Surgical Findings in van der Hoeve's Syndrome

Kosoy, J; Maddox, H. Edward

doi:10.1001/archotol.1971.00770060201002

Cited by 25 publications

(11 citation statements)

References 8 publications

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“…They are summarized and their relative frequencies compared in Table III. Fixed footplates were present in all our operated ears, as well as in all cases from the series, in which stapes surgery was performed, as reported by others 5, 17, 20, 27–32. Fractured stapes crura and atrophic crura are often reported in hearing impaired OI patients with middle‐ear surgery, as well as thickened and brittle footplates.…”

Section: Discussionsupporting

confidence: 81%

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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Section: Discussionsupporting

confidence: 81%

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

et al. 2009

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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%

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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“…Wullstein et al [8] reported that they treated three familial cases of this syndrome in a mother and her daughters by way of fenestration. Surgical and/or histological findings on this syndrome were also reported by several other authors [9][10][11] who applied stapes surgery techniques.…”

supporting

confidence: 68%

“…Otosclerotic changes were not observed around the oval window or promontory, although the footplate itself was totally replaced by oto sclerotic tissues. Kosoy and M add ox [11] reported that the footplate had a discrete margin and was only slightly fixed to the edges of the oval window in spite of total replacement of the footplate with soft chalky mounded bone. Their findings were in agreement with those in this case.…”

Section: Discussionmentioning

confidence: 99%

Surgical and Histological Findings of the Stapes Associated with Van der Hoeve Syndrome in a Japanese Boy

Morimitsu¹,

Hayashida²

1973

ORL

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A case of Van der Hoeve syndrome (osteogenesis imperfecta) in a Japanese boy is reported. Surgical observation revealed that the stapedial crura fractured spontaneously and displaced posteriorly, and the footplate was replaced totally by white, thick, soft bony tissues, although its fixation to the oval window was slight. It was noteworthy that the stapedial superstructure was retracted posteriorly by contraction of the stapedial muscle associated with sound stimuli. Histological examination revealed findings very similar to the so-called blue mantel of otosclerosis in the stapedial crus and the footplate. The genetic relationship between both diseases is discussed in reference to the incidences of both diseases in the Japanese.

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Surgical Findings in van der Hoeve's Syndrome

Cited by 25 publications

References 8 publications

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

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

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

Surgical and Histological Findings of the Stapes Associated with Van der Hoeve Syndrome in a Japanese Boy

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