Surgical interventions for Ménière's disease.

BACKGROUND: Ménière's disease is a condition that causes recurrent episodes of vertigo, associated with hearing loss and tinnitus. First-line treatments often involve dietary or lifestyle changes, medication or local (intratympanic) treatments. However, surgery may also be considered for people with persistent or severe symptoms. The efficacy of different surgical interventions at preventing vertigo attacks, and their associated symptoms, is currently unclear. OBJECTIVES: To evaluate the benefits and harms of surgical interventions versus placebo or no treatment in people with Ménière's disease. SEARCH METHODS: The Cochrane ENT Information Specialist searched the Cochrane ENT Register; Central Register of Controlled Trials (CENTRAL); Ovid MEDLINE; Ovid Embase; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials. The date of the search was 14 September 2022. SELECTION CRITERIA: We included randomised controlled trials (RCTs) and quasi-RCTs in adults with definite or probable Ménière's disease comparing ventilation tubes, endolymphatic sac surgery, semi-circular canal plugging/obliteration, vestibular nerve section or labyrinthectomy with either placebo (sham surgery) or no treatment. We excluded studies with follow-up of less than three months, or with a cross-over design (unless data from the first phase of the study could be identified).  DATA COLLECTION AND ANALYSIS: We used standard Cochrane methods. Our primary outcomes were: 1) improvement in vertigo (assessed as a dichotomous outcome - improved or not improved), 2) change in vertigo (assessed as a continuous outcome, with a score on a numerical scale) and 3) serious adverse events. Our secondary outcomes were: 4) disease-specific health-related quality of life, 5) change in hearing, 6) change in tinnitus and 7) other adverse effects. We considered outcomes reported at three time points: 3 to < 6 months, 6 to ≤ 12 months and > 12 months. We used GRADE to assess the certainty of evidence for each outcome.  MAIN RESULTS: We included two studies with a total of 178 participants. One evaluated ventilation tubes compared to no treatment, the other evaluated endolymphatic sac decompression compared to sham surgery.  Ventilation tubes We included a single RCT of 148 participants with definite Ménière's disease. It was conducted in a single centre in Japan from 2010 to 2013. Participants either received ventilation tubes with standard medical treatment, or standard medical treatment alone, and were followed up for two years. Some data were reported on the number of participants in whom vertigo resolved, and the effect of the intervention on hearing. Our other primary and secondary outcomes were not reported in this study. This is a single, small study and for all outcomes the certainty of evidence was low or very low. We are unable to draw meaningful conclusions from the numerical results. Endolymphatic sac decompression We also included one RCT of 30 participants that compared endolymphatic sac decompression with sham surgery. This was a single-centre study conducted in Denmark during the 1980s. Follow-up was predominantly conducted at one year, but additional follow-up continued for up to nine years in some participants. Some data were reported on hearing and vertigo (both improvement in vertigo and change in vertigo), but our other outcomes of interest were not reported. Again, this is a single, very small study and we rated the certainty of the evidence as very low for all outcomes. We are therefore unable to draw meaningful conclusions from the numerical results.  AUTHORS' CONCLUSIONS: We are unable to draw clear conclusions about the efficacy of these surgical interventions for Ménière's disease. We identified evidence for only two of our five proposed comparisons, and we assessed all the evidence as low- or very low-certainty. This means that we have very low confidence that the effects reported are accurate estimates of the true effect of these interventions. Many of the outcomes that we planned to assess were not reported by the studies, such as the impact on quality of life, and adverse effects of the interventions. Consensus on the appropriate outcomes to measure in studies of Ménière's disease is needed (i.e. a core outcome set) in order to guide future studies in this area and enable meta-analyses of the results. This must include appropriate consideration of the potential harms of treatment, as well as the benefits.

Acute truncal ataxia without nystagmus in patients with acute vertigo.

BACKGROUND AND PURPOSE: Differentiating between peripheral and central aetiologies can be challenging in patients with acute vertigo, given substantial symptom overlap. A detailed clinical history and focused physical eye movement examination such as the HINTS eye examination appear to be the most reliable approach to identify acute cerebellar/brainstem stroke, outperforming even acute brain imaging. We have observed, however, that isolated vertigo of central cause may be accompanied by acute truncal ataxia, in the absence of nystagmus. METHODS: We explored the frequency of ataxia without concurrent nystagmus in a cross section of patients with acute vertigo who presented to the emergency department at two centres in Argentina (Group A) and the UK (Group B). Patients underwent detailed clinical neuro-otological assessments (Groups A and B), which included instrumented head impulse testing and oculography (Group B). RESULTS: A total of 71 patients in Group A and 24 patients in Group B were included in this study. We found acute truncal ataxia-without nystagmus-in 15% (n = 14) of our overall cohort. Lesions involved stroke syndromes affecting the posterior inferior cerebellar artery, anterior inferior cerebellar artery, and superior cerebellar artery, thalamic stroke, cerebral hemisphere stroke, multiple sclerosis, and a cerebellar tumour. Additional oculomotor deficits did not reliably identify a central cause in these individuals, even with oculography. CONCLUSIONS: We have identified a significant subpopulation of patients with acute vertigo in whom the current standard approaches such as the HINTS examination that focus on oculomotor assessment may not be applicable, highlighting the need for a formal assessment of gait in this setting.

RFC1 expansions are a common cause of idiopathic sensory neuropathy.

After extensive evaluation, one-third of patients affected by polyneuropathy remain undiagnosed and are labelled as having chronic idiopathic axonal polyneuropathy, which refers to a sensory or sensory-motor, axonal, slowly progressive neuropathy of unknown origin. Since a sensory neuropathy/neuronopathy is identified in all patients with genetically confirmed RFC1 cerebellar ataxia, neuropathy, vestibular areflexia syndrome, we speculated that RFC1 expansions could underlie a fraction of idiopathic sensory neuropathies also diagnosed as chronic idiopathic axonal polyneuropathy. We retrospectively identified 225 patients diagnosed with chronic idiopathic axonal polyneuropathy (125 sensory neuropathy, 100 sensory-motor neuropathy) from our general neuropathy clinics in Italy and the UK. All patients underwent full neurological evaluation and a blood sample was collected for RFC1 testing. Biallelic RFC1 expansions were identified in 43 patients (34%) with sensory neuropathy and in none with sensory-motor neuropathy. Forty-two per cent of RFC1-positive patients had isolated sensory neuropathy or sensory neuropathy with chronic cough, while vestibular and/or cerebellar involvement, often subclinical, were identified at examination in 58%. Although the sensory ganglia are the primary pathological target of the disease, the sensory impairment was typically worse distally and symmetric, while gait and limb ataxia were absent in two-thirds of the cases. Sensory amplitudes were either globally absent (26%) or reduced in a length-dependent (30%) or non-length dependent pattern (44%). A quarter of RFC1-positive patients had previously received an alternative diagnosis, including Sjögren's syndrome, sensory chronic inflammatory demyelinating polyneuropathy and paraneoplastic neuropathy, while three cases had been treated with immune therapies.

Diagnosis and treatment choices of suspected benign paroxysmal positional vertigo: current approach of general practitioners, neurologists, and ENT physicians.

PURPOSE: Benign paroxysmal positional vertigo (BPPV) is a frequently underdiagnosed cause of vertigo, potentially due to the underuse of diagnostic and therapeutic canalith repositioning procedures (CRPs). We aimed to investigate self-reported use of the diagnostic and therapeutic approach to BPPV patients by Lithuanian neurologists, ear, nose, and throat (ENT) physicians, and general practitioners (GPs), and to explore potential reasons for the underuse of the maneuvers. METHODS: Neurologists, ENT physicians, and GPs were invited to complete a written questionnaire focused on diagnostic and therapeutic practices related to BPPV. Between-group differences and associations between responses were analyzed statistically. RESULTS: In total, 97 neurologists, 85 ENT physicians and 142 GPs (21.1%, 26.8%, and 5.7%, respectively, of all corresponding licensed Lithuanian physicians) completed the questionnaire. 24% of neurologists, 33% ENT physicians and 50% GPs do not perform diagnostic maneuvers for patients with suspected BPPV, and 28%, 61%, and 84%, respectively, do not perform CRPs. Years of clinical experience was a negative predictor of CRP performance [OR 0.97 (95% CI 0.95-0.99), p = 0.001]. Frequent reasons for not performing CRPs were time taken for the procedure, fear of provoking symptoms, and lack of knowledge. All physicians frequently ordered additional imaging or consultations for suspected BPPV and reported prescribing a range of medications. CONCLUSIONS: A significant proportion of Lithuanian neurologists, ENT physicians, and GPs do not employ diagnostic maneuvers and CRPs for BPPV patients, contrary to established guidelines. Lack of expertise and time available is a common culprit that leads to unnecessary drug prescribing and investigation.

Head-Jolting Nystagmus: Occlusion of the Horizontal Semicircular Canal Induced by Vigorous Head Shaking.

IMPORTANCE: We report a new syndrome, which we are calling head-jolting nystagmus, that expands the differential diagnosis of head movement-induced paroxysmal vertigo. OBSERVATIONS: Two male patients (65 and 58 years old) described rotational vertigo after violent and brief (1- to 2-second) oscillations of the head (head jolting) that triggered intense horizontal nystagmus lasting 45 seconds. Accelerations of the head required to induce these episodes could only be achieved by the patients themselves. In case 1, the episodes gradually disappeared over a 6-year period. In case 2, magnetic resonance imaging (3-T) suggested a filling defect within the left horizontal semicircular canal. He underwent surgical canal plugging in March 2013 that resolved the symptoms. CONCLUSIONS AND RELEVANCE: We attribute head-jolting nystagmus to dislodged material within the horizontal semicircular canal and provide a mechanistic model to explain its origin.