Abstract
The triad of blue sclera, osseous fragility, and hearing loss is known as van der Hoeve syndrome. Surgical results of conductive hearing loss in three cases involving four ears with this disease were analyzed.
Case 1: A 31-year-old male had a history of four fractures and blue sclera.His left ear, the surgically treated side, showed 64 dB hearing level with 28 dB sensorineural hearing loss.
Case 2: A 46-year-old female had a history of two fractures and blue sclera.Mixed hearing loss of 49 dB in the right ear and 51 dB in the left was demonstrated.Both ears were treated surgically.
Case 3: A 41-year-old female had a history of five fractures and blue sclera.She complained of bilateral hearing loss and tinnitus.Her right hearing level was 41 dB and left 40 dB, and the left ear was operated on.
Small fenestra stapedectomy was performed in all four ears.It was recongnized that the stapedial crura fractured spontaneously in two cases, fragile crura were also observed in the other two cases and in all cases, the stapedial footplates were immobile.An air-bone gap less than 15 dB and improvement of tinnitus were obtained in all the cases.
