Causes of Bilateral Vestibulopathy

Timothy C. Hain, MD Page last modified: January 5, 2008

Additional Disclaimer: This material is not written for legal use, including trial testimony.

Common Causes:

"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 -- including more than 200 cases diagnosed by the author, we have found gentamicin toxicity to be the predominant cause of bilateral vestibular loss. This closely 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 reasonabl 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. In the author's material, about 2% of all bilateral loss is due to bilateral sequential vestibular neuritis. 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.

Rare causes:

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.

Autoimmune inner ear disease or AIED undoubtedly causes a moderate number of cases of bilateral vestibular loss. 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.

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 patients on rare occasions less than 1% 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.

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.

Hereditary hearing loss syndromes such as DFNA9 can culminate in deafness and vestibular loss (Lemaire et al, 2003). There is a rare variant of bilateral loss that begins with migraine and episodic vertigo. This syndrome responds to acetazolamide (Baloh et al, 1994).

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.

References

• Baloh RW, Jacobson KJ, Fife T. Familial vestibulopathy: a new dominantly inherited syndrome. Neurology 1994:40:20-25
• Brandt T. Bilateral vestibulopathy revisited. Eur J. Med Res 1996:1:361-368
• Feneley, M. R. and P. Murthy (1994). "Acute bilateral vestibulo-cochlear dysfunction following occipital fracture." J Laryngol Otol 108(1): 54-6.
• Gillespie MB, Minor LB. Prognosis in bilateral vestibular hypofunction. Laryngoscope, 109, 1999, 35-41. 35 patient records were reviewed
• Lemaire FX, Feenstra L, Huygen PL, Fransen E, Devriendt K, Van Camp G, Vantrappen G, Cremers CW, Wackym PA and Koss JC (2003). "Progressive late-onset sensorineural hearing loss and vestibular impairment with vertigo (DFNA9/COCH): longitudinal analyses in a belgian family." Otol Neurotol 24(5): 743-8.
• Lyon MJ, Davis JR. Age-related blood flow and capillary changes in the rat utricular macula ... JARO 03:167-173, 2001
• Rinne T, and others. Bilateral loss of vestibular function: clinical findings in 53 patients. J. Neurol 1998:245:314-321
• Syms CA 3rd, House JW. Idiopathic Dandy's syndrome. Otol HNS 116(1);75-8, 1997.
• Watanabe, M., et al., [A case of superficial siderosis of the central nervous system with bilateral vestibular dysfunction]. No To Shinkei, 1997. 49(10): p. 931-5.
• Weekamp HH, Huygen PL, Merx JL, Kremer HP, Cremers CW and Longridge NS (2003). "Longitudinal analysis of hearing loss in a case of hemosiderosis of the central nervous system." Otol Neurotol 24(5): 738-42.
• Wim I. M. Verhagen, MD, PhD; Steven J. H. Bom, MD; Patrick L. M. Huygen, PhD; Erik Fransen, PhD; Guy Van Camp, PhD; Cor W. R. J. Cremers, MD, PhD Familial Progressive Vestibulocochlear Dysfunction Caused by a COCH Mutation (DFNA9) Arch Neurol, Vol. 57 No. 7, July 2000
• 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]