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

Gommeren, Hanne; Bosmans, Joyce; Moyaert, Julie; Mertens, Griet; Cras, Patrick; Engelborghs, Sebastiaan; Ombergen, Angelique Van; Gilles, Annick; Fransén, Erik; Berg, Raymond van de; JanssensdeVarebeke, Sebastien; Rompaey, Vincent Van

doi:10.1097/aud.0000000000001315

Cited by 8 publications

(8 citation statements)

References 80 publications

(127 reference statements)

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“…Most of the patient group with HL comprised persons with a heterozygous dominant-negative COCH gene variant, causing DFNA9 (53%) . This disorder has a progressive sensorineural HL and vestibular dysfunction that eventually evolves toward BV . Eighty-three percent of the participants with BV with NH had an idiopathic BV, often with a sudden onset.…”

Section: Discussionmentioning

confidence: 99%

Association of Bilateral Vestibulopathy With and Without Hearing Loss With Cognitive-Motor Interference

Danneels

Hecke

Leyssens

et al. 2023

JAMA Otolaryngol Head Neck Surg

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ImportanceThe past years, evidence suggested that the primary symptoms traditionally associated with bilateral vestibulopathy (BV) do not represent the full picture of this patient population. Recent literature also demonstrated cognitive impairment. However, although multitasking and dual-tasking are widely present in everyday activities, most of these studies assessed cognitive function only in single-task conditions.ObjectiveTo uncover the association of BV with and without hearing loss with cognitive and motor performance and cognitive-motor interference.Design, Setting, and ParticipantsThis prospective case-control study assessed persons with an isolated BV and persons with BV and a concomitant hearing loss compared with a healthy control group. Data were analyzed in December 2022. The study was conducted at Ghent University (Ghent, Belgium). Data collection took place between March 26, 2021, and November 29, 2022.Main Outcomes and MeasuresAll participants completed the 2BALANCE dual-task protocol, comprising a static and a dynamic motor task that was combined with 5 visual cognitive tasks. These cognitive tasks assessed mental rotation, visuospatial memory, working memory, response inhibition (executive function), and processing speed. All cognitive tasks were performed in a single-task condition (while seated) and in a dual-task condition (combined with a static and a dynamic motor task). The static task comprised balancing on a force platform with foam pad, and the dynamic task comprised walking at a self-selected speed on the GAITRite Walkway. Both motor tasks were performed in the single-task and dual-task condition.ResultsNineteen persons with BV and hearing loss (mean [SD] age, 56.70 [10.12] years; 10 women [52.6%]), 22 persons with an isolated BV (mean [SD] age, 53.66 [13.35] years; 7 women [31.8%]), and 28 healthy control participants were included (mean [SD] age, 53.73 [12.77] years; 12 women [42.9%]). Both patient groups had mental rotation and working memory impairment in a single-task condition and slower processing speed when walking (ie, during the dynamic dual-task condition). Additionally, the patient group with hearing loss had impaired visuospatial memory and executive function deficits in single-task and dual-task conditions, while this could only be elicited when performing a motor task in persons with isolated BV (ie, when dual-tasking).Conclusion and RelevanceThe findings of this case-control study suggest an association between vestibular function and cognitive and motor performance, even greater in persons with a concomitant hearing loss than in persons with an isolated BV.

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Section: Discussionmentioning

confidence: 99%

Association of Bilateral Vestibulopathy With and Without Hearing Loss With Cognitive-Motor Interference

Danneels

Hecke

Leyssens

et al. 2023

JAMA Otolaryngol Head Neck Surg

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“…The responsiveness of the RBANS-H will allow the establishment of a cognitive trajectory in a population with an inevitable evolution towards SNHL and BV, and possibly also dementia due to its increased risk for it and accelerated cognitive decline observed in this population 27…”

Section: Discussionmentioning

confidence: 99%

“…In contrast, homozygous loss-of-function mutations in COCH lead to autosomal recessive, early-onset, severe SNHL (DFNB110) 21 26. A recent cross-sectional study from Gommeren et al 27 found significantly worse results in patients with DFNA9 in cognitive performance compared with a cognitively healthy control group. This highlights the need for adequate treatment to prevent further cognitive decline and reduce dementia risk in this population.…”

Section: Introductionmentioning

confidence: 99%

Evaluation of hearing levels and vestibular function and the impact on cognitive performance in (pre)-symptomatic patients with DFNA9: protocol for a prospective longitudinal study (Rosetta study)

Gommeren,

Moyaert,

Bosmans

et al. 2023

BMJ Open

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IntroductionUntreated hearing loss is the largest potentially modifiable risk factor for dementia. Additionally, vestibular dysfunction has been put forward as a potential risk factor for accelerated cognitive decline. Patients with Deafness Autosomal Dominant 9 (DFNA9) present with progressive sensorineural hearing loss and bilateral vestibulopathy and show significantly worse results in cognitive performance compared with a cognitively healthy control group. This highlights the need for adequate treatment to prevent further cognitive decline. This study aims to determine how hearing and vestibular function evolve in (pre-)symptomatic carriers of the p.Pro51Ser mutation in theCOCHgene and how this impacts their cognitive performance and health-related quality of life.Methods and analysisA prospective, longitudinal evaluation of hearing, vestibular function and cognitive performance will be acquired at baseline, 1-year and 2-year follow-up. A total of 40 patients with DFNA9 will be included in the study. The study will be a single-centre study performed at the ORL department at the Antwerp University Hospital (UZA), Belgium. The control group will encompass cognitively healthy subjects, already recruited through the GECkO study. The primary outcome measure will be the Repeatable Battery for the Assessment of Neuropsychological Status adjusted for the Hearing-Impaired total score. Secondary outcome measures include Cortical Auditory-Evoked Potentials, vestibular assessments and health-related quality of life questionnaires. The expected outcomes will aid in the development of gene therapy by providing insight in the optimal time window for the application of gene therapy for the inner ear.Ethics and disseminationThe ethical committee of UZA approved the study protocol on 19 December 2022 (protocol number B3002022000170). All participants have to give written initial informed consent in accordance with the Declaration of Helsinki. Results will be disseminated to the public through conference presentations, lectures and peer-reviewed scientific publications.

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“…This study indicates that hearing loss is correlated with poorer scores on both the immediate memory and language subscales, as well as the total RBANS-H scale. Conversely, vestibular loss was found to be associated with reduced performance on the attention subscale of the RBANS-H ( 26 ).…”

Section: Discussionmentioning

confidence: 99%

Etiologies and hearing status in bilateral vestibulopathy: a retrospective study of 315 patients

Moyaert,

Dobbels,

Peetermans

et al. 2023

Front. Neurol.

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ImportanceThe development of a vestibular implant has reached milestones and seems to be a promising therapeutic tool for bilateral vestibulopathy (BV). Given the former lack of therapeutic options for BV, the disease has received scant attention in the previous research literature. It is therefore of major importance to gain more insight into the underlying pathology of BV. Furthermore, as some research groups specifically use a combined vestibulo-cochlear implant, the size of the group of BV patients with associated hearing loss is of special interest.ObjectivesThe study aimed to determine the definite and probable etiology in bilateral vestibulopathy (BV) patients and to report on their hearing status.DesignThis study involves multicenter retrospective study design.SettingThe research setting is at tertiary referral centers.ParticipantsConsecutive BV patients diagnosed at the Antwerp University Hospital between 2004 and 2018 at the Maastricht University Medical Center between 2002 and 2015 and at the Geneva University Hospital between 2013 and 2018, who met the BV diagnostic criteria of the Bárány Society.Main outcome measuresPrimary interests were the etiology and hearing status of BV patients. Moreover, the data of vestibular tests were examined (caloric irrigation, rotatory chair tests, and video-head impulse test).ResultsThe authors identified 315 BV patients, of whom 56% were male patients. Mean age at diagnosis was 58.6 ± 15.1 (range 7–91) years. The definite cause was determined in 37% of the patients and the probable cause in 26% of the patients. No cause was identified in 37% of BV patients. The largest subgroup included patients with genetic etiology (31%), most frequently COCH mutation. Only 21% of patients (n = 61) had bilateral normal hearing. Almost half of the patients (45%, n = 134) had profound hearing loss in at least one ear.ConclusionBV is a heterogeneous condition, with over a third of cases remaining idiopathic, and nearly three-quarters affected by hearing loss. COCH mutation is the most common non-idiopathic cause of BV in our population. Only 21% of our BV patients presented with bilateral normal hearing.

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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

Cited by 8 publications

References 80 publications

Association of Bilateral Vestibulopathy With and Without Hearing Loss With Cognitive-Motor Interference

Association of Bilateral Vestibulopathy With and Without Hearing Loss With Cognitive-Motor Interference

Evaluation of hearing levels and vestibular function and the impact on cognitive performance in (pre)-symptomatic patients with DFNA9: protocol for a prospective longitudinal study (Rosetta study)

Etiologies and hearing status in bilateral vestibulopathy: a retrospective study of 315 patients

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