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Pediatric Otolaryngology 543 G Velocardiofacial syndrome (Shprintzen syndrome, 22q11 deletion syndrome) Inheritance: Autosomal dominant Genetic loci: 22q11 deletion General: 10% related to DiGeorge syndrome (hypocalcemia, thymic aplasia) Relevant features: Velopharyngeal incompetence, excessive arched/cleft palate (overt or submucous), conductive listening to loss, square nasal root, distinguished nasal dorsum, deficiency of nasal alae, retrognathia, medial displacement of carotid artery (25%) Associated options: Mild developmental delay, short stature, cardiac de- fects (85%), elevated risk of psychiatric disorders, slender palms/fingers G Van der Woude syndrome Inheritance: Autosomal dominant Genetic loci: 1q32 (incomplete penetrance) Relevant features: cleft lip and/or cleft palate, lower lip pits, absent central/lateral incisors, canines, bicuspids Associated options: Developmental delay, hypospadia, cryptorchid- ism, hernias the next syndromes (where listening to loss is a main feature) are lined in Chapter 6. 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