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Microtia is a congenital deformity where the auricle (external ear) is underdeveloped. A completely undeveloped auricle is referred to as anotia. Because microtia and anotia have the same origin, it can be referred to as microtia-anotia. [1] Microtia can be unilateral (one side only) or bilateral (affecting both sides).
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.
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.
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.
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).
Otoplasty (surgery of the ear) was developed in ancient India and is described in the medical compendium, the Sushruta Samhita (Sushruta's Compendium, c. 500 AD).The book discussed otoplastic and other plastic surgery techniques and procedures for correcting, repairing and reconstructing ears, noses, lips, and genitalia that were amputated as criminal, religious, and military punishments.
The surgical option is cosmetic reconstruction of the external ear's normal shape and repair of the ear canal. In less severe cases, the reconstruction will be sufficient to restore hearing. In grades of anotia/microtia that affect the middle ear, the surgery with the use of a bone-anchored hearing aid (BAHA) will likely restore the hearing ...
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]