Association of Fibromuscular Dysplasia and Pulsatile Tinnitus: A Report of the US Registry for Fibromuscular Dysplasia

Abstract

Background Fibromuscular dysplasia (FMD) is a nonatherosclerotic arterial disease that has a variable presentation including pulsatile tinnitus (PT). The frequency and characteristics of PT in FMD are not well understood. The objective of this study was to evaluate the frequency of PT in FMD and compare characteristics between patients with and without PT. Methods and Results Data were queried from the US Registry for FMD from 2009 to 2020. The primary outcomes were frequency of PT among the FMD population and prevalence of baseline characteristics, signs/symptoms, and vascular bed involvement in patients with and without PT. Of 2613 patients with FMD who were included in the analysis, 972 (37.2%) reported PT. Univariable analysis and multivariable logistic regression were performed to explore factors associated with PT. Compared with those without PT, patients with PT were more likely to have involvement of the extracranial carotid artery (90.0% versus 78.6%; odds ratio, 1.49; P =0.005) and to have higher prevalence of other neurovascular signs/symptoms including headache (82.5% versus 62.7%; odds ratio, 1.82; P <0.001), dizziness (44.9% versus 22.9%; odds ratio, 2.01; P <0.001), and cervical bruit (37.5% versus 15.8%; odds ratio, 2.73; P <0.001) compared with those without PT. Conclusions PT is common among patients with FMD. Patients with FMD who present with PT have higher rates of neurovascular signs/symptoms, cervical bruit, and involvement of the extracranial carotid arteries. The coexistence of the 2 conditions should be recognized, and providers who evaluate patients with PT should be aware of FMD as a potential cause.

Prevalence and clinical characteristics of renovascular hypertension associated with fibromuscular dysplasia in China

“…In this study, the mean age at diagnosis of FMD was 26.9 years, which was similar to previous reports from other East Asian countries but much younger than the patients in the U.S. Registry (54.5 years) [4] and ARCADIA Registry (52.7 years) [26].…”

“…In this study, the mean age at diagnosis of FMD was 26.9 years, which was similar to previous reports from other East Asian countries but much younger than the patients in the U.S. Registry (54.5 years) [4] and ARCADIA Registry (52.7 years) [26]. The mean age at diagnosis of FMD in adults (>18 years) was 29.3 years and the duration of hypertension was about 4 years.…”

“…In the ARCADIA Registry [26], 84.5% of patients were female vs. 95.7% in the U.S. Registry [4]. These inconsistencies might be because patients enrolled in our study were all hypertensive patients with RAS diagnosed at a young age, while those with other symptoms suggesting intracranial arteries FMD without hypertension were left out.…”

“…Renal arteries are the most commonly involved vessels, although arteries in other vascular territories can also be affected. In the FMD cohort of the United States (U.S.) registry, there were 66.1% [4] with renal lesions vs. some 90.7% in the European/International registry [5]. Renal artery FMD is the second most frequent cause of renal artery stenosis (RAS) and renovascular hypertension is the most common clinical presentation of FMD [6,7], especially in children [8].…”