Stapes surgery in osteogenesis imperfecta patients |
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Authors: | R Dieler J Müller J Helms |
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Institution: | (1) Department of Otolaryngology-Head and Neck Surgery, University of Würzburg, Josef-Schneider-Strasse 11, D-97080 Würzburg, Germany |
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Abstract: | Osteogenesis imperfecta (OI), or the Van der Hoeve-de Kleyn syndrome, is a heterogeneous group of connective tissue disorders.
The key features in this disease are bone fragility with a tendency to spontaneous fractures and deformations. The classical
triad of symptoms involves a conductive and/or sensorineural hearing impairment together with a tendency to spontaneous bone
fractures and blue sclerae. Between January 1988 and December 1994, ear surgery was performed on eight ears of six OI patients
who presented with mixed hearing loss preoperatively. Pathological changes observed in the middle ear were atrophy and/or
fractures of the stapedial crura in combination with thickening and fixation of the stapes footplate. Partial stapedectomy
was performed in seven cases and a neo-window was created in the promontory of one patient when an overhanging facial canal
obscured visualization of the oval window niche. Pre- and postoperative bone conduction thresholds did not differ in any of
the patients. Postoperatively, mean values of the air-bone gap in the main speech frequency range were below 10 dB. Functional
results following stapes surgery in patients with otosclerosis during the same time interval (n = 857) did not differ significantly. These data indicate that stapes surgery in OI patients can be performed with the same
functional predictability as in otosclerosis patients, even though the underlying etiology is considerably different.
The results were presented in part at the 66th annual meeting of the German Society for Otorhinolaryngology-Head and Neck
Surgery (Deutsche Gesellschaft für Hals-Nasen-Ohrenheilkunde, Kopf- und Halschirurgie), Karlsruhe, 27–31 May 1995 |
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Keywords: | Osteogenesis imperfecta Otosclerosis Hearing loss Stapes surgery |
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