Microtia is a congenital deformity that affects one or both ears (a bilateral condition). It originates from the Greek term ‘micro-otia,’ meaning small ear and affects roughly one in every 8,000 to 10,000 births. A bilateral occurrence (microtia bilateral) depicts the deformity in both ears. Microtia bilateral is a rare occurrence, with incidence rates of one in 25,000 births.
This deformity takes on several forms, classified into four grades starting from grade I, which symbolizes a slightly small ear with identifiable structures and a small but open ear canal. Grade II represents a partially formed ear usually with a closed ear canal. Grade III, often the most common, is the “classic” microtia, where most of the ear (“pinna”) is missing. Lastly, grade IV, known as anotia, signifies a total absence of the ear.
The etiology of microtia bilateral remains largely unknown even though it is frequently linked to genetic forces. The deformity usually occurs during the first trimester of gestation, a period critical for ear development. Research links some causes to maternal environmental exposures during pregnancy, like certain medications, alcohol and drug abuse, maternal diabetes, or advanced maternal age, but confirmation has not been conclusive.
The prominence of microtia bilateral is more significant as it affect both ears, causing hearing difficulties. This creates challenges for speech and language development in children, which directly impacts their social development and academic performance. In addition to the cosmetic concerns, hearing impairment decreases the quality of life of those affected.
Microtia Bilateral and Treacher Collins Syndrome
In some instances, microtia bilateral occurs as a component of a syndrome, such as Treacher Collins Syndrome. This is a rare genetic disorder characterized by craniofacial deformities, which may include the underdevelopment or absence of the cheekbones, a very small jaw and chin (micrognathia), and a cleft palate. The occurrence of microtia with this syndrome emphasizes the need for genetic investigations in these cases.
Treacher Collins syndrome treatment focuses on addressing the individual symptoms and may include surgery for craniofacial abnormalities, hearing aids or cochlear implants for auditory problems, and speech therapy. When associated with microtia bilateral, the plan may encompass ear reconstruction surgery, which is usually carried out after the age of six when the ear has reached near-adult size. This reconstructive surgery often involves several stages to create a new ear. In cases where the ear canal is closed (aural atresia), a separate surgery may be performed to open the canal and improve hearing.
Detecting and Managing Microtia Bilateral
Microtia is often detected at birth due to its visible nature. In cases of microtia bilateral, newborn hearing screening is of utmost importance to assess the level of hearing loss and to plan early interventions. This may include a bone conduction hearing aid temporarily until the child is suitable for surgery.
To manage microtia bilateral, a multidisciplinary team approach is vital as it involves several specialties including audiology, otolaryngology, plastic surgery, speech therapy, genetics, and psychology. This holistic, patient-centered approach ensures that all physical, hearing, speech, and psychosocial needs of the child and their family are met. Early intervention with therapies and aids, coupled with parental education, plays a crucial role in the child’s overall developmental outcome.
While microtia bilateral presents significant physical and psychosocial challenges, advances in modern medicine have made it possible to contribute significantly to the quality of life of those affected. Increased understanding and awareness of this condition are crucial for early detection, intervention, support, and acceptance.