A Case of van der Hoeve Syndrome

Abstract

A case of van der Hoeve syndrome in a 7-year-old Japanese male is reported here. He demonstrated three symptoms: blue sclera, osteogenesis imperfecta and conductive hearing loss.
Pure tone audiometry showed a 61.7dB hearing loss in the left ear. Tympanogram was type Ad and stapedial reflex was absent. Therefore, dislocation of ossicular chain was suspected.
At surgery, it was observed that the bilateral stapedial crura had fractured spontaneously, but the stapedial footplate was mobile. Using tympanoplasty IV-i type, we reconstructed the conduction with an artificial ossicle. The patient obtained an improvement in his hearing level in the immediate postoperative period.