Audiologic and Otologic Clinical Manifestations of Loeys-Dietz Syndrome: A Heritable Connective Tissue Disorder
American Academy of Otolaryngology–Head and Neck Surgery Foundation
JunW. Jeon1*, Julie Christensen, MS2*, Jennifer Chisholm, AuD2, Christopher Zalewski, PhD2, Marjohn Rasooly, MSN3, Caeden Dempsey, MPH, MSN3, Alaina Magnani4, Pamela Frischmeyer-Guerrerio,MD, PhD3, Carmen C. Brewer, PhD2, and Hung Jeffrey Kim, MD1,5
Overview
Aortic and vascular complications are often the focus of patient care practices and research related to Loeys-Dietz syndrome (LDS), with some additional attention paid to topics like orthopedic issues, asthma, and food allergies. In this article, the authors describe their study of 36 children and adults with LDS types 1-4 and how the condition affected their hearing, ears, and face/head
Researchers found mild to moderate hearing loss in 69% of the study participants. A split uvula (the tissue at the back of the throat), hard cleft palate, and post-tympanostomy tympanic membrane perforation (when a hole exists between the external and middle ear) were more common in LDS-1 and LDS-2 than LDS-3 and LDS-4.
Based on their findings, the study team recommended healthcare professionals pay close attention to the hearing and ear health of individuals with LDS to manage existing issues and prevent further ones from developing.
American Academy of Otolaryngology–Head and Neck Surgery Foundation 2021 Reprints and permission: sagepub.com/journalsPermissions.nav
DOI: 10.1177/01945998211008899
http://otojournal.org