Is it possible to diagnose Posterior Semicircular Canal BPPV from the sitting position? The role of the Head Pitch Test and the upright tests along the RALP and LARP planes.

PURPOSE: The diagnosis of benign paroxysmal positional vertigo (BPPV) involving the posterior semicircular canal (PSC) is traditionally entrusted to positioning tests where patients are rapidly brought in the supine position. This prospective study aims to define the role of a diagnostic protocol for PSC-BPPV including only upright tests. MATERIALS AND METHODS: 109 patients with PSC-BPPV were enrolled. The Head Pitch Test (HPT) was carried out first. If uneventful, the patient's head was turned 45° to each side and bent back-and-forth along the plane aligning either with the right anterior-left posterior (RALP) or left anterior-right posterior (LARP) canals, thus performing the upright RALP / upright LARP (uRALP/uLARP) test. Nystagmus observed was used to predict the diagnosis, which was therefore confirmed by Dix-Hallpike tests. RESULTS: PSC-BPPV could be correctly diagnosed in 75.2% of cases with the sole HPT and in 87.2% of cases by adding the uRALP/uLARP test (Upright Protocol). The time elapsed from symptoms onset was closely related to the protocol sensitivity, as it reached 100% (64/64) in acute patients while decreased to 68.9% (31/45) in cases evaluated after 7 days (p < 0.001). CONCLUSIONS: Upright maneuvers could correctly diagnose PSC-BPPV in most cases. uRALP/uLARP test demonstrated to improve the sensitivity of the HPT, mainly in recent-onset BPPV.

Isolated horizontal canal hypofunction differentiating a canalith jam from an acute peripheral vestibular loss.

OBJECTIVES: To describe a unique case of acute vertigo presenting with spontaneous horizontal nystagmus (SHN) and a clinical picture consistent with right acute peripheral vestibular loss (APVL) in which an isolated hypofunction of a horizontal semicircular canal (HSC) permitted to detect a spontaneous canalith jam and treat the patient accordingly. METHODS: Case report and literature review. RESULTS: A 74-year old woman presented with acute vertigo, left-beating SHN and a clinical picture consistent with right APVL. Nevertheless, vestibular evoked myogenic potentials were normal with symmetrical amplitudes and the video head impulse test (vHIT) revealed an isolated hypofunction of the right HSC. After repeated head shakings, the supine roll test evoked bilaterally a positioning paroxysmal geotropic horizontal nystagmus suggesting benign paroxysmal positional vertigo involving the non-ampullated arm of the right HSC. vHIT and caloric testing confirmed restitution of HSC function after repositioning maneuvers. CONCLUSIONS: In case of acute vertigo with SHN, a complete functional assessment of vestibular receptors and afferents should always be given in order to avoid misdiagnosis. Canalith jam should be considered in case of spontaneous nystagmus and isolated canal hypofunction.

Skull Vibration-Induced Nystagmus in Superior Semicircular Canal Dehiscence: A New Insight into Vestibular Exploration-A Review.

The third window syndrome, often associated with the Tullio phenomenon, is currently most often observed in patients with a superior semicircular-canal dehiscence (SCD) but is not specific to this pathology. Clinical and vestibular tests suggestive of this pathology are not always concomitantly observed and have been recently complemented by the skull-vibration-induced nystagmus test, which constitutes a bone-conducted Tullio phenomenon (BCTP). The aim of this work was to collect from the literature the insights given by this bedside test performed with bone-conducted stimulations in SCD. The PRISMA guidelines were used, and 10 publications were included and analyzed. Skull vibration-induced nystagmus (SVIN), as observed in 55 to 100% of SCD patients, usually signals SCD with greater sensitivity than the air-conducted Tullio phenomenon (ACTP) or the Hennebert sign. The SVIN direction when the test is performed on the vertex location at 100 Hz is most often ipsilaterally beating in 82% of cases for the horizontal and torsional components and down-beating for the vertical component. Vertex stimulations are more efficient than mastoid stimulations at 100 Hz but are equivalent at higher frequencies. SVIN efficiency may depend on stimulus location, order, and duration. In SCD, SVIN frequency sensitivity is extended toward high frequencies, with around 400 Hz being optimal. SVIN direction may depend in 25% on stimulus frequency and in 50% on stimulus location. Mastoid stimulations show frequently diverging results following the side of stimulation. An after-nystagmus observed in 25% of cases can be interpreted in light of recent physiological data showing two modes of activation: (1) cycle-by-cycle phase-locked activation of action potentials in SCC afferents with irregular resting discharge; (2) cupula deflection by fluid streaming caused by the travelling waves of fluid displacement initiated by sound or vibration at the point of the dehiscence. The SVIN direction and intensity may result from these two mechanisms' competition. This instability explains the SVIN variability following stimulus location and frequency observed in some patients but also discrepancies between investigators. SVIN is a recent useful insight among other bedside examination tests for the diagnosis of SCD in clinical practice.

Audiovestibular Findings in Patients with Concurrent Superior Canal Dehiscence and Vestibular Schwannoma.

OBJECTIVE: To describe the clinical-instrumental findings in case of concurrent superior canal dehiscence (SCD) and ipsilateral vestibular schwannoma (VS), aiming to highlight the importance of an extensive instrumental assessment to achieve a correct diagnosis. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Five patients with concurrent SCD and VS. INTERVENTION: Clinical-instrumental assessment and imaging. MAIN OUTCOME MEASURE: Clinical presentation, audiovestibular findings, and imaging. RESULTS: The chief complaints were hearing loss (HL) and unsteadiness (80%). Other main symptoms included tinnitus (60%) and pressure-induced vertigo (40%). Mixed-HL was identified in three patients and pure sensorineural-HL in 1, including a roll-over curve in speech-audiometry in two cases. Vibration-induced nystagmus was elicited in all cases, whereas vestibular-evoked myogenic potentials showed reduced thresholds and enhanced amplitudes on the affected side in three patients. Ipsilesional weakness on caloric testing was detected in three patients and a bilateral hyporeflexia in one. A global canal impairment was detected by the video-head impulse test in one case, whereas the rest of the cohort exhibited a reduced function for the affected superior canal, together with ipsilateral posterior canal impairment in two cases. All patients performed both temporal bones HRCT scan and brain-MRI showing unilateral SCD and ipsilateral VS, respectively. All patients were submitted to a wait-and-scan approach, requiring VS removal only in one case. CONCLUSION: Simultaneous SCD and VS might result in subtle clinical presentation with puzzling lesion patterns. When unclear symptoms and signs occur, a complete audiovestibular assessment plays a key role to address imaging and diagnosis.

Posterior Semicircular Canal Dehiscence with Vestibulo-Ocular Reflex Reduction for the Affected Canal at the Video-Head Impulse Test: Considerations to Pathomechanisms.

Posterior semicircular canal dehiscence (PSCD) has been demonstrated to result in a third mobile window mechanism (TMWM) in the inner ear similar to superior semicircular canal dehiscence (SSCD). Typical clinical and instrumental features of TMWM, including low-frequency conductive hearing loss (CHL), autophony, pulsatile tinnitus, sound/pressure-induced vertigo and enhanced vestibular-evoked myogenic potentials, have been widely described in cases with PSCD. Nevertheless, video-head impulse test (vHIT) results have been poorly investigated. Here, we present six patients with PSCD presenting with a clinical scenario consistent with a TMWM and an impaired vestibulo-ocular reflex (VOR) for the affected canal on vHIT. In two cases, an additional dehiscence between the facial nerve and the horizontal semicircular canal (HSC) was detected, leading to a concurrent VOR impairment for the HSC. While in SSCD, a VOR gain reduction could be ascribed to a spontaneous "auto-plugging" process due to a dural prolapse into the canal, the same pathomechanism is difficult to conceive in PSCD due to a different anatomical position, making a dural herniation less likely. Alternative putative pathomechanisms are discussed, including an endolymphatic flow dissipation during head impulses as already hypothesized in SSCD. The association of symptoms/signs consistent with TMWM and a reduced VOR gain for the posterior canal might address the diagnosis toward PSCD.

Low-Frequency Air-Bone Gap and Pulsatile Tinnitus Due to a Dural Arteriovenous Fistula: Considerations upon Possible Pathomechanisms and Literature Review.

Low-frequency air-bone gap (ABG) associated with pulsatile tinnitus (PT) and normal impedance audiometry represents a common finding in patients with third window syndromes. Other inner disorders, including Meniere's disease (MD), perilymphatic fistula and intralabyrinthine schwannoma, might sometimes result in a similar scenario. On the other hand, PT is frequently associated with dural arteriovenous fistula (DAVF), while conductive hearing loss (CHL) is extremely rare in this clinical setting. A 47-year-old patient was referred to our center with progressive left-sided PT alongside ipsilateral fullness and hearing loss. She also experienced headache and dizziness. Otoscopy and video-oculographic examination were unremarkable. Conversely, a detailed instrumental audio-vestibular assessment revealed low-frequency CHL with normal impedance audiometry, slight left-sided caloric weakness, slightly impaired vestibular-evoked myogenic potentials on the left and normal results on the video-head impulse test, consistent with an MD-like instrumental profile. Gadolinium-enhanced brain MRI revealed an early enhancement of the left transverse sinus, consistent with a left DAVF between the left occipital artery and the transverse sinus, which was then confirmed by angiography. A trans-arterial embolization with Onyx glue was performed, resulting in a complete recession of the symptoms. Post-operatively, the low-frequency ABG disappeared, supporting the possible role of venous intracranial hypertension and abnormal pressure of inner ear fluids in the onset of symptoms and offering new insights into the pathomechanism of inner ear CHL.

Impaired Vestibulo-Ocular Reflex on Video Head Impulse Test in Superior Canal Dehiscence: "Spontaneous Plugging" or Endolymphatic Flow Dissipation?

Surgical plugging of the superior semicircular canal (SSC) represents an effective procedure to treat disabling symptoms in superior canal dehiscence (SCD), despite resulting in an impaired vestibulo-ocular reflex (VOR) gain for the SSC. On the other hand, SSC hypofunction on video head impulse test (vHIT) represents a common finding in patients with SCD exhibiting sound/pressure-induced vertigo, a low-frequency air-bone gap (ABG), and enhanced vestibular-evoked myogenic potentials (VEMPs). "Spontaneous canal plugging" has been assumed as the underlying process. Nevertheless, missing/mitigated symptoms and/or near-normal instrumental findings would be expected. An endolymphatic flow dissipation has been recently proposed as an alternative pathomechanism for SSC VOR gain reduction in SCD. We aimed to shed light on this debate by comparing instrumental findings from 46 ears of 44 patients with SCD exhibiting SSC hypofunction with post-operative data from 10 ears of 10 patients with SCD who underwent surgical plugging. While no difference in SSC VOR gain values was found between the two groups (p = 0.199), operated ears developed a posterior canal hypofunction (p = 0.002). Moreover, both ABG values (p = 0.012) and cervical/ocular VEMP amplitudes (p < 0.001) were significantly higher and VEMP thresholds were significantly lower (p < 0.001) in ears with SCD compared to operated ears. According to our data, canal VOR gain reduction in SCD should be considered as an additional sign of a third window mechanism, likely due to an endolymphatic flow dissipation.

Vestibular assessment in sudden sensorineural hearing loss: Role in the prediction of hearing outcome and in the early detection of vascular and hydropic pathomechanisms.

Introduction: Predicting hearing outcome in sudden sensorineural hearing loss (SSNHL) is challenging, as well as detecting the underlying pathomechanisms. SSNHL could be associated with vestibular damage since cochleo-vestibular structures share the same vascularization, along with being in close anatomical proximity. Whereas viral inflammations and autoimmune/vascular disorders most likely represent the involved aetiologies, early-stage Menière's disease (MD) can also present with SSNHL. Since an early treatment could beneficially influence hearing outcome, understanding the possible etiology plays a pivotal role in orienting the most appropriate treatment. We aimed to evaluate the extent of vestibular damage in patients presenting with SSNHL with or without vertigo, investigate the prognostic role of vestibular dysfunctions on hearing recovery and detect specific lesion patterns related to the underlying pathomechanisms. Methods: We prospectively evaluated 86 patients with SSNHL. Audio-vestibular investigation included pure-tone/speech/impedance audiometry, cervical/ocular-VEMPs, vHIT and video-Frenzel examination. White matter lesions (WML) were evaluated on brain-MRI. Patients were followed-up and divided into "SSNHL-no-vertigo," "SSNHL+vertigo" and "MD" subgroups. Results: Hearing was more impaired in "SSNHL+vertigo" patients who exhibited either down-sloping or flat-type audiograms, and was less impaired in "MD" where low frequencies were mostly impaired (p < 0.001). Otolith receptors were more frequently involved than semicircular canals (SCs). Although the "SSNHL-no-vertigo" subgroup exhibited the lowest vestibular impairment (p < 0.001), 52% of patients developed otolith dysfunctions and 72% developed nystagmus. Only "MD" subjects showed anterior SC impairment and upbeating spontaneous/positional nystagmus. They more frequently exhibited cervical-VEMPs frequency tuning (p = 0.036) and ipsilesional spontaneous nystagmus (p < 0.001). "SSNHL+vertigo" subjects presented with more frequently impaired cervical-VEMPs and posterior SC and with higher number of impaired receptors (p < 0.001). They mainly exhibited contralesional spontaneous and vibration-induced nystagmus (p < 0.05) and only they showed the highest WML score and "vascular" lesion patterns (p < 0.001). Concerning the outcomes, hearing was better in "MD" and worse in "SSNHL+vertigo" (p < 0.001). Hearing recovery was mostly affected by cervical-VEMPs impairment and the number of involved receptors (p < 0.05). Patients with "vascular" lesion patterns presented with the highest HL degree and WML score (p ≤ 0.001), while none of them exhibited a complete hearing recovery (p = 0.026). Conclusions: Our data suggest that vestibular evaluation in SSNHL can provide useful information on hearing recovery and underlying aetiologies.

Minimum Stimulus Strategy: A step-by-step diagnostic approach to BPPV.

Benign Paroxysmal Positional Vertigo (BPPV) is among the most common vestibular disorders, characterized by brief vertigo spells triggered by head position changes with abrupt onset and rapid decrease. BPPV is ascribed to otoconial matter dislodged from utricular macula and attached to the cupula of the affected semicircular canal (cupulolithiasis) or free-floating within its lumen (canalolithiasis). According to the vestibulo-ocular reflex pathophysiology, each cupular deflection, either exciting or inhibiting the corresponding ampullary afferents, generates the contraction of specific extraocular muscles couples leading to pathognomonic nystagmus. The Upright BPPV Protocol (UBP) is a diagnostic approach to BPPV conducted in the sitting position slowly bending the patient's head along the spatial axes, aiming to move canaliths by gravity within the involved semicircular canal, under continuous nystagmus monitoring by video-Frenzel goggles. UBP starts with the evaluation of pseudo-spontaneous nystagmus in the primary gaze position and continues with the upright Head Pitch Test (uHPT) by forward and backward head bendings along the pitch plane. The uHPT can indicate whether horizontal or vertical semicircular canal is involved. If horizontal canal is suspected, the upright Head Roll Test (uHRT) usually provides the diagnosis of the involved side and arm by tilting the patient's head rightward and leftward along the roll plane. Conversely, canalolithiasis involving the posterior semicircular canal can be diagnosed with the uHPT alone. Nevertheless, if necessary, the diagnostic sensitivity can be increased by head movements along the right anterior - left posterior (RALP) and left anterior - right posterior (LARP) canal planes (uRALP/uLARP test). Following the UBP, most BPPV form can be diagnosed in upright position, allowing clinicians to proceed immediately with proper physical treatment and avoiding unpleasant maneuvers to patients.

Spontaneous Upbeat Nystagmus and Selective Anterior Semicircular Canal Hypofunction on Video Head Impulse Test: A New Variant of Canalith Jam?

We describe a rare case of spontaneous upbeat nystagmus (UBN) attributable to a canalith jam involving the anterior semicircular canal (ASC) in a patient in whom comprehensive vestibular assessment was useful to identify the underlying pathomechanism. A 56-year-old woman with unsteadiness following repositioning procedures for left-sided benign paroxysmal positional vertigo (BPPV) presented with spontaneous UBN that showed slight right torsional components. A vestibular test battery detected isolated left ASC hypofunction on a video-head impulse test (Video-HIT). We postulated a persistent utriculopetal deflection of the left ASC cupula, which was attributable to entrapment of debris in a narrow canal tract, with consequent sustained inhibition of the ampullary afferents. Although spontaneous UBN receded after impulsive physical therapy, unsteadiness deteriorated into positional vertigo secondary to canalolithiasis involving the ipsilateral posterior canal. In our view, physical therapy possibly fragmented the canalith jam and released free-floating otoconia that eventually settled into the ipsilateral posterior canal. Video HIT revealed normalization of ASC hypofunction, and leftsided posterior canal canalolithiasis was successfully treated using appropriate repositioning procedures. We propose that a canalith jam involving the ASC should be considered in the differential diagnosis of spontaneous UBN, particularly in patients with a history of BPPV and isolated ASC hypofunction detected on video HIT.

Recovery of Regular Daily Physical Activities Prevents Residual Dizziness after Canalith Repositioning Procedures.

OBJECTIVE: Residual dizziness is a disorder of unknown pathophysiology, which may occur after repositioning procedures for benign paroxysmal positional vertigo. This study evaluates the relationship between regular daily physical activity and the development of residual dizziness after treatment for benign paroxysmal positional vertigo. STUDY DESIGN: Prospective observational cohort study. SETTING: Academic university hospital. METHODS: Seventy-one patients admitted with benign paroxysmal positional vertigo involving the posterior semicircular canal were managed with Epley's procedure. Three days after successful treatment, the patients underwent a telephone interview to investigate vertigo relapse. If the patients no longer complained of vertigo, they were asked about symptoms consistent with residual dizziness. Subsequently, they were asked about the recovery of physical activities they regularly performed prior to the onset of vertigo. RESULTS: Sixty-nine patients (age: 57.79 ± 15.05) were enrolled: five (7.24%) reported vertigo relapse whereas twenty-one of sixty-four non-relapsed patients (32.81%) reported residual dizziness. A significant difference in the incidence of residual dizziness was observed considering the patients' age (p = 0.0003). Of the non-relapsed patients, 46 (71.88%) recovered their regular dynamic daily activities after treatment and 9 (19.57%) reported residual dizziness, while 12 of the 18 patients (66.67%) who did not resume daily activity reported residual symptoms (p = 0.0003). A logistic regression analysis showed a significant association between daily activity resumption and lack of residual dizziness (OR: 14.01, 95% CI limits 3.14-62.47; p = 0.001). CONCLUSIONS: Regardless of age, the resumption of regular daily physical activities is associated with a lack of residual dizziness.

Spontaneous Jamming of Horizontal Semicircular Canal Combined with Canalolithiasis of Contralateral Posterior Semicircular Canal.

Spontaneous canalith jam is an uncommon form of benign paroxysmal positional vertigo mimicking acute vestibular neuritis. We described for the first time a spontaneous horizontal semicircular canalith jam associated with a typical canalolithiasis involving contralateral posterior semicircular canal (PSC), illustrating how the latter condition modified direction-fixed nystagmus during head movements. An 81-year-old woman with persistent vertigo referred to our center. Video-Frenzel examination showed horizontal direction-fixed right-beating nystagmus in primary gaze position, inhibited by visual fixation. She exhibited corrective saccades after leftward head impulses. Chin-to-chest positioning at the head-pitch test did not modify spontaneous nystagmus, whereas slight torsional components with the top pole of the eye beating toward the right ear appeared in backward head-bending, resulting in mixed horizontal-torsional nystagmus. At supine positioning tests, direction-fixed nystagmus turned into direction-changing geotropic horizontal nystagmus, which was stronger on the left side, while overlapping upbeat nystagmus with torsional right-beating components appeared on the right. Primary clinical findings were consistent with a left horizontal semicircular canalith jam, inducing a persistent utriculofugal cupular displacement, combined with a typical right-sided PSC-canalolithiasis. Once canalith jam crumbled, resulting in a non-ampullary arm canalolithiasis of the horizontal semicircular canal, both involved canals were freed by debris with appropriate repositioning procedures.

Bilateral Vestibulopathy in Neuroborreliosis.

OBJECTIVE: To describe a unique case of definite neuroborreliosis presenting with bilateral vestibulopathy (BV) due to simultaneous involvement of both vestibular systems highlighted by a complete assessment for all five vestibular receptors. PATIENT: A 72-year-old woman presented with disabling disequilibrium arisen about 4 weeks earlier and history of erythema migrans developing about 45 days before. INTERVENTIONS: Assessing all five vestibular receptors with the video-head impulse test (vHIT), the suppression head impulse paradigm (SHIMP) and vestibular evoked myogenic potentials (VEMPs), a severe bilateral vestibulopathy was diagnosed. IgG and IgM Borrelia-specific antibodies on patient serum and cerebrospinal fluid analysis confirmed the diagnosis of neuroborreliosis. Following diagnosis, a course of doxycycline was started and the patients received an individualized vestibular rehabilitation program. RESULTS: The patient exhibited slowly progressive improvements for disabling symptoms and the improving function of all five vestibular receptors was monitored with vHIT, SHIMP, and VEMPs over time. CONCLUSIONS: This is the first case report of bilateral vestibulopathy likely caused by neuroborreliosis. Although neurotologic involvement is an uncommon complication in this condition, clinicians should consider a vestibular testing battery when addressed by patient's history and bedside vestibular findings.

Fluctuating Posterior Canal Function in Benign Paroxysmal Positional Vertigo Depending on How and Where Otoconia Are Disposed.

OBJECTIVE: Though fluctuations in vestibular function represent a common finding in Menière's disease, we describe how benign paroxysmal positional vertigo (BPPV) may result in fluctuations of vestibulo-ocular reflex for the involved canal depending on the disposition of otoliths. PATIENT: A 54-year-old woman suffering from refractory posterior canal (PC)-BPPV resulting in fluctuating PC function. INTERVENTIONS: Diagnostic evaluation and rehabilitative treatment for BPPV involving the affected PC. MAIN OUTCOME MEASURES: Video-Frenzel and video-head impulse test (vHIT) findings before and after canalith repositioning procedures for PC-BPPV. RESULTS: BPPV involving the nonampullary arm of right PC was diagnosed based on presenting positional downbeat nystagmus and selective right PC hypofunction at the vHIT. During physical treatment, nystagmus first became positional paroxysmal upbeat likely due to a shift of debris into the ampullary arm of the canal, then turned to spontaneous downbeat nystagmus consistently with a plug effect exerted by particles entrapped within the nonampullary arm of PC and finally receded proving an otoliths fall within the utriculus. Simultaneously, vHIT documented fluctuations for right PC vestibulo-ocular reflex gain as it first increased to normal values, then severely declined and finally normalized, respectively. High-resolution computed tomography scan detected ipsilateral superior canal dehiscence. CONCLUSIONS: In accordance with recently reported vHIT findings in different types of BPPV, fluctuation of PC function could be likely explained by the effect of particles on cupular dynamic responses depending on the portion of the canal gradually involved. Superior canal dehiscence may have played a role facilitating otoliths mobilization by reducing labyrinthine impedance.

Case Report: Could Hennebert's Sign Be Evoked Despite Global Vestibular Impairment on Video Head Impulse Test? Considerations Upon Pathomechanisms Underlying Pressure-Induced Nystagmus due to Labyrinthine Fistula.

We describe a case series of labyrinthine fistula, characterized by Hennebert's sign (HS) elicited by tragal compression despite global hypofunction of semicircular canals (SCs) on a video-head impulse test (vHIT), and review the relevant literature. All three patients presented with different amounts of cochleo-vestibular loss, consistent with labyrinthitis likely induced by labyrinthine fistula due to different temporal bone pathologies (squamous cell carcinoma involving the external auditory canal in one case and middle ear cholesteatoma in two cases). Despite global hypofunction on vHIT proving impaired function for each SC for high accelerations, all patients developed pressure-induced nystagmus, presumably through spared and/or recovered activity for low-velocity canal afferents. In particular, two patients with isolated horizontal SC fistula developed HS with ipsilesional horizontal nystagmus due to resulting excitatory ampullopetal endolymphatic flows within horizontal canals. Conversely, the last patient with bony erosion involving all SCs developed mainly torsional nystagmus directed contralaterally due to additional inhibitory ampullopetal flows within vertical canals. Moreover, despite impaired measurements on vHIT, we found simultaneous direction-changing positional nystagmus likely due to a buoyancy mechanism within the affected horizontal canal in a case and benign paroxysmal positional vertigo involving the dehiscent posterior canal in another case. Based on our findings, we might suggest a functional dissociation between high (impaired) and low (spared/recovered) accelerations for SCs. Therefore, it could be hypothesized that HS in labyrinthine fistula might be due to the activation of regular ampullary fibers encoding low-velocity inputs, as pressure-induced nystagmus is perfectly aligned with the planes of dehiscent SCs in accordance with Ewald's laws, despite global vestibular impairment on vHIT. Moreover, we showed how pressure-induced nystagmus could present in a rare case of labyrinthine fistulas involving all canals simultaneously. Nevertheless, definite conclusions on the genesis of pressure-induced nystagmus in our patients are prevented due to the lack of objective measurements of both low-acceleration canal responses and otolith function.

Upright head roll test: A new contribution for the diagnosis of lateral semicircular canal benign paroxysmal positional vertigo.

Diagnosing the affected side in Benign Paroxysmal Positional Vertigo (BPPV) involving the Lateral Semicircular Canal (LSC) is often challenging and uncomfortable in patients with recent onset of vertigo and intense autonomic symptoms. The Minimum Stimulus Strategy (MSS) aims to diagnose side and canal involved by BPPV causing as little discomfort as possible to the patient. The strategy applied for LSC-BPPV includes the evaluation of pseudo-spontaneous nystagmus and oculomotor responses to the Head Pitch Test (HPT) in upright position, to the seated-supine test and to the Head Yaw Test (HYT) while supine. Matching data obtained by these tests enables clinicians to diagnose the affected side in LSC-BPPV. The purpose of this preliminary study is to propose a new diagnostic test for LSC-BPPV complimentary to the HPT, the Upright Head Roll Test (UHRT), to easily determine the affected ear and the involved arm in the sitting position and to evaluate its efficiency. Our results suggest that the UHRT can increase the sensitivity of the MSS without resorting to the HYT, thus reducing patient's discomfort.

A Possible Role of Video-Head Impulse Test in Detecting Canal Involvement in Benign Paroxysmal Positional Vertigo Presenting With Positional Downbeat Nystagmus.

OBJECTIVE: To describe the possible diagnostic role of video-head impulse test (vHIT) in patients presenting with positional downbeat nystagmus (PDN) due to benign paroxysmal positional vertigo (BPPV) involving the anterior canal (AC) or the non-ampullary arm of the posterior canal (PC). PATIENTS: Three patients presenting with positional vertigo, PDN, symmetrical cervical and ocular vestibular-evoked myogenic potentials, and selective deficit of the vestibulo-ocular reflex (VOR) gain for a single vertical canal on vHIT. INTERVENTIONS: Diagnostic evaluation and rehabilitative treatment for BPPV involving the deficient canals. MAIN OUTCOME MEASURES: Video-oculographic and vHIT findings before and after canalith repositioning procedures (CRP) for AC-BPPV and apogeotropic PC-BPPV. RESULTS: Each patient was treated with CRP for BPPV involving the hypoactive canal. In one case, symptoms receded with physical therapy, whereas two patients developed a paroxysmal positional upbeat nystagmus consistent with BPPV involving the ampullary arm of the ipsilateral PC and were treated with Epley CRP. Posttreatment evaluation showed resolution of symptoms and signs and restitution of canal function in all cases. CONCLUSIONS: PDN can be due to both peripheral and central vestibular pathologies. In case of BPPV, involvement of the non-ampullary arm of the PC is hardly distinguishable from contralateral AC canalolithiasis. In these situations, vHIT may play a key role in the differential diagnosis. Considering these findings, we propose to include vHIT in the test battery of patient with PDN, as it may provide clues to the differential diagnosis with central pathologies enabling the identification of the canal involved by BPPV.

Case Report: Filling Defect in Posterior Semicircular Canal on MRI With Balanced Steady-State Gradient-Echo Sequences After Labyrinthine Ischemia in the Common Cochlear Artery Territory as an Early Sign of Fibrosis.

We describe a rare case of posterior semicircular canal (PSC) fibrosis following acute labyrinthine ischemia in the territory supplied by the common cochlear artery (CCA) and review the relevant literature. A 71-year-old man with multiple vascular risk factors presented 12 days after the onset of acute vertigo and profound left-sided hearing loss. Right-beating spontaneous nystagmus with downbeat components elicited by mastoid vibrations and headshaking was detected. The video head impulse test (vHIT) revealed an isolated hypofunction of the left PSC, whereas vestibular evoked myogenic potentials (VEMPs) showed ipsilateral saccular loss. The clinical presentation and instrumental picture were consistent with acute ischemia in the territory supplied by left CCA. Compared to previous imaging, a new MRI of the brain with 3D-FIESTA sequences highlighted a filling defect in the left PSC, consistent with fibrosis. Hearing function exhibited mild improvement after steroid therapy and hyperbaric oxygen sessions, whereas vHIT abnormalities persisted over time. To the best of our knowledge, this is the only case in the literature reporting a filling defect on MRI, consistent with semicircular canal fibrosis following acute labyrinthine ischemia. Moreover, PSC fibrosis was related with poor functional outcome. We therefore suggest using balanced steady-state gradient-echo sequences a few weeks following an acute lesion of inner ear sensors to detect signal loss within membranous labyrinth consistent with post-ischemic fibrosis. Besides addressing the underlying etiology, signal loss might also offer clues on the functional behavior of the involved sensor over time. In cases of acute loss of inner ear function, a careful bedside examination supplemented by instrumental assessments, including vHIT and VEMPs, of vestibular receptors and afferents may be completed by MRI with balanced steady-state gradient-echo sequences at a later time to confirm the diagnosis and address both etiology and functional outcome.