Temporal bone and intracranial abnormalities in syndromic causes of hearing loss: an updated guide

Eur J Radiol. 2020 Feb;123:108803. doi: 10.1016/j.ejrad.2019.108803. Epub 2019 Dec 26.


Purpose: To describe in detail the temporal bone and brain findings in both common and rare syndromic causes of hearing loss, with the purpose of broadening among radiologists and enhance the current understanding of distinct imaging features in paediatric patients with syndromic hearing loss.

Methods: A detailed search of electronic databases has been conducted, including PubMed, Ovid Medline, Scopus, Cochrane Library, Google Scholar, National Institute for Health and Care Excellence (NICE), Embase, and PsycINFO.

Results: Syndromic causes of hearing loss are characterised by different and sometimes specific abnormalities in the temporal bone.

Conclusion: A complete knowledge of the image findings in the temporal bones, brain, skull and other body regions is critical for the optimal assessment and management of these patients.

Keywords: Brain malformations; Head and neck radiology; Inner ear malformations; Pediatric radiology; Syndromic hearing loss.

Publication types

  • Review

MeSH terms

  • Brain / abnormalities*
  • Brain / diagnostic imaging
  • Brain Diseases / complications*
  • Brain Diseases / diagnostic imaging
  • Child
  • Ear Diseases / complications*
  • Ear Diseases / diagnostic imaging
  • Hearing Loss / etiology*
  • Humans
  • Magnetic Resonance Imaging
  • Syndrome
  • Temporal Bone / abnormalities*
  • Temporal Bone / diagnostic imaging
  • Tomography, X-Ray Computed