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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.
Microtia is a congenital deformity where the auricle (external ear) is underdeveloped. A completely undeveloped pinna is referred to as anotia. 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).
Atresia, microtia, otitis media and other outer/middle ear abnormalities, as well as infants with sensorineural hearing loss, will require the use of bone-conduction ABR testing. Infants who have a considerable amount of amniotic fluid in their middle ear space may need to be tested with BCABR. This fluid usually disappears by 48 hours after birth.
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).
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 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 ...
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Exostoses, abnormal growth of bone within the ear canal; Tumor of the ear canal; Congenital stenosis or atresia of the external auditory canal (narrow or blocked ear canal). Ear canal stenosis & atresia can exist independently or may result from congenital malformations of the auricle such as microtia or anotia.