Vicente Honrubia

Benign positional vertigo Clinical and oculographic features in 240 cases

We report the clinical and oculographic features in 240 patients with benign positional vertigo (BPV). In each case, after a rapid position change from the sitting to head-hanging position, a stereotyped torsional paroxysmal positional nystagmus was visually observed and recorded with electronystagmography (ENG). The mean age of onset was 54 years, with a range of 11 to 84 years. In slightly more than one-half of the cases (122/240) a likely diagnosis was determined. The most common identifiable causes were head trauma (17%) and viral neurolabyrinthitis (15%). Females outnumbered males approximately two to one in the idiopathic group. Abnormalities on bithermal caloric testing were found in 47% of patients. Only two patients, both with well-documented neurologic disorders, had central signs on ENG. Our data are consistent with a peripheral, posterior semicircular canal origin of BPV.

Quantitative measurement of saccade amplitude, duration, and velocity

A method for rapid, accurate measurement of saccade amplitude, duration, and velocity (average and maximum) was developed as a functional test of the extraocular motor system. Recordings were made with a direct-current electro-oculographic system, and data analysis was performed on a laboratory digital computer. Saccade amplitude and duration were found to be linearly correlated in 25 normal subjects, with a mean slope of 2.7 msec per degree over a large amplitude range. In the same subjects, saccade amplitude and velocity (maximum or average) had a nonlinear relationship that was best fit by an exponential equation. The two constants of this equation adequately characterized the relationship between saccade amplitude and velocity and permitted rapid statistical comparison between normal and abnormal subjects.

Horizontal semicircular canal variant of benign positional vertigo

We report the clinical features and results of quantitative eye-movement testing in 13 patients with episodic positional vertigo and nonfatiguing direction-changing horizontal positional nystagmus (beating to the right with the head turned to the right and beating to the left with the head turned to the left). The benign history and lack of associated neurologic findings support a peripheral localization of the lesion. This syndrome probably represents a horizontal semicircular canal variant of benign positional vertigo. Free-floating debris in one horizontal canal may explain many of the clinical and oculographic findings.

Persistent direction‐changing positional nystagmus Another variant of benign positional nystagmus?

Positional nystagmus that does not fatigue, persists as long as the position is held, and changes direction in different head positions has typically been attributed to central vestibular lesions. We recently studied three patients who presented with positional nystagmus having these features but almost certainly of benign peripheral origin. All three had an initial history typical of benign positional vertigo and, in two, the persistent direction-changing positional nystagmus occurred after the patient underwent a maneuver to remove debris from the posterior semicircular canal. The positional nystagmus profile and clinical course are consistent with the debris leaving the posterior semicircular canal and becoming attached to the cupula of the horizontal semicircular canal.

The saccade velocity test

Scatter plots showing the amplitude versus velocity (maximum and average) relationship of horizontal saccades in 25 normal subjects and four groups of patients were statistically compared. Three patients with “subclinical” medial longitudinal fasciculus syndromes had significant slowing of adducting saccades, and two of these patients had unsuspected slowing of abducting saccades (although to a lesser degree). Five patients with olivopontocerebellar degeneration and three patients with myotonic dystrophy had significant slowing of saccades in both directions. Five patients with surgically documented acoustic neuromas did not have significant slowing despite brain-stem compression in three. It is concluded that the saccade velocity test can be a useful clinical tool in addition to its potential in clinical research.

Aging and the human vestibular nucleus.

Degenerative changes during aging have been identified in the inner ear and in the vestibular nerve, but not in the human vestibular nuclear complex (VNC). Therefore, the purpose of this study was to document quantitative morphometric changes within the VNC in humans as a function of age. The VNC of normal human subjects was examined for age-related changes using computer-based microscopy. Neuronal counts, nuclear volume, neuronal density, and nuclear length of the 4 vestibular nuclei were determined in 15 normal people, age 40 to 93 years. Based on a linear model, there was approximately a 3% neuronal loss per decade from age forty to ninety. VNC volume and neuronal density also decreased significantly with age, although to a lesser degree than did the number of neurons. Neuronal loss as a percentage of the total number of neurons was greatest in the superior vestibular nucleus and least in the medial vestibular nucleus. Despite the overall loss of neurons, the number of giant neurons (> 500 microns2) increased in older people. This increase in giant neurons could be traced to the accumulation of lipofuscin deposits in the cell somata. The overall rate of neuronal loss with aging in the VNC is comparable to that previously observed in hair cells, primary vestibular neurons, and cerebellar Purkinje cells, but is in contrast to prior reports of no age-related loss of neurons in other brain stem nuclei.

Idiopathic bilateral vestibulopathy

We report the clinical features of 22 patients with acquired bilateral vestibulopathy of unknown cause. All had either absent or markedly decreased responses to both caloric and rotational testing. They presented with dysequilibrium and imbalance, worse at night; most reported oscillopsia but none had associated hearing loss or other neurologic symptoms. Nine reported prior prolonged episodes of vertigo consistent with the diagnosis of bilateral sequential vestibular neuritis. Of the remaining 13, none had exposure to known ototoxins or a positive family history. Idiopathic bilateral vestibulopathy is an important cause of progressive imbalance in adults and should be considered even though hearing is normal.

Eye-Tracking and Optokinetic Nystagmus Results of Quantitative Testing in Patients with Well-Defined Nervous System Lesions

Eye-tracking and optokinetic nystagmus (OKN) abnormalities in patients with focal lesions of the nervous system are reviewed. Patients with peripheral labyrinthine lesions can have deficits in smooth pursuit and OKN, but they are rapidly compensated after an acute lesion. By contrast, patients with large, cerebellopontine angle tumors have progressive impairment of pursuit and OKN as the tumor enlarges. Abnormalities of saccadic eye movements suggest intrinsic central nervous system (CNS) dysfunction. Saccade accuracy is severely impaired with cerebellar lesions, while brain stem disease frequently results in a slowing of saccade maximum velocity. Smooth pursuit and OKN abnormalities are common with all types of CNS lesions. The pattern of eye-tracking and OKN abnormality can be useful in anatomically localizing nervous system lesions.

Quantification of the process of hair cell loss and recovery in the chinchilla crista ampullaris after gentamicin treatment.

The degree of ototoxic drug sensitivity and hair cell repair was determined in the chinchilla horizontal crista ampullaris after intraotic administration of gentamicin. Histological evaluation was made of 22 cristae ampullaris from one normal and six post‐treatment (PT) animal groups killed at 1, 4, 7, 14, 28, and 56 days. New hair cell production was quantified, using the dissector technique. Transmission electron microscopy was used to investigate the ultrastructural characteristics of the hair cells in the regenerated epithelium. At I day PT, type I and II hair cells presented cytoplasmic vacuolization, swollen nerve calyces and 20% of type I and 18% of type II hair cells were lost. At 4days PT, 95% of type I hair cells and 14% of type II hair cells had disappeared. In addition, most of the type II hair cells showed clumping of nuclear material. Nerve fibers were not found in the sensory epithelium, but were still observed below the basal lamina. Supporting cells appeared unaffected, maintaining their location in the crista. At 1 and 4 days PT, the damage to hair cells was more pronounced in the central region of the crista ampullaris. The degree of ototoxic damage at 7 days was similar to that of 14 days: no type I hair cells were present and most of the type II hair cells had disappeared; supporting cell nuclei began to occupy the apical part of the sensory epithelium and most of the nerve fibers had retracted. Quantitatively, 87 and 93% of type II hair cells were lost at 7 and 14 days PT, respectively. Initial signs of hair cell recovery began at 28 days PT; immature type II‐like hair cells appeared, supporting cell nuclei began to align at the base of the sensory epithelium and nerve fibers penetrating the basal lamina were observed. No type I hair cells were found, but 40% of the normal number of type II hair cells were present. Hair cells appeared to regenerate in the peripheral areas of the cristae ampullaris first. At 56 days PT, an increase in the number of mature type II hair cells was present, supporting cells were aligned at the base of the epithelium, and more nerve fibers appeared to penetrate the basal lamina to the sensory epithelium. Although type I hair cells were absent from the epithelium, 55% of the normal number of type II hair cells were present. At this time, more regenerated hair cells were located in the center of the cristae ampullaris as compared to the periphery. At the transmission electron microscopic level, type II hair cells at different stages of maturation were observed. Some exhibited mature stereocilia, a cuticular plate, and terminal endings with synaptic specialization opposing these hair cells. In conclusion, type I hair cells were more sensitive than type II hair cells to gentamicin intoxication (as they disappeared as early as 4days PT). After 56 days PT, the number of type II hair cells reached 55% of normal. No type I hair cells had regenerated at this time. These results demonstrate quantitatively the differential ototoxic sensitivity and regenerative capacity of hair cells.

The vestibular system in the elderly: Clinical implications

Cell loss, changes in synapse morphology, electrophysiologic alterations, and changes in the supporting microenvironment have all been noted in portions of the vestibular systems of aged animals and humans. Increased variability with age is also a prominent finding in many studies. Quantitative vestibular testing in humans has shown alterations with age as well, but decline with aging is not a prominent feature of all measures, and many reported studies are methodogically flawed. The connection between these observed aging changes and the increased incidence of dizziness and falls in the elderly is unproven, however, and clinicians should search carefully for specific disease processes in their elderly patients who present with vestibular symptoms.