PEDIATRICS TOP-UP: Preauricular Pits/Sinus (PPS)

Preauricular Pits/Sinus (PPS)

• Small indentations located anterior to the helix and superior to the tragus

• Can occur unilaterally (~50%) or bilaterally (~50%)

• Prevalence ranges between 1% and 10% depending on ethnicity

• Can occur in isolation with no increased risk of hearing impairment or renal         issues

• Can be associated with hearing impairment and organ malformations

• Branchio-oto-renal (BOR) syndrome:

–– Most common inherited syndrome causing hearing loss (autosomal dominant)

–– Clinical presentation: preauricular pits, sensorineural hearing loss (SNHL), branchial cysts (may present as holes/pits in the side of the neck or as tags/pits in front of the ear), renal anomalies

• Beckwith-Wiedemann syndrome:

–– Clinical presentation: macroglossia, asymmetric ear lobules or creases, omphalocele, Wilms tumor, hepatoblastoma.

–– Hearing loss can present later in childhood as conductive or mixed hearing loss

• PPS do not require surgical excision unless they are frequently draining or infected

• Passing of prenatal hearing screen should be confirmed in all patients

• Audiogram should be performed if there are other outer ear deformities or any evidence of genetic syndromes

• When to suggest renal ultrasound in children with ear anomalies when accompanied by any of the following:

–– Other known organ malformations

–– Family history of deafness and auricular and/or renal malformation

–– Maternal history of gestational diabetes mellitus