Advanced search
1 file | 267.44 KB

Temporal bone imaging in osteogenesis imperfecta patients with hearing loss

Freya Swinnen (UGent) , Jan Casselman (UGent) , Els De Leenheer (UGent) , Cor WRJ Cremers and Ingeborg Dhooge (UGent)
(2013) LARYNGOSCOPE. 123(8). p.1988-1995
Author
Organization
Abstract
Objectives: 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 (level of evidence: 4). Methods: CT images and audiograms of 17 hearing-impaired OI patients, aged 9-67 years, were analyzed retrospectively. In four patients, MR 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 (25/33 ears), oval window (23/33 ears) and round window (20/33 ears). Retrofenestral hypodensities were observed, affecting the cochlear turns (16/33 ears), facial nerve canal (10/33 ears) or semicircular canals (6/33 ears), or appearing like the fourth turn of the cochlea (11/33 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<0.05) and retrofenestral structures (P<0.01), respectively. Gadolinium-enhanced MR images demonstrated active lesions in three patients with mixed hearing loss or deafness. Conclusion: 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.
Keywords
Osteogenesis imperfecta, magnetic resonance imaging, temporal bone., computed tomography, COCHLEAR IMPLANTATION, CT DENSITOMETRY, STAPES SURGERY, OTOSCLEROSIS, CONGENITA, TARDA, HISTOPATHOLOGY, CAPSULE, EARS, TERM

Downloads

  • (...).pdf
    • full text
    • |
    • UGent only
    • |
    • PDF
    • |
    • 267.44 KB

Citation

Please use this url to cite or link to this publication:

Chicago
Swinnen, Freya, Jan Casselman, Els De Leenheer, Cor WRJ Cremers, and Ingeborg Dhooge. 2013. “Temporal Bone Imaging in Osteogenesis Imperfecta Patients with Hearing Loss.” Laryngoscope 123 (8): 1988–1995.
APA
Swinnen, F., Casselman, J., De Leenheer, E., Cremers, C. W., & Dhooge, I. (2013). Temporal bone imaging in osteogenesis imperfecta patients with hearing loss. LARYNGOSCOPE, 123(8), 1988–1995.
Vancouver
1.
Swinnen F, Casselman J, De Leenheer E, Cremers CW, Dhooge I. Temporal bone imaging in osteogenesis imperfecta patients with hearing loss. LARYNGOSCOPE. 2013;123(8):1988–95.
MLA
Swinnen, Freya, Jan Casselman, Els De Leenheer, et al. “Temporal Bone Imaging in Osteogenesis Imperfecta Patients with Hearing Loss.” LARYNGOSCOPE 123.8 (2013): 1988–1995. Print.
@article{3134790,
  abstract     = {Objectives: 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 (level of evidence: 4).
Methods: CT images and audiograms of 17 hearing-impaired OI patients, aged 9-67 years, were analyzed retrospectively. In four patients, MR 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 (25/33 ears), oval window (23/33 ears) and round window (20/33 ears). Retrofenestral hypodensities were observed, affecting the cochlear turns (16/33 ears), facial nerve canal (10/33 ears) or semicircular canals (6/33 ears), or appearing like the fourth turn of the cochlea (11/33 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{\textlangle}0.05) and retrofenestral structures (P{\textlangle}0.01), respectively. Gadolinium-enhanced MR images demonstrated active lesions in three patients with mixed hearing loss or deafness.
Conclusion: 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.},
  author       = {Swinnen, Freya and Casselman, Jan and De Leenheer, Els and Cremers, Cor WRJ and Dhooge, Ingeborg},
  issn         = {0023-852X},
  journal      = {LARYNGOSCOPE},
  language     = {eng},
  number       = {8},
  pages        = {1988--1995},
  title        = {Temporal bone imaging in osteogenesis imperfecta patients with hearing loss},
  url          = {http://dx.doi.org/10.1002/lary.23963},
  volume       = {123},
  year         = {2013},
}

Altmetric
View in Altmetric
Web of Science
Times cited: