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Subjective assessments of shortness of the ear should only be used if unavoidable. The commonly used term Microtia is a bundled term comprising decreased length and width surface area. Definition : Defect in the continuity of the helix, which may occur at any point along its length Figs. Normal anatomy of the helix. Variation in helix formation among the group of clinical geneticists involved in defining morphology.

If the defect or notch occurs at the junction of the superior and descending portions of the helix, it should be coded as Darwin notch of the helix. Definition : Linear, circumferential indentation in the convexity of the outer surface of the helix Fig. Comment : The crimp is usually found in the middle third of the descending helix. The helix has the appearance of having been pinched or flattened along its posterior margin. The crimp may distort the free margin of the helix.

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Definition : Continuum between the tragus and ascending helix, without any evidence of a posterior extension crus towards the concha Fig. Definition : Extension of the ridge of the crus helix across the ear and connection of the crus to the antihelix Fig. Definition : Widening, rather than tapering, of the crus at its posterior border near the antihelix Fig. Definition : Main axis of the crus of the helix perpendicular to the medial longitudinal axis of the ear, instead of sloping inferoposteriorly Fig. It is preferable to simply describe each component separately.

Helix, crus, hyperplastic: see Helix, crus, prominent. Helix, crus, hypertrophic: see Helix, crus, prominent.

Helix, crus, hypoplastic: see Helix, crus, underdeveloped. Helix, crus, hypotrophic: see Helix, crus, underdeveloped. Definition : Development of the crus helix to the same degree as an average antihelix stem or helix Fig. Degree of development of the crus helix. Judgment of prominence is highly subjective and may be influenced by the relative development of the other ear components. There appears to be a correlation between the length of the crus helix and its relative prominence, but these should be coded separately.

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Definition : Curving course of the crus of the helix, approaching or joining the antitragus Fig. Definition : The anterior origin of the crus encompasses the superior margin of the tragus, the crus overrides the upper portion of the conchal cavum and ends at the antihelix Fig. Tragal bridge of crus of helix. There appears to be a correlation between the length of the crus helix and its relative prominence. Definition : Small defect of the helical fold that lies at the junction of the superior and descending portions of the helix Fig.

Definition : Small expansion of the helical fold at the junction of the superior and descending portions of the helix Fig. Definition : Interruption between the ascending helix and the crus helix, allowing the ascending helix to be attached directly to the mastoid area Fig. Discontinuous ascending root of helix reprinted with permission from Park and Roh, Definition : Excessive curling of the helix edge, whereby the free edge is parallel to the plane of the ear Figs.

Comments : This is most often seen in the superior helix where it must be distinguished from a Lop ear where the usual convexity of the posterior border of the ear is lost. Helix folding is highly variable, and the range is illustrated in Figures 29 and Definition : Permanent indentation on the posteromedial aspect of the helix that may be sharply or indistinctly delineated Fig.

Comment : They are usually linear to a narrow oval in shape and may be single or multiple [Prescott and Hennekam, ]. Definition : Flattening instead of curving or rounded superior helix, allowing the superior helix to run more horizontally than usual Fig. Comment : This is not to be confused with Lop ear or Satyr ear and may represent an underdevelopment of the upper third of the pinna. This is usually associated with a short ascending helix.

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Definition : Underdevelopment of the helix that either affects the entire helix, or is localized Fig. Comment : Helix folding is highly variable, and the range is illustrated in Figures 29 and To use this term, the affected area must be too long to be considered a Cleft helix. Underdevelopment of part of the helix can lead to the impression that the scaphal area is enlarged.

Comments : See Attached lobe for a finding that should be distinguished from Absent lobe.

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Definition : Sharply demarcated, typically linear and approximately horizontal, indentations in the outer surface of the ear lobe Fig. Comment : Shallow grooves or indentations are quite common, especially in large lobes. Ear lobe creases may arise postnatally [Chitayat et al.

Posterior helical pits can be a related finding but should be assessed and coded separately. Definition : Attachment of the lobe to the side of the face at the lowest point of the lobe without curving upward Fig. Definition : Discontinuity in the convexity of the inferior margin of the lobe Fig. Comment : The cleft is often more visible if the lobe is pulled forward or when seen from behind.

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Tears acquired from earrings should be distinguished. Definition : Positioning of the anterior surface of the ear lobe in a more coronal plane than the remainder of the ear Fig. Comment : The lobe should be viewed from the front. This feature is distinct from the situation where the entire ear is forward facing and prominent as shown in Fig. The lobe normally lies more or less in the same plane as the remainder of the ear.

This feature should be distinguished from Uplifted lobe. Definition : Increased volume of the earlobe Fig. Variations in volume of the earlobe. Comments : All gradations in size of the earlobe may be seen from absent to clearly enlarged compared to average. This finding is highly variable, and the range is illustrated in Figure Lobe size increases throughout adulthood [Ferrario et al. Definition : Reduced volume of the earlobe Fig. Definition : Lateral surface of ear lobe faces superiorly Fig.

Definition : Anterior and inferior folding of the upper portion of the ear that obliterates triangular fossa and scapha Fig. Comments : Mild forms are limited to the superior ear, more severe forms affect the superior and posterior ear. The concha may be excessively concave. This should be distinguished from an Overfolded helix where the external contour of the ear is normal. Definition : Median longitudinal ear length greater than 2 SD above the mean and median ear width greater than 2 SD above the mean Fig.

Comment : This is acknowledged to be a bundled term but retained here because of its usefulness in practice.

Ear length is determined by the maximal distance from the superior aspect to the inferior aspect of the external ear. If only length is increased the term Long ear should be used. Normal values for ear width are available [Farkas, ]. The definitions of microtia below follow a widely used, surgically based, classification of ear anomalies outlined by Weerda As microtia indicates at least both decreased length and width, and in more severe forms it includes abnormal shape of structures, all forms are acknowledged to be bundled terms, but are retained here because they are well established.

Definition : Presence of all the normal ear components and the median longitudinal length more than 2 SD below the mean Fig. Comments : See Short ear for a discussion of altered ear length.

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A better assessment of size would include both length and width i. Normal values for width and length measurements are available [Farkas, ; Hall et al. Definition : Median longitudinal length of the ear more than 2 SD below the mean in the presence of some, but not all, parts of the normal ear Fig. Definition : Presence of some auricular structures, but none of these structures conform to recognized ear components Fig. Comments : This malformation is commonly associated with atresia of the external canal, but that anomaly should be coded separately.

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Complete absence of the ear should be coded as Anotia. Definition : Small indentation in the lower part of the ascending helix, concha, or in the crus helix Fig.