Complete Ocular Tilt Reaction Research Articles

Complete ocular tilt reaction with subjective visual vertical tilt in a patient with a medial prefrontal cortex lesion: a case report

A 61-year-old male patient undergoing chemotherapy for esophageal cancer presented with symptoms of rightward tilting while both sitting and walking. A neurological examination revealed a head tilt, skew deviation with hypertrophy of the left eye, and a rightward shift in his gait. No spontaneous nystagmus was observed, and the vestibulo-ocular reflex was normal. Magnetic resonance imaging of the head revealed a suspected metastatic lesion in the right medial prefrontal cortex. Following the initiation of levetiracetam treatment, the patient demonstrated marked improvement, with the resolution of both head tilt and skew deviation within 1 month. Traditionally, the ocular tilt reaction has been attributed to unilateral or asymmetric dysfunction of the graviceptive pathways extending from the utricle to the upper midbrain lesions. However, this case highlights the potential involvement of the prefrontal cortex in the ocular tilt reaction. Further research is warranted on the role of the prefrontal cortex within the vestibular system.

HINTS Examination in Acute Vestibular Neuritis: Do Not Look Too Hard for the Skew.

An ocular tilt reaction (OTR) is a triad of a skew deviation, head tilt, and ocular counter-roll that can be partial or complete. An OTR can occur anywhere along the utriculo-ocular motor pathways from the labyrinth to the interstitial nucleus of Cajal but is almost always central in origin. In acute vestibular neuritis (AVN), case reports have described patients with an OTR due to AVN, although it is unclear whether this examination finding is common or rare. The vestibular and ocular motor features of 7 patients presenting with AVN are described. Each of the 7 patients presented with typical features of AVN, including contralesional unidirectional spontaneous nystagmus and an ipsilesional abnormal head impulse test, although each patient also had a complete OTR. None of the patients had vertical diplopia or a skew deviation that was measurable with alternate cover testing (i.e., abnormal "test of skew" according to the Head Impulse, Nystagmus, Test of Skew examination); however, all had a subtle 1 prism diopter hyperphoria that was only measurable with a Maddox rod test. Seven cases of typical AVN with an OTR are presented, and in the authors' experience, the presence of a subtle OTR is a common feature of AVN in these patients.

Acute audiovestibular deficit with complete ocular tilt reaction and absent VEMPs

A patient presenting with vertical diplopia along with ocular tilt reaction (OTR) due to peripheral vestibular dysfunction is a rare occurrence. OTR is an eye-head postural reaction consisting of head tilt, skew deviation, conjugated eye cyclotorsion, and alteration of vertical perception, and is thought to occur with central lesions, mainly brainstem lesions. Here, we report a case of a patient who was suffering from left acute peripheral cochleovestibular loss. He had profound deafness and absence of caloric response on the left side. No central lesion was observed on magnetic resonance images. Neuro-ophthalmological examination showed OTR consisting of head tilt, skew deviation with left hypotropia, excyclotorsion, and tilt of the static visual vertical directed to the left side. Both utricular and saccular dysfunctions were identified by the absence of cervical vestibular evoked myogenic potential (cVEMP) and ocular VEMP (oVEMP) on the left side. Within a month, the OTR disappeared completely probably because of vestibular compensation. This is the first report to state that the peripheral otolith dysfunction causing reversible vertical diplopia was identified by objective examinations (VEMP).

Pearls & Oy-sters: Vestibular neuritis or not?

The ocular tilt reaction (OTR) is a central vestibular disorder that is characterized by the triad of head tilt, skew deviation, and ocular torsion. As the OTR frequently shows an incomplete manifestation, a head tilt can be the only apparent clinical sign of significant brainstem disorders. Hence, even a slight head tilt should be thoroughly investigated. A 48-year-old man presented after sudden onset of a severe, persistent rotatory vertigo associated with nausea, vomiting, and postural imbalance. Examination revealed a horizontal-torsional spontaneous nystagmus with the quick phases beating to the right ear. The nystagmus was attenuated by fixation as examined by Frenzel goggles. Head impulse test was positive to the left. When attempting to walk and during Romberg testing he fell to his left side. Except for a head tilt to the right (see video on the Neurology ® Web site at www.neurology.org), no other neurologic deficit was observed. The patient's medical history revealed no neurologic disorders. Quantitative caloric testing showed a canal paresis of the left vestibular organ. Taken together, the initial clinical presentation was highly suggestive of a left vestibular neuritis. However, due to the head tilt, an extended neuroophthalmologic examination was performed that revealed a discrete but complete OTR to the right with head tilt, mild skew deviation …

Otolith dysfunction in vestibular neuritis

To prospectively follow patients with vestibular neuritis (VN), to compare the recovery pattern of canal and otolith dysfunction, and to determine which tests best predict symptom recovery. Between March 2006 and December 2006, 51 consecutive patients with unilateral VN were enrolled within 7 days of onset (average 3 days). Otolith function tests included ocular torsion (OT), subjective visual vertical (SVV), and vestibular evoked myogenic potential (VEMP), and canal function tests included head-shaking nystagmus (HSN), caloric stimulation, and head-thrust testing. Patients returned for two follow-up evaluations at approximately 1 week and 6 weeks after the initial evaluation. On the first examination, all patients had abnormal HSN, caloric, and head-thrust test results, and at least one otolith-related test abnormality: abnormal tilt of SVV (48/51, 94%), abnormal OT (42/51, 82%), or abnormal VEMPs (25/51, 49%). The degree of SVV tilts correlated with the degree of OT for one or both eyes (p < 0.05). Skew deviation was observed in 7 patients (14%), and a complete ocular tilt reaction was detected in only 2 patients. On follow-up, otolith test results returned to normal more rapidly than canal test results. The head-thrust test was the best predictor of symptom recovery. Eighty percent of patients who continued to report dizziness at the last follow-up visit had a positive head-thrust test result, whereas only 10% of patients who were not dizzy had a positive head-thrust test result. Otolith-related test abnormalities improve more rapidly than canal-related test abnormalities after vestibular neuritis. If patients have a positive head-thrust test result on follow-up, they are more likely to be dizzy.

Medial Vestibular Nucleus Lesions in Wallenberg's Syndrome Cause Decreased Activity of the Contralateral Vestibular Cortex

Three patients with the clinical diagnosis of Wallenberg's syndrome caused by acute unilateral ischemic infarctions, which included the vestibular nucleus in the medullary brain stem and afferent vestibular pathways, were examined by positron emission tomography (PET) during caloric vestibular stimulation. They all had typical signs of vestibular dysfunction such as transient rotatory vertigo with vomiting at the onset, ipsiversive body and ocular lateropulsion, and a complete ocular tilt reaction with tilts of the subjective visual vertical. Compared with healthy volunteers, who show activation in a network of temporoparietal vestibular areas within both hemispheres, especially in the posterior insula and retroinsular region that contains the human homologue of the parietoinsular vestibular cortex (PIVC) in monkeys, the activation pattern of the patients with Wallenberg's syndrome was typically changed. During caloric irrigation of the ear ipsilateral to the side of the lesion, they showed no or significantly reduced activation in the contralateral hemisphere, whereas the activation pattern in the ipsilateral hemisphere appeared "normal." These results are compatible with bilateral ascending vestibular pathways from the vestibular nuclei to the vestibular cortex. The novel finding in all three patients was that the activation patterns were compatible with the assumption that only the crossing fibers from the medial vestibular subnucleus to the contralateral medial longitudinal fascicle were affected, but the ipsilateral vestibular thalamocortical projections via the superior vestibular subnucleus were spared. Thus, the activation pattern in the PET study may reflect the vestibular tonic imbalance within the vestibular nuclei circuitry at the cortical level.

Herpes zoster neuritis involving superior and inferior parts of the vestibular nerve causes ocular tilt reaction

A 43-year-old woman presented with peripheral facial nerve palsy, rotational vertigo, and hearing loss due to herpes zoster neuritis of the right seventh and eighth cranial nerves. Neuro-ophthalmologic examination showed a horizontal rotatory spontaneous nystagmus (fast phase) to the left and an ocular tilt reaction (OTR) to the right, a pattern of head tilt, skew deviation, ocular torsion, and tilt of perceived vertical. Ocular torsion was binocular but asymmetric with a predominant intorsion of the contralateral hypertropic eye (17° vs 7°). Since a complete OTR is rare in peripheral, but common in central vestibular disorders, involvement of the vestibular nucleus (due to a zoster meningoencephalitis) was first assumed. However, MRI scan (T1-weighted 2D-FLASH sequence) revealed contrast enhancement in both parts (pars superior and inferior) of the right vestibular nerve due to viral inflammation. The following conclusions can be drawn from the correlation of the neuro-ophthalmologic syndrome and MRI findings: (1) the horizontal component of spontaneous nystagmus is secondary to a horizontal semicircular canal paresis; (2) the rotatory component of the spontaneous nystagmus and the tonic OTR are secondary to a combined paresis of the anterior canal, the posterior canal, and the otoliths; and (3) the asymmetry of ocular torsion indicates incomplete loss of vestibular function with predominant involvement of the anterior semicircular canal.

Vestibular syndromes in the roll plane: Topographic diagnosis from brainstem to cortex

Central vestibular syndromes may be classified according to the three major planes of action of the vestibuloocular reflex, secondary to a lesional tone imbalance in either the horizontal yaw plane or the vertical pitch or roll plane. The clinical signs, both perceptual and motor, of a vestibular tone imbalance in the roll plane are ocular tilt reaction (OTR), ocular torsion, skew deviation and tilts of the perceived visual vertical (SVV). Either complete OTR or skew torsion without head tilt indicates a unilateral peripheral deficit of otolith input or a unilateral lesion of graviceptive brainstem pathways from the vestibular nuclei (crossing midline at the pontine level) to the interstitial nucleus of Cajal (INC) in the rostral midbrain. SVV tilts are the most sensitive sign of a vestibular tone imbalance in roll and occur with peripheral or central vestibular lesions from the labyrinth to the vestibular cortex. All tilt effects, perceptual, ocular motor and postural, are ipsiversive (ipsilateral eye undermost) with unilateral peripheral or pontomedullary lesions below the crossing of the graviceptive pathways. All tilt effects are contraversive (contralateral eye undermost) with unilateral pontomesencephalic brainstem lesions and indicate involvement of the medial longitudinal fasciculus or the rostral midbrain (INC). Unilateral lesions of vestibular structures rostral to the INC typically manifest with deviations of perceived vertical without concurrent eye-head tilt. OTR in unilateral paramedian thalamic infarctions indicates simultaneous ischemia of the paramedian rostral midbrain including the INC. Unilateral lesions of the posterolateral thalamus can cause thalamic astasia and moderate ipsiversive or contraversive SVV tilts, thereby indicating involvement of the vestibular thalamic subnuclei. Unilateral lesions of the parietoinsular vestibular cortex cause moderate, mostly contraversive SVV tilts. An SVV tilt found with monocular but not with binocular viewing is typical for a trochlear or oculomotor palsy rather than a supranuclear graviceptive brainstem lesion.

Thalamic infarctions: differential effects on vestibular function in the roll plane (35 patients).

We determined the subjective visual vertical (SVV), ocular torsion (OT), skew deviation, and lateral head tilt in 35 patients with acute thalamic infarctions (14 paramedian, 17 posterolateral, and four anterior polar) and in five patients with mesodiencephalic hemorrhages to obtain the tonic effects on vestibular function in the roll plane. Eight of 14 paramedian infarctions had complete ocular tilt reaction (OTR) with contraversive head tilt, skew deviation, OT, and SVV tilt. The OTR was due to ischemia of the rostral midbrain tegmentum, including the interstitial nucleus of Cajal (INC), and not to thalamic ischemia. Thus, the INC (and the rostral interstitial nucleus of the medial longitudinal fascicle) is the most rostral brainstem structure mediating eye-head coordination in roll. Eleven of 17 posterolateral infarctions exhibited moderate SVV tilts that were either ipsiversive or contraversive. In these 11 cases, vestibular thalamic nuclei (nucleus ventro-oralis intermedius, nucleus ventrocaudalis externus, and nucleus dorsocaudalis) were involved; infarctions in the remaining six were more ventromedial. Anterior polar infarctions did not affect vestibular function in roll.

Wallenberg's syndrome: lateropulsion, cyclorotation, and subjective visual vertical in thirty-six patients.

We measured the subjective visual vertical, cyclorotation of the eyes, and head and body lateropulsion in 36 patients with Wallenberg's syndrome. All patients exhibited significant tilts of the internal representation of the gravity vector, as indicated by deviation of subjective visual vertical ipsiversive to the lesion. Most patients (82%) had ipsiversive cyclorotation of one or both eyes, especially excyclotropia of the eye ipsilateral to the brainstem lesion. Twelve of 36 patients (33%), those with the most severe body lateropulsion, had a complete ocular tilt reaction consisting of head tilt, skew deviation, and cyclorotation in the roll plane. We hypothesize that deviation of subjective visual vertical, lateropulsion of the body, and cyclorotation of the eyes are the perceptual, the ocular motor, and the postural consequences of a common lesion of central vestibular pathways that subserve the vestibuloocular reflex in the roll plane. Lateropulsion in patients with Wallenberg's syndrome is interpreted as a postural consequence of an abnormal tilt of the internal representation of orientation in space.