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Oscillopsia of Dandy Revisited
Abstract & Commentary
By John J. Caronna, MD, Vice-Chairman, Department of Neurology, Cornell University Medical Center, Professor of Clinical Neurology, NewYork-Presbyterian Hospital. Dr. Caronna reports no financial relationship relevant to this field of study.
Synopsis: The most common cause of bilateral vestibulopathy remains ototoxicity from antibiotics, such as gentamicin.
Source: Zingler VC, et al. Causative Factors and Epidemiology of Bilateral Vestibulopathy in 255 Patients. Ann Neurol. 2007; 61: 524-532.
Walter Dandy (1886-1946), who bilaterally sectioned the vestibular nerve to treat Meniere's syndrome, was the first to recognize that total loss of labyrinthine function caused vision to become blurred when the head moves rapidly.1 Today, impairment or loss of function of both peripheral labyrinths or of both eighth nerves is rare.
Zingler and associates sought to determine the causes and epidemiology of bilateral vestibulopathy (BV). The diagnosis was suspected clinically in patients with the following symptoms:
1. Unsteadiness of gait, particularly in the dark or on uneven ground.
2. Apparent motion of the visual scene during head movements and locomotion (oscillopsia), and
3. Impaired spatial memory and navigation associated with hippocampal atrophy due to chronic loss of vestibular input.2
BV was confirmed by the head-thrust test3 and caloric irrigation of the external auditory canal with oculographic recordings. All patients underwent a standardized neuro-ophthalmological and neuro-otological examination, MRI or CT scans and laboratory tests. In a retrospective review, they found 255 patients (mean age 62 ± 16 years) diagnosed with BV between 1988 and 2005.
Of all patients, 27% (n = 70) suffered from complete BV. The remaining patients (n = 185) exhibited incomplete BV.
The definite cause of BV was defined in 24% and the probable cause in 25%.
Table: Patients with BV of Definite or Probable Cause (n = 125)*
|Cause of BV||Definitive or Probable Cause|
|Spinocerebellar ataxia, MSA||12||5|
|Familial inner ear disease||3||1|
*after Zingler, et al.
**Neurofibromatosis type 2, aspirin or furosemide toxicity, head trauma,
otosclerosis, hemosiderosis, and alcoholism
Episodes of vertigo preceded the manifestations of BV in 36% of patients (n = 91). BV developed slowly in 64% (n = 164). In the 51% of patients who suffered from idiopathic BV of unknown cause, symptoms more often were slowly progressive rather than sequentially progressive. Oscillopsia occurred in 44% and was more frequent in younger patients with an episodic or sequential course and with complete loss of peripheral vestibular function. Hypoacusis occurred bilaterally in only 25% and unilaterally in only 6% of all patients. Hearing loss appeared most often in patients with Cogan's syndrome (inflammatory eye disease, hearing loss, and vestibulopathy), meningitis, or Meniere's disease. In patients without hearing loss, BV generally developed progressively and often in combination with a cerebellar syndrome or a peripheral neuropathy, or both.
Cerebellar signs were present in 25% of patients. Cerebellar dysfunction was associated with peripheral neuropathy in 32% compared with 18% in BV patients without cerebellar signs.
Physicians who practiced in the days when streptomycin was a first-line antibiotic for treating bacterial infections such as endocarditis will remember patients who developed BV due to the ototoxicity of that drug. Sadly, ototoxic BV caused by antibiotic treatment with gentamicin alone or in combination with other ototoxic agents such as diuretics was still the most frequent cause of BV in this series. Zingler et al have pointed out that BV may develop insidiously without hearing loss but in association with other neurological findings. BV may be a feature of several spinocerebellar ataxias (SCAs) and of multisystem atrophy. Some patients showed signs of an axonal peripheral neuropathy. Therefore, as pointed out by Leigh and Thurbell in their editorial accompanying this report4 BV is a condition that neurologists need to be aware of and able to diagnose in patients presenting with problems with balance.
1. Dandy WE. Surg Gynecol Obstet. 1941; 72: 421-425.
2. Brandt T, et al. Brain. 2005; 128: 2732-2741.
3. Halmagyi GM, Curthoys IS. Arch Neurol. 1988; 45: 737-739.
4. Leigh RJ, Thurtell M. Ann Neurol. 2007; 61: 499-500.