Causes of Bilateral Vestibulopathy

Last revision: 1/2003

Timothy C. Hain, MDOscillopsia

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In our own clinical experience, including more than 100 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.

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).

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.

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). Occasionally patients with severe and protracted vertigo undergo bilateral vestibular nerve sections in an attempt to eliminate vertigo while preserving hearing.

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).

Bilateral vestibular loss from tumors is exceedingly rare. Occasional persons with von Recklinghausen's disease develop bilateral acoustic neuromas. The author of this brief review 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.

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.

Aging also causes bilateral vestibulopathy. 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).

Clinical Diagnosis

Rinne et al(1998)

N=53

Number of patients

Syms and House, 1995

17%

    16

<13%

    16

    Infection (Meningitis)

11%

    11

Autoimmune
9%  

    Vascular

 

    12

 

    6

 

    2

 

    2

    Unknown

21%

    34