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Branchial arch anomalies are one of the most common congenital anomalies and are usually unilateral in nature. The bilateral occurrence of more than one anomaly is not only rare, but its presence along with hearing loss results in branchiootic syndrome, which has important clinical and genetic implications. Branchiootic syndrome is a part of the spectrum of branchiootorenal syndrome, which is a rare autosomal dominant condition with incomplete penetrance characterized by the malformations of the external, middle, and inner ear, hearing loss, branchial fistulae, and renal abnormalities. We shall be discussing two such rare cases in this article.