A Case of van der Hoeve Syndrome

Accession number;04A0163650
Title;A Case of van der Hoeve Syndrome
Author; NIN FUMIAKI (Matsushitakinenbyoin Jibiinkoka) IOKA NOBUHITO (Matsushitakinenbyoin Jibiinkoka) HIRASUGI KAHEITA (Kyoto Prefect. Univ. of Med. Fac. of Med.) NAKAE SUSUMU (Matsushitakinenbyoin Jibiinkoka)
Journal Title;Practica Otologica (Kyoto)
Journal Code:G0710A
ISSN:0032-6313
VOL.97;NO.3;PAGE.201-204(2004)
Figure&Table&Reference;FIG.6, REF.17
Pub. Country;Japan
Language;Japanese
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 61.7 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. (author abst.)