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Benign Paroxysmal Positional Vertigo (BPPV) is one of the most prevalent peripheral vestibular disorders seen in specialized dizziness clinics. Despite being a well-understood condition with effective treatment options, BPPV remains associated with significant diagnostic delays and healthcare costs. If proven reliable, telemedicine approaches could help address these challenges by improving diagnostic accessibility and efficiency. To investigate the interobserver agreement in BPPV diagnosis, when using eye movement recordings. Six vestibular medicine specialists (Specialist 1, 2, 3, 4, 5, 6) were recruited to participate in this study. The specialists were asked to evaluate the recordings of 240 patient cases who underwent assessment for BPPV (first assessment). After viewing the recordings of each case, they were required to make a BPPV diagnosis. Five specialists (2, 3, 4, 5, 6) agreed to repeat the procedure twice, to additionally evaluate the intraobserver agreement (second assessment). The proportion of agreement and Cohen’s kappa were calculated for both interobserver and intraobserver agreement. Furthermore, agreement with the original diagnoses was evaluated. The interobserver agreement between experts was fair to moderate with a Cohen’s kappa value of 0.40 (CI 95% [0.35, 0.45]) and a proportion of agreement of 60% (CI 95% [54, 67]). Specialists 2, 4, and 5 exhibited substantial intraobserver agreement. In contrast, Specialist 3 demonstrated fair intraobserver agreement, while Specialist 6 showed almost perfect intraobserver agreement. Regarding the first assessment, agreement with the original diagnoses ranged from fair to substantial, with kappa values between 0.40 and 0.70, and corresponding percentages between 58 and 78%. Similar results were observed for the second assessment. The interobserver agreement between specialists diagnosing BPPV using eye movement recordings, was fair to moderate. The suboptimal agreement could be related to missing clinical information (e.g. patient history and symptoms during positional maneuvers). Future studies should incorporate this information and reassess interobserver agreement.

[...]we would like to thank the Authors, as they offered us the opportunity to clarify our precise position on the topic. [...]true is it that a normal SSC VOR-gain has been detected even postoperatively in some patients submitted to surgical plugging, likely due to preserved cupular deflections after high-frequency head impulse, which is allowed by an endolymphatic redistribution within the remaining membranous canal [16,17,18,19]. [...]we totally agree with them in that the impaired SSC VOR-gain on vHIT cannot be considered a general rule for any natural evolution of an SCD. For illustrative purposes, we enclose the case of a patient with left SCD exhibiting, besides low-frequency air-bone gap (ABG) and abnormally enhanced VEMPs on the left side (Figure 1A–D), a normal VOR-gain for the dehiscent SSC as long as the head peak velocity remains within 150°/s (i.e., a value which is considered absolutely consistent with the current recommendations on the use of the vHIT in the plane of vertical canals [23]) (Figure 1E), but broadly dropping to deficiency ranges (<0.7) when increasing the head impulse velocity over 200°/s (Figure 1F). [...]not only the dehiscent SSC is clearly impaired, unlike the contralateral SSC which is tested at the same velocity, but also a clear VOR-gain impairment for the functionally-coupled contralateral PSC can be detected (likely resulting from the loss of the push-pull mechanism), which is missing in the first vHIT traces with lower head velocities. [...]in this latter paper, we aimed to propose to go beyond the concept of a spontaneous auto-plugging as the cause of the SSC VOR-gain, and we introduced a different and alternative idea: the dissipation of the ampullofugal endolymphatic flow during the head impulses (Figure 2B) [13,14]. The first is that, when comparing instrumental signs of patients with surgically plugged SCD to a group of SCD patients who did not undergo surgery, we observed significant differences in VEMPs and ABG in our investigation, while the VOR-gain for the dehiscent SSC at the vHIT was roughly similar in both groups. [...]we realized how the high-velocity VOR-gain of some dehiscent PSC was impaired even though the anatomic position of the PSC precludes a plugging exerted by the surrounding structures.

The treatment of patients with rheumatoid arthritis (RA) has dramatically changed in the past 30 years. Currently, numerous conventional, biologic, and targeted synthetic DMARDs have been licensed and used following recommendations provided by international and national scientific societies. However, the availability of biosimilars and the increasing necessity of savings impacted on the local/national prescription of these drugs. The information provided by data sheet of every single drug is a decisive factor on the choice of a certain treatment merged with the patient’s profile. Thus, our purpose was to construct a rational algorithm for the treatment strategy in RA according to costs and the product leaflet of the biologic and targeted-synthetic DMARDs currently licensed in Italy. We used the most recent available recommendations and then we performed a review of the literature considering all the factors that are known to influence drug safety/effectiveness. All these factors were considered in the context of the data sheets of currently available originators and biosimilars.

Objectives: Typical BPPV forms are widespread and easily diagnosed disorders. However, some forms of labyrinthine lithiasis can differ from the typical BPPV paradigm, showing their own signs and symptoms and resulting in variable therapeutic responses. The aim of this retrospective study is to describe the incidence of the so-called atypical forms compared to the more common BPPV, describing their clinical behavior. Methods: This retrospective study analyzed clinical and instrumental data of 139 patients evaluated over a 12-month period at a referral center. Patients were divided into two groups. The first group (Group A) included patients with so-called “typical” and unilateral labyrintholithiasis, while the second group (Group B) included patients with so-called “atypical” forms. Results: Based on clinical characteristics, 82 patients were assigned to group A while 57 (51.01%) to group B. In group A, resolution of the clinical picture required fewer sessions and a smaller number of therapeutic maneuvers than in group B (p < 0.001). Furthermore, in group A, resolution of symptoms was observed immediately after one of the therapeutic maneuvers performed in 74.07% of cases, while in group B, resolution of the clinical picture was observed during one of the follow-up visits in 39.66% of cases (p < 0.001). Conclusions: Although considered rare, “atypical” forms have an increased prevalence in tertiary centers. The location of the canaliths within the labyrinth can be hypothesized based on the pattern of nystagmus, which serves as a guide for treatment.

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.

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.

Vestibular atelectasis (VA) is a rare clinical entity characterized by a collapse of the endolymphatic space resulting in vestibular loss with the possible onset of positional and/or sound/pressure-induced vertigo. It could be idiopathic or secondary to other inner-ear diseases including Meniere’s disease (MD). A collapse of the membranous labyrinth involving the semicircular canals (SCs) and the utricle represents its distinctive histopathological feature. While specific radiological patterns consistent with VA have been described on contrast-enhanced MRI with delayed acquisitions, an impairment of the blood–labyrinthine barrier (BLB) could be detected in several disorders leading to vestibular loss. We conducted a narrative review of the literature on VA focusing on the putative pathomechanisms accounting for positional and sound/pressure-induced nystagmus despite unilateral vestibular loss (UVL) in this condition, providing two novel cases of VA. Both patients presented with a clinical picture consistent with unilateral MD that rapidly turned into progressive UVL and positional and/or sound/pressure-induced vertigo. In both cases, the posterior SC was initially impaired at the video-head impulse test (vHIT) and both cervical and ocular VEMPs were initially reduced. Progressively, they developed unsteadiness with paretic spontaneous nystagmus, an impairment also for the lateral and anterior SCs, caloric hypo/areflexia and VEMPs areflexia. They both exhibited ipsilesional nystagmus to sound/pressure stimuli and in one case a persistent geotropic direction-changing positional nystagmus consistent with a “light cupula” mechanism involving the lateral SC of the affected side. A collapse of the membranous labyrinthine walls resulting in contact between the vestibular sensors and the stapes footplate could explain the onset of nystagmus to loud sounds and/or pressure changes despite no responses to high- and low-frequency inputs as detected by caloric irrigations, vHIT and VEMPs. On the other hand, the onset of positional nystagmus despite UVL could be explained with the theory of the “floating labyrinth”. Both patients received contrast-enhanced brain MRI with delayed acquisition exhibiting increased contrast uptake in the pars superior of the labyrinth, suggesting an impairment of the BLB likely resulting in secondary VA. A small intralabyrinthine schwannoma was detected in one case. VA should always be considered in case of positional and/or sound/pressure-induced vertigo despite UVL.

(1) Background: Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is characterized by late-onset cerebellar ataxia, bilateral vestibulopathy, and sensory neuronopathy mostly due to biallelic RFC1 expansion. (2) Objectives: The aim of this case series is to describe vestibular, gait, and speech alterations in CANVAS via a systematic approach. (3) Methods: All patients (n = 5) underwent a standardized clinical–instrumental examination, including the perceptual and acoustic analysis of speech, instrumental gait, and balance analysis (posturographic data were acquired using a force plate [Kistler, Winterthur, Switzerland] while 3D gait analysis, inclusive of surface electromyography, was acquired using a motion capture system [SMART DX, BTS Bioengineering, Milan, Italy], a wireless electromyograph [FreeEMG, BTS Bioengineering, Milan, Italy]), and vestibular assessment with video-oculography. (4) Results: Five patients were included in the analysis: three females (patients A, B, C) and two males (patients D and E) with a mean age at evaluation of 62 years (SD ± 15.16, range 36–74). The mean age of symptoms’ onset was 55.6 years (SD ± 15.04, range 30–68), and patients were clinically and instrumentally evaluated with a mean disease duration of 6.4 years (SD ± 0.54, range 6–7). Video-Frenzel examination documented spontaneous downbeat nystagmus enhanced on bilateral gaze in all patients, except for one presenting with slight downbeat nystagmus in the supine position. All patients exhibited different degrees of symmetrically reduced VOR gain for allsix semicircular canals on the video-head impulse test and an unexpectedly normal (“false negative”) VOR suppression, consistent with combined cerebellar dysfunction and bilateral vestibular loss. Posturographic indices were outside their age-matched normative ranges in all patients, while 3D gait analysis highlighted a reduction in ankle dorsiflexion (limited forward rotation of the tibia over the stance foot during the stance phase of gait and fatigue of the dorsiflexor muscles) and variable out-of-phase activity of plantar flexors during the swing phase. Finally, perceptual-acoustic evaluation of speech showed ataxic dysarthria in three patients. Dysdiadochokinesis, rhythm instability, and irregularity were observed in the oral diadochokinesis task. (5) Conclusions: CANVAS is a recently discovered syndrome that is gaining more and more relevance within late-onset ataxias. In this paper, we aimed to contribute to a detailed description of its phenotype.

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.

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.