Ear Surgery in Osteogenesis Imperfecta: Clinical Findings and Short-term and Long-term Results

Garretsen, Ton J. T. M.; Cremers, W. R. J.

doi:10.1001/archotol.1990.01870030081014

Cited by 40 publications

(33 citation statements)

References 19 publications

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“…Total loss of hearing did not occur in our series but has been described in a few cases by others [Patterson and Stone, 1970;Kosoy and Maddox, 1971;Brosnan et al, 1977;Garretsen and Cremers, 1990;van der Rijt and Cremers, 2003;Kuurila et al, 2004].…”

Section: Audiometric Resultssupporting

confidence: 55%

“…Both AC and BC thresholds demonstrate negligible shifts from the short-to long-term follow-up. Studies including long-term postoperative audiometry mentioned a mean threshold shift of 8 dB in the long term without change in the ABG [Shea and Postma, 1982;Pedersen, 1985;Garretsen and Cremers, 1990;Ferekidis et al, 2000]. Despite a longer follow-up period, the average threshold increased by only 3.5 dB in our subjects, whereas the ABG remained constant.…”

Section: Audiometric Resultscontrasting

confidence: 47%

“…A good agreement is observed between the present and our former series regarding the frequency of occurrence of the different surgical observations. Summing both our series and comparing them with the large series of Shea and Postma [1982], Pedersen [1985] and Garretsen and Cremers [1990] reflects similarities in magnitude as well as in surgical findings ( table 2 ). The most frequently encountered peculiarity is a fixed footplate, which may be thickened or brittle.…”

Section: Primary Surgery Intraoperative Findingsmentioning

confidence: 62%

“…In former days, the diagnosis of OI has put off otologists to perform stapes surgery because of a higher risk of complications, such as bleeding of the mucosa, ossicular atrophy and the progressive character of the sensorineural hearing loss component inherent to the disease [Cremers and Garretsen, 1989], though, during the last few decades, reports on the results of stapes surgery in larger series of OI patients [Shea and Postma, 1982;Pedersen 1985;Cremers and Garretsen, 1989;Garretsen and Cremers, 1990;van der Rijt and Cremers, 2003;Kuurila et al, 2004;Vincent et al, 2005;Swinnen et al, 2009] have shown that a successful reduction in hearing thresholds may be expected in the majority of the OI patients with conductive hearing loss. Nevertheless, a few cases of total deafness following surgery have been reported [Garretsen and Cremers, 1990;van der Rijt and Cremers, 2003;Kuurila et al, 2004], as well as less beneficial results when revision surgery is concerned [Shea and Postma, 1982;Garretsen and Cremers, 1991b;van der Rijt and Cremers, 2003].…”

Section: Introductionmentioning

confidence: 99%

“…Nevertheless, a few cases of total deafness following surgery have been reported [Garretsen and Cremers, 1990;van der Rijt and Cremers, 2003;Kuurila et al, 2004], as well as less beneficial results when revision surgery is concerned [Shea and Postma, 1982;Garretsen and Cremers, 1991b;van der Rijt and Cremers, 2003]. Furthermore, the hearing gain tends to be eliminated in the long term because of progression of the sensorineural component which is inherent to the disease and occurs in both operated and nonoperated ears [Shea and Postma, 1982;Pedersen, 1985;Garretsen and Cremers, 1990;Ferekidis et al, 2000]. We expanded our previously published results on genetic, surgical and audiometric findings in 15 ears [Swinnen et al, 2009] with a larger sample of operated OI ears retrospectively obtained from several clinics in Belgium and the Netherlands.…”

Section: Introductionmentioning

confidence: 99%

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

Swinnen

Leenheer²,

Coucke³

et al. 2012

Audiol Neurotol

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Intraoperative findings of stapes surgery in 34 ears from 22 patients with genetically confirmed osteogenesis imperfecta (OI) are reported, as well as the audiometric results after the longest postoperative follow-up published to date. Twenty-nine out of 34 ears underwent primary stapes surgery and 5 ears revision surgery. Postoperative audiometric follow-up ranged from 6 months to 37 years. Stapes footplates were fixed in all ears. Additionally, footplates were thickened or fragile, stapes crura atrophic or fractured, and middle ear mucosae thickened or hypervascularized. Short-term postoperative audiometry revealed improved hearing and reduced air-bone gaps in 28/29 primary operated ears and in all revision cases. In the 22 ears with long-term postoperative follow-up (mean duration: 16 years), hearing gain was still significant at the latest audiometric evaluation. Independently of the patients being diagnosed with OI type I or IV and independently of the underlying OI genotype, beneficial results are obtained in the majority of OI patients undergoing primary or revision stapes surgery for reduction of conductive hearing loss components caused by stapes footplate fixation. Despite the progressive course of the concomitant sensorineural component, hearing gain remains beneficial over several decades.

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Section: Audiometric Resultssupporting

confidence: 55%

Section: Audiometric Resultscontrasting

confidence: 47%

Section: Primary Surgery Intraoperative Findingsmentioning

confidence: 62%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

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

Swinnen

Leenheer²,

Coucke³

et al. 2012

Audiol Neurotol

View full text Add to dashboard Cite

show abstract

Audiometric, surgical, and genetic findings in 15 ears of patients with osteogenesis imperfecta

et al. 2009

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In general, stapes surgery is successful in resolving the conductive hearing loss in OI patients, even in the long term. Hearing loss in OI is mostly of the mixed type, and the sensorineural component is reported to be progressive. Stapedotomy, by improving the hearing level, may facilitate the rehabilitation with a hearing aid. Because the identified mutation could be located in the COL1A1 gene in all patients, conductive hearing loss in OI caused by stapes fixation is possibly linked to a mutation in this gene. Laryngoscope, 2009.

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Temporal bone imaging in osteogenesis imperfecta patients with hearing loss

et al. 2013

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Objectives/Hypothesis Osteogenesis imperfecta (OI) is an autosomal‐dominant connective‐tissue disorder, predominantly characterized by bone fragility. Conductive hearing loss develops in half of the OI patients and often progresses to mixed loss. Findings of computed tomography (CT) and magnetic resonance (MR) imaging of the temporal bone in the largest series of OI patients to date are presented and correlated with the audiograms. Study Design Retrospective case series. Methods CT images and audiograms of 17 hearing‐impaired OI patients, aged 9 to 67 years, were analyzed retrospectively. In four patients, MR imaging was performed as well. Imaging abnormalities were correlated with type and severity of hearing loss deduced from the audiograms. Results CT revealed fenestral hypodense foci in the fissula ante fenestram (25 of 33 ears), oval window (23 of 33 ears), and round window (20 of 33 ears). Retrofenestral hypodensities were observed, affecting the cochlear turns (16 of 33 ears), facial nerve canal (10 of 33 ears), or semicircular canals (6 of 33 ears), or appearing like the fourth turn of the cochlea (11 of 33 ears). The site of hypodensities corresponded to the type of hearing loss in 72.2% of the OI ears. The air‐bone gap and bone‐conduction thresholds showed significant positive associations with the number of affected fenestral (P < .05) and retrofenestral structures (P < .01), respectively. Gadolinium‐enhanced MR images demonstrated active lesions in three patients with mixed hearing loss or deafness. Conclusions The site of hypodensities on temporal bone CT images in OI corresponds to presence and type of hearing loss determined by audiometry. The more severe the hearing loss, the more affected temporal bone structures in OI. Level of Evidence 4. Laryngoscope, 123:1988–1995, 2013

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Ear Surgery in Osteogenesis Imperfecta: Clinical Findings and Short-term and Long-term Results

Cited by 40 publications

References 19 publications

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

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

Audiometric, surgical, and genetic findings in 15 ears of patients with osteogenesis imperfecta

Temporal bone imaging in osteogenesis imperfecta patients with hearing loss

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