Temporal bone and intracranial abnormalities in syndromic causes of hearing loss: an updated guide. Issue 123 (February 2020)

• Record Type: Journal Article
• Authors: D'Arco, Felice; Youssef, Adam; Ioannidou, Evangelia; Bisdas, Sotirios; Pinelli, Lorenzo; Caro-Dominguez, Pablo; Nash, Robert; Siddiqui, Ata; Talenti, Giacomo
• Is Part Of: European journal of radiology. Issue 123(2020)
• Publication Date: 2020-02
• Subjects: Pediatric radiology -- Head and neck radiology -- Syndromic hearing loss -- Inner ear malformations -- Brain malformations; Medical radiology -- Periodicals; Radiology -- Periodicals; Radiologie médicale -- Périodiques; Medical radiology; Periodicals; 616.075705
• DOI: 10.1016/j.ejrad.2019.108803
• Languages: English
• ISSNs: 0720-048X

Abstract

Highlights: Syndromic causes of HL may have specific findings that needs to be recognized. New classification of cochlear malformation has been introduced. Associated brain and body findings helps the diagnosis. Abstract: 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.