Timothy C. Hain, MD Page last modified: August 19, 2018
Additional Disclaimer: This material is not written for legal use, including trial testimony.
About 28/100,000 people in the US have bilateral vestibular loss (Ward et al, 2013). We have recently reviewed the subject of bilateral vestibular loss (see Hain, Cherchi and Yacovino, 2018).
At Chicago Dizziness and Hearing, we have seen many patients with bilateral vestibulopathy. The figure below shows data from a 176 patient subset of our entire database compiled in 2014. This data is being actively updated and does not include all of our patients, as this is a big project. These are all patients referred for clinical diagnosis. It does not include patients referred through medicolegal review activity.
As can easily be seen, the majority are "idiopathic", and the second largest group are aminoglycoside ototoxicity. There is also a moderate literature concerning other similar clinic collections (see below).
Rinne et al(1998)
Zingler et al (2007)
Syms and House, 1995
Number of patients
Chicago Dizziness and Hearing, 2014
Infection (Meningitis, CJD, bilateral vestibular neuritis)
Autoimmune, including Cogan's
While we have not updated this table, as of 2018, the numbers are similar.
"Unknown" -- no diagnosis - -is the most common category for all patients -- roughly accounting for 50% of all cases.
In our own clinical experience, a mixed otology-neurology setting -- we have found gentamicin toxicity to be a common cause of bilateral vestibular loss (see pie chart above). This resembles the experience of Gillespie and Minor at Johns Hopkins (see references, Otology setting), who found 66% of their 35 cases caused by ototoxins. Syms and House (1995), at the House Ear institute in Los Angeles, reported a different experience, with more patients diagnosed with Meniere's disease or "vascular" causes.
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.
Nevertheless, in all reports, ototoxicity is the single most common diagnosed cause.
Bilateral sequential vestibular neuritis is a significant cause of bilateral vestibular loss. In these cases, the loss is not complete. While the lateral canals may be unresponsive on both sides, there often is evidence of retained posterior canal function, as well as presumably saccule function. Curiously, Zingler et al reported no patients at all with this condition.
Aging also causes bilateral vestibulopathy, although it is only rarely severe. Roughly, at the age of 80, half of the cells in the vestibular ganglion have died. Blood flow to the inner ear is also reduced with aging (Lyon and Davis, 2001). This reduction presumably contributes to the invariable loss of balance function that occurs as people age. It can be diagnosed with rotatory chair testing.
Like Syms and House, Rinne et al (1998) and Brandt (1996) found about 11% of bilateral loss to derive from meningitis. In persons with bilateral loss 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.
We have not seen many patients with meningitis at Chicago Dizziness and Hearing, perhaps this is because in the years where we collected patients, Meningitis has become rarer due to availability of antibiotics.
Rare causes of bilateral vestibulopathy:
Meniere's disease, while associated with repeated attacks of vertigo, rarely causes bilateral vestibular loss. Hearing seems to be more sensitive to the disease process of Meniere's than does vestibular function. Some patients diagnosed as Meniere's disease may have their disorder due to autoimmune processes (see below). In some instances the treatments for Meniere's disease may result in bilateral loss. This is not unheard of as many persons with Meniere's develop bilateral disease. Situations may occur where there are bilateral vestibular nerve sections, a nerve section 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.
Autoimmune inner ear disease or AIED undoubtedly causes a small number of cases of bilateral vestibular loss. (Lucieer et al., 2016). At present the diagnosis of AIED is difficult as there are no reliable laboratory tests for AIED. A combination of a progressive pattern of bilateral vestibular loss, absence of other reasonable causes, response to steroids or other immunomodulator drugs, and coincidence of other autoimmune disorders is 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.
Head trauma is an uncommon cause of bilateral vestibular paresis, and in the very few reported cases, there is also hearing disturbance (e.g. Fenneley et al, 1994). We have encountered this condition in about 5% of our very large population. Occasionally patients with severe and protracted vertigo undergo bilateral vestibular nerve sections in an attempt to eliminate vertigo while preserving hearing.
Migraine associated bilateral vestibular loss. We have encountered this pattern in roughly 1.5% of our patient population. We are dubious that this is a real association. It may be just chance.
Another rare cause of bilateral loss is superficial siderosis, an iron deposition usually related to repeated bleeding associated with a tumor or a vascular malformation in the brainstem or cerebellum (Watanabe, 1997). Hearing and vestibular loss gradually progresses over many years (Weekamp et al. 2003). Superficial siderosis can be easily diagnosed on MRI from the characteristic hypointense areas where iron has been deposited. Cochlear implantation may not work in superficial siderosis (Wood et al, 2008).
Wernicke's Encephalopathy, which is due to acute thiamine deficiency (often in the context of alcoholism), was recently reported to be a rare cause of a partial bilateral vestibulopathy, mainly affecting the horizontal canals. (Lee et al, 2018). This was established using VHIT -- these authors reported 5 new patients and reviewed a world literature including a total of 17 patients. Wernickes damages many parts of the brainstem, as well as parts of the cerebral cortex, and attributing a particular finding to a particular structure can be tricky. Wernicke's is rare in the author's clinical context (Chicago Illinois), and he has seen one case in the last 30 years. Presumably there is more Wernickes where alcoholism is more common.
As Wernicke's often affects the horizontal eye muscles, and often is characterized by bilateral 6th nerve palsies, we think it might be difficult to separate damage to the oculomotor system from damage to the vestibular system. This is because in the VHIT test, vestibular driven eye movements need to travel at the same speed as small saccades. In other words, we are suggesting that perhaps the VHIT test is confounding eye movement weakness with vestibular weakness. We are also dubious that a metabolic disorder such as Wernicke's could selectively affect the lateral canals, by "enhanced vulnerability of the medial vestibular nuclei neurons to thiamine deficiency", and spare the vertical canal neurons, as was suggested by Lee et al (2018). The evidence for this occuring is sparse. A study that compares VHIT recordings made in each eye, would be interesting. We would also like to have ocuolomotor function documented in these patients. More for someone to study !
a Video of a case of upbeating nystagmus due to Wernicke's encephalopathy. This nystagmus is probably due to damage to the NPH nucleus, not the vestibular nucleus, as these patients usually have very odd patterns of gaze holding. Nevertheless, if gaze holding depends on the vestibular nucleus, this is still concievable.
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)
Yersinia has been reported on one occasion (Bucheler and Lowenheim, 1997) . Yersinia is related to the bubonic plague.
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 vestibular loss, without hearing loss
There is a rare variant of bilateral vestibular loss that begins with migraine and episodic vertigo. This syndrome responds to acetazolamide (Baloh et al, 1994). We have found a few of these in our own practice.
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).
Hereditary hearing loss and vestibular loss syndromes
DFNA9 (also called COCH) can culminate in deafness and vestibular loss (Bom, 1999; Lemaire et al, 2003; Verhagen et al, 2000). According to Usami et al, the COCH gene seems to mainly be involved with hearing rather than vestibular symptoms (Usami et al, 2003).
According to Jen, DFNA11 can also cause vestibular disturbances similar to Meniere's.
Hereditary neuropathies with evidence of an vestibular impact.
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.
Charcot Marie Tooth is a much more common inherited polyneuropathy than Refsum disease. Poretti et al (2013) reported reduction of VHIT gain and increased cVEMP latencies in 15 patients with CMT. This supports the conjecture that CMT is a cause of bilateral vestibular impairment. This may be related to the underlying pathophysiology of demyelination.
Presumably there are many other cases like this involving other hereditary as well as acquired neuropathic illnesses.
Bilateral vestibular loss from tumors is exceedingly rare. Occasional persons with von Recklinghausen's disease develop bilateral acoustic neuromas. The author of this page has seen one patient in whom an acoustic neuroma was responsible for unilateral loss on one side, combined with presumed vestibular neuritis on the other side.
Bilateral vestibular loss from surgery is also unusual. This may result from bilateral vestibular nerve section, or a combination of nerve section on one side and gentamicin on the other. We have encountered several patients with this over the years, and it accounts for about 1% of our population.