“…Chromosome locus 8q11.1–q13.3 has been reported as possible culprit [ 5 8 10 11 ]. Bearing in mind that the external ear originates from fusion of 6 hillocks of His: 3 caudal borders of the first brachial arch (gives rise to tragus and the anterior crus of helix) and 3 caudal border of the second branchial arch (gives rise to rest of the auricle) [ 1 3 5 6 8 9 10 11 ], it is no surprise the disease manifests in various types of syndromes and these include Branchio-Oto-Renal Syndrome, Branchio-Oto-Ureteral Syndrome, Branchi-Otic Syndrome, Branchio-Oto Costal Syndrome, Tetralogy of Fallot, and clinodactyly, Steatocytoma multiplex, rare syndrome of bilateral defects, deafness, preauricular sinus, external ear anomaly and comissural lip pit syndrome, Cat Eye syndrome, Wartenburg's Syndrome, Floating-Harbour Syndrome of unusual phenotype, Trisomy 22 mosaicisim, and Full Trisomy 22 [ 5 8 ]. Concomitant congenital anomalies related to hearing loss accounts for 1.7% of preauricular sinus, while renal problems are associated with 2.6% of the disease.…”