Nystagmus in children with primary brain tumours in Denmark between 2007 and 2017.

BACKGROUND: The aim of the study was to evaluate the prevalence, clinical characteristics, and diagnostic importance of nystagmus in children with brain tumours. METHODS: A nation-wide retrospective review of all children diagnosed with a brain tumour between January the 1st, 2007 and December 31st, 2017, in Denmark. Data is based on information from the Danish Childhood Cancer Registry, hospital records from paediatric- and ophthalmological departments, and records from private ophthalmologists. RESULTS: Nystagmus was observed in 13.7% (60/437) of children with a brain tumour. In 50/60 children (83.3%) nystagmus was an incidental finding at the clinical examination and only in 10/60 children (16,7%) were nystagmus noticed by patient/caregivers prior to the clinical examination. In 38/60 children nystagmus was observed before the brain tumour diagnosis, most often (16/38, 42%) the same day as the diagnosis was made. In 22/60 children nystagmus was found after the brain tumour diagnosis (prior to any treatment) with a median of four days (range 0-47) after the brain tumour diagnosis. Nystagmus was most commonly binocular (56/60, 93.3%) and gaze-evoked (43/60, 71.7%). The median number of additional symptoms and/or clinical findings was five (range 0-11). CONCLUSION: Nystagmus is frequent in children with brain tumours and is typically accompanied by other symptoms and clinical signs. However, nystagmus is often first recognized by the ophthalmologist late in the time course. Therefore, raising awareness of the importance of looking for nystagmus in children with unspecific neurological symptoms might contribute to increased suspicion of brain tumour and thereby faster diagnosis.

Do monosymptomatic stroke patients with dizziness present a vestibular syndrome without nystagmus? An underestimated entity.

BACKGROUND AND PURPOSE: Vestibular symptoms are common in emergency department (ED) patients and have various causes, including stroke. Accurate identification of stroke in patients with vestibular symptoms is crucial for timely management. We conducted a prospective cross-sectional study from 2015 to 2019 to determine stroke prevalence and associated symptoms in ED patients with vestibular symptoms, aiming to improve diagnosis and outcomes. METHODS: As part of the DETECT project, we screened 1647 ED patients with acute vestibular symptoms. Following a retrospective analysis of 961 head and neck magnetic resonance imaging (MRI) scans, we included 122 confirmed stroke cases and assessed them for vestibular signs and symptoms. RESULTS: Stroke prevalence in dizzy patients was 13% (122/961 MRI scans). Most patients (95%) presented with acute vestibular symptoms with or without nystagmus, whereas 5% had episodic vestibular syndrome (EVS). Nystagmus was present in 50% of stroke patients. Eighty percent had a purely posterior circulation stroke, and nystagmus was absent in 46% of these patients. Seven patients (6%) had lesions in both the anterior and posterior circulation. Vertigo was experienced by 52% regardless of territory. CONCLUSIONS: A stroke was identified in 13% of ED patients presenting with acute vestibular symptoms. In 5%, it was EVS. Most strokes were in the posterior circulation territory; vertigo occurred with similar frequency in anterior and posterior circulation stroke, and absence of nystagmus was common in both.

Incidence, characteristics, and neuroanatomical substrates of vestibular symptoms in supratentorial stroke.

The incidence and characteristics of acute vestibular symptoms, responsible structures, and lateralization of the causative lesions in supratentorial stroke remain unknown. This study aimed to determine the incidence, clinical features, and anatomical correlation of acute vestibular symptoms in supratentorial stroke. We performed a prospective, multicenter, observational study that had recruited patients with supratentorial stroke from the neurology clinics of referral-based four university hospitals in Korea. All patients received a constructed neuro-otological evaluations, and neuroimaging. We analyzed the incidence of acute vestibular symptoms, abnormal ocular motor and vestibular function tests, and stroke lesions. Of 1301 patients with supratentorial stroke, 48 (3.7%) presented with acute vestibular symptoms, and 13 of them (1%) had the vestibular symptoms in isolation. In patients with acute vestibular symptoms, abnormal findings included spontaneous nystagmus (5%), impaired horizontal smooth pursuit (41%), and abnormal tilt of the subjective visual vertical (SVV) (20%). Video head impulse and caloric tests were normal in all the patients. There was no clear correlation between acute vestibular symptoms and involvement of specific vestibular cortex. In patients with unilateral stroke, there was also no lateralization of the causative lesions of acute vestibular symptoms (left vs. right; 52 vs. 48%), even in patients with vertigo (left vs. right; 58 vs. 42%). This study demonstrates that the incidence of acute vestibular symptoms in supratentorial stroke is 3.7%, with being isolated in 1%. The widespread lesions responsible for acute vestibular symptoms implicate diffuse multisensory cortical-subcortical networks in the cerebral hemispheres without a lateralization.

Demographic and Clinical Characteristics of 600 Children With Nystagmus.

PURPOSE: To characterize a large, international cohort of children with nystagmus. METHODS: Data were collected from a single-center, prospectively developed database on patients with nystagmus. Outcome variables for this study included: (1) demographic characteristics, (2) nystagmus type, (3) clinical characteristics, (4) associated ophthalmic conditions, (5) associated non-ophthalmic conditions, (6) special testing findings, and (7) treatments. RESULTS: Six hundred patients from 38 states and 30 countries were included. Age ranged from birth to younger than 18 years (mean: 15.2 years), 58% were female, 55% were race other than White, 75% had infantile nystagmus syndrome, 17% had neurologically significant nystagmus, 7% had fusion maldevelopment nystagmus syndrome, 64% had strabismus, 56% had an anomalous head posture, 94% had a significant refractive error, 64% had an associated ophthalmic abnormality (excluding ametropia), and 45% had an associated systemic condition. Special testing showed abnormalities in 67% and 95% had treatment directed at their nystagmus. CONCLUSIONS: The most common diagnosis in this cohort is neurologically non-significant infantile nystagmus syndrome. Eye movement recordings provide a path toward accurate diagnosis and classification. There was a high prevalence of underlying ocular and/or systemic conditions requiring ophthalmic special testing as part of a diagnostic routine. Clinical treatments are available to most patients. [J Pediatr Ophthalmol Strabismus. 2023;60(6):406-410.].

Strabismus and Nystagmus in Patients With Pediatric Cataracts: Study Using Insurance Claims Data.

PURPOSE: To describe the characteristics and prevalence of strabismus and nystagmus in children diagnosed with cataracts using a national insurance claims database. DESIGN: Population-based retrospective cohort study. METHODS: Patients aged <13 years diagnosed with cataracts (traumatic cataracts excluded) and enrolled continuously in their health care program for ≥5 years after their first cataract diagnosis were identified in a retrospective review of 66 million charts in Optum's de-identified Clinformatics Data Mart Database between 2003 and 2015. Patients were categorized based on age of their first diagnosed cataract, and if cataract surgery was performed. Clinical and demographic factors associated with the occurrence of strabismus and nystagmus were evaluated. RESULTS: Of 1636 children diagnosed with cataract, 434 (26.5%) and 109 (6.7%) were diagnosed with strabismus and nystagmus, respectively. Both strabismus and nystagmus were more common in those who underwent cataract surgery (P < .001) and in patients diagnosed with cataract at ≤12 months of age (P < .001). Survival analysis demonstrated that strabismus and nystagmus may be diagnosed 8 years after the initial cataract diagnosis. Cox proportional hazard regression analyses revealed strabismus was associated with cataract surgery, nystagmus, and the diagnosis with cataract at ≤12 months and cataract surgery at >12 months. CONCLUSIONS: As strabismus and nystagmus occur more frequently in children diagnosed with cataracts necessitating cataract surgery, regular long-term follow-up is crucial for these children to monitor for the development of strabismus and nystagmus.

Systemic and Ophthalmic Manifestations in Different Types of Refractive Errors in Patients with Down Syndrome.

Background and Objective: This study aims to investigate the prevalence of systemic and ophthalmic manifestations in different refractive groups in children and young adults with Down syndrome (DS). Materials and Methods: The study was a population-based, cross-sectional study that included 141 Caucasian children and young adults with DS. They were classified into the following three groups: myopia DS group (37 subjects, mean age 15.8 years), emmetropia DS group (41 subjects, mean age 11.7 years) and hyperopia DS group (63 subjects, mean age 10.9 years). The participants underwent inspection, slit-lamp examination, cycloplegic refraction, ocular alignment and ocular motility examination. Ten systemic manifestations were analyzed. Results: There was no difference in the prevalence of any systemic manifestations between the groups. Considering the ophthalmic manifestations, there was statistical difference in the distribution of proportions among the three groups for nystagmus (p = 0.011), iris-stromal atrophy (p = 0.048) and strabismus (p = 0.031). The prevalence of strabismus in our DS myopia group was 35.1%, and in DS hyperopia group 38.1%. Conclusions: The results of our study suggest that DS children and young adults with any refractive error do not have a higher chance of additional systemic manifestations. Myopia in DS was associated with a higher prevalence of nystagmus and iris stromal atrophy, whereas astigmatism was found to be more frequent in hyperopia.

Impact of the COVID-19 pandemic on individuals with nystagmus and an exploration of public assumptions about the condition: an electronic questionnaire study.

PURPOSE: Nystagmus is a disorder characterized by uncontrolled, rhythmic oscillations of the eyes. It often causes reduced visual function beyond reduced visual acuity alone. There is a paucity of literature regarding the public understanding of nystagmus, and there are no published data on the impact of the COVID-19 pandemic on people living with the condition. This study explores the self-reported impact of the COVID-19 pandemic on those with nystagmus, and examines both public understanding of how nystagmus affects people who have it and the perceptions of public understanding by those with the condition and their carers. METHODS: A qualitative questionnaire was designed following a stakeholder engagement process. This questionnaire was advertised via social media platforms and charity websites to achieve widespread recruitment. Data were collected between November and December 2020. Participants were divided into two groups based on their response to the question: "Do you, or anyone you know well, have nystagmus?". Questions were posed to participants in a purpose-built, branching survey. The resulting data were analyzed using descriptive and inferential statistical methods. RESULTS: One thousand six hundred forty-five respondents were recruited, of which 849 (51.6%) answered "Yes" to the initial filtering question. Analysis showed that, broadly, public understanding of nystagmus differs from the perception of it by those with nystagmus and their carers, that the COVID-19 pandemic has had a significant impact on those with nystagmus, and that respondents who have met someone with nystagmus, even briefly, tend to have a greater understanding of the impact of the condition. CONCLUSION: This study highlights the lack of public awareness regarding nystagmus and suggests opportunities to increase the awareness of nystagmus without the need for extensive knowledge of the condition. The COVID-19 pandemic has posed additional difficulties for those living with nystagmus, which is likely to be comparable among those with similar ocular disorders.

Prevalence of "Reversal Nystagmus" in Benign Paroxysmal Positional Vertigo.

PURPOSE: To investigate the prevalence of reversal nystagmus in individuals with benign paroxysmal positional vertigo (BPPV). STUDY DESIGN: Prevalence of reversal nystagmus was assessed in 28 subjects with unilateral posterior canal BPPV, canalithiasis type. Six trials of Dix-Hallpike testing were completed for each subject. RESULTS: Reversal nystagmus was present in 129 out of 167 Dix-Hallpike maneuvers that were performed (77.2%). In 19 trials where nystagmus was absent with the dependent position of Dix-Hallpike testing, reversal nystagmus was nonetheless demonstrated in 11 trials (57.9%). CONCLUSION: Reversal nystagmus is commonly demonstrated in individuals with posterior canal BPPV, canalithiasis type. It is frequently evoked even when there is no nystagmus with the dependent position of Dix-Hallpike testing. Observation of reversal nystagmus may enhance the identification of BPPV during Dix-Hallpike testing.

Central nystagmus in progressive supranuclear palsy: A neglected clinical feature?

BACKGROUND: Progressive supranuclear palsy (PSP) features parkinsonism characterized by early postural instability, falls and prominent eye movement abnormalities that consist of saccadic slowing, followed by gaze limitation. Nystagmus is not considered typical for PSP, being more commonly associated with multiple system atrophy. OBJECTIVES: To describe the prevalence and phenomenology of nystagmus in patients with PSP. METHODS: 42 patients with probable PSP underwent detailed clinical eye movement examination. Patients with nystagmus performed video-nystagmography. T-test, Chi-Square test and Wilcoxon signed-rank test were used to test differences in demographic data, disease duration and PSP subtype between patients with and without nystagmus, and for analysis of video-nystagmographic data. RESULTS: Among 42 patients with PSP, we identified 15 patients (35,7%) with gaze-evoked nystagmus, predominantly horizontal. Clinically, 10/15 patients had symmetrical or asymmetrical gaze - evoked nystagmus (Type 1), while 5/15 patients had dissociated gaze-evoked nystagmus related to internuclear ophthalmoplegia (Type 2). Nystagmus and eye movement abnormalities were further characterized by video-nystagmography. There was no significant difference in age, disease duration or PSP subtypes between patients with and without nystagmus. CONCLUSION: Central nystagmus is present in more than a third of patients with progressive supranuclear palsy. It may present as symmetrical or asymmetrical gaze-evoked nystagmus or as dissociated gaze-evoked nystagmus related to internuclear ophthalmoplegia and probably arises from neurodegeneration of the neural integrator. Nystagmus in PSP has been a hitherto under-described feature and its presence should not deter clinicians from a diagnosis of PSP.

Clinical spectrum of non-syndromic microphthalmos, anophthalmos and coloboma in the paediatric population: a multicentric study from North India.

AIMS: To describe the clinical features, visual acuity and causes of ocular morbidity in children (0-18 years) with microphthalmos, anophthalmos, and coloboma (MAC) from North India. METHODS: A retrospective study conducted between October 2017 and September 2018 in three tertiary eye institutes, part of the Bodhya Eye Consortium with consensus led common pro formas. Children with complete clinical data and without syndromic/systemic involvement were included. The clinical phenotype was divided into isolated ocular coloboma (CB), coloboma with microcornea (CBMC), colobomatous microphthalmos (CBMO), non-colobomatous microphthalmos (MO) and anophthalmos (AO). RESULTS: A total of 532 children with MAC were examined. Seventeen records were excluded due to incomplete data (0.2%). 515 children (845 eyes) were included: 54.4% males and 45.6% females. MAC was unilateral in 36% and bilateral in 64%. CB, CBMC, CBMO, MO and AO were seen in 26.4%, 31%, 22%, 8% and 12.5% of eyes, respectively. Nystagmus was found in 40%, strabismus in 23%, cataract in 18.7% and retinal detachment in 15%. Best-corrected visual acuity (BCVA) of <3/60 was seen in 62.4% eyes. Blindness (BCVA <3/60 in better eye) was seen in 42.8% of bilateral patients. Those with microcornea or microphthalmos with coloboma had worse BCVA (p<0.001). There were regional differences in the type of MAC phenotype presenting to the three institutes. CONCLUSION: The MAC group of disorders cause significant ocular morbidity. The presence of microcornea or microphthalmos with coloboma predicts worse BCVA. The variation of the MAC phenotype with the district of origin of the patient raises questions of aetiology and is subject to further studies.

Nystagmus in Ramsay Hunt syndrome with or without dizziness.

BACKGROUND: The presence of dizziness has been reported as a negative prognostic factor for recovery of facial palsy in Ramsay Hunt syndrome (RHS). The aim of this study was to investigate the incidence and patterns of nystagmus in RHS patients without dizziness, and discuss possible mechanisms. We also compared the severity and prognosis of facial palsy between RHS patients with and without dizziness. METHODS: From January 2014 to January 2019, 36 patients diagnosed with RHS (27 with dizziness and 9 without dizziness) were included. Patterns of nystagmus were examined and categorized using video-nystagmography. House-Brackmann(HB) grade of facial palsy was compared between RHS patients with and without dizziness. RESULTS: Not only RHS patients with dizziness exhibited nystagmus in most cases (96%, 26 of 27) but also as many as 67% (6 of 9) of RHS patients without dizziness exhibited nystagmus, though the intensity was remarkably weak. In both groups of RHS with and without dizziness, direction-fixed nystagmus and direction-changing positional nystagmus were observed. Initial HB grade and recovery of facial palsy after treatment were not significantly different between RHS with and without dizziness. CONCLUSION: Various patterns of nystagmus including direction-fixed and positional direction-changing nystagmus were observed in RHS patients, and inflammation of the vestibular nerve and inner ear end organs may be responsible for the production of nystagmus in these patients. The results support that the evaluation of vestibular function may be necessary even in RHS patients who do not complain of dizziness or vertigo.

Oculocerebrorenal syndrome of Lowe: Survey of ophthalmic presentations and management.

BACKGROUND: Lowe syndrome is a rare X-linked disease that is characterized by renal dysfunction, developmental delays, congenital cataracts and glaucoma. Mutations in the oculocerebral renal syndrome of Lowe (OCRL) gene are found in Lowe syndrome patients. Although loss of vision is a major concern for families and physicians who take care of Lowe syndrome children, definitive cause of visual loss is still unclear. Children usually present with bilateral dense cataracts at birth and glaucoma, which occurs in more than half of cases, either concurrently or following cataract surgery. MATERIALS AND METHODS: A retrospective review was conducted on the prevalence and characteristics of ocular findings among families of patients with Lowe syndrome with 137 uniquely affected individuals. RESULTS: Of 137 patients, all had bilateral congenital cataracts. Nystagmus was reported in 69.3% of cases, glaucoma in 54.7%, strabismus in 35.0%, and corneal scar in 18.2% of patients. Glaucoma was reported as the most common cause of blindness (46%) followed by corneal scars (41%). Glaucoma occurred in 54.7% of patients and affected both eyes in the majority of cases. Of these patients, 55% underwent surgery for glaucoma, while the remaining patients used medications to control their eye pressure. Timolol and latanoprost were the most commonly used medications. Although trabeculectomy and goniotomy are commonly used for pressure management, aqueous tube shunts had the best outcomes. CONCLUSION: Ocular manifestations in individuals with Lowe syndrome and carriers with OCRL mutation are reported which may help familiarize clinicians with the ocular manifestations and management of a rare and complex syndrome.

The association of head shaking nystagmus with head-bending and lying-down nystagmus in horizontal canal benign paroxysmal positional vertigo.

BACKGROUND: In benign paroxysmal positional vertigo (BPPV), the otolithic debris may alter the dynamics of the endolymph or cupula during head-shaking. This dynamic may generate head-shaking nystagmus (HSN) but exact pathomechanism of HSN in BPPV has not been elucidated. The association of positional nystagmus induced by head-bending or lying-down with HSN may help to understand the dynamics of HSN. OBJECTIVE: To assess the presence, pattern, and relationship with head-bending nystagmus (HBN) and lying-down nystagmus (LDN) of HSN in horizontal canal (HC)-BPPV. METHODS: We recruited 173 patients with HC-BPPV (76 geotropic and 97 apogeotropic). We analyzed the pattern of HSN, and correlation with HBN and LDN. RESULTS: Half of patients (83/173, 48%) with HC-BPPV showed HSN. The directional preponderance of HSN was also not found in patients with geotropic or apogeotropic HC BPPV (p = 0.488). The presence of HSN was related with the occurrence of HBN in both geotropic (p = 0.005) and apogeotropic type (p = 0.001). The direction of HSN was same with HBN and was opposite to LDN in both geotropic and apogeotropic type. CONCLUSIONS: HSN was frequently found in patients with HC-BPPV and related with HBN and LDN. HSN in BPPV might be contributed by the otolith movements related with endolymph dynamics.

A Variation in FGF14 Is Associated with Downbeat Nystagmus in a Genome-Wide Association Study.

Downbeat nystagmus (DBN) is a frequent form of acquired persisting central fixation nystagmus, often associated with other cerebellar ocular signs, such as saccadic smooth pursuit or gaze-holding deficits. Despite its distinct clinical features, the underlying etiology of DBN often remains unclear. Therefore, a genome-wide association study (GWAS) was conducted in 106 patients and 2609 healthy controls of European ancestry to identify genetic variants associated with DBN. A genome-wide significant association (p < 5 × 10-8) with DBN was found for a variation on chromosome 13 located within the fibroblast growth factor 14 gene (FGF14). FGF14 is expressed in Purkinje cells (PCs) and a reduction leads to a decreased spontaneous firing rate and excitability of PCs, compatible with the pathophysiology of DBN. In addition, mutations in the FGF14 gene cause spinocerebellar ataxia type 27. Suggestive associations (p < 1 × 10-05) could be detected for 15 additional LD-independent loci, one of which is also located in the FGF14 gene. An association of a region containing the dihydrofolate reductase (DHFR) and MutS Homolog 3 (MSH3) genes on chromosome 5 was slightly below the genome-wide significance threshold. DHFR is relevant for neuronal regulation, and a dysfunction is known to induce cerebellar damage. Among the remaining twelve suggestive associations, four genes (MAST4, TPPP, FTMT, and IDS) seem to be involved in cerebral pathological processes. Thus, this GWAS analysis has identified a potential genetic contribution to idiopathic DBN, including suggestive associations to several genes involved in postulated pathological mechanisms of DBN (i.e., impaired function of cerebellar PCs).

Unusual Locations of Benign Paroxysmal Positional Vertigo: Rare Entity or Regular Occurrence?

INTRODUCTION: According to the literature and our recent experience, even if patients present with symptoms strongly suggestive of benign paroxysmal positional vertigo (BPPV), the observed positional nystagmus does not always correspond to any previously described typical location. The aim of this multicentric study was to evaluate the frequency of both typical and less common forms of BPPV. MATERIAL AND METHODS: All consecutive patients presenting with BPPV in two hospitals between November 2016 and October 2017 were included. For each subject, answers to a standardized evaluation form and a recorded videonystagmoscopy were obtained by two otorhinolaryngologists. Appropriate diagnostic and therapeutic maneuvers were performed. RESULTS: A total of 532 patients were studied and 491 cases of typical BPPV were found: 370 cases of canalolithiasis of the posterior semicircular canal (SCC), 3 cases of canalolithiasis of the anterior SCC, 107 cases of canalolithiasis of the lateral SCC, and 11 cases of cupulolithiasis of the lateral SCC. Fourty one patients reported symptoms suggestive of BPPV with positional nystagmus that could correspond to unusual locations of otoconia in the SCC: 18 cases of canalolithiasis in the anterior ampulla of the lateral SCC, 16 cases of posterior short arm canalolithiasis, four cases of pseudo-spontaneous nystagmus in canalolithiasis of the lateral SCC, and three cases of canalolithiasis of the posterior third of SCC. DISCUSSION: Unusual BPPV accounted for almost 8% of BPPV treated in our clinics; it is paramount to know how to diagnose them, to carry out the appropriate therapeutic maneuvers and relieve these patients.

Evaluating the impact of information and support for people with nystagmus in the digital age: A patient and carer questionnaire study.

Background: Nystagmus is a disorder of rhythmic, involuntary oscillations of the eyes and can be classified as either infantile or acquired. Whether it occurs in isolation or as a part of other visual or neurological disorders, it causes significant visual dysfunction and problems with social functioning. In this study, we seek to understand ways in which people with nystagmus are currently supported across the UK and identify any geographical variations or disconnects between current practice and best practice, as judged by patients and their carers.Methods: A nationwide, qualitative, cross-sectional, questionnaire study of people with nystagmus and their carers. Recruitment was achieved through specialist clinics, charity events, online advertisements and social media calls. Data was gathered using five, age-appropriate questionnaires which were completed and returned to the research team between November 2016 and August 2018.Results: 184 respondents were included (89 carers, 47 patients aged 4-10 years, 5 aged 11-14 years, 4 aged 15-17 years and 39 > 18 years). Notably, respondents rated social media as the best source of information they have received, even compared with face-to-face consultation with medical professionals. Additionally, only 33% of the respondents had been offered visual impairment support. Notably, patterns of clinical practice and patient experience emerged according to geographical location, particularly provision of initial information and ongoing VI support.Conclusions: This study highlights a significant variation in the support and information received by people in the UK with nystagmus. It also supports the role of charities and increasingly, social media in the provision of patient information. The study also highlights the need for standardized guidelines for the management of patients with nystagmus, particularly with regard to support and information.

Comorbid Cupulolithiasis Is Rare in Patients With Horizontal Semicircular Canal Canalolithiasis.

OBJECTIVES: Considering that otolith particles pass through the canal until attaching to the cupula in the canal-side horizontal semicircular canal (HSCC) cupulolithiasis, comorbidity of HSCC canalolithiasis and cupulolithiasis may occur. We aimed to investigate the incidence of comorbidity of cupulolithiasis in patients with HSCC canalolithiasis and to improve treatment efficacy. STUDY DESIGN: Retrospective study. SETTING: Tertiary referral academic center. PATIENTS: Ninety-seven consecutive patients with HSCC canalolithiasis between March 2017 and March 2019 were included. MAIN OUTCOME MEASURES: Coexistence of HSCC cupulolithiasis was hierarchically investigated. 1) Spontaneous reversal of initial nystagmus is observed bilaterally in a head-roll test (HRT), 2) nystagmus is in the same direction at each of the three times the supine position was tested, and 3) both bowing and leaning nystagmus with opposite direction are observed. RESULTS: Of 97 patients with HSCC canalolithiasis, 2 patients (2%) had comorbid HSCC cupulolithiasis. CONCLUSIONS: Although coexistence of HSCC canalolithiasis and cupulolithiasis should be considered when spontaneous reversal of nystagmus direction is observed without position change during a HRT, the incidence of coexistence is very low. However, canalith repositioning maneuvers for both canalolithiasis and cupulolithiasis should be performed in cases with comorbidity.

Acute vestibular asymmetry disorder: a new disease entity in acute vestibular syndrome?

BACKGROUND: Acute vestibular syndrome (AVS) is characterized by the rapid onset of vertigo, nausea, vomiting and gait unsteadiness, which lasts for days. AIMS/OBJECTIVES: We report cases as acute vestibular asymmetry disorder (AVAD), with presentations that mimic vestibular neuritis (VN) but without central lesions. MATERIALS AND METHODS: We retrospectively reviewed records of patients presenting with acute spontaneous vertigo lasting more than 24 h from January 2011 to June 2016. Among 341 patients, five showed different findings that did not indicate either VN or stroke. We analyzed the clinical features and vestibular assessments of these patients. RESULTS: All five patients showed spontaneous nystagmus continuing for several days. However, head impulse tests (HITs) did not reveal a corrective saccade. Brain magnetic resonance imaging showed no abnormal lesions. The bithermal caloric test revealed directional preponderance without canal paresis. Finally, the slow harmonic test of the rotatory chair revealed unilateral high gain and phase within the normal range, but a significantly asymmetric response was observed. No patients showed recurrence during follow-up. CONCLUSIONS AND SIGNIFICANCE: Our study suggests that a normal HIT in AVS is not always a dangerous sign indicating an acute stroke. From our observations, we propose that AVAD would be a new disease entity within AVS.

Diagnosis strategy and Yacovino maneuver for anterior canal-benign paroxysmal positional vertigo.

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.