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

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...

Full description

Bibliographic Details
Main Authors: Julie Moyaert, Bieke Dobbels, Olivier Peetermans, Bram Boon, Florence Lucieer, Nils Guinand, Griet Mertens, Annick Gilles, Paul van de Heyning, Angelica Pérez Fornos, Raymond van de Berg, Vincent Van Rompaey
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-11-01
Series:Frontiers in Neurology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fneur.2023.1271012/full
_version_ 1797448588531335168
author Julie Moyaert
Julie Moyaert
Bieke Dobbels
Bieke Dobbels
Olivier Peetermans
Bram Boon
Florence Lucieer
Nils Guinand
Griet Mertens
Griet Mertens
Annick Gilles
Annick Gilles
Paul van de Heyning
Paul van de Heyning
Angelica Pérez Fornos
Raymond van de Berg
Raymond van de Berg
Vincent Van Rompaey
Vincent Van Rompaey
author_facet Julie Moyaert
Julie Moyaert
Bieke Dobbels
Bieke Dobbels
Olivier Peetermans
Bram Boon
Florence Lucieer
Nils Guinand
Griet Mertens
Griet Mertens
Annick Gilles
Annick Gilles
Paul van de Heyning
Paul van de Heyning
Angelica Pérez Fornos
Raymond van de Berg
Raymond van de Berg
Vincent Van Rompaey
Vincent Van Rompaey
author_sort Julie Moyaert
collection DOAJ
description 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.
first_indexed 2024-03-09T14:12:42Z
format Article
id doaj.art-51416d9bcd354df0acacf9ded2700843
institution Directory Open Access Journal
issn 1664-2295
language English
last_indexed 2024-03-09T14:12:42Z
publishDate 2023-11-01
publisher Frontiers Media S.A.
record_format Article
series Frontiers in Neurology
spelling doaj.art-51416d9bcd354df0acacf9ded27008432023-11-29T05:51:31ZengFrontiers Media S.A.Frontiers in Neurology1664-22952023-11-011410.3389/fneur.2023.12710121271012Etiologies and hearing status in bilateral vestibulopathy: a retrospective study of 315 patientsJulie Moyaert0Julie Moyaert1Bieke Dobbels2Bieke Dobbels3Olivier Peetermans4Bram Boon5Florence Lucieer6Nils Guinand7Griet Mertens8Griet Mertens9Annick Gilles10Annick Gilles11Paul van de Heyning12Paul van de Heyning13Angelica Pérez Fornos14Raymond van de Berg15Raymond van de Berg16Vincent Van Rompaey17Vincent Van Rompaey18Department of Translational Neurosciences, Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumDepartment of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, Edegem, BelgiumDepartment of Translational Neurosciences, Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumDepartment of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, Edegem, BelgiumDepartment of Translational Neurosciences, Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumDepartment of Translational Neurosciences, Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumDivision of Balance Disorders, Department of Otorhinolaryngology and Head and Neck Surgery, Faculty of Health Medicine and Life Sciences, School for Mental Health and Neuroscience, Maastricht University Medical Center, Maastricht, NetherlandsService of Otorhinolaryngology Head and Neck Surgery, Department of Clinical Neurosciences, Geneva University Hospitals, Geneva, SwitzerlandDepartment of Translational Neurosciences, Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumDepartment of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, Edegem, BelgiumDepartment of Translational Neurosciences, Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumDepartment of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, Edegem, BelgiumDepartment of Translational Neurosciences, Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumDepartment of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, Edegem, BelgiumService of Otorhinolaryngology Head and Neck Surgery, Department of Clinical Neurosciences, Geneva University Hospitals, Geneva, SwitzerlandDivision of Balance Disorders, Department of Otorhinolaryngology and Head and Neck Surgery, Faculty of Health Medicine and Life Sciences, School for Mental Health and Neuroscience, Maastricht University Medical Center, Maastricht, NetherlandsFaculty of Physics, Tomsk State Research University, Tomsk, RussiaDepartment of Translational Neurosciences, Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumDepartment of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, Edegem, BelgiumImportanceThe 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.https://www.frontiersin.org/articles/10.3389/fneur.2023.1271012/fullbilateral vestibulopathyhearing lossCOCH proteinhumancausalityMeniere disease
spellingShingle Julie Moyaert
Julie Moyaert
Bieke Dobbels
Bieke Dobbels
Olivier Peetermans
Bram Boon
Florence Lucieer
Nils Guinand
Griet Mertens
Griet Mertens
Annick Gilles
Annick Gilles
Paul van de Heyning
Paul van de Heyning
Angelica Pérez Fornos
Raymond van de Berg
Raymond van de Berg
Vincent Van Rompaey
Vincent Van Rompaey
Etiologies and hearing status in bilateral vestibulopathy: a retrospective study of 315 patients
Frontiers in Neurology
bilateral vestibulopathy
hearing loss
COCH protein
human
causality
Meniere disease
title Etiologies and hearing status in bilateral vestibulopathy: a retrospective study of 315 patients
title_full Etiologies and hearing status in bilateral vestibulopathy: a retrospective study of 315 patients
title_fullStr Etiologies and hearing status in bilateral vestibulopathy: a retrospective study of 315 patients
title_full_unstemmed Etiologies and hearing status in bilateral vestibulopathy: a retrospective study of 315 patients
title_short Etiologies and hearing status in bilateral vestibulopathy: a retrospective study of 315 patients
title_sort etiologies and hearing status in bilateral vestibulopathy a retrospective study of 315 patients
topic bilateral vestibulopathy
hearing loss
COCH protein
human
causality
Meniere disease
url https://www.frontiersin.org/articles/10.3389/fneur.2023.1271012/full
work_keys_str_mv AT juliemoyaert etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT juliemoyaert etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT biekedobbels etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT biekedobbels etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT olivierpeetermans etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT bramboon etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT florencelucieer etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT nilsguinand etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT grietmertens etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT grietmertens etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT annickgilles etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT annickgilles etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT paulvandeheyning etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT paulvandeheyning etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT angelicaperezfornos etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT raymondvandeberg etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT raymondvandeberg etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT vincentvanrompaey etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients
AT vincentvanrompaey etiologiesandhearingstatusinbilateralvestibulopathyaretrospectivestudyof315patients