Patients who have severe manifestations of the syndrome frequently have upper airway obstruction and require a tracheostomy and/or surgical elongation of the lower jaw at a very young age. This condition is always bilateral and tends to be an inherited syndrome and involves underdevelopment of the lower eyelids, the cheekbones, and the lower jaw. An Earlobe is present but is in the wrong location.Īnother syndrome where microtia may be present is Treacher Collins Syndrome. Like isolated microtia, hemifacial microsomia can also occur, in a minority of patients, on both sides of the face.Ī patient with classic microtia and aural atresia (absent ear canal). This condition is also tremendously variable and involves underdevelopment, to some degree, of all the structures on one side of the face, including the ear, the jaw bones, the fullness of the cheek tissue, function of the facial nerve and the movement of the facial muscles. For example, hemifacial microsomia is also known as the "1st and 2nd branchial arch syndrome" and the "oto-mandibular syndrome" as well as by other names. This condition has several other names which makes it difficult to research for patients and families. The most common condition in which microtia accompanies other anomalies is called "hemifacial microsomia". In approximately 10% patients, however, the ear deformity occurs in conjunction with other facial abnormalities. In most patients microtia and aural atresia occur as isolated conditions. The inner ear, on the other hand, develops with the brain, and is almost always normal in patients with microtia and aural atresia.ĭiagram of Normal External Ear Is Microtia an Isolated Condition? Microtia and aural atresia tend to occur together because the outer ear and the middle ear develop together in fetal life. No wonder the sound can’t get through! These patients also have structural abnormalities of the middle ear itself with absence of the eardrum and incomplete formation of the tiny middle ear bones, which normally allow conduction of hearing through the middle ear. In other words, the outer ear is completely separated from the middle ear by bone. #Aural atresia skin#Patients who lack the ear canal are not just missing a “hole” in the skin they also have no canal through in the skull. It is important to note that patients who have atresia in both ears will be sufficiently hard of hearing to be totally dependent on a hearing aid in all situations (See below) Patients who hear on only one side, however, have difficulty localizing sounds and have difficulty distinguishing sounds in situations where there is background noise (play-ground, classrooms, parties etc). Because of the normal hearing on the opposite side, these patients usually have no trouble hearing when someone speaks loudly to them in a quiet surrounding environment or in one-on-one conversations. Patients who have aural atresia do not have good hearing on that side but usually have completely normal hearing on the opposite side. The most common situation, however, is the patient with microtia AND aural atresia. The reverse can also be true: some patients have aural atresia but a normal appearing external ear. Patients who have microtia usually, but not always, have aural atresia. The term aural atresia refers to the absence of the ear canal and ear drum. Other patients have a low hairline and additional maneuvers are required to avoid a reconstructed ear that is partially or completely covered with hair. Some patients have a hairline that is in normal position, leaving appropriate, hairless skin for the reconstructed ear. While some patients appear to have a true ear canal, these canals are almost always blind and to not communicate with the middle ear (more on this below under Aural Atresia). Some patients have no evidence of an ear canal and some have a blind canal. Some patients have small, almost normal appearing ears while others have completely absent ears. As will be discussed below, microtia is tremendously variable. The unilateral form is much more common, occurring in approximately 90% of patients. Microtia can occur on one side only (unilateral) or on both sides ("bilateral). The term microtia literally means “small ear” but in reality indicates a small, abnormally shaped or absent external ear.
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