Conductive hearing loss in Beckwith-Wiedemann syndrome

Beckwith-Wiedemann syndrome is a rare genetic overgrowth syndrome presentin g with organomegaly, abdominal wall defects, macroglossia, and postnatal hy poglycemia. Head and neck manifestations of this abnormality include flame news of the forehead and characteristic sulci of the ear lobe. We present a 7-year-old child with Beckwith-Wiedemann syndrome and a rare finding of co nductive hearing loss on both sides due to congenital malleus and stapedial fixation. Small fenestra stapedotomy and mobilization of malleus fixation in the epitympanum improved the childs hearing. The bony fixation of the ma lleus and stapes is explained as atavism of the processus anterior mallei a nd peripheral lamina stapedialis in embryological development. (C) 1999 Els evier Science Ireland Ltd. All rights reserved.