Recovery Nystagmus in Vestibular Neuritis with Minimal Canal Paresis. Clinical Observation and Interpretation

Recovery nystagmus in vestibular neuritis patients is a reversal of spontaneous nystagmus direction, beating towards the affected ear, observed along the time course of central compensation. It is rarely registered due either to its rarity as a phenomenon per se, or to the fact that it is missed between follow-up appointments. The aim of the manuscript is to describe in detail a case of recovery nystagmus found in an atypical case of vestibular neuritis and discuss pathophysiology and clinical considerations regarding this rare finding. A 26-year-old man was referred to our Otorhinolaryngology practice reporting “dizziness” sensation and nausea in the last 48 h. Clinical examination revealed left beating spontaneous nystagmus (average slow phase velocity aSPV 8.1°/s) with absence of fixation. The head impulse test (H.I.T.) was negative. Cervical vestibular evoked myogenic potentials (cVEMP) and Playtone audiometry (PTA) were normal. Romberg and Unterberger tests were not severely affected. A strong directional preponderance to the left was found in caloric vestibular test with minimal canal paresis (CP 13%) on the right. The first follow-up consultation took place on the 9th day after the onset of symptoms. Right beating weak (aSPV 2.4°/s) spontaneous nystagmus was observed with absence of fixation, whereas a strong right directional preponderance (DP) was found in caloric vestibular test. A brain MRI scan was ordered to exclude central causes of vertigo, which was normal. The patient was seen again completely free of symptoms 45 days later. He reported feeling dizzy during dynamic movements of the head and trunk for another 15 days after his second consultation. The unexpected observation of nystagmus direction reversal seven days after the first consultation is a typical sign of recovery nystagmus. Recovery nystagmus (RN) is centrally mediated and when found, it should always be carefully assessed in combination with the particularities of vestibular neuritis.

Nystagmus: Diagnosis, Topographic Anatomical Localization and Therapy

Nystagmus is defined as rhythmic, most often involuntary eye movements. It normally consists of a slow (pathological) drift of the eyes, followed by a fast central compensatory movement back to the primary position (refixation saccade). The direction, however, is reported according to the fast phase. The cardinal symptoms are, on the one hand, blurred vision, jumping images (oscillopsia), reduced visual acuity and, sometimes, double vision; many of these symptoms depend on the eye position. On the other hand, depending on the etiology, patients may suffer from the following symptoms: 1. permanent dizziness, postural imbalance, and gait disorder (typical of downbeat and upbeat nystagmus); 2. if the onset of symptoms is acute, the patient may experience spinning vertigo with a tendency to fall to one side (due to ischemia in the area of the brainstem or cerebellum with central fixation nystagmus or as acute unilateral vestibulopathy with spontaneous peripheral vestibular nystagmus); or 3. positional vertigo. There are two major categories: the first is spontaneous nystagmus, i.e., nystagmus which occurs in the primary position as upbeat or downbeat nystagmus; and the second includes various types of nystagmus which are induced or modified by certain factors. Examples are gaze-evoked nystagmus, head-shaking nystagmus, positional nystagmus, and hyperventilation-induced nystagmus. In addition, there are disorders similar to nystagmus, such as ocular flutter or opsoclonus. The most common central types of spontaneous nystagmus are downbeat and upbeat, infantile, pure torsional, pendular fixation, periodic alternating, and seesaw nystagmus. Many types of nystagmus allow a precise neuroanatomical localization: for instance, downbeat nystagmus, which is most often caused by a bilateral floccular lesion or dysfunction, or upbeat nystagmus, which is caused by a lesion in the midbrain or medulla. Examples of drug treatment are the use of 4-aminopyridine for downbeat and upbeat nystagmus, memantine or gabapentin for pendular fixation nystagmus, or baclofen for periodic alternating nystagmus. In this article we are focusing on nystagmus. In a second article we will focus on central ocular motor disorders, such as saccade or gaze palsy, internuclear ophthalmoplegia, and gaze-holding deficits. Therefore, these types of eye movements will not be described here in detail.

Central Ocular Motor Disorders: Clinical and Topographic Anatomical Diagnosis, Syndromes and Underlying Diseases

The key to the diagnosis of ocular motor disorders is a systematic clinical examination of the different types of eye movements, including eye position, spontaneous nystagmus, range of eye movements, smooth pursuit, saccades, gaze-holding function, vergence, optokinetic nystagmus, as well as testing of the function of the vestibulo-ocular reflex (VOR) and visual fixation suppression of the VOR. This is like a window which allows you to look into the brain stem and cerebellum even if imaging is normal. Relevant anatomical structures are the midbrain, pons, medulla, cerebellum and rarely the cortex. There is a simple clinical rule: vertical and torsional eye movements are generated in the midbrain, horizontal eye movements in the pons. For example, isolated dysfunction of vertical eye movements is due to a midbrain lesion affecting the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), with impaired vertical saccades only or vertical gaze-evoked nystagmus due to dysfunction of the Interstitial nucleus of Cajal (INC). Lesions of the lateral medulla oblongata (Wallenberg syndrome) lead to typical findings: ocular tilt reaction, central fixation nystagmus and dysmetric saccades. The cerebellum is relevant for almost all types of eye movements; typical pathological findings are saccadic smooth pursuit, gaze-evoked nystagmus or dysmetric saccades. The time course of the development of symptoms and signs is important for the diagnosis of underlying diseases: acute: most likely stroke; subacute: inflammatory diseases, metabolic diseases like thiamine deficiencies; chronic progressive: inherited diseases like Niemann-Pick type C with typically initially vertical and then horizontal saccade palsy or degenerative diseases like progressive supranuclear palsy. Treatment depends on the underlying disease. In this article, we deal with central ocular motor disorders. In a second article, we focus on clinically relevant types of nystagmus such as downbeat, upbeat, fixation pendular, gaze-evoked, infantile or periodic alternating nystagmus. Therefore, these types of nystagmus will not be described here in detail.

Implication of Vestibular Hair Cell Loss of Planar Polarity for the Canal and Otolith-Dependent Vestibulo-Ocular Reflexes in Celsr1–/– Mice

Introduction: Vestibular sensory hair cells are precisely orientated according to planar cell polarity (PCP) and are key to enable mechanic-electrical transduction and normal vestibular function. PCP is found on different scales in the vestibular organs, ranging from correct hair bundle orientation, coordination of hair cell orientation with neighboring hair cells, and orientation around the striola in otolithic organs. Celsr1 is a PCP protein and a Celsr1 KO mouse model showed hair cell disorganization in all vestibular organs, especially in the canalar ampullae. The objective of this work was to assess to what extent the different vestibulo-ocular reflexes were impaired in Celsr1 KO mice.Methods: Vestibular function was analyzed using non-invasive video-oculography. Semicircular canal function was assessed during sinusoidal rotation and during angular velocity steps. Otolithic function (mainly utricular) was assessed during off-vertical axis rotation (OVAR) and during static and dynamic head tilts.Results: The vestibulo-ocular reflex of 10 Celsr1 KO and 10 control littermates was analyzed. All KO mice presented with spontaneous nystagmus or gaze instability in dark. Canalar function was reduced almost by half in KO mice. Compared to control mice, KO mice had reduced angular VOR gain in all tested frequencies (0.2–1.5 Hz), and abnormal phase at 0.2 and 0.5 Hz. Concerning horizontal steps, KO mice had reduced responses. Otolithic function was reduced by about a third in KO mice. Static ocular-counter roll gain and OVAR bias were both significantly reduced. These results demonstrate that canal- and otolith-dependent vestibulo-ocular reflexes are impaired in KO mice.Conclusion: The major ampullar disorganization led to an important reduction but not to a complete loss of angular coding capacities. Mildly disorganized otolithic hair cells were associated with a significant loss of otolith-dependent function. These results suggest that the highly organized polarization of otolithic hair cells is a critical factor for the accurate encoding of the head movement and that the loss of a small fraction of the otolithic hair cells in pathological conditions is likely to have major functional consequences. Altogether, these results shed light on how partial loss of vestibular information encoding, as often encountered in pathological situations, translates into functional deficits.

Vibration-induced nystagmus in patients with chronic unilateral Meniere's disease

Background and Aim: Meniere's disease (MD) is one of the inner ear disorders associated with fluctuating hearing loss, vertigo, ear fullness, and tinnitus. Vestibular stimulation delas with the int­egrity of the peripheral vestibular system and may cause nystagmus due to the functional asy­mmetry between right and lef peripheral vesti­bular system. This study aimed to assess the vibration-induced nystagmus (VIN) in patients with chronic unilateral MD and investigate the effectiveness of this test in detecting the affected ear in these patients. Methods: This study was conducted on 29 pati­ents with chronic unilateral MD. For this pur­pose, spontaneous nystagmus (SN) and VIN at frequencies of 30 Hz and 100 Hz were recorded by videonystagmography test under five recor­ding conditions. The vibratory stimulation was presented to both healthy and affected ears. Coll­ected were analyzed in SPSS v.22 software. Results: Vibratory stimulation compared to the unstimulated condition, revealed a significant difference in eye movements for both healthy and affected ears. Moreover, the difference between VIN and SN in the affected ear was much greater than in the healthy ear. Conclusion: In patients with chronic unilateral MD, 100 Hz vibratory stimulation of the affected ear induces more reliable nystagmus than 30 Hz stimulation and unstimulated condition. The VIN test can be used for the evaluation of the vesti­bular system function and is a promising techni­que to detect the MD ear. Keywords: Meniere's disease; spontaneous nystagmus; vibration-induced nystagmus; chronic; definite; vestibular vibrator

VESTIBULAR NEURONITIS

Vestibular neuronitis is an acute vestibular syndrome due to inflammation of the vestibular nerve characterized by the typical symptoms of acute rotatory vertigo accompanied by nausea, vomiting, and symptoms of balance disorders. The incidence of vestibular neuronitis is about 3.5 per 100,000 people. The exact etiology of this vestibular neuronitis is unknown. However, based on existing evidence, vestibular neuronitis is associated with viral infections of the upper respiratory tract and herpes zoster infection. The clinical manifestations of vestibular neuronitis are persistent rotatory vertigo accompanied by oscillopsia, horizontal-rotatory peripheral vestibular spontaneous nystagmus on the healthy side, and a tendency to fall on the affected side. Diagnosis of vestibular neuronitis can be made by clinical diagnosis, through history, physical examination, and special examinations. Through these examinations, the differential diagnosis of vestibular neuronitis should be excluded, such as Meniere's disease, labyrinthitis, benign paroxysmal positional vertigo, and vertigo due to central lesions such as cerebellar infarction. Management of vestibular neuronitis is in the form of symptomatic therapy with vestibular suppressants, antivertigo, and redirect to relieve the symptoms that arise, then causative therapy can be done by administering corticosteroids, and in patients, physiotherapy can be done to improve vestibular function.

Pulse-synchronous torsional nystagmus

Purely torsional spontaneous nystagmus almost always has a central vestibular cause. We describe a man with spontaneous pulse-synchronous torsional nystagmus in which the clockwise component corresponded to his pulse upswing, in keeping with a peripheral vestibular cause; following imaging we diagnosed left-sided superior canal dehiscence syndrome. Identifying pulse synchronicity of spontaneous nystagmus may help to distinguish central from peripheral vestibular torsional nystagmus, and is readily confirmed at the bedside using Frenzel’s glasses and a pulse oximeter.