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Enlarged vestibular aqueducts and other inner-ear abnormalities in patients with Down syndrome

  • C M Clark (a1), H H Patel (a1), S G Kanekar (a2) and H Isildak (a1)
Abstract
Background:

Histopathological anomalies of inner-ear structures in individuals with Down syndrome have been well documented; however, few studies have examined the radiological features.

Methods:

A retrospective study was conducted of temporal bone computed tomography images in 38 individuals (75 ears) with Down syndrome to evaluate the prevalence of inner-ear abnormalities and assess vestibular aqueduct widths.

Results:

Inner-ear anomalies were identified in 20 of the 38 individuals (52.6 per cent). Seven of the 75 temporal bones (9.3 per cent) were found to have higher than previously reported. A dilated internal auditory canal and vestibule were more common among the present study group, while prior studies have demonstrated internal auditory canal stenosis and decreased vestibule size.

Conclusion:

Down syndrome patients exhibit a high prevalence of dysplastic inner-ear features that confer substantial risk of sensorineural hearing loss. Computed tomography is a useful screening aid to detect inner-ear abnormalities, particularly enlarged vestibular aqueducts, which cause preventable sensorineural hearing loss in this population.

Copyright
Corresponding author
Address for correspondence: Dr Huseyin Isildak, Department of Surgery, Division of Otolaryngology – Head and Neck Surgery, Pennsylvania State University, College of Medicine, 500 University Drive, H091, Hershey, PA 17033-0850, USA Fax: +01 717 531 4907 E-mail: hisildak@hmc.psu.edu
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The Journal of Laryngology & Otology
  • ISSN: 0022-2151
  • EISSN: 1748-5460
  • URL: /core/journals/journal-of-laryngology-and-otology
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