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Temporal bone imaging in osteogenesis imperfecta patients with hearing loss
Freya K R Swinnen1, Jan W Casselman, Els M R De Leenheer
1Department of Otorhinolaryngology, Ghent University Hospital, Ghent, Belgium. freya.swinnen@UGent.be
The Laryngoscope
|February 14, 2013
Summary
Osteogenesis imperfecta (OI) patients often experience hearing loss. Temporal bone CT scans reveal hypodensities correlating with hearing loss type and severity, indicating more affected structures with worse hearing.
Area of Science:
- Radiology
- Otolaryngology
- Genetics
Background:
- Osteogenesis imperfecta (OI) is a genetic disorder causing bone fragility.
- Hearing loss, often conductive and progressing to mixed, affects approximately 50% of OI patients.
Purpose of the Study:
- To present computed tomography (CT) and magnetic resonance (MR) imaging findings of the temporal bone in OI patients.
- To correlate imaging abnormalities with audiometric findings in a large cohort of OI patients.
Main Methods:
- Retrospective analysis of CT and audiograms from 17 hearing-impaired OI patients (aged 9-67).
- MR imaging was performed in four patients.
- Correlation of temporal bone imaging abnormalities with hearing loss type and severity.
Main Results:
- CT revealed hypodense foci in fenestral (fissula ante fenestram, oval, round windows) and retrofenestral (cochlear turns, facial nerve canal, semicircular canals) regions in a significant proportion of ears.
- The location of hypodensities correlated with the type of hearing loss in 72.2% of OI ears.
- Increased number of affected fenestral and retrofenestral structures showed significant positive associations with air-bone gap and bone-conduction thresholds, respectively.
- Enhanced MR imaging identified active lesions in patients with mixed hearing loss or deafness.
Conclusions:
- Temporal bone CT hypodensities in OI patients correspond to the presence and type of hearing loss.
- The severity of hearing loss in OI is directly related to the number of affected temporal bone structures.
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