An accessory auricle is a congenital anomaly typically seen as a skin coloured nodule, most frequently just to the front of the

ear In vertebrates, an ear is the organ that enables hearing and (in mammals) body balance using the vestibular system. In humans, the ear is described as having three parts: the outer ear, the middle ear and the inner ear. The outer ear co ...

Signs and symptoms

The general presentation is of a skin-covered nodule, papule, or nodule of the skin surface, usually immediately anterior to the auricle. However, it may be anywhere within the periauricular tissues. Bilateral presentation can be seen.

Genetics

A study of a family with 11 affected showed the accessory auricle were inherited in an

autosomal An autosome is any chromosome that is not a sex chromosome. The members of an autosome pair in a diploid cell have the same morphology, unlike those in allosomal (sex chromosome) pairs, which may have different structures. The DNA in autosome ...

dominant manner.

Diagnosis

The lesions presents as a nodule or papule, either sessile or pedunculated. They may be soft or have a cartilaginous structure. By histologic examination, it is a recapitulation of normal external auricle. There will be skin, cartilaginous structures, and cartilage (although the last is not seen in all variants of this disorder). Some investigators believe that the tragus is the only hillock which is derived from the first

branchial arch Branchial arches or gill arches are a series of paired bony/ cartilaginous "loops" behind the throat ( pharyngeal cavity) of fish, which support the fish gills. As chordates, all vertebrate embryos develop pharyngeal arches, though the eve ...

. This is clearly suggestive that true cases of accessory auricle represent a true duplication of the hillocks that were part of the second branchial arch. The second ear appears as a mirror image folded forward and lying on the posterior cheek.

Differential diagnosis

These structures are distinctly different from squamous papilloma and benign

teratoma A teratoma is a neoplasia, tumor made up of several types of biological tissue, tissue, such as hair, muscle, Human tooth, teeth, or bone. Teratomata typically form in the tailbone (where it is known as a sacrococcygeal teratoma), ovary, or test ...

Classification

The several components or degrees of development range from an ear tag, preauricular appendage, preauricular tag, or accessory tragus, to

supernumerary Supernumerary means "exceeding the usual number". Supernumerary may also refer to: * Supernumerary actor, a performer in a film, television show, or stage production who has no role or purpose other than to appear in the background, more common ...

ears or polyotia. It is a relatively common

congenital anomaly A birth defect is an abnormal condition that is present at birth, regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can range from mild to severe. Birth def ...

of the first

or second branchial arches. Other anomalies may be present concurrently, including

cleft palate A cleft lip contains an opening in the upper lip that may extend into the nose. The opening may be on one side, both sides, or in the middle. A cleft palate occurs when the palate (the roof of the mouth) contains an opening into the nose. The ...

cleft lip A cleft lip contains an opening in the upper lip that may extend into the nose. The opening may be on one side, both sides, or in the middle. A cleft palate occurs when the palate (the roof of the mouth) contains an opening into the nasal cavi ...

, or mandibular hypoplasia. There is a known association with

Goldenhar syndrome Goldenhar syndrome is a rare congenital defect characterized by incomplete development of the ear, nose, soft palate, lip and mandible on usually one side of the body. Common clinical manifestations include limbal dermoids, preauricular skin tag ...

(oculo-auriculo-vertebral syndrome) and with

Wildervanck syndrome Wildervanck syndrome or cervico-oculo-acoustic syndrome comprises a triad of: * Duane syndrome Duane syndrome is a congenital rare type of strabismus most commonly characterized by the inability of the human eye, eye to move outward. The syndrom ...

. There may also be an association with

congenital cartilaginous rest of the neck Congenital cartilaginous rest of the neck (CCRN) is a minor and very rare congenital cutaneous condition characterized by branchial arch remnants that are considered to be the cervical variant of accessory tragus. It resembles a rudimentary Pinna ...

Management

Simple surgical excision is curative. The recommended treatment is that the skin is peeled off the extra-auricular tissue and protruding cartilage remnants are trimmed. Normal appearance is achieved in majority of cases. The reconstruction successful in true cases of accessory auricle, as it also is in individuals with auricular appendages.

Epidemiology

These lesions usually present in neonates, although they may not come to clinical attention until adulthood (for cosmetic reasons). There is no gender predilection. They are present in approximately 3–6 per 1000 live births.

References

External links

{{DEFAULTSORT:Accessory Tragus Ear Cutaneous congenital anomalies Genetic disorders with OMIM but no gene Congenital disorders of ears