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273:, and is characterized by craniofacial deformities; malformed or absent ears are also seen in this syndrome. The effects may be mild, undiagnosed to severe, leading to death. Because the ear defects are much different in this disorder and not only affect the outer ear, but the middle ear as well, reconstructive surgery may not help with the child's hearing and in this case a
283:: A rare congenital birth defect that causes abnormalities of facial development. Also known as oculoauricular dysplasia. The facial anomalies include underdeveloped, asymmetric half of the face. The defect is capable of affecting tissue, muscle, and the underlying bone structure of the side of the face with the abnormality.
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results from malformation or suppression of the auricular hillocks, which are small swellings on the embryonic visceral arches or the beginnings of the external ears; the small swellings are derived from the first and second pharyngeal arches. Because the ears and the kidneys develop simultaneously,
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The isolated cause, a cause not associated with a syndrome, of anotia or microtia is not known, though it is believed to be of genetic basis. Developmentally, anotia/microtia occurs when certain tissues associated with the auricle do not develop. This rare defect may occur as part of a syndrome or as
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The treatment will vary with the different grades, but the most common is a surgical repair. 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
302:(BAHA) will likely restore the hearing. The BAHA may be surgically implanted onto the skull which would allow for some hearing repair by conduction through the skull bone. "This allows sound vibrations to travel through bones in the head to the inner ear."
186:, or outer portion of the ear. Throughout fetal development, the hillocks will move from the sides of the neck to the sides of the head. Simultaneously, in the seventh week of development, the auditory tube begins to form out of the tympanic membrane.
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205:, or ear drum and several little bones that are moved by the sound waves that have entered the ear via the canal. These movements are very small, like vibrations and are transmitted to the inner ear.
174:, an extension of the early hind brain. By the fourth week of development, the otic placodes invaginate, or sink inward forming pits which close themselves off for the outer surface
91:, in which a small part of the auricle is present. Anotia and microtia may occur unilaterally (only one ear affected) or bilaterally (both ears affected). This deformity results in
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The inner ear contains a structure called the cochlea, which contains small hair- like cells that respond to sound information and transmits it via nerve impulses down the
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289: : (AMS) A rare genetic disorder characterized by various physical anomalies which affect the craniofacial area, the skin, the fingers, and the genitals.
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and begin forming the inner ear labyrinthe on the inside. Outer ear development begins in about the fifth week of human embryonic development. Upon the
182:, auricle hillocks begin to form. By the seventh week, the three pairs of hillocks have enlarged differentiated and fused together to start forming the
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Schoenwolf, Gary C., and
William J. Larsen. Larsen's Human Embryology. 4th ed. Philadelphia: Churchill Livingstone/Elsevier, 2009. p601-2. Print.
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Most severe type, anotia; all external structures of the ears are absent. Defects affecting the external ear such as the
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Ear development begins in about the third week of human embryonic development, beginning with the formation of the
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BAHA: An implantable hearing device. It is the only hearing aid device that works via direct bone conduction.
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263:"20% to 40% of children with microtia/anotia will have additional defects that could suggest a syndrome."
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Small amount of basic, soft tissue ear structure lacking cartilage; auricle is abnormal in appearance.
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The human ear is divided into three sections. Each section has its own specialized function.
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would be best. BAHA will only work, however if the inner ear and nerve are intact.
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The auricle is in a hook or 'S' shape; external ear is only moderately abnormal.
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anotia/microtia that affect the middle ear, the surgery with the use of a
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External portion of the ear is small in size; auricle structure is normal
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children with ear defects are often checked for kidney defects at birth.
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269:: (TCS) A congenital disorder caused by a defective protein known as
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The outer ear acts like a funnel and takes in the sound.
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may be too technical for most readers to understand
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339:. Minnesota Department of Health. Archived from
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337:Children and Youth with Special Health Needs
166:Bilateral anotia in a 10-year-old boy, 1904
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79:that involves the complete absence of the
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150:Learn how and when to remove this message
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132:make it understandable to non-experts
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221:Defects of the ear: anotia/microtia
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486:malformations and deformations of
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368:Baha- implantable hearing device
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287:Ablepharon macrostomia syndrome
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570:Congenital disorders of ears
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72:("no ear") describes a rare
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16:For the insect genus, see
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300:bone-anchored hearing aid
275:bone-anchored hearing aid
267:Treacher-Collins syndrome
226:an isolated abnormality.
201:The middle ear holds the
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370:. Johns Hopkins Medicine
93:conductive hearing loss
87:. This contrasts with
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43:Illustration of anotia
400:at Wikimedia Commons
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18:Anotia (planthopper)
281:Goldenhar syndrome
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549:Mondini dysplasia
544:Accessory auricle
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398:Anotia (disorder)
396:Media related to
364:"Hopkins Hearing"
343:on 4 January 2013
333:"Anotia/Microtia"
259:Related syndromes
203:tympanic membrane
180:pharyngeal arches
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372:. Retrieved
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374:25 November
347:20 November
212:and to the
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309:References
74:congenital
293:Treatment
242:Type III:
85:ear canal
77:deformity
49:Specialty
564:Category
521:Position
507:Microtia
502:Macrotia
248:Type IV:
236:Type II:
176:ectoderm
140:May 2014
97:deafness
89:microtia
426:C537772
271:treacle
252:auricle
230:Type I:
184:auricle
126:Please
81:auricle
512:Anotia
70:Anotia
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29:Anotia
537:Other
214:brain
495:Size
488:ears
421:MeSH
376:2012
349:2012
444:NIH
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Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
