Preauricular Sinuses Clinical Presentation

Updated: Jan 27, 2020
  • Author: Jeffrey Weinberg, MD; Chief Editor: William D James, MD  more...
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Presentation

History

Most people with preauricular sinuses are asymptomatic. Only one third of persons are aware of their malformations. In one study of 31 patients, once the lesions became apparent, about 9.2 years (on average) passed before they sought medical care.

Some patients with preauricular sinuses present with chronic intermittent drainage of purulent material from the opening. Draining sinuses are prone to infection. Once infected, these sinuses rarely remain asymptomatic, often developing recurrent acute exacerbations.

Patients with preauricular sinuses may present with facial cellulitis or ulcerations located anterior to the ear. These ulcerations are often treated without recognition of the primary source, and the preauricular sinus remains unnoticed.

Subsequent to infection, a patient with preauricular sinuses may develop scarring and disfigurement.

Infants of diabetic mothers are at increased risk for the oculo-auriculo-vertebral sequence, which includes sinuses. [5]

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Physical Examination

The preauricular sinus usually presents as a small dell adjacent to the anterior margin of the ascending limb of the helix, as noted in the image below.

Small dell adjacent to the ear demonstrates the pr Small dell adjacent to the ear demonstrates the preauricular sinus.

Unless they are actively infected or have previously been infected with subsequent scarring, they are only small openings in the external ear. If associated conditions are present, one might see external ear anomalies, such as flop ears. Physical examination may reveal associated branchiogenic fistulas and/or hearing loss. [6]

In 2006, Saltzmann and Lissner [7] reported an unusual case of familial punctal atresia with apparent genetic linkage to bilateral preauricular sinuses that lacked any comorbid syndromic features, which is usually not the case.

Choi et al, [8] in 2007, noted that what is termed the preauricular sinus can occur in the postauricular area. Sinuses occurring in the postauricular areas seem to have a lower rate of recurrence after surgery (0%) than those in the preauricular area (2.2%).

Associated conditions

Conditions associated with preauricular sinuses include subcondylar impaction of a third molar, renal malformations, [9] hearing loss, branchiogenic fistulas, commissural lip pits (3.8% of patients with these have preauricular sinuses), and external ear anomalies; however, these conditions rarely occur.

Cleft palate, spina bifida, imperforate anus, renal hypoplasia or renal agenesis, reduplication of the duodenum, undescended testes, and umbilical hernias are reported associations.

Preauricular sinuses are involved in the following syndromes: Treacher Collins syndrome; branchio-oto-renal (BOR) syndrome; hemifacial microsomia syndrome; a syndrome consisting of facial steatocystoma multiplex associated with pilar cysts and bilateral preauricular sinuses; and a syndrome that includes preauricular sinuses, conductive deafness, commissural lip pits, and external ear abnormalities. BOR syndrome consists of conductive, sensorineural, or mixed hearing loss; preauricular pits; structural defects of the outer, middle, or inner ear; renal anomalies; lateral cervical fistulas, cysts, or sinuses; and/or nasolacrimal duct stenosis or fistulas. Hemifacial microsomia syndrome can include preauricular sinuses, facial nerve palsy, sensorineural hearing loss, microtia or anotia, cervical appendages containing cartilage, and other defects. [10]

A report and review in 2012 described a 6-year-old girl with off and on left and right discharging neck swellings. [11] MRI documented branchial cleft fistulae on the right and left sides. She had removal of the brachial fistula and a tonsillectomy and recovered fully.

Associated facial pathology

Preauricular sinuses can be associated with facial pathology. In one case, a preauricular sinus associated with a congenital cholesteatoma resulted in a facial palsy by impinging on the facial nerves.

Wound infections after rhytidectomy have also been associated.

Calculi can develop in the preauricular sinuses, resulting in infection.

Other findings

A novel autosomal dominant syndrome consisting of hypertelorism, punctal pits, preauricular sinus, and deafness (HPPD) located on 14q31 has been noted. [12]

Other constellations of conditions that occur with preauricular sinuses include natal teeth, kidney stones, and atopic dermatitis. [13]

Jimena et al describe a female aged 6 years with left- and right-draining anterior neck puncta, moderately severe bilateral hearing loss and a preauricular sinus, and moderately severe bilateral hearing loss with concomitant first and bilateral second branchial fistulas concurrent with simultaneous nonfamilial branchio-otic syndrome. The study found this was the third such case in the literature. [14]

Congenital periauricular fistulas may be seen as variations of preauricular sinuses. [3]

The co-incidence of branchial arch anomalies (branchial cysts, branchial fistulas), renal anomalies, and hearing loss together can be termed the Melnick-Fraser syndrome, also described as branchio-oto-renal (BOR) syndrome. A case from Pakistan in a female neonate who presented with profound deafness, branchial sinuses, preauricular pits, and renal hypoplasia was noted in 2014 by Jalil et al. [15]

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