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Aural atresia is the underdevelopment of the middle ear and canal and usually occurs in conjunction with microtia. Atresia occurs because patients with microtia may not have an external opening to the ear canal, though. However, the cochlea and other inner ear structures are usually present.
The several components or degrees of development range from an ear tag, preauricular appendage, preauricular tag, or accessory tragus, to supernumerary ears or polyotia. [7] It is a relatively common congenital anomaly of the first branchial arch or second branchial arches.
Burt Brent is a retired reconstructive plastic surgeon best known for his work in reconstructing the absent outer ear. He built upon the techniques of his mentor, Dr. Radford Tanzer [1] of the Mary Hitchcock Clinic at Dartmouth Medical School and repaired ear defects in 1,800 patients, most of them children born with ear deformities such as microtia.
Because microtia and anotia have the same origin, it can be referred to as microtia-anotia. [16] Microtia can be unilateral (one side only) or bilateral (affecting both sides). Microtia occurs in 1 out of about 8,000–10,000 births. In unilateral microtia, the right ear is most commonly affected.
CHARGE syndrome (formerly known as CHARGE association) is a rare syndrome caused by a genetic disorder.First described in 1979, the acronym "CHARGE" came into use for newborn children with the congenital features of coloboma of the eye, heart defects, atresia of the nasal choanae, restricted growth or development, genital or urinary abnormalities, and ear abnormalities and deafness. [1]
A conductive hearing loss along with middle ear disease is most commonly seen in patients with Pfeiffer syndrome; although, there have been reports of mixed hearing loss as well. The hearing loss is most typically caused by stenosis or atresia of the auditory canal, middle ear hypoplasia and ossicular hypoplasia (Vallino-Napoli, 1996).
Ear abnormalities are common, including small holes in the skin just in front of the ears (preauricular pits, or sinuses). The external auricle of the ear is typically malformed, with possibly severe microtia with atresia of the external auditory canal present. Deafness may occur, but severe hearing loss is uncommon; the occurrence of mild ...
Goldenhar syndrome is a rare congenital defect characterized by incomplete development of the ear, nose, soft palate, lip and mandible on usually one side of the body. Common clinical manifestations include limbal dermoids, preauricular skin tags and strabismus. [1]