Bilateral Vestibulopathy - Current Knowledge and Future Directions to Improve its Diagnosis and Treatment

Bilateral vestibulopathy was recently defined by the Barany society. Unlike in unilateral loss of vestibular function where compensation is typical, many patients with bilateral vestibulopathy experience chronic oscillopsia due to failure of the vestibulo-ocular reflex and gait instability due to failure of vestibulo-spinal reflexes. Other vestibular projections such as those to higher level cognitive processes like spatio-temporal navigation and autonomic reflexes may also be affected and are active areas of investigation. Bilateral vestibulopathy is estimated to affect 21 in 100,000 people and in its most severe forms can have profound impacts on quality of life and lead to increased risk of falls. There are numerous potential contributing factors including aminoglycoside antibiotics like gentamicin, but also genetic causes such as mutations in the COCH gene or syndromes such as neurofibromatosis type 2 or cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS). Its prevalence may be underappreciated, as common diagnoses like diabetes or presbyvestibulopathy also can lead to bilateral vestibular failure. Many cases, however, remain idiopathic. The diagnosis of bilateral vestibulopathy is often delayed due to patients’ nonspecific symptoms and clinicians’ unfamiliarity with the diagnosis. This delayed diagnosis places patients at risk for unnecessary diagnostic tests and delayed initiation of treatment. Diagnostic tools to quantify the functional integrity of all parts of the vestibular organs are now available in specialized clinics. These novel tests offer new opportunities to characterize patterns of vestibular impairment in distinct disorders leading to bilateral vestibulopathy. Treatment of bilateral vestibulopathy has included vestibular rehabilitation and employing falls prevention strategies; nevertheless, many patients fail to improve or even worsen over time. Newer treatments including hair cell regeneration and vestibular implants may provide benefit, and clinical trials are ongoing. With the advent of new therapies, there is urgency to define and better understand patients with bilateral vestibulopathy. This Research Topic will highlight new developments in the understanding of bilateral vestibulopathy and its potential treatments.

In order to address the latest research in bilateral vestibulopathy, this Research Topic will include themes such as an exploration of the large class of patients with bilateral vestibulopathy currently considered idiopathic, by identifying novel pathophysiologic mechanisms. Other themes will include a historical perspective on the early recognition of the syndrome of bilateral vestibulopathy, the impact of bilateral vestibular impairment on quality of life, and how advances in diagnostics are refining our understanding of what it means to have bilateral vestibulopathy. New developments in treatment strategies for patients with bilateral vestibulopathy will also be featured. The Topic Editors welcome original research, reviews, perspectives, opinions and case reports to be submitted to the Research Topic.