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Objective: to report the first case series of Brazilian children diagnosed with Kleefstra syndrome, present a possible phenotype expansion to the syndrome and to raise physicians' awareness for this rare disease., Results: seven patients with confirmed KS were evaluated, including 5 males and 2 females. Abnormal prenatal findings were observed in 4 patients. Most patients were born at term, with normal birth measurements. All patients had neurodevelopmental delay and 6 evolved with intellectual disability. Hearing loss was present in 57.1% of patients and 28.7% had congenital heart disease. In males, cryptorchidism was present in 75%. Despite the facial dysmorphisms, only 2 out of 7 patients had a pre-test clinical suspicion of KS. One specific patient presented bilateral agenesis of the semicircular canals, a very rare ear manifestation in Kleefstra syndrome, representing a possible phenotype expansion of the syndrome., Conclusion: this report aims to promote awareness among physicians evaluating patients in a context of neurodevelopmental delay or congenital malformations, especially congenital heart defects. We also highlight a possible phenotype expansion of the syndrome, with a case of semicircular anomaly, not reported in this syndrome so far., Competing Interests: Declaration of competing interest nothing to declare., (Copyright © 2024 The Authors. Published by Elsevier Masson SAS.. All rights reserved.)

Purpose: Most of Superior Semicircular Canal Dehiscence (SSCD) are located in the apical region of the SSC. However, in a small number of cases, it may be situated in the medial wall, causing the SSC to contact with the superior petrosal sinus (SPS). The aim of this study is to describe four patients with SSCD involving the superior petrosal sinus (SSCD-SPS) and to perform a review of the literature., Methods: Observational retrospective study of patients diagnosed of SSCD-SPS in a tertiary referral center. A systematic review was made, identifying 7 articles in the literature. Clinical presentation, complementary test (pure-tone audiometry, PTA; vestibular evoked myogenic potential, VEMP; computed tomography, CT), therapeutic management and outcomes were reported., Results: Four new cases of SSCD-SPS are reported, in three of them a transmastoid plugging was performed. 54 patients with SSCD-SPS (57 dehiscences) were reported in the literature. The most frequent symptoms were aural pressure (57.41%) and vertigo provoked by pressure/Valsalva (55.55%). Conductive hearing loss was the most common finding in PTA (47.37%). Abnormally low thresholds were observed in 59.46% of reported VEMP. Transmastoid approach was used in ten cases, middle fossa approach in four, round window reinforcement in one, and occlusion of the SPS using coils in two., Conclusions: Within SSCD, we have encountered a rare subtype characterized by its medial wall location in close proximity to the SPS. This subgroup needs special consideration as it has shown its own distinct characteristics. Regarding therapeutic management, we advocate a transmastoid approach., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Determine if superior canal dehiscence (SCD) found on flat-panel CT increases the risk for other defects in the otic capsule., Study Design: Retrospective cohort study., Setting: Tertiary care center., Patients: One hundred ears (50 with SCD and 50 matched controls without SCD)., Interventions: Flat-panel CT imaging., Main Outcome Measures: (1) Prevalence of other dehiscences in SCD ears, (2) dehiscences in controls, and (3) otic capsule thickness in other reported dehiscence locations (cochlea-carotid, lateral semicircular canal [SCC] and mastoid, facial nerve-lateral SCC, vestibular aqueduct, posterior SCC-jugular bulb, posterior SCC-posterior fossa). Between-group comparisons were considered significant at p < 0.007 after applying the Bonferroni correction for multiple comparisons., Results: Not including the SCD, there was a mean of 0.04 additional dehiscences in the SCD group (n = 2/50, 4%) and 0.04 non-SCD dehiscences in the controls (n = 2/50, 4%, p > 0.007). In the SCD group, there was one dehiscence between the cochlea and carotid artery and one between the posterior SCC and posterior fossa. The control group had one enlarged vestibular aqueduct and one dehiscence between the facial nerve and lateral SCC. As a group, SCD ears had wider vestibular aqueducts (0.68 ± 0.20 vs 0.51 ± 0.30 mm, p < 0.007) and thinner bone between the posterior SCC and posterior fossa (3.12 ± 1.43 vs 4.34 ± 1.67 mm, p < 0.007). The bone between the facial nerve and lateral SCC was thicker in SCD ears (0.77 ± 0.23 vs 0.55 ± 0.27 mm, p < 0.007) and no different for cochlea-carotid, and lateral SCC and mastoid (p > 0.007)., Conclusions: SCD does not increase the likelihood of a second dehiscence in the same otic capsule. SCD patients may have congenitally thinner otic capsule bones compared to controls, particularly near the posterior SCC, where the vestibular aqueduct may be enlarged., Competing Interests: Conflicts of Interest: The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript; or in the decision to publish the results., (Copyright © 2024, Otology & Neurotology, Inc.)

Benign paroxysmal positional vertigo (BPPV) is a common vestibulopathy and involves failed dissolution and dislocation of calcium carbonate crystals into the semicircular canal. This causes short-lasting vertigo during changes in head position. Oftentimes, BPPV can be resolved within a single clinic visit, but secondary to many known risk factors, BPPV can recur. This case report follows a patient with extremely frequent recurrences despite a lack of known risk factors. A 55-year-old female experienced BPPV in December 2022, with successful canalith repositioning treatment from otolaryngology. On having a recurrence in March 2023, the patient underwent videonystagmography including caloric testing, and MRI, all of which showed normal findings besides left posterior-canal BPPV. From December 2022 to February 2024, the patient had 13 recurrences, each treated to resolution, confirmed by repeating positional tests and per subjective report for at least 2 weeks following. The incidence of BPPV recurrence is reported higher in females, however, this could not be interpreted as a causative factor. Though many other risk factors are documented in literature, this patient's history, demographics, imaging, and blood tests were all negative. This case report highlights a gap in knowledge of vestibular pathophysiology, as this patient's high rate of recurrence remains unexplained.

Superior Semicircular Canal Dehiscence (SSCD) is a recently-defined developmental defect may be associated with several craniofacial anomalies such as Cleft Lip/Palate (CLP). The present study aimed to compare subjects with unilateral and bilateral CLP and normal controls in terms of Superior Semicircular Canal (SSC) bone thickness and pattern., A total of 238 Cone Beam Computed Tomography (CBCT) images were collected from 52 unilateral Cleft Lip and Palate (UCLP) subjects (104 temporal bones) and 38 Bilateral Cleft Lip and Palate (BCLP) (76 temporal bones) subjects and 148 healthy controls (296 temporal bones). The SSC bone thickness was measured twice and validated by a maxillofacial radiologist. The samples were then classified into five categories based on bone thickness: papyraceous or thin, normal, thick, pneumatized, and dehiscence. After all, the UCLP, BCLP, and normal control groups were compared concerning the SSC pattern and thickness., The results revealed no significant difference among the three groups regarding the SSC pattern and thickness based on gender. The SSC patterns ( P value = .001) and SSC thickness (0.01) were strongly correlated to the cleft type. The thinnest bone thickness and the highest incidence of SSCD were observed among the subjects with BCLP., The results showed a significant association between the SSC patterns and SSC thickness and the study groups., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: This study investigates the importance of bone density, surface area, and diameter of anatomical structures of the superior semicircular canal (SSC), lateral semicircular canal (LSC), posterior semicircular canal (PSC), utricle, and saccule in patients diagnosed with superior semicircular canal dehiscence (SSCD)., Materials and Methods: The bone density, surface area, and diameter of SSC, LSC, PSC, utricle, and saccule were measured and compared between the SSCD group and control group. Fifteen ears in the SSCD group and 60 ears in the control group were evaluated. Additionally, within the SSCD group, the dehiscent and healthy sides were evaluated independently., Results: SSC's bone density was significantly lower in the SSCD group compared to the control group (p = 0.008). No significant differences were found in surface area and diameter between the groups (p > 0.05). While most of the anatomical structures showed no significant difference in bone density between dehiscent and healthy ears (p > 0.05), SSC bone density was significantly lower in affected ears (p = 0.000) in SSCD group., Conclusion: Based on the data obtained in this study, bone density and anatomical structure may be useful in patients diagnosed with SSCD., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Competing Interests: The authors disclose no conflicts of interest.

Purpose: Radiographic review of pathologies that associate with third window syndrome., Methods: Case series and literature review., Results: Eight unique third window conditions are described and illustrated, including superior, lateral, and posterior semicircular canal dehiscence; carotid-cochlear, facial-cochlear, and internal auditory canal-cochlear dehiscence, labyrinthine erosion from endolymphatic sac tumor, and enlarged vestibular aqueduct., Conclusion: The present study highlights the characteristic imaging features and symptoms to differentiate third window pathologies for expedient diagnosis and management planning., Competing Interests: Declaration of competing interest The authors report no relevant conflict of interest in submitting this article for publication., (Copyright © 2023. Published by Elsevier Inc.)

Background, Aims: Stapes footplate thickness measurement using ultra-high-resolution CT has been described only in the lateral semicircular canal plane. The purpose of this study was to compare stapes footplate thickness between the lateral semicircular canal and stapes axial planes in patients with otosclerosis compared to controls., Material and Methods: We performed a retrospective single-center study of patients undergoing high-resolution temporal bone CT. Two radiologists measured stapes footplate thickness in both the lateral semicircular canal and stapes axial planes., Results: Between February 2020 and October 2022, we collected 81 ears from 49 patients (75% of women; mean age 51.22 ± 16.6 years, 17 otosclerosis, and 64 controls). In the stapes axial plane, there was a significant anterior thickening in otosclerosis patients (Reader 1: 0.52 ± 0.12 [0.3-0.7] vs. 0.41 ± 0.08 [0.3-0.6], p  = 0.001; Reader 2: 0.54 ± 0.06 [0.5-0.7] vs. 0.39 ± 0.08 [0.2-0.6], P  < 0.001) compared to controls. These differences were not significant using the lateral semicircular canal plane., Conclusion: The stapes footplate was thickened at its AC in otosclerosis patients using only the stapes axial plane., Significance: We propose to use the stapes axial plane instead of the lateral semicircular canal plane when analyzing the stapes.

Superior canal dehiscence syndrome (SCDS) is a vestibular disorder in which the presence of a pathological third window in the labyrinth causes several vestibular and cochlear symptoms. Herein, we review the diagnostic criteria and treatment of SCDS. The cause of SCDS is considered to be congenital or acquired; however, its etiology is not well known. Symptoms: Vertigo and/or oscillopsia induced by loud sounds (Tullio phenomenon) or stimuli that change the middle ear or intracranial pressure (fistula symptoms) with vestibular symptoms and hyperacusis and aural fullness with cochlear symptoms are characteristic clinical complaints of this syndrome. Neurological tests: Vertical-torsional eye movements can be observed when the Tullio phenomenon or fistula symptoms are induced. Conductive hearing loss with both a decrease in the bone conduction threshold at lower frequencies and an increase in the air conduction threshold at lower frequencies may be present on audiometry. Cervical and/or ocular vestibular evoked myogenic potentials are effective in strongly suspecting the presence of a pathologic third window in the labyrinth. Computed tomography (CT) imaging: High-resolution CT findings with multiplanar reconstruction in the plane of the superior semicircular canal consistent with dehiscence indicate SCDS. The Pöschl view along the plane of the superior semicircular canal and the Stenvers view perpendicular to it are recommended as CT imaging conditions. Findings from all three major diagnostic categories (symptoms, neurological tests, and/or CT imaging) are needed to diagnose SCDS. The surgical approaches for SCDS are as follows: the 1) middle cranial fossa approach, 2) transmastoid approach, and 3) round window and oval window reinforcement. Each technique has advantages and disadvantages., Competing Interests: Declaration of Competing interest The authors declare that there is no conflict of interest., (Copyright © 2023. Published by Elsevier B.V.)

Background and Purpose: The radiologic prevalence of superior semicircular canal dehiscence in the asymptomatic population has been widely studied, but less is known about the rates of other forms of third window dehiscence. Per the existing literature, the radiologic prevalence of cochlear-facial nerve dehiscence, for example, exceeds that seen in histologic studies, suggesting that conventional CT is unreliable for cochlear-facial dehiscence. These studies relied on nonisometric CT acquisitions, however, and underused multiplanar reformatting techniques, leading to false-positive findings. Our purpose was to determine the rate of cochlear-facial dehiscence and other non-superior semicircular canal third window dehiscences on optimized CT in asymptomatic patients., Materials and Methods: Sixty-four-channel temporal bone CT scans from 602 patients in emergency departments were assessed for cochlear-facial and other non-superior semicircular canal third window dehiscences by using high-resolution, multiplanar oblique reformats. Confidence intervals for dehiscence prevalence were calculated using the Newcombe 95% interval confidence method., Results: Of 602 patients, 500 were asymptomatic, while 102 had an imaging indication consistent with possible third window syndrome (symptomatic). Eight asymptomatic patients (1.6%) had cochlear-facial dehiscence, while 43 (8.4%) had jugular bulb-vestibular aqueduct dehiscence. There was no statistically significant difference between the prevalence of cochlear-facial dehiscence or jugular bulb-vestibular aqueduct dehiscence in asymptomatic patients compared with symptomatic patients. Cochlear-carotid canal, cochlear-internal auditory canal, and cochlear-petrosal sinus dehiscences were not observed., Conclusions: Sixty-four-channel CT with multioblique reformatting is sensitive and specific for identifying cochlear-facial dehiscence, with rates similar to those in postmortem series. Jugular bulb-vestibular aqueduct dehiscence is a common incidental finding and is unlikely to produce third window physiology. Other non-superior semicircular canal third window dehiscences are rare in asymptomatic patients., (© 2023 by American Journal of Neuroradiology.)

Objective: To determine the accuracy of 1.5 Tesla magnetic resonance imaging in identifying the bony defect in superior semi-circular canal dehiscence syndrome., Methods: A retrospective case-control study was conducted in tertiary university settings. The study included 12 patients with a definite diagnosis of superior semi-circular canal dehiscence syndrome and a control group comprising 12 non-superior semi-circular canal dehiscence syndrome patients, all with available magnetic resonance imaging data. The imaging scans were anonymised, and reviewed blindly and independently by three experienced radiologists., Results: There was low sensitivity and specificity, with average values of 47 per cent (range, 16.7-66.7 per cent) and 69 per cent (range, 66.7-75 per cent) respectively. Cohen's kappa was 0.75, indicating substantial inter-rater reliability., Conclusion: Given the low accuracy of 1.5T magnetic resonance imaging in identifying the bony defect in superior semi-circular canal dehiscence syndrome, despite its value in inner-ear imaging, computed tomography or equivalent should be used when superior semi-circular canal dehiscence syndrome is suspected.

Otic capsule dehiscences create a pathological third window in the inner ear that results in a dissipation of the acoustic energy consequent to the lowered impedance. Superior semicircular canal dehiscence (SSCD) was identified by Minor et al in 1998 as a syndrome leading to vertigo and inner ear conductive hearing loss. The authors also reported the relation between the dehiscence and pressure- or sound-induced vertigo (Tullio's phenomenon). Prevalence rates of SSCD in anatomical studies range from 0.4% to 0.7% with a majority of patients being asymptomatic. The observed association with other temporal bone dehiscences, as well as the propensity toward a bilateral or contralateral "near dehiscence," raises the question of whether a specific local bone demineralization or systemic mechanisms could be considered. The present report regard a case of a patient with a previous episode of meningitis, with a concomitant bilateral SSCD and tegmen tympani dehiscence from the side of meningitis. The patient was affected by dizziness, left moderate conductive hearing loss, and pressure/sound-induced vertigo. Because of disabling vestibular symptoms, the patient underwent surgical treatment. A middle cranial fossa approach allowed to reach both dehiscences on the symptomatic side, where bone wax and fascia were used for repair. At 6 months from the procedure, hearing was preserved, and the vestibular symptoms disappeared.

Objective: To assess clinical symptoms and rehabilitation of anterior canal benign paroxysmal positional vertigo (aBPPV) in comparison to horizontal canal BPPV (hBPPV) and posterior canal BPPV (pBPPV)., Patients and Methods: The study included 22 patients with aBPPV, 450 patients with pBPPV and 88 patients with hBPPV from total 640 patients with definite BPPV. Rehabilitation of BPPV included different manual repositioning maneuvers. In case of unsuccessful reposition during the visit the patients practiced various home-based repositioning maneuvers and underwent reposition in multipositional mechanical chair. Clinical symptoms and repositioning process in patients with aBPPV were compared to patients with hBPPV and pBPPV, nystagmus was analyzed in aBPPV during the rehabilitation., Results: ABPPV is a rare type of otolithiasis (3.4% of all BPPVcases). aBPPV typically has no association with middle and inner ear pathology and head trauma. Clinical symptoms in aBPPV are equal to pBPPV and less severe than in hBPPV. In aBPPV cases dominates cupulolithiasis type of canal involvement, which leads to treatment resistance and complex rehabilitation process, which includes repeat visits, multiple repeat maneuvers by specialist, home-based treatment and use of multipositional mechanical chair. Residual dizziness with duration more than a week after successful reposition occurs more often in patients with aBPPV compared to hBPPV and pBPPV cases. In aBPPV cases atypical change of nystagmus could be observed which could be due to canal switch from anterior to posterior canals, primary location of otoliths in common crus of vertical canals or masking chronic otolithiasis of anterior short arm of posterior canal.

The current theory in physiopathology of benign paroxysmal positional vertigo is the mechanical theory, namely the cupulolithiasis-canalolithiasis theory. Repositioning maneuvers based on this theory has now taken place in therapy. However, mechanical theory is insufficient to explain some clinical situations and cannot fully enlighten the physiopathology. Mechanical theory is based on very few histological studies. Currently, these few articles are still used for reference. Anatomically, there are uncertainties that need to be explained in this theory. In this literature review, the histological and anatomical evidence is reviewed and the value of mechanical theory in benign paroxysmal positional vertigo physiopathology has been questioned. Studies suggest that the debris in the semicircular canals is caused by degeneration due to aging and may not be responsible for the symptoms in benign paroxysmal positional vertigo. Some patients with debris in semicircular canals do not have benign paroxysmal positional vertigo symptomatology, while some patients without debris may have benign paroxysmal positional vertigo symptomatology. Experimental and histological findings suggest that vestibulopathy due to inflammation caused by neurotropic viruses may lead to benign paroxysmal positional vertigo picture. For all these reasons, in benign paroxysmal positional vertigo physiopathology, there must be other factors besides particle debris in semicircular canals.

Objectives: (1) To determine how otologic/neurotologic surgeons counsel patients with superior semicircular canal dehiscence (SSCD). (2) To understand the plethora of presenting symptoms associated with SSCD and appropriate management. (3) To suggest appropriate management; oftentimes avoiding surgery., Methods: This was a survey study of both community and academic physicians. A 23-question survey was distributed to all members of the American Neurotological (ANS) and American Otologic Societies (AOS) via email in the Fall of 2018. A total of 54 responses were received from a possible pool of 279 for a response rate of 19.4%. Inferences were made about the population through sample proportions and confidence intervals., Results: All respondents use computed tomography (CT) in diagnosing SSCD and 11.1% use CT exclusively. Cervical vestibular evoked myogenic potential (VEMP; 77.8%) are used more often than ocular VEMPs (38.9%). Magnetic resonance imaging (7.4%) is used infrequently; 96.3% of surgeons surveyed have seen patients with SSCD on imaging that are asymptomatic. Following surgical treatment, respondents reported balance issues and mild-to-moderate high-frequency sensorineural hearing loss (88.4%); 32.6% reported that the majority (>50%) of their patients needed further intervention after surgery, typically aggressive vestibular rehabilitation., Conclusions: There is a discrepancy in the systematic approach to SSCD between both the surgeons and the published literature. Patients with SSCD on ultra-high-resolution CT may have myriad symptoms while others are asymptomatic, and surgery may lead to additional complications. We will present a methodical recommendation to assist in the management of patients with SSCD depending upon their symptoms. This may improve patient selection, counseling, and outcomes.

Objective: To describe a case of benign paroxysmal positional vertigo (BPPV) resulting in reversible horizontal semicircular canalith jam successfully treated with horizontal canal occlusion. A brief literature review of similar cases was performed., Methods: Case report and literature review., Results: A 68-year-old female presented with apogeotropic positional nystagmus, attributed to reversible horizontal canalith jam mimicking cupulolithiasis that was refractory to tailored repositioning maneuvers across months. She was unable to work due to the severity of her symptoms. She underwent surgical occlusion of the affected canal with immediate resolution of her symptoms. A literature review revealed similar cases of canalith jam mimicking cupulolithiasis., Conclusions: Reversible canalith jam, in which particles moving with horizontal head position alternate between obstructing the semicircular canal and resting on the cupula, can mimic signs of cupulolithiasis. This variant of BPPV can be effectively managed with surgical canal occlusion should symptoms fail to resolve after tailored repositioning maneuvers.

The aim of this study was to reveal clinical features of benign paroxysmal positional vertigo (BPPV) through comparing idiopathic BPPV and BPPV secondary to vestibular neuritis (VN). The clinical data of the 189 BPPV patients admitted to our tertiary care hospital including otolaryngological, audiological, vestibular, neurological, and radiological evaluations were reviewed. Patients diagnosed with idiopathic BPPV (n = 145) and BPPV secondary to VN (n = 44) were grouped as I and II, respectively. The clinical data of 2 groups were compared. The findings of the study showed that the patients with secondary BPPV due to VN are much younger, have symptoms of only posterior semicircular canal involvement, and require more treatments compared to patients with idiopathic BPPV. The clinical features of patients with BPPV secondary to VN and idiopathic BPPV differ on several aspects. More extensive studies are needed to investigate the underlying etiology in patients with BPPV encountered after VN.

Background: The presence of endolymphatic hydrops (ELH) in patients with Meniere's disease (MD) is considered a pathological hallmark., Aims/objectives: We aimed to conduct a quantitative volumetric measurement of inner ear ELH in patients with unilateral MD (uMD). The values of uMD with and without herniation into the posterior semi-circular canal (h-PSC) and the lateral semi-circular canal (h-LSC) were compared using 3 D magnetic resonance imaging., Material and Methods: This study included 130 individuals (47 controls and 83 patients with uMD). We measured the total fluid space (TFS) and endolymphatic space (ELS) volumes. We also evaluated the ELS/TFS volume ratios (%)., Results: The ELS/TFS volume ratios in the inner ear, cochlea, and vestibule were significantly different between the affected and contralateral sides in patients with h-PSC. Moreover, the ELS/TFS volume ratios of the inner ear, vestibule, and semi-circular canals in the affected ear were significantly higher in patients with h-PSC than in those without h-PSC. The vestibular ELS/TFS volume ratio in the affected ear was significantly higher in patients with h-LSC than in those without h-LSC., Conclusions and Significance: H-LSC is present in extended vestibular ELH. However, this is a result of ELH progression in the inner ear.

We investigated whether cholesteatoma is associated with sensorineural hearing loss (SNHL) and the effects of a lateral semicircular canal (LSCC) fistula, destruction of stapes, localization of cholesteatoma, and air-bone gap (ABG) size on SNHL. The charts of 159 patients who had received surgery for unilateral cholesteatoma were examined retrospectively. In all patients, air conduction and bone conduction (BC) thresholds in both ears were measured at 500, 1000, 2000, and 4000 Hz. Differences in BC thresholds between ears with cholesteatoma and contralateral ears were calculated. Demographics, localization of cholesteatoma, presence of LSCC fistula, condition of stapes, and ABG size on the affected ear were evaluated. There were significantly greater BC thresholds in ears with cholesteatoma than in normal ears for each frequency. Comparing the average BC differences at the different cholesteatoma locations, there were significant differences between the tympanic cavity + all mastoid cell group and attic, attic + antrum, and tympanic cavity + antrum groups. The BC differences at 4000 Hz were significantly high in patients with LSCC fistula. There were no significant relationships between the condition of the stapes and BC differences at any frequencies. There were significant correlations between average ABG and BC threshold differences at all frequencies. A significant relationship was found between cholesteatoma and SNHL. Patients with advanced cholesteatoma had significantly higher levels of SNHL. The BC threshold differences increased with increases in the ABG.

Background: There have been no useful imaging methods to diagnose benign paroxysmal positional vertigo (BPPV), a common cause of vertigo, depending on the characteristic symptom., Objective: To visualize horizontal canal (HC) BPPV using 3DCT and assess its clinical usefulness., Subjects and Methods: Ten BPPV patients were diagnosed with distinct BPPV, canalolithiasis, and cupulolithiasis of the HC (hc-BPPV, hc-BPPV-cu), which were definitely diagnosed on the basis of criteria of BPPV by the Barany Society and 10 healthy subjects without a history of dizziness were investigated using 3DCT with several different CT window values (CTWVs)., Results: The HCs of BPPV patients were clearly visualized and the luminal aspects showed differences among ears with cupulolithiasis, canalolithiasis and no symptoms healthy subjects., Conclusions and Significance: 3DCT images visualized the characteristic changes of the HC of patients with BPPV compared to healthy subjects. The HC images were coincident with the clinical condition of cupulolithiasis and canalolithiasis. This imaging technique is clinically useful for diagnosing, treating and assessing the prognosis of HC BPPV.

In vestibular schwannoma (VS) patients hearing outcome and surgery related risks can vary and depend on the nerve of origin. Preoperative origin differentiation between inferior or superior vestibular nerve may influence the decision on treatment, and the selection of optimal treatment and counselling modalities. A novel scoring system based on functional tests was designed to predict the nerve of origin for VS and was applied to a large number of consecutive patients. A prospective, double blind, cohort study including 93 patients with suspected unilateral VS was conducted at a tertiary referral centre. Preoperatively before tumor resection a functional test battery [video head-impulse test (vHIT) of all semicircular canals (SCC)], air-conducted cervical/ocular vestibular evoked myogenic potential tests (cVEMP, oVEMP), pure-tone audiometry, and speech discrimination was applied. Sensitivity and specificity of prediction of intraoperative finding by a preoperative score based on vHIT gain, cVEMP and oVEMP amplitudes and asymmetry ratios was calculated. For the prediction of inferior vestibular nerve origin, sensitivity was 73% and specificity was 80%. For the prediction of superior vestibular nerve origin, sensitivity was 60% and specificity was 90%. Based on the trade-off between sensitivity and specificity, optimized cut-off values of - 0.32 for cVEMP and - 0.11 for oVEMP asymmetry ratios and vHIT gain thresholds of 0.77 (anterior SCC), 0.84 (lateral SCC) and 0.80 (posterior SCC) were identified by receiver operator characteristic curves. The scoring system based on preoperative functional tests improves prediction of nerve of origin and can be applied in clinical routine.

This study aims to explore the long-term efficacy of triple semicircular canal plugging (TSCP) in the treatment of intractable ipsilateral delayed endolymphatic hydrops (DEH), so as to provide an alternative therapy for this disease. Forty-eight patients diagnosed with ipsilateral DEH referred to vertigo clinic of our hospital between Dec. 2010 and Dec. 2017, were included in this study for retrospective analysis. All patients were followed up for 2 years. Vertigo control and auditory functions were measured and analyzed. Pure tone audiometry, caloric test, and vestibular evoked myogenic potential (VEMP) were performed in two-year follow-up. Forty-five patients who accepted intratympanic gentamicin (26.7 mg/mL) twice given one week apart were selected as a control group. The total control rate of vertigo in TSCP group was 97.9% (47/48) in the two-year follow-up, with complete control rate of 83.3% (40/48) and substantial control rate of 14.6% (7/48). The rate of hearing loss was 22.9% (11/48). The total control rate of vertigo in intratympanic gentamicin group was 80.0% (36/45), with complete control rate of 57.8% (26/45) and substantial control rate of 22.2% (10/45), and the rate of hearing loss was 20.0% (9/45). The vertigo control rate of TSCP was significantly higher than that of intratympanic gentamicin (χ 2  = 6.01, p < 0.05). There was no significant difference of hearing loss rate between two groups. (χ 2  = 0.12, p > 0.05). TSCP, which can reduce vertiginous symptoms in patients with intractable ipsilateral DEH, represents an effective therapy for this disorder.

Objective: The characteristics of typical posterior canal-benign paroxysmal positional vertigo-canalolithiasis (P-BPPV-Can) are well known. We found 10 patients with a new variant of P-BPPV-Can: Reverse (Rev)-P-BPPV-Can. The characteristics and pathophysiology of Rev-P-BPPV-Can are discussed., Methods: For 4 years and 9 months, 10 patients with Rev-P-BPPV-Can (9 women and 1 man; mean age: 73 years) visited our clinic. Within the same period, 268 patients with unilateral typical P-BPPV-Can were treated. During the Dix-Hallpike and Epley maneuvers, nystagmus was recorded using an infrared charge-coupled device camera mounted on a pair of spectacles., Results: The patients exhibited entirely opposite direction of nystagmus from typical P-BPPV-Can in both the Dix-Hallpike head position and upright sitting position. The patients had typical characteristics of nystagmus due to canalolithiasis, such as latency, duration of <1 min, linear-torsional nystagmus, and fatigability. Rev-P-BPPV-Can appeared after the Epley maneuver for typical P-BPPV-Can; in other patients, Rev-P-BPPV-Can changed to typical P-BPPV-Can. The affected sides of Rev-P-BPPV-Can were decided by those of typical P-BPPV-Can., Conclusion: Canalolithiasis in the non-ampullary distal portion of the posterior semicircular canal may be a potential pathophysiology of Rev-P-BPPV-Can. The Epley maneuver has little effect for treating Rev-P-BPPV-Can, but spontaneous transition to typical P-BPPV-Can or spontaneous recovery is expected., (Copyright © 2020 Oto-Rhino-Laryngological Society of Japan Inc. Published by Elsevier B.V. All rights reserved.)

Objective: To test the hypothesis that patterns of semicircular canal (SCC) and otolith impairment in unilateral vestibular loss depend on the underlying disorders, we analyzed peripheral-vestibular function of all 5 vestibular sensors., Methods: For this retrospective case series, we screened the hospital video-head-impulse test database (n = 4,983) for patients with unilaterally impaired SCC function who also received ocular vestibular-evoked myogenic potentials and cervical vestibular-evoked myogenic potentials (n = 302). Frequency of impairment of vestibular end organs (horizontal/anterior/posterior SCC, utriculus/sacculus) was analyzed with hierarchical cluster analysis and correlated with the underlying etiology., Results: Acute vestibular neuropathy (AVN) (37.4%, 113 of 302), vestibular schwannoma (18.2%, 55 of 302), and acute cochleovestibular neuropathy (6.6%, 20 of 302) were most frequent. Horizontal SCC impairment (87.4%, 264 of 302) was more frequent ( p < 0.001) than posterior (47.4%, 143 of 302) and anterior (37.8%, 114 of 302) SCC impairment. Utricular damage (58%, 175 of 302) was noted more often ( p = 0.003) than saccular impairment (32%, 98 of 302). On average, 2.6 (95% confidence interval 2.48-2.78) vestibular sensors were deficient, with higher numbers ( p ≤ 0.017) for acute cochleovestibular neuropathy and vestibular schwannoma than for AVN, Menière disease, and episodic vestibular syndrome. In hierarchical cluster analysis, early mergers (posterior SCC/sacculus; anterior SCC/utriculus) pointed to closer pathophysiologic association of these sensors, whereas the late merger of the horizontal canal indicated a more distinct state., Conclusions: While the extent and pattern of vestibular impairment critically depended on the underlying disorder, more limited damage in AVN and Menière disease was noted, emphasizing the individual range of loss of function and the value of vestibular mapping. Likely, both the anatomic properties of the different vestibular end organs and their vulnerability to external factors contribute to the relative sparing of the vertical canals and the sacculus., (© 2020 American Academy of Neurology.)

Aim: The aim of the present study was to evaluate the effect of isotretinoin (ISO) on peripheral vestibular system using vHIT., Material and Method: This is a prospective study in which 30 patients administered ISO treatment with the diagnosis of acne vulgaris was evaluated. Following ear nose and throat, examination, audiological and vestibular evaluations were carried out. vHIT tests were conducted before and three months after the use of ISO (0.5-0.75 mg/kg/day). In addition, all participants underwent perceptual vertigo and dizziness tests before and three months after the use of ISO., Results: In vHIT evaluation of all patients, no overt saccade, covert saccade and spontaneous nystagmus finding was observed. Gain and asymmetry were compared before and after the use of ISO: No significant difference was found between lateral semicircular canal, anterior, and posterior semi-circular and symmetry measurements made before ISO use and those made three months after it (p = 1.00; p = 0.99; p = 0.66). Similarly, there was no significant difference in asymmetry values of vertical semicircular canals measured before ISO and three months after it (p = 0.90; p = 0.76). No statistically significant difference was found in vertigo, nausea and dizziness in terms of responses before and 3 months after ISO use (p = 0.063; p = 0.031; p = 0.063)., Conclusion: Although the studies demonstrating the effect of ISO on cochlea and symptoms occurring during treatment such as nausea, vomiting and vertigo suggest that it may exert effects on peripheral vestibular system, the present study indicates that it has no short terms effects on structures in peripheral vestibular system and vestibuloocular reflex pathways., Competing Interests: Declaration of competing interest None. There is no financial support or funding. There is no financial support or conflict of interest in the present study., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: This study aimed to evaluate the association between cochlear nerve canal dimensions and semicircular canal abnormalities and to determine the distribution of bony labyrinth anomalies in patients with cochlear nerve canal stenosis., Method: This was a retrospective study in which high-resolution computed tomography images of paediatric patients with severe-to-profound sensorineural hearing loss were reviewed. A cochlear nerve canal diameter of 1.5 mm or less in the axial plane was classified as stenotic. Semicircular canals and other bony labyrinth morphology and abnormality were evaluated., Results: Cochlear nerve canal stenosis was detected in 65 out of 265 ears (24 per cent). Of the 65 ears, 17 ears had abnormal semicircular canals (26 per cent). Significant correlation was demonstrated between cochlear nerve canal stenosis and semicircular canal abnormalities (p < 0.01). Incomplete partition type II was the most common accompanying abnormality of cochlear nerve canal stenosis (15 out of 65, 23 per cent)., Conclusion: Cochlear nerve canal stenosis is statistically associated with semicircular canal abnormalities. Whenever a cochlear nerve canal stenosis is present in a patient with sensorineural hearing loss, the semicircular canal should be scrutinised for presence of abnormalities.

Objectives: There are 3 subtypes of lateral canal benign paroxysmal positional vertigo. Persistent geotropic positional nystagmus is due to a light cupula, heavy cupula produces apogeotropic positional nystagmus, and canalolithiasis accounts for transient geotropic positional nystagmus. The aims of this study were to determine the recurrence rate and to examine the number of times of recurrence in each of the 3 subtypes., Methods: Subjects were patients with light cupula (n = 47), heavy cupula (n = 48), and canalolithiasis (n = 45). We investigated the number of times of recurrence and determined the subtypes of a recurrent vertigo attack over the period of 5 years., Results: The subtype of a recurrent vertigo attack was not always the same as the subtype at the first visit. The recurrence rate of light cupula group was 72.3%, that of heavy cupula group was 20.8%, and that of canalolithiasis group was 28.9%. Some patients experienced recurrence more than once. The mean value and standard deviation of the number of times of recurrence in light cupula group was 2.5 ± 1.3 times, that in heavy cupula group was 1.5 ± 0.7 times, and that in canalolithiasis group was 1.5 ± 0.7 times., Conclusions: The recurrence rate in patients with light cupula is much higher than that in patients with heavy cupula or canalolithiasis. The number of times of recurrence in patients with light cupula is greater than that in patients with heavy cupula or canalolithiasis., Competing Interests: Declaration of Competing Interest We declare that we have no conflict of interest., (Copyright © 2019 Oto-Rhino-Laryngological Society of Japan Inc. Published by Elsevier B.V. All rights reserved.)

Purpose: Adolescent idiopathic scoliosis (AIS) is a three-dimensional spinal structural deformity that occurs in otherwise normal individuals. Although curve progression and severity vary amongst individuals, AIS can lead to significant cosmetic and functional deformity. AIS etiology has been determined to be genetic, however, exact genetic and biological processes underlying this disorder remain unknown. Vestibular structure and function have potentially been related to the etiopathogenesis of AIS. Here, we aimed to characterize the anatomy of the semicircular canals (SCC) within the vestibular system through a novel approach utilizing T2-weighted magnetic resonance images (MRI)., Methods: Three dimensional, MRI-based models of the SCCs were generated from AIS subjects (n = 20) and healthy control subjects (n = 19). Linear mixed models were used to compare SCC morphological measurements in the two groups. We compared side-to-side differences in the SCC measurements between groups (group*side interaction)., Results: Side-to-side differences in the lateral SCC were different between the two groups [false discovery rate adjusted p-value: 0.0107]. Orientation of right versus left lateral SCC was significantly different in the AIS group compared to the control group [mean side-to-side difference: -4.1°, 95% CI: -6.4° to -1.7°]. Overall, among subjects in the AIS group, the left lateral SCC tended to be oriented in a more horizontal position than subjects in the control group., Significance: Asymmetry within the SCCs of the vestibular system of individuals with AIS potentially results in abnormal efferent activity to postural muscles. Consequences of this muscular activity during periods of rapid growth, which often coincides with AIS onset and progression, warrant consideration., Competing Interests: The authors have declared that no competing interests exist.

Background: Individuals with superior semi-circular canal syndrome often describe vestibular symptoms elicited by loud sounds, as well as other pressure-induced symptoms. They also often report other symptoms, including autophony, hyperacusis, cognitive dysfunction, spatial disorientation, anxiety and migraine headaches. Symptoms occur due to the presence of a 'third window' created by the dehiscence of the superior semi-circular canal. This case report describes a minimally invasive technique to provide soft reinforcement of the round window., Case Report: Our patient underwent a permeatal procedure whereby the tympanic membrane was raised to allow inspection of the middle ear. The round window niche was identified and the round window membrane was reinforced with fat. The mucosa of the bony meatus leading to the round window was then disrupted before the application of a double layer of perichondrium to allow further reinforcement., Conclusion: The case provides support for the use of 'soft reinforcement' as a simple and effective technique to treat the symptoms of superior canal dehiscence syndrome.

Background: Superior semicircular canal dehiscence (SSCD) is a rare inner ear disorder; currently, it is unknown whether the etiopathology underlying this structural irregularity affects neighboring structures. The goal is to investigate the prevalence of bone thinning in areas of the middle cranial fossa (MCF) floor in SSCD and non-SSCD patients., Methods: This retrospective study analyzed 100 patients from March 2011 to June 2017 at a tertiary referral center. 100 patients undergoing 118 SSCD repair surgeries (18 bilateral) were identified. 12 SSCD ears were excluded due to lack of pre-operative computed tomography (CT) scans or history of prior SSCD repair at an outside facility. Non-SSCD ears were identified from routinely-obtained CT scans for temporal bone fracture (fractured sides excluded) for a total of 101 ears; 26 non-SSCD ears were excluded due to lack of high-resolution imaging., Results: Univariate analyses reveal that SSCD diagnosis is associated with higher rates of geniculate ganglion (GG) dehiscence compared with non-SSCD controls (42.7 vs. 24%; χ 2 (1) = 9.69,P = 0.008). Individuals with SSCD depicted significantly thinner bone overlying the geniculate ganglion (GG) (0.23 ± 1.2 mm) compared to controls (0.28 ± 1.8 mm, (t(1 6 4)) = 2.1, P = 0.04). SSCD patients presented thinner bone overlying the internal auditory canal (IAC) (0.33 ± 1.3 mm) compared to patients without SSCD (0.46 ± 1.6 mm, (t(2 5 7) = 6.4, P < 0.001)., Conclusions: The increased prevalence of dehiscence of the MCF in this cohort of SSCD patients compared to non-SSCD patients suggests that the etiology underlying SSCD affects surrounding structures., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

The aim of this study was to evaluate clinical and neurophysiological characteristics of Persistent postural-perceptual dizziness (PPPD) in a tertiary vertigo clinic. This was a cross-sectional study that included consecutive patients examined in the Vertigo clinic of the University Hospital Center Zagreb, Croatia. The following data were extracted from the electronic hospital records: age, sex, the duration of symptoms, initial trigger event, results of the caloric testing, video head impulse test (vHIT) for all six semicircular canals and ocular and cervical vestibular evoked myogenic potentials (oVEMP and cVEMP). During the study period 147 consecutive patients with dizziness were examined and 28 (19%) were diagnosed with PPPD, 68% of them were women and the mean age was 59.5 ± 15 years. The median duration of symptoms was 23 months. The most common initial event was vestibular neuritis in 39.3% of patients, followed by benign paroxysmal positional vertigo in 10.7% of patients. Caloric testing was performed in 25 patients. It revealed six cases of unilateral canal paresis. vHIT was performed in 24 patients. There were 13 pathological responses with three cases of lateral canal dysfunction, two cases of posterior, one case of anterior and seven cases of multiple canals affection. VEMP was performed in 23 patients. There were five isolated oVEMP pathologies, one isolated cVEMP pathology and 11 findings of a combined oVEMP and cVEMP pathology. This study provides clinical and neurophysiological data on PPPD and indicates the utility of complete neurophysiological assessment of vestibular function in this group of patients., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Objective: To investigate the clinical characteristics and therapeutic effect of benign paroxysm positional vertigo（BPPV） secondary to middle ear surgery. Method: A total of 1 126 patients who underwent tympanoplasty or radical mastoidectomy due to chronic suppurative otitis media and middle ear cholesteatoma in our hospital from January 2014 to December 2018 were collected. Clinical data of BPPV within 1 month after surgery were collected, The incidence, incidence side, involved semicircular canal, onset time, age of onset, and duration of operation of secondary BPPV after middle ear surgery were analyzed. All patients with secondary BPPV were treated by manual reduction, and the efficacy was evaluated 1 day, 1 week, and 1 month after reduction. Result: 2.13% （24 cases） of patients had secondary BPPV after operation, among which 2 cases were parietal incidence. 18 cases were involved in posterior semicircular canals and 6 cases were horizontal semicircular canals. The onset time was 1-12 days after the operation, with an average of （3.29±2.44） days. The mean age of onset was （51.62±10.15） years old, and there was no statistically significant difference between the age of patients without BPPV after middle ear surgery （ P >0.05）. The average operating time was （97.29±14.78） minutes, showing no statistically significant difference compared with patients in the group without BPPV （ P >0.05）. Fourteen cases （58.3%） were cured and 10 cases were improved after 1 day evaluation. Evaluated 1 week after treatment, 19 cases （79.17%） were cured and 5 cases were improved. Evaluated 1 month after treatment, all patients were cured without recurrence. Conclusion: BPPV secondary to middle ear surgery often appears 3 days after surgery, and the posterior semicircular canal of the operative ear is most commonly involved. Age and operation duration have no significant influence on the incidence of BPPV, and satisfactory therapeutic effect can be obtained through manipulative reduction., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

The vestibular system in the inner ear senses angular head manoeuvres by endolymph fluid which deforms a gelatinous sensory structure (the cupula). We constructed computer models that include both the endolymph flow (using CFD modelling), the cupula deformation (using FEM modelling), and the interaction between both (using fluid-structure interaction modelling). In the wide utricle, we observe an endolymph vortex. In the initial time steps, both the displacement of the cupula and its restorative forces are still small. As a result, the endolymph vortex causes the cupula to deform asymmetrically in an S-shape. The asymmetric deflection increases the cupula strain near the crista and, as a result, enhances the sensitivity of the vestibular system. Throughout the head manoeuvre, the maximal cupula strain is located at the centre of the crista. The hair cells at the crista centre supply irregularly spiking afferents, which are more sensitive than the afferents from the periphery. Hence, the location of the maximal strain at the crista may also increase the sensitivity of the semicircular canal, but this remains to be tested. The cupula overshoots its relaxed position in a simulation of the Dix-Hallpike head manoeuvre (3 s in total). A much faster head manoeuvre of 0.222 s showed to be too short to cause substantial cupula overshoot, because the cupula time scale of both models (estimated to be 3.3 s) is an order of magnitude larger than the duration of this manoeuvre.

We encountered an extremely rare case where a patient with cat eye syndrome (CES) who presented with symptoms of posterior semicircular canal dehiscence (PSCD). CES is a rare genetic disorder, resulting from duplication of chromosome 22. Patients may present with variable phenotypes, including characteristic of coloboma, heart defect, periauricular skin pit/tag, microtia, anal atresia and mildly retarded mental development in some cases. PSCD is also a disease of the inner ear, where patients present with third window signs and symptoms due to lack of bony coverage. PSCD is usually associated with a high riding jugular bulb and fibrous dysplasia. In this study, we report a new otologic finding in CES patient as an association of PSCD and high jugular pulp. We describe the work up and its findings and the management of this patient. SIMILAR CASES PUBLISHED:: None.

Objective: To analyze and discuss the parameters and clinical significance of nystagmus in patients with benign positional paroxysmal vertigo (BPPV) of posterior semicircular canal. Methods: The subjects of the study were 564 BPPV patients diagnosed with posterior semicircular canal canalithis (PSC-can) from January 2016 to July 2017 in Tianjin No.1 Central Hospital, including 186 males and 378 females, with a median age of 57 years. The induced nystagmus in Dix-Hallpike test was recorded by video nystagmuo graph(VNG), and the direction, latency, duration time and intensity characteristics of nystagmus were compared with the position of hanging and sitting.SPSS17.0 software was used for statistical analysis. Results: Vertical torsional nystagmus was both induced with the position of hanging and sitting during Dix-Hallpike test. The vertical direction of the induced nystagmus was upward and downward respectively. The latency, duration time and intensity of lesion side were L(2.65±1.92; 1.44±1.24), D(14.90±10.46; 15.28±8.06), and P(29.75±21.26; 14.08±9.48). The latency and intensity in hanging position were higher than those of sitting. The intensity rate was about 2∶1, with statistically significant difference ( t= 13.831, and 17.296, P all<0.01). The direction of the induced nystagmus was opposite to turning in HSC-Can BPPV. The intensity turning to normal side was larger than lesion side obviously. The intensity rate was about 2∶1, the difference was statistically significant ( t= 17.296, P< 0.01). There was no statistical difference of nystagmus during time between the two positions( t= -0.735, P> 0.05). Conclusions: The nystagmus intensity rate in Dix-Hallpike test between hanging and sitting position of lesion side in PSC-Can is 2∶1, which conforms to the Ewald's law. The direction, latency and intensity of nystagmus can be used as a reference index for the localization diagnosis of PSC-Canotolith.

Previous studies have looked at differences in predisposing factors, symptomology, treatment options, and outcomes in patients with SSCD experiencing audiologic and vestibular symptoms, however this study utilizes data from the largest series of SSCD patients by a single pair of neurosurgeon and head and neck surgeon to date. The objective was to determine what pre-operative factors, if any, contribute to post-operative outcomes in SSCD patients. A retrospective chart review collected patient demographics, preoperative symptoms, and postoperative symptoms. Nonparametric tests were run using IBM® SPSS® Statistics. Fisher's Exact Tests, Spearman's rho, and McNemar's test for paired comparison of binary measures were performed, with a significance level of P < 0.05. A total of 156 SSCD surgeries were performed within a cohort of 119 patients. The majority of patients were female (n = 75, 63.0%). The median age was 55 years (±12.7 years) and median follow-up length was 0.46 months (range: 0.03-59.5 months). Increased postoperative dizziness and hearing loss was significantly correlated with females (P = .048, P = .041). Additionally, males reported significantly improved postoperative hearing (P = .044) with confirmatory audiometry. Serum ionized calcium levels inversely correlated with age using spearman's coefficient (r s  = -.260 P = .037). Postsurgical autophony was significantly associated with bilateral SSCD (P = .01). In conclusion, differences in outcomes between patients may have to do with bilateralism of SSCD or gender effects. Proposed theories concerning a "two hit" hypothesis and about calcium feedback regulation in SSCD may play a role in these findings. Understanding differences between symptomology will help facilitate discussions with future patients., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Objectives: Positional nystagmus can be related to various kinds of disorders. The current study aims to compare the direction-changing horizontal positional nystagmus (DCPN) characteristics in horizontal canal canalolithiasis (HC-canalolithiasis), heavy cupula of the horizontal canal (HC-Hcu), and light cupula of the horizontal canal (HC-Lcu), especially the temporal patterns of positional nystagmus in three disorders., Methods: 52 patients (22 males, 30 females; mean age, 49.6 years) presenting with geotropic or apogeotropic DCPN were enrolled, and they were divided into HC-canalolithiasis, HC-Hcu, or HC-Lcu groups according their nystagmus characteristics. We compared their latency, time constant, peak slow-phase velocity (SPV), time to reach peak SPV intensity (T peak ), and time to decay to half-peak intensity (T 1/2peak )., Results: The time to reach peak SPV did not differ significantly between the HC-Hcu (23.1 ± 8.6 s) and HC-Lcu (24.4 ± 9.9 s) groups (p = 0.733), but was significantly longer than that of the HC-canalolithiasis group (5.4 ± 3.5 s; p ≤ 0.001). The peak intensity did not differ among the canalolithiasis (36.4 ± 20.6º/s), HC-Hcu (30.1 ± 23.6º/s), and HC-Lcu (21.4 ± 12.7º/s) groups (p = 0.133). The onset latency also had no statistical difference among three groups (p = 0.200). The nystagmus patterns of HC-Lcu and HC-Hcu groups were similar, including latency, peak SPV intensity, T peak , T 1/2peak , and SPV in 20 s, 40 s, 60 s, 80 s., Conclusions: The nystagmus characteristics of HC-Hcu and HC-Lcu are similar, except for the fact that movement was in opposite directions, suggesting that HC-Hcu and HC-Lcu may result from a similar pathophysiological mechanism (cupulopathy) differing from that underlying canalolithiasis.

Third window defects have increasingly been identified as a cause of vertigo. These defects are bony dehiscences that occur in the bony labyrinth, resulting in abnormal pressure gradient in the inner ear fluids leading to sound (Tullio's phenomenon) or pressure (Hennebert's sign) induced vertigo. The superior semicircular canal dehiscence syndrome is a well-described entity in this regard, however defects of the posterior semicircular canal are rare and may have overlapping symptomatology. We describe the history, clinical profile and management of a patient who had importunate symptoms despite being on conservative management for a year and had resolution of vestibular symptoms following surgical management., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To compare outcomes for patients undergoing a transmastoid approach versus a middle fossa craniotomy approach with plugging and/or resurfacing for repair of superior semicircular canal dehiscence. Outcome measures include symptom resolution, hearing, operative time, hospital stay, complications, and revision rates., Study Design: Multicenter retrospective comparative cohort study., Settings: Three tertiary neurotology centers., Subjects and Methods: All adult patients undergoing repair for superior canal dehiscence between 2006 and 2017 at 3 neurotology centers were included. Demographics and otologic history collected by chart review. Imaging, audiometric data, and vestibular evoked myogenic potential measurements were also collected for analysis., Results: A total of 68 patients (74 ears) were included in the study. Twenty-one patients underwent middle fossa craniotomy repair (mean age, 47.9 years), and 47 underwent transmastoid repair (mean age, 48.0 years). There were no significant differences in age or sex distribution between the groups. The transmastoid group experienced a significantly shorter duration of hospitalization and lower recurrence rate as compared with the middle fossa craniotomy group (3.8% vs 33%). Both groups experienced improvement in noise-induced vertigo, autophony, pulsatile tinnitus, and nonspecific vertigo. There was no significant difference among symptom resolution between groups. Additionally, there was no significant difference in audiometric outcomes between the groups., Conclusion: Both the transmastoid approach and the middle fossa craniotomy approach for repair of superior canal dehiscence offer symptom resolution with minimal risk. The transmastoid approach was associated with shorter hospital stays and lower recurrence rate as compared with the middle fossa craniotomy approach.

Objective: To investigate the diagnosis strategy of anterior canal-benign paroxysmal positional vertigo (AC-BPPV) and the therapeutic effects of the Yacovino maneuver., Methods: The clinical data of 40 AC-BPPV patients were collected. The nystagmus characteristics induced by the Dix-Hallpike (D-H) and straight head-hanging (SHH) tests, the diagnostic methods used, and the effectiveness of the Yacovino maneuver for the treatment of AC-BPPV were all retrospectively analyzed., Results: Among the 40 cases analyzed, 19 patients had simple AC-BPPV, 11 patients had AC-posterior canal BPPV, and 10 patients had AC-horizontal canal BPPV. D-H and SHH tests showed down-beating nystagmus in 26 and 33 patients, respectively, and showed down-beating and torsional nystagmus in 14 and 7 patients, respectively. AC-BPPV was diagnosed in 15 patients based on the presence of typical BPPV in other canals, in 9 patients based on typical disease history and the results of position tests, in 6 patients based on effectiveness of the treatment with the Yacovino maneuver, in 4 patients based on the treatment effectiveness and the presence of typical BPPV in other canals, in 3 patients based on the treatment effectiveness and the follow-up outcome, in 2 patients based on the typical BPPV in other canals and occurrence of canal conversion, and in 1 patient based on the treatment effectiveness and occurrence of canal conversion. Thirteen patients with canalolithiasis and four patients with cupulolithiasis were cured after the initial Yacovino maneuver treatment. Twenty-one patients with canalolithiasis and seven patients with cupulolithiasis were cured following 1 week of treatment., Conclusions: The effectiveness of the Yacovino maneuver, the follow-up outcome, the presence of typical BPPV in other canals, and the occurrence of canal conversions contribute to AC-BPPV diagnosis. The Yacovino maneuver was found to be more effective in AC-BPPV patients with canalolithiasis than in those with cupulolithiasis.

Background: Superior semicircular canal dehiscence syndrome (SSCD) is a current diagnosis that is due to a loss of bone covering the superior semicircular canal (SSC). This results in pressure-/sound- induced vertigo and oscillopsia., Objective: To find the variation of the thickness of the bone that covers the Superior Semicircular Canal with relation to age and gender among the Chinese descents., Materials and Methods: Three hundred and eleven temporal bone Cone Beam Computed Tomography (CBCT) images of patients who attended Otology clinic at Second Hospital of Shandong University from January, 2017 to April, 2018 were retrospectively studied. The images were reconstructed in the line of Poschl and the thinnest area of the bone covering the SSC was taken., Results: We included 172 (55.31%) females and 139 (44.69%) males. Mean age was 41 years. Overall mean difference in thickness was found to be -0.0210. There was no significant difference between the female and male bone thickness (p = .7113). With age the mean difference was 0.0801 (p = .1557) which was not statistically significant., Conclusion and Significance: There was no significant change in bone thickness with advancing age. CBCT is the best method of assessing SSCD.