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Causes of Bilateral Vestibulopathy

Timothy C. Hain, MD
Last updated 5/30/2002

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.

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

    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.

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 (Baloh et al, 1994). Also, 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 gene (DFNA9), on chromosome 14.

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. In this study, 35 patient records were reviewed.
  • 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
  • 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

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