785:
to heal an open wound closed. Repairing aural atresia is a very detailed and complicated surgical procedure which requires an expert in atresia repair. While complications from this surgery can arise, the risk of complications is greatly reduced when using a highly experienced otologist. Atresia patients who opt for surgery will temporarily have the canal packed with gelatin sponge and silicone sheeting to prevent closure. The timing of ear canal reconstruction (canalplasty) depends on the type of external ear (Microtia) repair desired by the patient and family. Two surgical teams in the USA are currently able to reconstruct the canal at the same time as the external ear in a single surgical stage (one stage ear reconstruction).
765:
ear canal, no eardrum, and the small ear bones (malleus/hammer, incus/anvil, and stapes/stirrup) are underdeveloped. "Usually" is in parentheses because rarely, a child with atresia also has a malformation of the inner ear leading to a sensorineural hearing loss (as many as 19% in one study). Sensorineural hearing loss is caused by a problem in the inner ear, the cochlea. Sensorineural hearing loss is not correctable by surgery, but properly fitted and adjusted hearing amplification (hearing aids) generally provide excellent rehabilitation for this hearing loss. If the hearing loss is severe to profound in both ears, the child may be a candidate for a
809:
large enough to provide the necessary donor tissue, some surgeons wait until the patient is 8 years of age; however, some surgeons with more experience with this technique may begin the surgery on a child aged six. The major advantage of this surgery is that the patient's own tissue is used for the reconstruction. This surgery varies from two to four stages depending on the surgeon's preferred method. A novel one stage ear reconstruction technique is performed by a few select surgeons. One team is able to reconstruct the entire external ear and ear canal in one operation.
95:
777:
unilateral aural atresia) on scholastic performance. If atresia surgery or some form of amplification is not used, special steps should be taken to ensure that the child is accessing and understanding all of the verbal information presented in school settings. Recommendations for improving a child's hearing in the academic setting include preferential seating in class, an FM system (the teacher wears a microphone, and the sound is transmitted to a speaker at the child's desk or to an ear bud or hearing aid the child wears), a
136:
641:
847:) screws and can be placed simultaneously. The biggest advantage over any surgery is having a prosthetic ear that allows the affected ear to appear as normal as possible to the natural ear. The biggest disadvantage is the daily care involved and knowing that the prosthesis is not real. In 2022, success of transplantation of a 3D bioprinted auricle made from the microtia patient's own cells was reported, also achieving a first in
357:
815:
This is a 1–2 stage surgery that can start at age 3 and can be done as an outpatient without hospitalization. Using the porous framework, which allows the patient's tissue to grow into the material and the patient's own tissue flap, a new ear is constructed in a single surgery. A small second surgery
863:
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 grade of microtia
784:
It is important to note that not all children with aural atresia are candidates for atresia repair. Candidacy for atresia surgery is based on the hearing test (audiogram) and CT scan imaging. If a canal is built where one does not exist, minor complications can arise from the body's natural tendency
764:
The hearing loss associated with congenital aural atresia is a conductive hearing loss—hearing loss caused by inefficient conduction of sound to the inner ear. Essentially, children with aural atresia have hearing loss because the sound cannot travel into the (usually) healthy inner ear—there is no
808:
This surgery may be performed by specialists in the technique. It involves sculpting the patient's own rib cartilage into the form of an ear. Because the cartilage is the patient's own living tissue, the reconstructed ear continues to grow as the child does. In order to be sure that the rib cage is
797:
The age when outer ear surgery can be attempted depends upon the technique chosen. The earliest is 7 for Rib
Cartilage Grafts. However, some surgeons recommend waiting until a later age, such as 8–10 when the ear is closer to adult size. External ear prostheses have been made for children as young
772:
Unilateral sensorineural hearing loss was not generally considered a serious disability by the medical establishment before the nineties; it was thought that the afflicted person was able to adjust to it from birth. In general, there are exceptional advantages to gain from an intervention to enable
788:
In cases where a later surgical reconstruction of the external ear of the child might be possible, positioning of the BAHA implant is critical. It may be necessary to position the implant further back than usual to enable successful reconstructive surgery – but not so far as to compromise hearing
776:
Children with unilateral sensorineural hearing loss often require years of speech therapy in order to learn how to enunciate and understand spoken language. What is truly unclear, and the subject of an ongoing research study, is the effect of unilateral conductive hearing loss (in children with
169:
The etiology of microtia in children remains uncertain but there are some cases that associate the cause of microtia with genetic defects in multiple or single genes, altitude, and gestational diabetes. Risk factors gathered from studies include infants born underweight, male sex, women
71:
740:
At birth, lower grade microtia is difficult to visually diagnose with a physical exam. While higher grade microtia can be visually diagnosed due to noticeable abnormalities. Infants that have noticeable abnormalities are closely monitored by physicians and hearing specialists.
773:
hearing in the microtic ear, especially in bilateral microtia. Children with untreated unilateral sensorineural hearing loss are more likely to have to repeat a grade in school and/or need supplemental services (e.g., FM system – see below) than their peers.
123:. 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. It may occur as a complication of taking
834:
to mirror the other ear. Prosthetic ears can appear very realistic. They require a few minutes of daily care. They are typically made of silicone, which is colored to match the surrounding skin and can be attached using either
789:
performance. If the reconstruction is ultimately successful, it is easy to remove the percutaneous BAHA abutment. If the surgery is unsuccessful, the abutment can be replaced and the implant re-activated to restore hearing.
2033:
2018:
999:
Guo, Rui et al. “Epidemiological
Analysis and the Nomogram for Possible Risk Factors for Severe Microtia.” The Journal of craniofacial surgery vol. 32,2 (2021): e184-e189. doi:10.1097/SCS.0000000000007068
2144:
761:
of the middle ear can be done to elucidate its development and clarify which patients are appropriate candidates for surgery to improve hearing. For younger individuals, this is done under sedation.
1843:
Tanner PB; Mobley SR. (2006). "External
Auricular and Facial Prosthetics: A Collaborative Effort of the Reconstructive Surgeon and Anaplastologist. Auricular Surgery: Aesthetic and Reconstructive".
1058:
Zhang, Qing-guo; Zhang, Jiao; Yu, Pei; Shen, Hao (October 2009). "Environmental and genetic factors associated with congenital microtia: a case-control study in
Jiangsu, China, 2004 to 2007".
157:
Grade III: Absence of the external ear with a small peanut-like vestige structure and an absence of the external ear canal and ear drum. Grade III microtia is the most common form of microtia.
781:(BAHA), or conventional hearing aids. Age for BAHA implantation depends on whether the child is in Europe (18 months) or the US (age 5). Until then it is possible to fit a BAHA on a softband
757:
Typically, testing is first done to determine the quality of hearing. This can be done as early as in the first two weeks with a BAER test (Brain Stem
Auditory Response Test). At age 5–6,
1879:"A Multicenter, Single Arm, Prospective, Open-Label, Staged Study of the Safety and Efficacy of the AuriNovo Construct for Auricular Reconstruction in Subjects With Unilateral Microtia"
1762:
1405:
Nicholson N, Christensen L, Dornhoffer J, Martin P, Smith-Olinde L (2011). "Verification of speech spectrum audibility for pediatric baha softband users with craniofacial anomalies".
1681:
Nagata S (1994). "Modification of the Stages in Total
Reconstruction of the Auricle: Part I. Grafting the Three-Dimensional Costal Cartilage Framework for Lobule-Type Microtia".
174:, and medication use while pregnant. Genetic inheritance has not been fully studied but in the few studies available, it has shown to occur during the early stages of pregnancy.
2137:
820:. This surgery should only be performed by experts in the techniques involved. The use of porous polyethylene implants for ear reconstruction was initiated in the 1980s by
1448:
Verhagen CV, Hol MK, Coppens-Schellekens W, Snik AF, Cremers CW (2008). "The Baha
Softband. A new treatment for young children with bilateral congenital aural atresia".
2130:
876:. It is also occasionally associated with kidney abnormalities (rarely life-threatening), and jaw problems, and more rarely, heart defects and vertebral deformities.
494:
317:
45:
24:
1833:. Implantate für die rekonstruktive Chirurgie der Nase und des Ohres. Sammelwerk=Laryngo-Rhino-Otologie. Vol.86. 2007. Page 67–76. DOI=10.1055/s-2007-966301
230:
224:
2244:
843:
screws inserted into the skull to which the prosthetic is attached with a magnetic or bar/clip type system. These screws are the same as the BAHA (
35:
207:
1766:
568:
147:
Grade I: A less than complete development of the external ear with identifiable structures and a small but present external ear canal
1990:
864:
usually correlates to the degree of development of the middle ear. Microtia is usually isolated, but may occur in conjunction with
1257:
Kountakis SE, Helidonis E, Jahrsdoerfer RA (1995). "Microtia grade as an indicator of middle ear development in aural atresia".
688:
2239:
848:
282:
2048:
1788:
Reinisch JF, Lewin S (2009). "Ear reconstruction using a porous polyethylene framework and temporoparietal fascia flap".
909:
654:
723:
625:
299:
201:
434:
1378:
Bess FH, Tharpe AM (1988). "Performance and
Management of Children with Unilateral Sensorineural Hearing Loss".
1953:
Bennun RD, Mulliken JB, Kaban LB, Murray JE (December 1985). "Microtia: a microform of hemifacial microsomia".
943:
Luquetti, Daniela V.; Heike, Carrie L.; Hing, Anne V.; Cunningham, Michael L.; Cox, Timothy C. (January 2012).
712:
311:
258:
718:
323:
1901:
1613:(1992). "Auricular Repair with Autogenous Rib Cartilage Grafts: Two Decades of Experience with 600 Cases".
924:
Pretest self assessment and review for the USMLE, pediatrics, 12th edition, question 84, general pediatrics
649:
2110:
573:
530:
292:
263:
700:
370:
218:
195:
873:
844:
778:
749:
The goal of medical intervention is to provide the best form and function to the underdeveloped ear.
706:
519:
2122:
1570:(1999). "Technical Advances with Autogenous Rib Cartilage Grafts—A Personal Review of 1,200 Cases".
94:
2059:
729:
582:
597:
400:
1239:
De la Cruz A, Kesser BW (1999). "Management of the
Unilateral Atretic Ear". In Pensak M (ed.).
633:
484:
408:
344:
307:
274:
150:
Grade II: A partially developed ear (usually the top portion is underdeveloped) with a closed
1486:
Jahrsdoerfer RA, Kesser BW (1995). "Issues on Aural
Atresia for the facial Plastic Surgeon".
865:
438:
329:
181:
428:
349:
192:
171:
49:
28:
1335:
Bess FH, Tharpe AM (1986). "Case History Data on Unilaterally Hearing-Impaired Children".
1133:
Bly, Randall A.; Bhrany, Amit D.; Murakami, Craig S.; Sie, Kathleen C.Y. (November 2016).
547:
Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-hip dysplasia syndrome
8:
2153:
2037:
1270:
562:
108:
2042:
1978:
1830:
1813:
1706:
1638:
1511:
1430:
1360:
1317:
1167:
1134:
1091:
977:
944:
869:
821:
588:
555:
442:
404:
394:
365:
1740:
2218:
2213:
2070:
1970:
1966:
1931:
1860:
1817:
1805:
1744:
1698:
1694:
1630:
1626:
1587:
1583:
1546:
1542:
1503:
1465:
1422:
1387:
1352:
1348:
1309:
1274:
1221:
1213:
1172:
1154:
1083:
1075:
1040:
1032:
1027:
1010:
982:
964:
692:
578:
513:
381:
375:
244:
115:(external ear) is underdeveloped. A completely undeveloped auricle is referred to as
112:
83:
1982:
1710:
1642:
1515:
1434:
1364:
1321:
816:
is performed in 3–6 months if needed for minor adjustments. Medpor was developed by
2099:
1962:
1852:
1797:
1736:
1690:
1622:
1579:
1538:
1495:
1457:
1414:
1344:
1301:
1266:
1203:
1162:
1146:
1095:
1067:
1022:
972:
956:
766:
611:
546:
446:
88:
17:
1878:
2064:
1461:
1305:
1071:
831:
659:
269:
240:
2075:
1208:
1191:
550:
525:
488:
335:
252:
187:
2027:
1856:
1150:
813:
Reconstruct the ear using a polyethylene plastic implant (also called Medpor):
2233:
1292:
Vrabec JT, Lin JW (2010). "Inner Ear Anomalies in Congenital Aural Atresia".
1217:
1158:
1079:
1036:
968:
817:
665:
629:
618:
592:
533:
with relative macrocephaly and with or without cardiac or endocrine anomalies
390:
287:
236:
135:
119:. Because microtia and anotia have the same origin, it can be referred to as
1109:
2197:
1935:
1864:
1809:
1801:
1748:
1591:
1550:
1499:
1469:
1426:
1313:
1225:
1176:
1087:
1044:
986:
885:
607:
478:
451:
422:
414:
248:
143:
According to the Altman-classification, there are four grades of microtia:
1974:
1702:
1634:
1507:
1391:
1356:
1278:
960:
889:
471:
2010:
1926:
Huston Katsanis S, Cutting GR (July 2004). "Treacher Collins Syndrome".
543:
Neurodevelopmental disorder with speech impairment and dysmorphic facies
1724:
1610:
1567:
1481:
1479:
1192:"Could 3D bioprinted tissues offer future hope for microtia treatment?"
603:
537:
1727:(2000). "The Team Approach to Treating the Microtia-Atresia Patient".
1659:
Firmin F (1992). "Microtie Reconstruction par la Technique de Brent".
640:
2152:
2094:
682:
676:
465:
1476:
1404:
1011:"Descriptive epidemiology of anotia and microtia, Hawaii, 1986-2002"
2171:
2105:
1418:
840:
836:
801:
For auricular reconstruction, there are several different options:
509:
214:
151:
124:
78:
Unilateral grade III microtia (more often affecting the right ear).
39:
1447:
860:
758:
418:
356:
318:
Ectrodactyly, ectodermal dysplasia, and cleft lip-palate syndrome
2053:
913:
70:
2181:
2022:
1781:
503:
458:
116:
1529:
Tanzer RC (1959). "Total Reconstruction of the External Ear".
1256:
644:
Microtia in a 50-year-old woman with Saethre-Chotzen syndrome
1902:"Doctors Transplant Ear of Human Cells, Made by 3-D Printer"
942:
672:
279:
188:
Alpha thalassemia-X-linked intellectual disability syndrome
177:
Microtia is also feature of many conditions and syndromes:
304:
Diamond-Blackfan anemia 15 with mandibulofacial dysostosis
2157:
1952:
495:
Microcephalic osteodysplastic primordial dwarfism type II
1243:. New York: Thieme Medical Publishers. pp. 381–385.
154:
external ear canal producing a conductive hearing loss.
1925:
1450:
International Journal of Pediatric Otorhinolaryngology
1009:
Forrester, Mathias B.; Merz, Ruth D. (December 2005).
360:
Microtia in a 10-year-old girl with Goldenhar syndrome
1676:
1674:
1654:
1652:
1241:
Controversies in Otolaryngology–Head and Neck Surgery
2000:
1605:
1603:
1601:
1562:
1560:
1132:
892:, was born with grade III microtia of his right ear.
1842:
830:An auricular (ear) prosthesis is custom made by an
231:
Blepharophimosis - intellectual disability syndrome
225:
Blepharophimosis - intellectual disability syndrome
1671:
1649:
1485:
1196:International Journal of Surgery (London, England)
1598:
1557:
2231:
1238:
1057:
139:Bilateral grade III microtia in a 9-year-old boy
1139:Facial Plastic Surgery Clinics of North America
1252:
1250:
2138:
1919:
1787:
1765:. Cedars-Sinai Medical Center. Archived from
1008:
376:Hennekam lymphangiectasia-lymphedema syndrome
160:Grade IV: Absence of the total ear or anotia.
949:American Journal of Medical Genetics. Part A
719:Triglyceride storage disease with ichthyosis
499:Microcephaly 6, primary, autosomal recessive
1247:
2145:
2131:
1377:
1334:
569:Osteopathia striata with cranial sclerosis
470:Mandibulofacial dysostosis-macroblepharon-
164:
93:
69:
1528:
1291:
1207:
1166:
1026:
976:
685:, congenital symmetric circumferential, 2
413:Intellectual developmental disorder with
1729:Otolaryngologic Clinics of North America
1680:
1658:
697:Spondyloepiphyseal dysplasia, Cantu type
639:
355:
134:
2245:Diseases of the ear and mastoid process
1723:
1609:
1566:
945:"Microtia: Epidemiology & Genetics"
769:(beyond the scope of this discussion).
668:and Facioauriculothoracic malformations
2232:
1189:
384:12 with or without pancreatic agenesis
341:Fanconi anemia complementation group L
2126:
1899:
1881:. clinicaltrials.gov. 15 October 2021
1763:"MEDPOR Reconstruction For Mictrotia"
1407:The Cleft Palate-Craniofacial Journal
855:
617:Primordial dwarfism-immunodeficiency-
1683:Plastic & Reconstructive Surgery
1615:Plastic & Reconstructive Surgery
1572:Plastic & Reconstructive Surgery
1531:Plastic & Reconstructive Surgery
1271:10.1001/archotol.1995.01890080053010
938:
936:
934:
932:
930:
332:, junctional 6, with pyloric atresia
283:congenital disorder of glycosylation
1995:Grabb & Smith's Plastic Surgery
1661:Annals Chirurgie Plastica Esthetica
910:Online Mendelian Inheritance in Man
903:
888:, vocalist and rhythm guitarist of
806:Rib Cartilage Graft Reconstruction:
598:Periventricular nodular heterotopia
401:Intellectual developmental disorder
13:
2156:malformations and deformations of
1946:
1060:Plastic and Reconstructive Surgery
445:congenita, genital anomalies, and
14:
2256:
1900:Rabin, Roni Caryn (2 June 2022).
1190:Thomas, Daniel J. (August 2016).
1110:"Microtia (Concept Id: C0152423)"
927:
679:-intellectual disability syndrome
636:-intellectual disability syndrome
536:Neurodevelopmental disorder with
275:Chromosome 1p36 deletion syndrome
130:
127:(isotretinoin) during pregnancy.
1967:10.1097/00006534-198512000-00010
1695:10.1097/00006534-199402000-00001
1627:10.1097/00006534-199209000-00001
1584:10.1097/00006534-199908000-00001
1543:10.1097/00006534-195901000-00001
1349:10.1097/00003446-198602000-00004
1028:10.1111/j.1741-4520.2005.00080.x
879:
724:X-linked intellectual disability
626:spondyloepimetaphyseal dysplasia
464:Mandibulofacial dysostosis with
298:Diamond-Blackfan anemia 14 with
1893:
1871:
1845:Facial Plast Surg Clin North Am
1836:
1824:
1755:
1717:
1522:
1441:
1398:
1371:
1328:
1285:
1259:Arch Otolaryngol Head Neck Surg
1232:
792:
556:Oculoauriculovertebral spectrum
435:Intrauterine growth retardation
1183:
1126:
1102:
1051:
1002:
993:
918:
849:3D bioprinting for transplants
387:Holoprosencephaly 13, X-linked
312:hypergonadotropic hypogonadism
1:
1741:10.1016/s0030-6665(05)70286-3
896:
324:Epidermolysis bullosa simplex
202:spondylometaphyseal dysplasia
2240:Congenital disorders of ears
1991:Ear Reconstruction: Microtia
1462:10.1016/j.ijporl.2008.06.009
1306:10.1097/mao.0b013e3181f7ab62
1072:10.1097/PRS.0b013e3181b454d8
744:
735:
713:Trichohepatoenteric syndrome
689:Spondyloepiphyseal dysplasia
288:Complete trisomy 21 syndrome
259:Branchiooculofacial syndrome
7:
531:Neurodevelopmental disorder
522:deficiency, nuclear type 11
477:Mandibulofacial dysostosis-
196:faciodigitogenital syndrome
10:
2261:
1209:10.1016/j.ijsu.2016.06.036
752:
300:mandibulofacial dysostosis
16:For the orchid genus, see
15:
2206:
2190:
2164:
2085:
2004:
1989:Thorne, Charles (2013) "
1857:10.1016/j.fsc.2006.01.003
1294:Otology & Neurotology
1151:10.1016/j.fsc.2016.06.011
1135:"Microtia Reconstruction"
874:Treacher-Collins Syndrome
845:bone anchored hearing aid
779:bone-anchored hearing aid
707:Treacher Collins syndrome
655:Scalp-ear-nipple syndrome
427:Intellectual disability,
264:Branchiootorenal syndrome
82:
77:
68:
63:
1997:, 7th ed. Pages 283–294.
914:Microtia-Anotia - 600674
650:Saethre-Chotzen syndrome
409:behavioral abnormalities
326:5C, with pyloric atresia
208:Bartsocas-Papas syndrome
583:cleidocranial dysplasia
574:Pallister-Hall syndrome
520:Mitochondrial complex 3
338:complementation group F
293:Diamond-Blackfan anemia
165:Causes and risk factors
1802:10.1055/s-0029-1239448
1500:10.1055/s-2008-1064543
1488:Facial Plastic Surgery
701:Townes-Brocks syndrome
645:
512:-imperforation of the
485:Methylmalonic aciduria
457:Larsen-like syndrome,
361:
345:Fine-Lubinsky syndrome
308:Dilated cardiomyopathy
140:
1955:Plast. Reconstr. Surg
1851:(2): 137–45, vi–vii.
866:hemifacial microsomia
643:
439:metaphyseal dysplasia
359:
330:Epidermolysis bullosa
138:
1735:(6): 1353–65, viii.
1114:www.ncbi.nlm.nih.gov
1015:Congenital Anomalies
961:10.1002/ajmg.a.34352
730:Yunis-Varon syndrome
200:Autosomal recessive
172:gravidity and parity
109:congenital deformity
563:primordial dwarfism
558:with radial defects
213:Bilateral microtia-
193:Autosomal recessive
46:the butterfly genus
25:the gastropod genus
2086:External resources
1906:The New York Times
1831:Alexander Berghaus
1769:on 2 February 2017
1380:Cand Audiol Supple
870:Goldenhar Syndrome
856:Related conditions
822:Alexander Berghaus
693:joint dislocations
646:
634:fourth metatarsals
589:uniparental disomy
443:adrenal hypoplasia
429:autosomal dominant
371:Hemifacial atrophy
366:Goldenhar syndrome
362:
270:CHARGE association
182:46,XY sex reversal
141:
2227:
2226:
2219:Mondini dysplasia
2214:Accessory auricle
2120:
2119:
1790:Facial Plast Surg
579:Parietal foramina
514:nasolacrimal duct
405:dysmorphic facies
382:Holoprosencephaly
102:
101:
58:Medical condition
2252:
2147:
2140:
2133:
2124:
2123:
2002:
2001:
1986:
1940:
1939:
1923:
1917:
1916:
1914:
1912:
1897:
1891:
1890:
1888:
1886:
1875:
1869:
1868:
1840:
1834:
1828:
1822:
1821:
1785:
1779:
1778:
1776:
1774:
1759:
1753:
1752:
1721:
1715:
1714:
1678:
1669:
1668:
1656:
1647:
1646:
1607:
1596:
1595:
1564:
1555:
1554:
1526:
1520:
1519:
1483:
1474:
1473:
1445:
1439:
1438:
1402:
1396:
1395:
1375:
1369:
1368:
1332:
1326:
1325:
1300:(9): 1421–1426.
1289:
1283:
1282:
1254:
1245:
1244:
1236:
1230:
1229:
1211:
1187:
1181:
1180:
1170:
1130:
1124:
1123:
1121:
1120:
1106:
1100:
1099:
1066:(4): 1157–1164.
1055:
1049:
1048:
1030:
1006:
1000:
997:
991:
990:
980:
940:
925:
922:
916:
907:
767:cochlear implant
691:with congenital
612:sinus arrhythmia
561:Osteodysplastic
447:immunodeficiency
352:perinatal lethal
204:, Megarbane type
98:
97:
89:Medical genetics
73:
61:
60:
2260:
2259:
2255:
2254:
2253:
2251:
2250:
2249:
2230:
2229:
2228:
2223:
2202:
2186:
2160:
2151:
2121:
2116:
2115:
2081:
2080:
2013:
1949:
1947:Further reading
1944:
1943:
1924:
1920:
1910:
1908:
1898:
1894:
1884:
1882:
1877:
1876:
1872:
1841:
1837:
1829:
1825:
1786:
1782:
1772:
1770:
1761:
1760:
1756:
1722:
1718:
1679:
1672:
1657:
1650:
1608:
1599:
1565:
1558:
1527:
1523:
1484:
1477:
1446:
1442:
1403:
1399:
1376:
1372:
1337:Ear and Hearing
1333:
1329:
1290:
1286:
1255:
1248:
1237:
1233:
1188:
1184:
1131:
1127:
1118:
1116:
1108:
1107:
1103:
1056:
1052:
1007:
1003:
998:
994:
941:
928:
923:
919:
908:
904:
899:
882:
858:
832:anaplastologist
828:Ear Prosthesis:
795:
759:CT or CAT scans
755:
747:
738:
726:, van Esch type
660:Seckel syndrome
540:and poor growth
395:facial clefting
350:Gaucher disease
241:choanal atresia
239:abnormalities,
167:
133:
121:microtia-anotia
92:
59:
56:
12:
11:
5:
2258:
2248:
2247:
2242:
2225:
2224:
2222:
2221:
2216:
2210:
2208:
2204:
2203:
2201:
2200:
2194:
2192:
2188:
2187:
2185:
2184:
2179:
2174:
2168:
2166:
2162:
2161:
2150:
2149:
2142:
2135:
2127:
2118:
2117:
2114:
2113:
2102:
2090:
2089:
2087:
2083:
2082:
2079:
2078:
2067:
2056:
2045:
2030:
2014:
2009:
2008:
2006:
2005:Classification
1999:
1998:
1987:
1948:
1945:
1942:
1941:
1918:
1892:
1870:
1835:
1823:
1780:
1754:
1716:
1670:
1648:
1621:(3): 355–374.
1597:
1578:(2): 319–334.
1556:
1521:
1494:(4): 274–277.
1475:
1456:(10): 1455–9.
1440:
1419:10.1597/08-178
1397:
1370:
1327:
1284:
1246:
1231:
1182:
1145:(4): 577–591.
1125:
1101:
1050:
1021:(4): 119–124.
1001:
992:
955:(1): 124–139.
926:
917:
901:
900:
898:
895:
894:
893:
881:
878:
857:
854:
853:
852:
825:
810:
794:
791:
754:
751:
746:
743:
737:
734:
733:
732:
727:
721:
716:
710:
704:
698:
695:
686:
680:
669:
663:
657:
652:
638:
637:
622:
615:
601:
595:
585:
576:
571:
566:
559:
553:
551:point mutation
544:
541:
534:
528:
526:Myhre syndrome
523:
517:
506:
500:
497:
492:
489:homocystinuria
482:
475:
468:
462:
455:
454:-like syndrome
449:
432:
425:
411:
398:
388:
385:
379:
373:
368:
354:
353:
347:
342:
339:
336:Fanconi anemia
333:
327:
321:
315:
305:
302:
296:
290:
285:
277:
272:
267:
261:
256:
253:hypothyroidism
237:Branchial arch
234:
228:
222:
211:
205:
198:
190:
185:
166:
163:
162:
161:
158:
155:
148:
132:
131:Classification
129:
100:
99:
86:
80:
79:
75:
74:
66:
65:
57:
36:the vole genus
9:
6:
4:
3:
2:
2257:
2246:
2243:
2241:
2238:
2237:
2235:
2220:
2217:
2215:
2212:
2211:
2209:
2205:
2199:
2196:
2195:
2193:
2189:
2183:
2180:
2178:
2175:
2173:
2170:
2169:
2167:
2163:
2159:
2155:
2148:
2143:
2141:
2136:
2134:
2129:
2128:
2125:
2112:
2108:
2107:
2103:
2101:
2097:
2096:
2092:
2091:
2088:
2084:
2077:
2073:
2072:
2068:
2066:
2062:
2061:
2057:
2055:
2051:
2050:
2046:
2044:
2040:
2039:
2035:
2031:
2029:
2025:
2024:
2020:
2016:
2015:
2012:
2007:
2003:
1996:
1992:
1988:
1984:
1980:
1976:
1972:
1968:
1964:
1961:(6): 859–65.
1960:
1956:
1951:
1950:
1937:
1933:
1929:
1922:
1907:
1903:
1896:
1880:
1874:
1866:
1862:
1858:
1854:
1850:
1846:
1839:
1832:
1827:
1819:
1815:
1811:
1807:
1803:
1799:
1795:
1791:
1784:
1768:
1764:
1758:
1750:
1746:
1742:
1738:
1734:
1730:
1726:
1720:
1712:
1708:
1704:
1700:
1696:
1692:
1689:(2): 221–30.
1688:
1684:
1677:
1675:
1666:
1662:
1655:
1653:
1644:
1640:
1636:
1632:
1628:
1624:
1620:
1616:
1612:
1606:
1604:
1602:
1593:
1589:
1585:
1581:
1577:
1573:
1569:
1563:
1561:
1552:
1548:
1544:
1540:
1536:
1532:
1525:
1517:
1513:
1509:
1505:
1501:
1497:
1493:
1489:
1482:
1480:
1471:
1467:
1463:
1459:
1455:
1451:
1444:
1436:
1432:
1428:
1424:
1420:
1416:
1412:
1408:
1401:
1393:
1389:
1385:
1381:
1374:
1366:
1362:
1358:
1354:
1350:
1346:
1342:
1338:
1331:
1323:
1319:
1315:
1311:
1307:
1303:
1299:
1295:
1288:
1280:
1276:
1272:
1268:
1264:
1260:
1253:
1251:
1242:
1235:
1227:
1223:
1219:
1215:
1210:
1205:
1201:
1197:
1193:
1186:
1178:
1174:
1169:
1164:
1160:
1156:
1152:
1148:
1144:
1140:
1136:
1129:
1115:
1111:
1105:
1097:
1093:
1089:
1085:
1081:
1077:
1073:
1069:
1065:
1061:
1054:
1046:
1042:
1038:
1034:
1029:
1024:
1020:
1016:
1012:
1005:
996:
988:
984:
979:
974:
970:
966:
962:
958:
954:
950:
946:
939:
937:
935:
933:
931:
921:
915:
911:
906:
902:
891:
887:
884:
883:
880:Notable cases
877:
875:
871:
867:
862:
861:Aural atresia
850:
846:
842:
838:
833:
829:
826:
823:
819:
818:John Reinisch
814:
811:
807:
804:
803:
802:
799:
790:
786:
782:
780:
774:
770:
768:
762:
760:
750:
742:
731:
728:
725:
722:
720:
717:
714:
711:
708:
705:
702:
699:
696:
694:
690:
687:
684:
681:
678:
675:-dysmorphism-
674:
670:
667:
666:Short stature
664:
661:
658:
656:
653:
651:
648:
647:
642:
635:
631:
630:short stature
627:
623:
620:
619:lipodystrophy
616:
613:
609:
605:
602:
599:
596:
594:
593:chromosome 14
590:
586:
584:
580:
577:
575:
572:
570:
567:
564:
560:
557:
554:
552:
548:
545:
542:
539:
535:
532:
529:
527:
524:
521:
518:
515:
511:
507:
505:
501:
498:
496:
493:
490:
486:
483:
480:
476:
473:
469:
467:
463:
460:
456:
453:
450:
448:
444:
440:
436:
433:
431:1, 43, and 53
430:
426:
424:
420:
416:
412:
410:
406:
402:
399:
396:
392:
391:Hypertelorism
389:
386:
383:
380:
377:
374:
372:
369:
367:
364:
363:
358:
351:
348:
346:
343:
340:
337:
334:
331:
328:
325:
322:
319:
316:
313:
309:
306:
303:
301:
297:
294:
291:
289:
286:
284:
281:
278:
276:
273:
271:
268:
265:
262:
260:
257:
254:
250:
246:
242:
238:
235:
232:
229:
226:
223:
220:
216:
212:
209:
206:
203:
199:
197:
194:
191:
189:
186:
183:
180:
179:
178:
175:
173:
159:
156:
153:
149:
146:
145:
144:
137:
128:
126:
122:
118:
114:
110:
106:
96:
90:
87:
85:
81:
76:
72:
67:
62:
54:
52:
47:
43:
42:
37:
33:
31:
26:
22:
20:
2198:Low-set ears
2176:
2104:
2093:
2069:
2058:
2047:
2032:
2017:
1994:
1958:
1954:
1927:
1921:
1909:. Retrieved
1905:
1895:
1883:. Retrieved
1873:
1848:
1844:
1838:
1826:
1796:(3): 181–9.
1793:
1789:
1783:
1771:. Retrieved
1767:the original
1757:
1732:
1728:
1719:
1686:
1682:
1664:
1660:
1618:
1614:
1575:
1571:
1534:
1530:
1524:
1491:
1487:
1453:
1449:
1443:
1413:(1): 56–65.
1410:
1406:
1400:
1383:
1379:
1373:
1343:(1): 14–19.
1340:
1336:
1330:
1297:
1293:
1287:
1265:(8): 885–6.
1262:
1258:
1240:
1234:
1199:
1195:
1185:
1142:
1138:
1128:
1117:. Retrieved
1113:
1104:
1063:
1059:
1053:
1018:
1014:
1004:
995:
952:
948:
920:
905:
886:Paul Stanley
859:
827:
812:
805:
800:
796:
793:External ear
787:
783:
775:
771:
763:
756:
748:
739:
683:Skin creases
624:Progressive
608:ectrodactyly
510:eye coloboma
479:microcephaly
452:Isotretinoin
423:speech delay
415:macrocephaly
393:, microtia,
249:hearing loss
219:cleft palate
176:
168:
142:
120:
104:
103:
50:
40:
29:
18:
1928:GeneReviews
1537:(1): 1–15.
472:macrostomia
233:, Ohdo type
53:(butterfly)
32:(gastropod)
2234:Categories
2154:Congenital
2071:DiseasesDB
1773:19 October
1119:2023-11-18
897:References
610:-deafness-
604:Phocomelia
538:spasticity
227:, MKB type
111:where the
2095:eMedicine
1818:260135290
1218:1743-9159
1202:: 43–44.
1159:1064-7406
1080:1529-4242
1037:0914-3505
969:1552-4825
745:Treatment
736:Diagnosis
677:hypotonia
587:Paternal
549:due to a
508:Microtia-
502:Microtia-
491:type cblF
84:Specialty
2191:Position
2177:Microtia
2172:Macrotia
2106:Orphanet
2100:ped/3003
1983:25652076
1936:20301704
1865:16750771
1810:19809950
1749:11449792
1711:39161797
1643:24327009
1592:10654675
1551:13633474
1516:34448986
1470:18667244
1435:46151637
1427:20180710
1386:: 75–9.
1365:11839568
1322:23454415
1314:21113986
1226:27353851
1177:27712823
1088:19935299
1045:16359491
987:22106030
912:(OMIM):
841:titanium
839:or with
837:adhesive
621:syndrome
614:syndrome
565:, type 1
516:syndrome
481:syndrome
474:syndrome
466:alopecia
419:seizures
397:syndrome
314:syndrome
255:syndrome
221:syndrome
215:deafness
152:stenotic
125:Accutane
105:Microtia
64:Microtia
51:Microtia
41:Microtus
30:Microtis
19:Microtis
2065:D065817
1975:4070453
1911:19 July
1885:19 July
1725:Brent B
1703:8310014
1635:1513882
1611:Brent B
1568:Brent B
1508:9046615
1392:3067327
1357:3949096
1279:7619415
1168:5950715
1096:3388083
978:3482263
753:Hearing
703:1 and 2
632:-short
320:1 and 3
245:athelia
113:auricle
21:(plant)
2182:Anotia
2054:600674
2043:744.23
1981:
1973:
1934:
1863:
1816:
1808:
1747:
1709:
1701:
1667:: 119.
1641:
1633:
1590:
1549:
1514:
1506:
1468:
1433:
1425:
1390:
1363:
1355:
1320:
1312:
1277:
1224:
1216:
1175:
1165:
1157:
1094:
1086:
1078:
1043:
1035:
985:
975:
967:
798:as 5.
709:1 to 3
671:Short
504:Anotia
459:B3GAT3
421:, and
251:, and
117:anotia
91:
48:, and
2207:Other
2111:83463
2076:29876
2028:Q17.2
1979:S2CID
1814:S2CID
1707:S2CID
1639:S2CID
1512:S2CID
1431:S2CID
1361:S2CID
1318:S2CID
1092:S2CID
581:with
403:with
107:is a
2165:Size
2158:ears
2060:MeSH
2049:OMIM
2038:9-CM
1971:PMID
1932:PMID
1913:2022
1887:2022
1861:PMID
1806:PMID
1775:2011
1745:PMID
1699:PMID
1631:PMID
1588:PMID
1547:PMID
1504:PMID
1466:PMID
1423:PMID
1388:PMID
1353:PMID
1310:PMID
1275:PMID
1222:PMID
1214:ISSN
1173:PMID
1155:ISSN
1084:PMID
1076:ISSN
1041:PMID
1033:ISSN
983:PMID
965:ISSN
953:158A
890:Kiss
673:ulna
487:and
461:type
407:and
280:COG1
2034:ICD
2019:ICD
1993:".
1963:doi
1853:doi
1798:doi
1737:doi
1691:doi
1623:doi
1580:doi
1576:104
1539:doi
1496:doi
1458:doi
1415:doi
1345:doi
1302:doi
1267:doi
1263:121
1204:doi
1163:PMC
1147:doi
1068:doi
1064:124
1023:doi
973:PMC
957:doi
872:or
591:of
2236::
2109::
2098::
2074::
2063::
2052::
2041::
2026::
2023:10
1977:.
1969:.
1959:76
1957:.
1930:.
1904:.
1859:.
1849:14
1847:.
1812:.
1804:.
1794:25
1792:.
1743:.
1733:33
1731:.
1705:.
1697:.
1687:93
1685:.
1673:^
1663:.
1651:^
1637:.
1629:.
1619:90
1617:.
1600:^
1586:.
1574:.
1559:^
1545:.
1535:23
1533:.
1510:.
1502:.
1492:11
1490:.
1478:^
1464:.
1454:72
1452:.
1429:.
1421:.
1411:48
1409:.
1384:30
1382:.
1359:.
1351:.
1339:.
1316:.
1308:.
1298:31
1296:.
1273:.
1261:.
1249:^
1220:.
1212:.
1200:32
1198:.
1194:.
1171:.
1161:.
1153:.
1143:24
1141:.
1137:.
1112:.
1090:.
1082:.
1074:.
1062:.
1039:.
1031:.
1019:45
1017:.
1013:.
981:.
971:.
963:.
951:.
947:.
929:^
868:,
441:,
437:,
417:,
295:10
247:,
243:,
44:,
38:,
34:,
27:,
23:,
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2139:t
2132:v
2036:-
2021:-
2011:D
1985:.
1965::
1938:.
1915:.
1889:.
1867:.
1855::
1820:.
1800::
1777:.
1751:.
1739::
1713:.
1693::
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1645:.
1625::
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1582::
1553:.
1541::
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1498::
1472:.
1460::
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1417::
1394:.
1367:.
1347::
1341:7
1324:.
1304::
1281:.
1269::
1228:.
1206::
1179:.
1149::
1122:.
1098:.
1070::
1047:.
1025::
989:.
959::
851:.
824:.
715:1
662:7
628:-
606:-
600:9
378:2
310:-
266:1
217:-
210:1
184:4
55:.
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
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