Autosomal dominant duplication of the renal collecting system, hearing loss, and external ear anomalies: A new syndrome?

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Abstract
We report two families in which propositi had severe bilateral sensorineural hearing loss, a preauricular pit or tag, and duplication of the ureters or bifid renal pelvices. Other relatives had one or more of these anomalies in a pattern suggesting autosomal dominant inheritance with reduced penetrance and variable expressivity. We suggest the term “branchio‐oto‐ureteral syndrome” to designate this condition.