Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS)

An exceptional case of bilateral vestibular areflexia complicating acute otitis media

“…Oscillopsies during head and body movements are also major symptoms [2,8]. The absence of an intense rotatory character of the vertigo as it would be during the brutal vestibular deafferentations is due to the concomitant involvement of the two vestibules [2,[6][7][8]. In our case, the signi icant imbalance dominated the clinical picture.…”

“…The post-otitis infectious etiology of BVA is rare [2,4,5], and is seen mainly in the course of chronic otitis media [6]. Indeed, only three cases of otitis was found in the small Chinese series of 42 patients with bilateral vestibular weakness [4], but no case secondary to otitis was noted in Hain, et al series of 213 patients followed for BVP [2].…”

“…Vestibulo-ocular (VOR) and vestibulo-spinal re lex de icits during BVA lead to impaired orientation, navigation and spatial memory during movements of the head at high acceleration, which results in an oscillopsia induced by head movement and reduced dynamic visual acuity [2,[6][7][8]. In the absence of visual and proprioceptive signals, patients lose their sense of verticality and become disoriented.…”

A clinical case of cerebellar ataxia with neuropathy and vestibular areflexia syndrome in the presence of polycythemia vera

“…After some time the unsteady gait and faltering steps appeared when walking. The patient has been using a cane since 2016 Abdominal ultrasound test showed the following dimensions of the spleen: 12.6×5.5 cm, area -50 cm 2 . Taking into account a variety of schools of ultrasound diagnostic tests in medical establishments [7], both linear dimensions and the area of the largest section were measured.…”

“…Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome -CANVAS) -is a hereditary disease with a late onset that can be transmitted by an autosomal dominant or recessive type, the gene is not detected [1,2]. The clinical picture is determined by a variety of symptoms: cerebellar lesion, bilateral reduction of vestibular functions, and sensory polyneuropathy.…”