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{{short description|Clinical sign of congenital conditions}}
{{Infobox medical condition (new)
{{Infobox medical condition (new)
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'''Low-set ears''' are ears with depressed positioning of the [[pinnae]] two or more [[standard deviations]] below the population average.<ref>{{cite journal |vauthors=Sivan Y, Merlob P, Reisner SH |title=Assessment of ear length and low set ears in newborn infants |journal=J. Med. Genet. |volume=20 |issue=3 |pages=213–5 |date=June 1983 |pmid=6876114 |pmc=1049049 |doi= 10.1136/jmg.20.3.213|url=}}</ref>
'''Low-set ears''' are a [[Clinical examination|clinical]] feature in which the [[Ear|ears]] are positioned lower on the head than usual. They are present in many [[Congenital condition|congenital conditions]]. Low-set ears are defined as the [[outer ears]] being positioned two or more [[standard deviations]] lower than the population average.<ref name=":0">{{cite journal |vauthors=Sivan Y, Merlob P, Reisner SH |title=Assessment of ear length and low set ears in newborn infants |journal=J. Med. Genet. |volume=20 |issue=3 |pages=213–5 |date=June 1983 |pmid=6876114 |pmc=1049049 |doi= 10.1136/jmg.20.3.213}}</ref> Clinically, if the point at which the [[Helix (ear)|helix]] (curved upper part) of the [[outer ear]] meets the [[cranium]] is at or below the line connecting the inner [[Canthus|canthi]] of eyes (the [[bicanthal plane]]), the ears are considered low set.<ref>{{Cite journal|last=Lewis|first=Mary L.|date=2014-09-01|title=A Comprehensive Newborn Exam: Part I. General, Head and Neck, Cardiopulmonary|url=https://www.aafp.org/afp/2014/0901/p289.html|journal=American Family Physician|volume=90|issue=5|pages=289–296|issn=0002-838X}}</ref><ref name=":0" />


It can be associated with conditions such as:
Low-set ears can be associated with conditions such as:
*[[Down syndrome]]<ref name="urlDowns Syndrome">{{cite web |url=http://www.patient.co.uk/showdoc/40000383/ |title=Down's Syndrome |format= |website= |accessdate=}}</ref>
*[[Down syndrome]]<ref name="urlDowns Syndrome">{{cite web |url=http://www.patient.co.uk/showdoc/40000383/ |title=Down's Syndrome }}</ref>
*[[Turner syndrome]]
*[[Turner syndrome]]
*[[Noonan syndrome]]<ref>{{Cite journal | pmid = 6884370 | year = 1983 | last1 = Sanchez-Cascos | first1 = A. | title = The Noonan syndrome | volume = 4 | issue = 4 | pages = 223–229 | journal = European Heart Journal | doi = 10.1093/oxfordjournals.eurheartj.a061452 }}</ref>
*[[Noonan syndrome]]<ref>{{Cite journal | pmid = 6884370 | year = 1983 | last1 = Sanchez-Cascos | first1 = A. | title = The Noonan syndrome | volume = 4 | issue = 4 | pages = 223–229 | journal = European Heart Journal | doi = 10.1093/oxfordjournals.eurheartj.a061452 }}</ref>
*[[Patau syndrome]]<ref name="Ostler2004">{{cite book|author=H. Bruce Ostler|title=Diseases of the eye and skin: a color atlas|url=https://books.google.com/books?id=jewuK_B-3bcC&pg=PA72|accessdate=13 April 2010|year=2004|publisher=Lippincott Williams & Wilkins|isbn=978-0-7817-4999-2|page=72}}</ref>
*[[Patau syndrome]]<ref name="Ostler2004">{{cite book|author=H. Bruce Ostler|title=Diseases of the eye and skin: a color atlas|url=https://books.google.com/books?id=jewuK_B-3bcC&pg=PA72|access-date=13 April 2010|year=2004|publisher=Lippincott Williams & Wilkins|isbn=978-0-7817-4999-2|page=72}}</ref>
*[[DiGeorge syndrome]]<ref name="CrocettiBarone2004">{{cite book|author1=Michael Crocetti|author2=Michael A. Barone|author3=Frank A. Oski|title=Oski's essential pediatrics|url=https://books.google.com/books?id=I3Kh1cNJxyUC&pg=PA675|accessdate=27 October 2010|year=2004|publisher=Lippincott Williams & Wilkins|isbn=978-0-7817-3770-8|page=675}}</ref>
*[[DiGeorge syndrome]]<ref name="CrocettiBarone2004">{{cite book|author1=Michael Crocetti|author2=Michael A. Barone|author3=Frank A. Oski|title=Oski's essential pediatrics|url=https://books.google.com/books?id=I3Kh1cNJxyUC&pg=PA675|access-date=27 October 2010|year=2004|publisher=Lippincott Williams & Wilkins|isbn=978-0-7817-3770-8|page=675}}</ref>
*[[Cri du chat syndrome]]
*[[Cri du chat syndrome]]
*[[Edwards syndrome]]
*[[Edwards syndrome]]
*[[Fragile X syndrome]]
*[[Fragile X syndrome]]
*[[Okamoto syndrome]]

It is usually bilateral, but it can be unilateral in [[Goldenhar syndrome]].<ref>{{Cite GPnotebook|275447838|ear (low set)}}</ref>
It is usually [[Bilateral symmetry|bilateral]], but it can be unilateral (one sided) in [[Goldenhar syndrome]].<ref>{{Cite GPnotebook|275447838|ear (low set)}}</ref>

A lethal form of low-set ears manifests in [[otocephaly]], a very rare disorder characterized by [[agnathia|absence of the mandible (agnathia)]] and partial or total merging of the ears at the midline (synotia). Due to the extent of craniofacial malformations, fetuses with otocephaly never survive to birth. Otocephaly occurs 1 in every 70,000 embryos.<ref>{{cite journal|last1=Jagtap|first1=Sunil V|year=2015|title=Otocephaly: Agnathia- Microstomia-Synotia Syndrome– A Rare Congenital Anomaly|journal=Journal Of Clinical And Diagnostic Research|doi=10.7860/jcdr/2015/13636.6444|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4606241}}</ref>


==See also==
==See also==

Latest revision as of 00:15, 31 January 2023

Low-set ears
SpecialtyMedical genetics Edit this on Wikidata

Low-set ears are a clinical feature in which the ears are positioned lower on the head than usual. They are present in many congenital conditions. Low-set ears are defined as the outer ears being positioned two or more standard deviations lower than the population average.[1] Clinically, if the point at which the helix (curved upper part) of the outer ear meets the cranium is at or below the line connecting the inner canthi of eyes (the bicanthal plane), the ears are considered low set.[2][1]

Low-set ears can be associated with conditions such as:

It is usually bilateral, but it can be unilateral (one sided) in Goldenhar syndrome.[7]

See also

[edit]

References

[edit]
  1. ^ a b Sivan Y, Merlob P, Reisner SH (June 1983). "Assessment of ear length and low set ears in newborn infants". J. Med. Genet. 20 (3): 213–5. doi:10.1136/jmg.20.3.213. PMC 1049049. PMID 6876114.
  2. ^ Lewis, Mary L. (2014-09-01). "A Comprehensive Newborn Exam: Part I. General, Head and Neck, Cardiopulmonary". American Family Physician. 90 (5): 289–296. ISSN 0002-838X.
  3. ^ "Down's Syndrome".
  4. ^ Sanchez-Cascos, A. (1983). "The Noonan syndrome". European Heart Journal. 4 (4): 223–229. doi:10.1093/oxfordjournals.eurheartj.a061452. PMID 6884370.
  5. ^ H. Bruce Ostler (2004). Diseases of the eye and skin: a color atlas. Lippincott Williams & Wilkins. p. 72. ISBN 978-0-7817-4999-2. Retrieved 13 April 2010.
  6. ^ Michael Crocetti; Michael A. Barone; Frank A. Oski (2004). Oski's essential pediatrics. Lippincott Williams & Wilkins. p. 675. ISBN 978-0-7817-3770-8. Retrieved 27 October 2010.
  7. ^ "ear (low set)". GPnotebook.
[edit]


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