with congenital microtia, characterised by a rudimentary pinna. This was associated with Hemifacial Microsomia, a syndromic condition characterised by poor facial development of the affected side. He previously underwent an unsuccessful attempt at reconstruction at a government hospital, which unfortunately resulted in further deformity and scarring, making him self-conscious and beginning to avoid his friends.

He underwent corrective surgery (using his rib cartilage) in two stages at Narayana Hospital. The initial surgery was complicated by the presence of scar tissue from the previous operation, which resulted in a loss of normal skin pliability, which is essential to the success of the procedure. The deformed cartilage and scarred ear tissue were removed in the first operation, and a skin pocket was created. The patient's ribs were taken from his chest. The ribs were cut, assembled, and meticulously carved to create a framework for the new ear. It was then placed inside the skin pocket. A small piece of rib cartilage was stored in the patient's chest. Then, six months later, the banked rib cartilage was used to elevate the new ear. Tissue and skin grafts taken from the scalp were employed to cover this extra rib cartilage, thereby providing projection for the ear. The newly created ear was finally attached to the existing earlobe to complete the reconstruction.

Although some residual asymmetry remains compared to the normal ear, it brought me great joy to see him walk into my outpatient department yesterday without his ears covered for the first time.

Case Analysis

Microtia ('small ear') is a congenital deformity in which the external ear, or pinna, is malformed and underdeveloped. It may present with a wide spectrum of findings ranging from complete absence of the external ear (anotia) to mild changes in the shape of the ear. Microtia is more commonly unilateral, with an incidence of around 1 in 5000 live births. It is more common in Asians and Indians.

In most cases, microtia is an isolated finding. However, it can be associated with several syndromes, most commonly with Hemifacial Microsomia (HFM), which is characterised by poor development of the affected side of the face.

Although patients often present for the first consultation as infants, corrective surgical reconstruction for microtia is ideally performed at 8-10 years of age. This is because i) Sufficient thoracic cartilage stock is absent before this age and ii) at 8-10 years of age, the growth of the normal ear is almost complete, thereby allowing a more accurate reconstruction.

In severe cases of microtia, the ear canal may be absent, leading to conductive hearing loss on the affected side. However, the inner ear is rarely involved; therefore, these patients have at least some hearing in the affected ear. Applying hearing aids within weeks of birth can correct hearing loss and allow normal speech to develop. Still, they are associated with the disadvantages of a cumbersome external device and social stigma. While surgical correction of the conduction problem may be performed to improve hearing, these procedures are associated with high complication rates and should be reserved for bilateral cases. When surgical correction to improve hearing is contemplated, it should be performed after auricular reconstruction to prevent scarring of the ear region.

Conclusion

Microtia or 'small ear' is a relatively common congenital disorder characterised by a malformed, underdeveloped external ear. While there may be some hearing loss, the main issue is this condition's external appearance and social embarrassment. However, it is possible to recreate the external ear by surgical means using tissue from the patient's body, ideally performed at 8-10 years of age.

Frequently Asked Questions

Q1. What causes microtia?
A1. Microtia is primarily a congenital condition, meaning it is present at birth. The exact cause is often unknown, but it can result from genetic factors, environmental influences, or a combination of both.

Q2. How common is microtia?
A2. Microtia occurs in approximately 1 in every 6,000 to 12,000 live births worldwide. However, the prevalence may vary depending on geographic location and population demographics.

Q3. Can microtia affect hearing?
A3. Yes, microtia can impact hearing, especially if the middle and inner ear structures are also affected. Due to ear canal and middle ear abnormalities, conductive hearing loss is common in individuals with microtia.

Q4. What treatment options are available for microtia?
A4. Treatment options for microtia may include hearing aids to improve hearing function and surgical reconstruction of the external ear (pinna). Surgical procedures such as ear reconstruction using autologous rib cartilage or prosthetic ear implants are commonly used to enhance the appearance of the affected ear.

Q5. Is microtia associated with other health conditions?
A5. Microtia can be isolated or associated with other congenital anomalies or syndromes, such as Treacher-Collins syndrome or Goldenhar syndrome. It's essential for individuals diagnosed with microtia to undergo a thorough medical evaluation to assess for any associated health concerns.

References:

https://my.clevelandclinic.org/health/diseases/21063-microtia