The major features of the branchio-oto-renal syndrome are branchial remnants, ear anomalies and renal dysplasia. Investigation of the family of an infant who presented with renal failure and features of the syndrome led to the recognition of renal disease in 2 siblings. 2 other patients with severe renal involvement are presented. The necessity for vigorous investigation of other family members when cases are identified is emphasized.
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eacute et al. (2008) studied this question.