Conductive hearing loss in Beckwith-Wiedemann syndrome.
Beckwith-Wiedemann syndrome is a rare genetic overgrowth syndrome presenting with organomegaly, abdominal wall defects, macroglossia, and postnatal hypoglycemia. Head and neck manifestations of this abnormality include flame nevus 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 conductive 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 child's hearing. The bony fixation of the malleus and stapes is explained as atavism of the processus anterior mallei and peripheral lamina stapedialis in embryological development.
Schick
B
B
Department of Ear, Nose and Throat Diseases, Head-, Neck- and Facial Plastic Surgery, Communication Disorders, Academic Teaching Hospital of the University of Marburg, Fulda, Germany.
Brors
D
D
Prescher
A
A
Draf
W
W
D001506:Beckwith-Wiedemann Syndrome / Q000150:complications; D002648:Child; D004429:Ear Ossicles / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery; D005260:Female; D006314:Hearing Loss, Conductive / Q000150:complications / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D013201:Stapes Surgery; D014057:Tomography, X-Ray Computed