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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).
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]
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).
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.
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.
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).
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.
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.