Grades of Microtia and Its Relation To Goldenhar Syndrome
Microtia is a congenital deformity affecting the external ear (pinna) in which the baby’s ear is underdeveloped. A relatively rare condition, Microtia can occur in one or both ears. The term ‘Microtia’ comes from Latin and translates to ‘little ear’. Statistics suggest that Microtia affects about 1 in every 9,000 babies born globally.
Microtia is usually noticeable at birth and categorized into four grades based on the severity of the malformation. Understanding these
is essential in making decisions about corrective treatments and surgeries.
Grade 1 Microtia
In Grade 1 Microtia, the external ear appears smaller but maintains a noticeable structure that closely mirrors a typical ear. The size may be smaller, and some elements may be slightly malformed. In some cases, Grade 1 Microtia may also include a narrow or blocked ear canal.
Grade 2 Microtia
Grade 2 Microtia presents more notable malformations than Grade 1. In this case, the external ear’s structure may be more significantly distorted, with elements of the ear like the lobe or helix not clearly defined. The ear canal may be blocked or narrowed, affecting the individual’s hearing ability.
Grade 3 Microtia
This grade is sometimes referred to as ‘classic’ Microtia. The external ear is significantly underdeveloped or small, often taking the shape of a peanut. Key elements, the ear canal and middle ear, are generally absent, causing conductive hearing loss. This is the most common form of Microtia.
Grade 4 Microtia
Also known as Anotia, Grade 4 is the most severe form of Microtia, where the external ear is completely absent. Just a small piece of soft tissue or nubbin may be visible in place of the ear.
Microtia is often associated with another condition called the Goldenhar Syndrome. This is a rare congenital defect characterized by incomplete development of the ear, nose, lip, soft palate, and mandible. It usually affects one side of the body but can impact both sides. The involvement of the ear ranges from minor anomalies to complete absence of the ear (anotia). There could be other associated anomalies like renal abnormalities, vertebral anomalies, facial nerve palsy, amongst others.
In conclusion, understanding the grades of Microtia not only helps parents to better comprehend the state of their child’s condition, but it’s also critical for doctors to make better treatment choices and surgical plans. As part of a potential syndrome such as Goldenhar Syndrome, obtaining genetic counseling may also be helpful.