Van der Hoeve 症候群の一家系

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The authors reported one family of typical van der Hoeve's syndrome which showed bilateral progressive conductive deafness, with blue sclera and multiple fractures in their past history. Surgery was done in one case of this family. The stapedial crus was fractured at the connectied portion to the plate and displaced toward the posterior margin of the oval window. The stapedial foot plate was replaced by an amount of white soft bone. But the fixation with the oval window was very slight, showing a good movement with manipulation. It was very interesting that the stapes superstructure was retracted vividly by a contraction of the stapedial muscle by sound stimulation. The plate was removed and replaced with a vein graft. The stapedial muscle was cut off and the posterior crus of the stapes was placed on the vein graft. An appreciable increase in hearing was noted in the operated side.
The authors emphasized that the surgical treatment should be indicated in van der Hoeve's syndrome.