Cochleovestibular involvement in patients with Fabry disease: data from the multicenter cohort FFABRY

Asquier-Khati, Antoine; Mauhin, Wladimir; Michel, Guillaume; Gendre, Adrien; Durant, C.; Lavigne, Christian; Maillard, Hélène; Lacombe, Didier; Willems, Marjolaine; Lidove, Olivier; Masseau, A.

doi:10.1007/s00405-021-07173-x

Cited by 2 publications

(3 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Our study also showed that peripheral vertigo occurred in 28.3% of patients with Fabry disease. Prior studies have documented high rates of vertigo or dizziness among persons with Fabry disease, ranging between 10.7% and 54% [ 11 – 14 ], without, however, distinguishing true vertigo from non-specific dizziness, and between peripheral causes of vertigo from central lesion-caused vertigo. Patients with Fabry disease may be more susceptible to cardiovascular or cerebrovascular disease [ 15 ], which may also cause dizziness and fainting, mimicking vertigo from vestibular diseases.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with Fabry disease may be more susceptible to cardiovascular or cerebrovascular disease [ 15 ], which may also cause dizziness and fainting, mimicking vertigo from vestibular diseases. Nevertheless, patients with Fabry disease have manifested high susceptibility to impaired vestibular tests such as head impulse test (HIT) [ 14 , 16 , 17 ], electro-/video-nystagmography (ENG/VNG) [ 14 , 18 ], caloric testing [ 16 , 18 , 19 ], or vestibular evoked myogenic potentials (VEMP) [ 16 , 19 ]. Progressive deterioration of vestibular function is also reported, affecting 80% of men and 77% of women when assessed with head impulse testing [ 16 ].…”

Section: Discussionmentioning

confidence: 99%

“…Ganglion cells exhibited a ballooning appearance with granulating nuclei in the vestibular ganglia. Some studies did not found involvement of eighth cranial nerve, and postulated that cochleovestibular ischemia caused by lipid accumulations in the endothelial cells may explain the vertigo [ 14 , 19 ].…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Increased prevalence of peripheral vestibular disorder among patients with Fabry disease

Yang,

Xirasagar,

Cheng

et al. 2024

Orphanet J Rare Dis

View full text Add to dashboard Cite

Background Although peripheral vestibular disorder is a non-fatal complication of Fabry disease, fatalities have been reported in some case reports and case series. To date, no studies have examined the relative risk of peripheral vestibular disorder in patients with Fabry disease compared to the general population without the condition. Due to the high prevalence of Fabry disease in East Asia and the potential shared pathogenic pathways between Fabry disease and vasculopathy, we conducted a study using a nationwide population-based dataset to compare the prevalence of peripheral vestibular disorder between patients with Fabry disease and matched comparison patients. Methods Data was sourced from Taiwan’s Longitudinal Health Insurance Database 2010. this study consists of 11,668 sampled patients, 2917 study patients with Fabry disease and 8751 propensity-score-matching comparison patients. We conducted multiple logistic regression analysis to study the association between peripheral vestibular disorder and Fabry disease. Results The study identified notable differences in the prevalence of various vestibular disorders between the study and comparison groups. Specifically, there was a 7.2% increased prevalence of peripheral vestibular disorder in the study group (28.3%) compared to the comparison group (20.9%), Meniere’s disease (5.4% vs. 3.7%), benign paroxysmal positional vertigo (5.1% vs. 3.3%), and other/ unspecified peripheral vestibular dizziness (15.6% vs. 11.8%) (all p < 0.001). The odds ratios for PVD, MD, BPPV, and other PVD were 1.44 (95% CI = 1.29–1.60), 1.50 (95% CI = 1.23–1.83), 1.59 (95% CI = 1.30–1.95), and 1.40 (95% CI = 1.24–1.58), respectively, among the Fabry disease group relative to the comparison group after adjusting for age, monthly income, geographic location, urbanization level, hyperlipidemia, diabetes, coronary heart disease, and hypertension. Conclusion This study found that patients with Fabry disease had increased prevalence of peripheral vestibular disorder.

show abstract

Section: Discussionmentioning

confidence: 99%