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Blackwell Publishing, Ltd.

CLINICAL REVIEW
Pediatric Dermatology Vol. 21 No. 3 191 196, 2004

The Preauricular Sinus: A Review of


its Clinical Presentation, Treatment,
and Associations
Noah S. Scheinfeld, M.D., J.D., Nanette B. Silverberg, M.D., Jeffrey M. Weinberg, M.D.,
and Valerie Nozad, D.O.
Department of Dermatology, St. Lukes-Roosevelt Hospital Center and Beth Israel Medical Center, New York, New York

Abstract: Preauricular sinuses (ear pits) are common congenital abnor-


malities. Usually asymptomatic, they manifest as small dells adjacent to
the external ear near the anterior margin of the ascending limb of the helix,
most frequently on the right side. Preauricular sinuses can be either
inherited or sporadic. When inherited, they show an incomplete autosomal
dominant pattern with reduced penetrance and variable expression. They
may be bilateral, increasing the likelihood of being inherited, in 2550% of
cases. Preauricular sinuses are features of other conditions or syndromes
in 3 10% of cases, primarily in association with deafness and branchio-
oto-renal (BOR) syndrome. When other congenital anomalies coexist with
these sinuses, auditory testing and renal ultrasound should be con-
sidered. Sinuses may become infected, most commonly with gram-positive
bacteria, in which case their exudates should be cultured and appropriate
antibiotics administered. Recurrent infection is a clear indication for com-
plete excision and provides the only definitive cure. Recurrence rates after
surgery range from 9% to 42%. Meticulous excision by an experienced
head and neck surgeon minimizes the risk of recurrence.

Preauricular sinuses (ear pits) are a common congenital is usually on the auricle. Sinuses have been reported to
Address correspondence to Noah S. Scheinfeld, M.D., Department of Dermatology, St. Lukes Hospital, 1020 Amsterdam Ave., Suite 11D, Apt 9E, New York, NY 10025, or e-mail: nss32@columbia.edu.

abnormality. First described by Heusinger in 1864, they are occur along the lateral surface of the helicine crus and the
frequently noted on routine physical examination and most superior posterior margin of the helix, tragus, or lobule.
affected patients seek no treatment. Their incidence varies Preauricular sinuses are usually narrow and short,
globally and is estimated in the United States at 0.10.9%, in occasionally arborizing and following a tortuous course near
England at 0.9% (1), in Taiwan at 1.62.5% (2), and in some the external ear. They are usually found lateral, superior,
areas of Africa at 410% (3). Sinuses manifest as small dells and posterior to the facial nerve and the parotid gland,
(width less than 3 mm) adjacent to the external ear, usually into which they can rarely extend. In almost all cases, the
located at the anterior margin of the ascending limb of the duct connects to the perichondrium of the auricular carti-
helix, most frequently on the right side (Fig. 1) (4). The lage. The extent of lesions can be assessed using methyl-
term preauricular is a misnomer because the sinus opening ene blue staining and a probe (5).

Address correspondence to Noah S. Scheinfeld, M.D., Department


of Dermatology, St. Lukes Hospital, 1020 Amsterdam Ave., Suite 11D,
Apt 9E, New York, NY 10025, or e-mail: nss32@columbia.edu.

191
192 Pediatric Dermatology Vol. 21 No. 3 May/June 2004

Figure 2. Squamous epithelium with hyperkeratosis and


parakeratosis (hematoxylin-eosin; magnification 200).

Figure 3. Duct filled with smegma-like material (hematoxylin-


Figure 1. Preauricular sinus adjacent to the external ear eosin; magnification 100).
located at the anterior margin of the ascending limb of the
helix, on the right side in an 18-year-old African American.

HISTOLOGY
Stratified squamous epithelium showing hyperkeratosis
and parakeratosis (Fig. 2) lines the duct of the sinus and
may be filled with a smegma-like material (Fig. 3) and
may contain sebaceous glands or sebocytes (Fig. 4), sweat
glands, and hair follicles (6). The surrounding tissue can
contain plasma cells, lymphocytes, and neutrophils. If
severe inflammation has occurred, the epithelial lining is
often replaced by granulation tissue.

COMPLICATIONS Figure 4. Cells with isolated sebocytes (hematoxylin-


eosin; magnification 400).
Preauricular sinuses can become infected, most commonly
with Staphylococcal species and less commonly with
Proteus, Streptococcus, and Peptococcus species (7). Edema, can have atypical presentations that include wound infection
erythema, fluid drainage, and pain are common signs of after rhytidectomy (8) and submandibular abscesses (9).
infection. Fluid drained from a sinus should be cultured Other conditions (10) and lesions can coexist with pre-
and appropriate antibiotics administered. Sinus infections auricular sinuses providing nests for infection, including
Scheinfeld et al: Preauricular Sinus 193

epidermal cysts (11), pseudocyts of the auricle (12), and


INHERITANCE AND ASSOCIATED
calculi (13). Many believe that recurrent infection is an
SYNDROMES
indication for performing surgery, while others think that
even asymptomatic sinuses should be excised because of Preauricular sinuses are both sporadic and inherited.
their uncertain behavior (14). When inherited, they show an incomplete autosomal
dominant pattern with reduced penetrance and variable
expression. The sinuses are bilateral in 2550% of cases,
SURGERY and bilateral sinuses are more likely to be inherited.
Complete excision of the pit and sinus tract provides Preauricular sinuses are rarely associated with deafness or
the only definitive way to prevent complications from renal problems. In the only large study looking directly
sinuses. Meticulous dissection of the sinus under general at ear anomalies (involving 58,000 children with ear
anesthesia by an experienced head and neck surgeon deformities), auricular sinuses were not associated with
minimizes the risk of recurrence (15). Some have any risk of renal anomalies; moreover, of 446 preauricular
evaluated (16) and advocated a supra-auricular approach sinuses, only 15 were associated with syndromes (25).
(wide local excision) to minimize recurrences (17). One series of cases of branchial cleft anomalies found that
However, in most cases, because the cyst is adherent to 60% had preauricular sinuses (26). The occurrence of
the cartilage of the helical crus and failure to remove a these two conditions in a mother and her two children has
small section of cartilage at that point of adherence can also been reported (27).
be the cause of recurrence (18), careful excision in an The branchio-oto-renal (BOR) syndrome is associated
approach anterior to the crus and tragus, if needed, will with preauricular sinuses. An autosomal dominant
give all the exposure necessary. disorder, BOR syndrome affects 2% of profoundly deaf
Surgery should occur only after a preauricular sinus children and is caused by mutations in the human
infection has resolved. Wide excision is useful if the EYA1 gene (28). BOR syndrome consists of conductive,
edema and inflammation associated with a recurrent sinus sensorineural, or mixed hearing loss; preauricular pits;
infection cannot be reduced by antibiotics. Surgical structural defects of the outer, middle, or inner ear; renal
experience in dealing with sinuses is the key to successful anomalies and failure; lateral cervical fistulas, cysts, or
treatment and removal. Recurrence after surgery has been sinuses; and/or nasolacrimal duct stenosis or fistulas (29).
reported at between 9% and 42% (19). Study of the tissue of the temporal bones of an infant with
preauricular pits, cervical fistula, and deafness revealed
abnormalities in the middle ear, the vestibular system, and
the cochlea (30). Other syndromes (some of which have
EMBRYOLOGY
been reported separately but might in fact overlap with
Preauricular sinuses originate during embryogenesis (20). BOR) can be associated with preauricular sinuses as well
The auricle arises from the first and second branchial (Table 1).
arches during the sixth week of gestation. The eustachian
tube and middle ear arise from the first pharyngeal pouch.
Tissue from the first and second brachial clefts develops EVALUATION OF SYSTEMIC ASSOCIATIONS
into six auditory hillocks, which unite to form the external OF PREAURICULAR PITS
ear (21). Specifically, three hillocks arise from the caudal
border of the first branchial arch, and three arise from the Preauricular pits can be associated with hearing and renal
cephalic border of the second branchial arch (the hillocks problems, and auditory testing (31) and renal ultrasound
of His), which should unite during the next few weeks of appear to be useful in patients who have associated
embryogenesis (22). syndromes. Some investigators, however, recommend
Three theories provide explanations for the formation that renal ultrasonography be performed on all children
of preauricular sinuses that: they result from defective with a preauricular sinus (32,33). A larger study sug-
fusion of the six auricular hillocks resulting in preaur- gested that a renal ultrasound should be performed in
icular fistulas; they are relics of incomplete closure of patients with isolated preauricular sinuses accompanied
the dorsal part of the first pharyngeal groove; and they by one or more of the following: another malformation
develop from isolated ectodermal folding during auricular or dysmorphic feature, a family history of deafness,
development (23). Merlob and Aitkin (24) have posited auricular and/or renal malformations, or a maternal
that sinuses are a marker of teratogenic exposures and history of gestational diabetes. In the absence of these
suggest that a decrease in their prevalence in Israel might findings, this study concluded that renal ultrasonography
be a marker for decreased exposure to teratogens. is not indicated (34).
194 Pediatric Dermatology Vol. 21 No. 3 May/June 2004

TABLE 1. Syndromes Associated with Preauricular Sinus


Syndromes/conditions Manifestations Comments

BOR syndrome Conductive, sensorineural, or mixed hearing loss; BOR is caused by mutations in
preauricular pits; structural defects of the outer, middle, the human EYA1 gene
or inner ear; renal anomalies and failure; lateral cervical
fistulas, cysts, or sinuses; and/or nasolacrimal duct
stenosis or fistulas
Branchio-otic (BO) syndrome (35) Branchial anomalies, preauricular pits, and hearing loss,
with no renal dysplasia
Branchio-oto-ureteral syndrome (36) Severe bilateral sensorineural hearing loss, a preauricular pit Two families reported
or tag, and duplication of the ureters or bifid renal pelves
Branchio-oto-costal syndrome (37) Branchial arch anomalies, hearing loss, ear and commissural
lip pits, and rib anomalies
Branchio-oculo-facial syndrome (38) Abnormal upper lip, which resembles a poorly repaired cleft Branchio-oculo-facial syndrome and
lip, malformed nose with broad bridge and flattened tip, BO/BOR syndromes are distinct
lacrimal duct obstruction, malformed ears, and branchial entities (39)
cleft sinuses and/or linear skin lesions behind the ears
Hemifacial microsomia syndrome Bilateral preauricular sinuses, facial steatocystoma multiplex
associated with pilar cysts (40)
Sensorineural hearing loss, facial palsy, microtia or
anotia, cervical appendages containing cartilage, and
other defects (41)
Lip pits Preauricular sinuses, conductive deafness, commissural lip No inner ear abnormalities are identified on
pits, and external ear abnormalities (42) computed tomography (CT) scans (43)
Bilateral defects, male transmission Bilateral cervical branchial sinuses, bilateral preauricular Three generations of male-to-male
sinuses, bilateral malformed auricles, and bilateral transmission reported
hearing impairment (44)
Tetralogy of Fallot and clinodactyly Characteristic facial appearance, preauricular pits, fifth Autosomal dominant transmission
finger clinodactyly, and tetralogy of Fallot have been
reported (45)
Ectodermal dysplasia Preauricular pits, tetra-amelia, ectodermal dysplasia,
hypoplastic lacrimal ducts and sacs opening toward the
exterior, peculiar face, and developmental retardation (46)
Waardenburg syndrome Bilateral preauricular sinuses, Waardenburg syndrome
without a white forelock with syndactyly, absence of
the fourth left toe, and dacryocystitis (47)
Incomplete trisomy 22 (48) Complex congenital heart defect, membranous anal atresia A female infant with growth failure,
without fistula, distal limb hypoplasia, partial cutaneous microcephaly, hypertelorism,
syndactyly between second and third toes, and a left epicanthal folds, preauricular pit,
preauricular pit congenital heart defect, hypotonia,
and delayed development had trisomy
22 mosaicism (46,XX/47,XX, +22)
in cultured skin fibroblasts but not
in blood lymphocytes (49)
Complete trisomy 22 (50) Primitive and low-set ears, bilateral preauricular pit, One case reported. The child
broad nasal bridge, antimongoloid palpebral fissures, developed signs of congenital heart
macroglossia, enlarged sublingual glands, cleft palate, disease and died at the age of 10 weeks
micrognathia, clinodactyly of the fifth fingers, hypoplastic
fingernails, hypoplastic genitalia, short lower limbs,
bilateral sandal gap, and deep plantar furrows

CONCLUSION syndromes are being elaborated and should generate new


insight into their etiology. Recognition of the appearance,
Preauricular sinuses are usually asymptomatic and isolated,
course, and associations of sinuses will allow physicians
but may still invoke management and evaluation questions.
to optimize patient care.
Symptomatic sinuses that drain fluid (i.e., infected) need
to be cultured and treated with appropriate antibiotics. If
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