Causes of Bilateral Vestibulopathy

Timothy C. Hain, MD

Last updated 10/2012

In our own clinical experience, including at least 50 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), who found 66% of their 35 cases caused by ototoxins. Zingler et al also found ototoxins to be the most common cause, responsible for 13 percent of cases.

Syms and House (1995), at the House Ear Institute in Los Angeles, reported a different experience, with far more patients diagnosed with Meniere’s disease or “vascular” causes. We have no good explanation for this difference other than referral patterns. The following table lists the clinical diagnosis statistics from the Syms and House study.

Clinical Diagnosis Number of Patients
Ototoxic 16
Meniere’s Disease 16
Infection 11
Vascular 12
Trauma 6
Hereditary 2
Tumor 2
Unknown 34

Like Syms and House, Rinne et al (1998) and Brandt (1996) found about 11% of bilateral loss to derive from meningitis. Zingler et al reported that 5 percent of cases were due to meningitis.

Genetic causes have also been suggested. A pedigree has been reported with a progressive vestibular impairment having a phenotype similar to Meniere’s disease. This was traced to a mutation of the COCH genet (DFNA9), on chromosome 14. In a study by Jen et al., several families with inherited bilateral vestibulopathy were found to have a mutation on chromosome 6.

There are several rare causes of bilateral vestibulopathy. Head trauma is an uncommon cause of bilateral vestibular paresis, and in the very few reported cases, there is also hearing disturbance (for example, Fenneley et al, 1994).

There is a rare variant of bilateral loss that begins with migraine and episodic vertigo. This syndrome responds to acetazolamide (Jen, 2008; Baloh et al, 1994). A case of bilateral vestibulopathy due to aspirin ingestion has also been reported (Strupp, 2003).

Some have hypothesized that bilateral vestibulopathy is due to an autoimmune cause. Arbusow et al. identified antibodies against the membranous labyrinth in some patients. Currently, this is still theoretical.

In many cases of bilateral vestibulopathy, the underlying cause cannot be determined. Undetermined cases were reported to account for 20 percent (Brandt, 1996) and 50 percent (Zingler, 2007) by some authors.

A moderately-sized study discusses the causes and epidemiology of bilateral vestibulopathy in 255 patients Reich (2007).

References

  • Arbusow V, Strupp M, Dieterich M, et al. Serum antibodies against membranous labyrinth in patients with “idiopathic” bilateral vestibulopathy. J Neurol. 1998;245(3):132-6.
  • 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. In this study, 35 patient records were reviewed.
  • Jen JC, Wang H, Lee H, et al. Suggestive linkage to chromosome 6q in families with bilateral vestibulopathy. Neurology. 2004;63(12):2376-9
  • Reich SG, Boatman-Reich d. Causative factors and epidemiology of bilateral vestibulopathy in 255 patients. Ann Neurol. 2007;62(5):530.
  • Strupp M, Jahn K, Brandt T. Another adverse effect of aspirin; bilateral vestibulopathy. J Neurol Neurosurg Psychiatry. 2003;74(5):691.
  • Syms CA 3rd, House JW. Idiopathic Dandy’s syndrome. Otol HNS 116(1);75-8,1997
  • 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, Cnyrim C, Jahn K et al. Causative factors and epidemiology of bilateral vestibulopathy in 255 patients. Ann Neurol. 2007;61(6):524-32.