Congenital Ear Abnormalities


What is it?

The ears are made entirely of cartilage covered by skin. They hold a very prominent position on the head and have a very recognizable shape. As a result, even minor abnormalities can be quite obvious, and throw off the balance of the face. This can attract unwanted attention or teasing from peers, and affect your child’s self-esteem. A wide variety of ear abnormalities exist. “Constricted ear” occurs when the top of the ear (helical rim) is folded over, wrinkled, tight, or missing its normal curves. Sometimes this is also called “lop or cup ear”. If the constricted ear is diagnosed early enough, it can usually be treated effectively with ear molding. Later in life, treatment would require a formal otoplasty. “Stahls ear” refers to an extra fold or crease in the upper part of the ear, and can sometimes make the ear appear pointed like “Spock ears”. Stahls ear is treated similarly to constricted ear. “Cryptotia” refers to when the top of the ear appears as though it is buried in the surrounding skin. It can be treated with ear molding up to 5 years of age. This is because all of the normal cartilage is present, and treatment mainly requires stretching of the surrounding skin to allow the cartilage to be released and take on its normal shape. It can also be treated with local skin flaps or grafts. “Prominent ears” refers to what we typically think of as large ears that “stick out”. Usually, the top of the ear is also missing its normal folds. Prominent ears can also be treated with ear molding or a formal otoplasty. Last is “microtia”.

Microtia refers to a condition where the external ear is underdeveloped or absent (anotia). It is graded based on severity. The most common type is Grade III or “lobular” where there is a peanut-shaped piece of skin and cartilage present without recognizable structures. Microtia can occur without other medical problems and is also a common part of other conditions like hemifacial microsomia and Goldenhar Syndrome. In more severe forms of microtia, the ear canal can be abnormal or absent, and the inner ear bones can also be abnormal. This will affect your child’s hearing. Depending on the degree of hearing loss, your child may need a type of hearing aid or may need surgery. Making the outer part of the ear usually requires complete or near complete reconstruction.

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