This bipolar disease involves head and neck with
auricular pits (77%); deafness that is perceptive, conductive, or mixed
(89%); narrow auditory canal; and branchial cleft or cysts (63%).
Cleft palate or uvula, facial paresis, microphthalmia, and absent
nasolacrimal duct can be observed. Occasional deep prognathism.
Genitourinary tract is the second pole of the disease (66%), ranging from
minor dysplasia (double ureters, hydronephrosis, polycystic kidneys,
supernumerary, ectopic or small kidneys) to bilateral renal agenesis.