Vol.97 No.3 contentsPRACTICA OTO-RHINO-LARYNGOLOGICA
Vol. 97 No. 3 March 2004
A Case of van der Hoeve Syndrome
Fumiaki Nin, Nobuhito Ioka and Susumu Nakae
(Matsushita Memorial Hospital)
Kaheita Hirasugi
(Kyoto Prefectural University of Medicine)
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 61.7 dB 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.
Key words : van der Hoeve syndrome, osteogenesis imperfecta, dislocation of ossicular chain, tympanoplasty IV-i type