Please use this identifier to cite or link to this item:
http://hdl.handle.net/1942/40728
Title: | Accelerated Cognitive Decline Associated With Hearing Loss and Bilateral Vestibulopathy: Insights From a Prospective Cross-Sectional Study Using the Repeatable Battery for the Assessment of Neuropsychological Status Adjusted for the Hearing Impaired in the DFNA9 Population | Authors: | Gommeren, Hanne Bosmans, Joyce Moyaert, Julie Mertens, Griet Cras, Patrick Engelborghs, Sebastiaan Van Ombergen, Angelique Gilles, Annick Fransen, Erik van de Berg, Raymond JANSSENS DE VAREBEKE, Sebastien Van Rompaey, Vincent |
Issue Date: | 2023 | Publisher: | LIPPINCOTT WILLIAMS & WILKINS | Source: | EAR AND HEARING, 44 (4) , p. 697 -709 | Abstract: | Background:DeaFNess Autosomal dominant 9 (DFNA9) is a hereditary disorder known to affect both hearing and vestibular function in its carriers. Its phenotype is characterized by progressive sensorineural hearing loss (SNHL) and vestibular dysfunction evolving towards bilateral vestibulopathy (BV) by the 3rd to 5th life decade. Recent studies have identified the impact of hearing loss and vestibular dysfunction on cognitive functioning. Objective:The main objective of this study was to investigate how the cognitive functioning of carriers of the p.Pro51Ser variant in the COCH gene is affected by the disease and compare these results with a matched healthy control group. Study design:Forty-six carriers of the pathogenic p.Pro51Ser variant in the COCH gene were included in this study, of which 38 met the Barany Society criteria and were thus diagnosed with BV. All subjects were between the age of 22 and 72 years old. Each control was individually matched based on age, gender, and education level. A cognitive, vestibular, and hearing assessment was performed in all subjects. All participants completed the Repeatable Battery for the Assessment of Neuropsychological Status, adjusted for the Hearing Impaired (RBANS-H), a cognitive test battery that includes subtests probing Immediate and Delayed Memory, Visuospatial/Constructional, Language, and Attention. Results:Overall, the DFNA9 patients demonstrated significantly lower scores on the Immediate Memory subscale and lower Total Scale scores than their healthy matched controls. The total sample was divided into two groups: age Conclusion:This cross-sectional study found that DFNA9 patients demonstrated cognitive deficits in comparison with their healthy matched controls. The DFNA9 group aged >= 55 years old obtained significantly lower scores on the Total Scale and Attention subscale. This finding; however, was not observed for the age group younger than 55 years old. Further research is needed on the individual trajectory of SNHL and vestibular function, and how hearing rehabilitation affects cognitive functioning. | Notes: | Gommeren, H (corresponding author), Univ Antwerp, Fac Med & Hlth Sci, Dept Translat Neurosci, Campus Drie Eiken,Univ Pl 1, B-2610 Antwerp, Belgium. hanne.gommeren@uantwerpen.be |
Keywords: | Cognition;DeaFNess Autosomal Dominant 9;Hearing impairment;Vestibular dysfunction | Document URI: | http://hdl.handle.net/1942/40728 | ISSN: | 0196-0202 | e-ISSN: | 1538-4667 | DOI: | 10.1097/AUD.0000000000001315 | ISI #: | 001008065300004 | Rights: | 2022 The Authors. Ear & Hearing is published on behalf of the American Auditory Society, by Wolters Kluwer Health, Inc.This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. | Category: | A1 | Type: | Journal Contribution |
Appears in Collections: | Research publications |
Files in This Item:
File | Description | Size | Format | |
---|---|---|---|---|
accelerated_cognitive_decline_associated_with.4 (1).pdf | Published version | 755.07 kB | Adobe PDF | View/Open |
Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.