Neonatology Book



Congenital Abnormality

  • Congenital Ear Anomaly

Jaw Disorders

Neck Disease


Congenital Ear Anomaly

Aka: Congenital Ear Anomaly, Congenital Ear Malformation, Low-Set Ears, Low Set Ears, Congenital Small Ears, Microtia, Pre-auricular Skin Tag
  1. See Also
    1. Newborn Head and Neck Exam
  2. Definitions
    1. Low-Set Ears
      1. Pinna falls below the horizontal level of the medial canthus of the eye (or line drawn from the lateral canthus to the occipital protuberance)
      2. Ear Pinna is rotated posteriorly
      3. Caused by several congenital disorders (see below)
      4. Associated with hearing deficit (obtain Newborn Hearing Screening) and genitourinary anomalies (obtain renal Ultrasound)
    2. Microtia (Congenital Small Ears)
      1. Small, undeveloped pinna or absent pinna in the case of anotia
      2. May be associated with hearing deficit (obtain Newborn Hearing Screening)
    3. Pre-auricular Skin Tags, ear pits, fissures or sinuses
      1. Growths or defects at the anterior Ear Pinna
      2. May be associated with hearing deficits (obtain Newborn Hearing Screening)
      3. Renal Ultrasound not indicated unless other dysmorphic features, Teratogen Exposure, deafness Family History or maternal diabetes
  3. Causes: Low-Set Ears
    1. Trisomy 21 (Down Syndrome)
    2. Trisomy 18
  4. Causes: Microtia
    1. CHARGE Syndrome
    2. Goldenhar Syndrome
    3. Treacher-Collins Syndrome
  5. References
    1. Lewis (2014) Am Fam Physician 90(5): 289-96 [PubMed]

Congenital small ears (C0152423)

Concepts Congenital Abnormality (T019)
MSH D065817
ICD9 744.23
ICD10 Q17.2
SnomedCT 247242007, 35045004
Italian Microzia
Japanese 小耳症, ショウジショウ
English MICROTIA, microtia, microtia (physical finding), ear auricle malformations microtia, microtia was noted, microtia (diagnosis), Microtia, Microtias, Microtia (disorder), Congenital small ears
Portuguese MICROTIA, Microtia
French MICROTIE, Microtie
Czech Mikrocie
Korean 작은귀증
Hungarian Microtia
Dutch Microtie, microtie
Spanish microtia (trastorno), microtia, Microtía
German Mikrotie
Derived from the NIH UMLS (Unified Medical Language System)

Low set ears (C0239234)

Concepts Congenital Abnormality (T019)
ICD10 Q17.4
SnomedCT 204260001, 95515009
Italian Orecchie ad impianto basso
Japanese 低位耳介, テイイジカイ
English low set ears (physical finding), low set ears, ear auricle malformations low set ears, ears low set, Low-set ear, Low-set ears, Low set ears, Low set ears (disorder), low; set ears,, low; set ears, set ears; low
Czech Nízko posazené uši
Hungarian Alacsonyan ülő fülek
Dutch implantatie; oorschelp laag, laag; implantatie oorschelp, laag; implantatie, oorschelpen, oren laag aangezet
Spanish implantación baja de las orejas (trastorno), implantación baja de las orejas, Implantación baja de las orejas
Portuguese Orelhas de inserção baixa
French Oreilles basses implantées
German niedrig ansetzende Ohren
Derived from the NIH UMLS (Unified Medical Language System)

Congenital ear anomaly NOS (disorder) (C0266589)

Concepts Congenital Abnormality (T019)
ICD9 744.3
ICD10 Q17.9
SnomedCT 268312004, 268311006, 204262009, 156903001, 156906009, 275259005, 8380000
English Unspecified congenital anomaly of ear, Congenital ear anomaly NOS, Congenital malformation of ear, unspecified, Ear anomalies, CUP EAR, EAR MALFORMATION, congenital deformity of ear (diagnosis), congenital deformity of ear, Congenital ear malformation NOS, Ear malformation NOS, Congenital ear anomaly NOS (disorder), Ear anomaly NOS, Congenital anomaly of ear NOS, Congenital anomaly;ear, congenital ear malformation, malformation of ear, birth defects ear, ear malformations, congenital anomaly ear, ear malformation, congenital ear anomaly, Unspecified anomaly of ear, Ear -congenital anomaly, Malformation of ear, Congenital anomaly of ear, Congenital deformity of ear, Congenital anomaly of ear (disorder), Congenital abnormality of ear, Ear malformation, Congenital malformation of ear, Congenital malformation of ear (disorder), deformity; ear, congenital, ear; anomaly, ear; deformity, congenital, anomaly; ear, Congenital anomaly of ear, NOS, Congenital deformity of ear, NOS, Malformation of ear, NOS, congenital anomaly of the ear
Italian Malformazione dell'orecchio, Anomalie congenite dell'orecchio NAS, Malformazione dell'orecchio NAS, Anomalie congenite non specificate dell'orecchio
Dutch oormisvorming NAO, niet-gespecificeerde congenitale ooranomalie, oormisvorming NAO, congenitaal, Aangeboren afwijking oor, anomalie; oor, misvorming; oor, congenitaal, oor; anomalie, oor; misvorming, congenitaal, Congenitale misvorming van oor, niet gespecificeerd, oormisvorming
French Malformation congénitale de l'oreille SAI, Anomalie congénitale de l'oreille non précisée, Malformation de l'oreille SAI, MALFORMATION DE L'OREILLE, Malformation de l'oreille
German unspezifische kongenitale Anomalie des Ohrs, kongenitale Missbildung des Ohrs NNB, Missbildung des Ohrs NNB, Angeborene Fehlbildung des Ohres, nicht naeher bezeichnet, OHRENMISSBILDUNG, Missbildung des Ohrs
Portuguese Malformação do ouvido NE, Malformação congénita do ouvido NE, Anomalia congénita NE do ouvido, MALFORMACAO DO OUVIDO, Malformação da orelha
Spanish Anomalía congénita no especificada del oído, Malformación congénita del oído NEOM, Malformación del oído NEOM, MALFORMACION OIDO, anomalía congénita del oído, SAI, anomalía congénita del oído, SAI (trastorno), anomalía congénita del oído (trastorno), anomalía congénita del oído, deformidad congénita del oído, malformación del oído, Malformación del oído, malformación congénita del oído (trastorno), malformación congénita del oído
Japanese 先天性耳奇形NOS, 耳奇形, 詳細不明の耳の先天異常, 耳奇形NOS, センテンセイジキケイNOS, センテンセイミミキケイNOS, ミミキケイ, ジキケイNOS, ショウサイフメイノミミノセンテンイジョウ, ジキケイ, ミミキケイNOS
Czech Malformace ucha NOS, Malformace ucha, Vrozená malformace ucha NOS, Blíže neurčená vrozená anomálie ucha
Korean 상세불명의 귀의 선천 기형
Hungarian Veleszületett fül malformatio k.m.n., Fül malformatio, Fül malformatio k.m.n., Fül veleszületett nem meghatározott anomaliája
Derived from the NIH UMLS (Unified Medical Language System)

Preauricular skin tag (C1860816)

Concepts Finding (T033)
English Preauricular skin tags, skin tag of preauricular area of ear (physical finding), skin tag of preauricular area of ear, Preauricular skin tag
Czech Preaurikulární kožní přívěsek
Dutch preauriculaire skintag
French Tatouage préauriculaire
German Praeaurikulaeres Akrochordon
Hungarian Preauricular bőrkinövés
Italian Appendici cutanee preauricolari
Spanish Apéndices preauriculares
Japanese 副耳, フクミミ, フクジ
Portuguese Apêndices pré-auriculares
Derived from the NIH UMLS (Unified Medical Language System)

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