What is bilateral vestibulopathy

Bilateral vestibulopathy

1 definition

A bilateral vestibulopathy is a bilateral disease of the organ of equilibrium, which leads to a partial or complete failure of the organ. It belongs to the vertigo syndromes.

2 causes

In over 50% of the total no cause can be determined (idiopathic). In these cases a degenerative disease can be assumed. The most common identifiable causes are:

Other possible causes of bilateral vestibulopathy include:

  • ENT operations
  • cerebellar diseases: CANVAS syndrome, spinocerebellar ataxias, multiple system atrophies
  • Labyrinthitis (e.g. streptococci, Mycobacterium tuberculosis)
  • Tumors: bilateral acoustic neuromas in neurofibromatosis type 2, non-Hodgkin lymphoma, meningiosis carcinomatosa, tumor infiltration of the skull base
  • Autoimmune diseases: Cogan's syndrome, neurosarcoid, Behcet's disease, cerebral vasculitis, systemic lupus erythematosus, polychondritis, rheumatoid arthritis, polyarteritis nodosa, granulomatosis with polyangiitis, giant cell arteritis, antiphospholipid syndrome
  • Neuropathies: vitamin B12 deficiency, vitamin B6 deficiency, hereditary sensory and motor neuropathy (HSMN IV)
  • bilateral sequential vestibular neuritis
  • congenital malformations: e.g. Usher syndrome
  • superficial siderosis
  • bilateral petrous bone fracture
  • Paget's disease
  • Macroglobulinemia
  • vertebrobasilar dolichoectasia

3 clinic

Patients with bilateral vestibulopathy usually complain of movement-dependent vertigo and an unsteady gait and stance, which increases in the dark and on uneven ground. Those affected are usually symptom-free when sitting or lying down. About 40% of patients notice blurring when walking and when moving their head (oscillopsia). Spatial memory and navigation disorders also occur, often accompanied by hippocampal atrophy.

4 diagnosis

For the diagnosis of bilateral vestibulopathy, at least two of the following 4 criteria must be present:

  • Unsteadiness when walking or standing and / or
    • motion-induced blurred vision or oscillopsia and / or
    • Worsening vertigo in the dark and / or on uneven ground
  • No symptoms when sitting or lying down without translational movements
  • Bilaterally reduced / missing function of the horizontal vestibulo-ocular reflex (VOR): testing by head impulse test according to Halmagyi and Curthoys or caloric testing
  • Lack of a better explanation from another illness

In the head impulse test, both right and left head rotation shows refixation saccades as an expression of the high frequency deficit of the VOR. If the result is unclear, video oculography can also be performed. The VOR reading test with determination of the decrease in visual acuity when the head is turned (Dynamic Visual Acuity) is also useful diagnostically. In addition, there are no disorders of the oculomotor system, except in patients with CANVAS syndrome or other cerebellar oculomotor disorders.

The stance and gait tests are largely normal when the eyes are open. When the eyes are closed, there is a swaying of the body in the Romberg standing test and when walking tightrope. The bilateral vestibulopathy can continue with or without hearing impairment.

Depending on the cause, there are other characters, e.g .:

5 differential diagnoses

6 therapy

Bilateral vestibulopathy due to an autoimmune origin can be treated symptomatically with the help of immunosuppressants. Glucocorticoids (e.g. high-dose steroid therapy in Cogan's syndrome) and possibly azathioprine or cyclophosphamide are suitable for this. It is crucial to promote central compensation through physical therapy.

7 prophylaxis

Patients with kidney failure, old age or familial ototoxic susceptibility should only receive aminoglycosides under strict indications. A combination of ototoxic drugs must be avoided.