Figure 1:

Pedigree and clinical characteristics of the index family. (A) Pedigree. (B) Ultrasonographic images of right kidneys from patient III-1 (A 82.6 mm), patient IV-2 (A 44.7 mm), and patient IV-1 (A 19.5 mm). Note the prominent kidney allograft next to the hypotrophic and dysplastic right kidney in patient IV-1. (C) Close-up images of left ears from indicated patients with preauricular pits highlighted by dashed circles. (D) Audiograms from patient III-1 showing functional deafness of the right ear and combined sensorineural hearing loss on the left side. Red color, right ear; blue color, left ear; downward arrow, no response; circle, air conduction of the right ear; arrow pointing right, bone conduction of the right ear; cross, air conduction of the left ear; arrow pointing left, bone conduction of the left ear; dash, contralaterally masked air conduction; zig zag, contralaterally masked bone conduction.