Bilateral vestibulopathy causes 4 gas planets

Zingler et al (2007) found ototoxicity to account for about 20% of their diagnosed patients (they only diagnosed 50% of their cases — so there were only 10% overall). This is a very low percentage. Zingler also noted a large # of patients with cerebellar signs. These differences in proportion probably reflects referral patterns — in the patients from Zingler et al, seen in a neurology clinic – -there would reasonable be a higher proportion of patients with neurological problems. Perhaps another clinic in Munich (the setting of the Zingler study) sees patients with ototoxicity. Their report is misleading in that it does not establish that bilateral loss patients, in general, have cerebellar problems. This is simply not a reasonable thought as the hair cells of the inner ear have little to do with the cerebellum. Practically, it is difficult to accumulate sufficient patients in a population based framework, so this question will probably go unanswered.

following meningitis, hearing is ordinarily also profoundly affected. This can be clearly established if the MRI/CT-Tbone studies show labyrinthitis ossificans – – basically petrification of the inner ear. On MRI, there is no fluid seen on T2. On CT, there is bone rather than fluid seen in the canals. This pattern is simlar to that seen on Cogan’s syndrome.

combined with labyrinthectomy, etc. Even though Chicago Dizziness and Hearing has thousands of Meniere’s patients, we have almost none with bilateral vestibular loss. It would not seem that Meniere’s is a very likely cause of bilateral loss.

used to make the diagnosis in these patients. We have encountered only a single patient with sequential bilateral vestibular loss (and hearing loss), associated with relapsing polychondritis (an autoimmune disorder). As of 2018, we had encountered 8 patients out of about 180 with a reasonable suspicion of autoimmune.

Lyme disease is rarely associated with bilateral vestibular loss. Recently, van Leewen et al reported on Lyme and Bilateral loss (van Leeuwen, van Kooten, & de Cock, 2016), in a single patient. They reviewed the literature and stated that their case was the only one. There are actually also some other sporadic case reports of lyme (Farshad-Amacker, Scheffel, Frauenfelder, & Alkadhi, 2013)

There are many reports of Syphilis causing hearing and vestibular symptoms together. (Garcia-Berrocal et al, 2006; Steckelberg & McDonald, 1984). We would be dubious that Syphilis could cause isolated bilateral loss. As of 2018, we have encountered -0- patients with syphilis causing bilateral loss. It can’t be very common. Hereditary conditions: Hereditary vestibular loss, without hearing loss

There is also a potential syndrome, called CANVAS, of cerebellar ataxia combined with bilateral vestibular loss, as well as a sensory neuropathy. Whether nor not this is a distinct entity or simply a chance occurrence is argued (Szmulewitz et al, 2011). Other cerebellar syndromes with bilateral vestibular loss such as SCA-3 (Machado Joseph), SCA-7, and Friedreich ataxia have overlapping symptoms. There are very very few of these patients.

Jen (2009) reviewed a tiny collection of families with inherited bilateral vestibulopathy with normal hearing. These included 3 dutch patients reported by Verhagen and colleagues(1987), the 3 families reported by Baloh (see above), and a Swedish family reported in 2003 by Brandtberg. In the Swedish family, most had normal VEMPs (implying that it may actually have been bilateral vestibular neuritis).

Recently Taylor and others reported a single case of a vestibular neuropathy due to Refsum disease. (2018). This is an incredibly rare genetic disorder characterized by a progressive neuropathy. This patient had nearly normal vestibular function, but the presence of a vestibular neuropathy was elucidated from delays in evoked potential testing. We think this case illustrates that one can have bilateral vestibular nerve damage, without classic signs of bilateral vestibular loss. This may be of relevance to other neuropathic disorders thought to affect vestibular function such as CANVAS, and Charcot Marie Tooth.

• Usami S1, Takahashi K, Yuge I, Ohtsuka A, Namba A, Abe S, Fransen E, Patthy L, Otting G, Van Camp G.Mutations in the COCH gene are a frequent cause of autosomal dominant progressive cochleo-vestibular dysfunction, but not of Meniere’s disease. Eur J Hum Genet. 2003 Oct;11(10):744-8

• Zingler VC and others. Causitive factors and epidemiology of bilateral vestibulopathy in 255 patients. Ann Neurol; 61: 524-532, 2007. [Comment: this is an odd paper that suggests that cerebellar signs are commonly found in persons with BV. This may be specific to the German population that was studied here]