Pediatrics

Causes of Low Set Ears in Infants



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"Causes of Low Set Ears in Infants"
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Ear development can be influenced by both genetic as well as other factors during the fetal life and newborn babies can show differences in the appearance of their ear lobes or the pinna, its size, shape, positioning…etc. Usually, the outer rim of the ear, the helix, will meet the skull at a level which is above a horizontal plane at the level of inner corners of both eyes and if the ears appear two standard deviations below, it would be considered as ‘low set ears’. In many instances, the condition is considered to be part of many other symptoms which might be indicative of a congenital abnormality.

Thus, there are several congenital abnormalities that will show ‘low set ears’ as one of its characteristic features in combination with other inherited signs and some of these conditions are,

*Downs syndrome

*Turner syndrome

*Noonan syndrome

*Goldenhar syndrome

Downs syndrome:

The condition is also known as ‘trisomy 21’ and is a common chromosomal defect seen in babies born to elderly women or else in babies born to parents who are blood relatives. The incidence of Downs syndrome would rise with the increased maternal age and there will be many other signs associated with this chromosomal defect. Thus, apart from the ‘low set ears’, the condition will also have sings such as epicanthic folds, flat nasal bridge, slanting eyes, protruding large tongue, mental retardation, heart defects…etc.

Turner syndrome:

Being another chromosomal abnormality, the main underlying problem seems to be a missing chromosome which relates to ‘gender’ determination or the ‘X’ chromosome. The ‘low set ears’ would be one of the characteristic features of this condition and some of the other associated signs of Turner syndrome would be,

*Short stature

*Webbed neck

*Flat, shield shaped chest

*Ambiguous genitals…etc.

Noonan syndrome:

This condition can be considered as a variation of Turner syndrome and apart from the ‘low set ears’, it will be characterized by short stature, indented chest, heart defects, learning problems as well as impaired blood clotting. The condition can also mimic certain other congenital disorders such as ‘fetal alcohol syndrome’ and ‘leopard syndrome’ where distinguishing between the disorders needed to be done through ‘karyotyping’.

Goldenhar syndrome:

This is relatively a rare congenital development disorder characterized by development failures of the ear, mainly on one side of the skull. The condition can also be associated with other congenital malformations of the nose, palate, lip as well as the mandible.

Thus, it would be evident that ‘low set ears’ does not usually appear in isolation and in such instances, other congenital abnormalities needs to be excluded and this finding per se would not determine the presence of a congenital abnormality as well.

More about this author: Dr Pandula Siribaddana

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