Stapes Surgery in Patients with Osteogenesis Imperfecta

Abstract

Osteogenesis imperfecta is not in itself a contraindication to stapedectomy. Thirty stapedectomies were performed on 24 patients with osteogenesis imperfecta. Thin ossicles, crural fractures, and thick, mushy, granular footplates predominate in this condition. Deficient, short crura that did not contact the footplate were noted in three patients; this is possibly a new clinical observation. Three times in this small series the endosteum was so thick that it was possible to fenestrate the soft, granular, mush-like footplate without invading the vestibule. Extreme caution in handling the incus is necessary. Conductive hearing loss can be relieved through stapedectomy in patients with osteogenesis imperfecta with about the same level of predictability as in those with otosclerosis.

Stapedotomy in Osteogenesis Imperfecta: A Prospective Study of 23 Consecutive Cases

“…Since then, several authors have presented their stapedectomy results (7,8,12,19,20). In this series, a postoperative ABG of less than 10 dB was achieved in 85.7% of cases, and absence of overclosure was observed as reported by the majority of authors (2,7,8,11,12,21). Results of other authors are presented in Table 6.…”

“…It is characteristic of osteogenesis imperfecta that the footplate is often very thick and soft (17). The overall incidence of obliterated footplate in osteogenesis imperfecta is reported to vary from 27 to 80%, with a mean incidence of 51% (1,(10)(11)(12)19,20). The severity of the footplate involvement is related to a higher incidence of failure.…”

Stapes Surgery in Osteogenesis Imperfecta: Analysis of Postoperative Hearing Loss

“…The results were usually good. [38][39][40][41][42] The operative findings and the short-term and longterm results of stapes surgery are reported, especially in the three largest studies. 39.40.42 This article presents an analysis of the disappointing results among the 58 ears in the Dutch series of stapes operations for osteogenesis imperfecta.…”

Stapes Surgery in Osteogenesis Imperfecta: Retrospective Analysis of 34 Operated Ears

“…In addition, the ossicles may be fractured or atrophic, in particular the stapes crura. In our series atrophic or fractured crura always coexisted with bony ankylosis of the stapes footplate, whereas other authors have associated sudden hearing loss and a large preoperative ABG in a few OI patients with crural fractures in combination with a mobile footplate [Armstrong, 1984;Dieler et al, 1997;Albahnasawy et al, 2001]. The middle ear mucosa covering the stapes footplate or promontory may be thickened or hypervascularized, which may end up in excessive bleeding during surgery.…”

“…Coagulation of the mucosa around the promontory by use of the laser may also reduce the liability to abundant bleeding. Although it was not reported in the other large series and is more frequently encountered when revision surgery is concerned, an atrophic or even fractured long process of the incus may be observed in a few primary operated ears [Patterson and Stone, 1970;Armstrong, 1984].…”