Intratympanic dexamethasone in Manières disease and symptom control.

BACKGROUND: Manières disease (MD) is a chronic inner ear disease characterized by recurrent vertigo and fluctuation in auditory symptoms. Vertigo spells have a sudden onset and are difficult for patients to handle. Therefore, treating a patient with MD is still a challenge for clinicians. AIMS: This study aims to analyse the short-term effects of intratympanic dexamethasone (ITD) on the various symptoms of unilateral MD. MATERIALS AND METHODS: The study comprised 27 patients with unilateral MD and severe vertigo who failed medication therapy. Treatment was with ITD as an alternative to destructive therapy. Treatment is evaluated after four months. RESULTS: Significant improvements were measured with Dizziness Handicap Inventory (DHI), Tinnitus Handicap Inventory (THI), frequency of vertigo attacks longer than 20 min, Functional Level Scale (FLS), and tinnitus sensation measured by the Analog Visual Scale (AVS). Patients with severe symptoms grading with DHI and THI experienced the most improvement. Patients have achieved substantial vertigo control in 73%. CONCLUSION: ITD application shows improvement in controlling vertigo and tinnitus in patients under exacerbation in MD. SIGNIFICANCE: It is a promising non-destructive addition to the 'stepwise treatment concept' in MD and can be used as a first-line treatment in vertigo control.

Internet-based vestibular rehabilitation versus standard care after acute onset vertigo: a study protocol for a randomized controlled trial.

BACKGROUND: Dizziness and vertigo affect around 15% of adults annually and represent common reasons for contacting health services, accounting for around 3% of all emergency department visits worldwide. Vertigo is also associated with excessive use of diagnostic imaging and emergency care and decreased productivity, primarily because of work absenteeism. Vestibular rehabilitation is an evidence-based treatment for chronic dizziness and supervised group exercise therapy has recently been shown to be effective after vestibular neuritis, a common cause of acute onset vertigo. However, such interventions are not readily available and there is a need for more easily accessible tools. The purpose of this study is to investigate the effects on vestibular symptoms of a 6-week online vestibular rehabilitation tool after acute onset vertigo, with the aim of aiding vestibular rehabilitation by presenting a more accessible tool that can help to reduce recovery time. METHODS: Three hundred twenty individuals diagnosed with acute vestibular syndrome (AVS) will be recruited from multiple hospitals in Sweden and the effects of an online vestibular rehabilitation tool, YrselTräning, on vestibular symptoms after acute onset vertigo will be compared to standard care (written instructions leaflet) in a two-armed, evaluator-blinded, multicenter randomized controlled trial. The primary outcome will be the Vertigo Symptom Scale Short Form (VSS-SF) score at 6 weeks after symptom onset. Secondary outcomes include effects of the intervention on activities of daily living, mood and anxiety, vestibular function recovery, mobility measures, health economic effects, and the reliability of the Swedish VSS-SF translation. DISCUSSION: Participants using the online vestibular rehabilitation tool are expected to recover earlier and to a greater extent from their symptoms as compared to standard care. Since up to 50% of people with AVS without treatment develop persistent symptoms, effective treatment of AVS will likely lead to a higher quality of life and help reduce the societal costs associated with dizziness and vertigo. TRIAL REGISTRATION: Clinicaltrials.gov NCT05056324 . Registered on September 24, 2021.

Stereocilin gene variants associated with episodic vertigo: expansion of the DFNB16 phenotype.

Vestibular disorders comprise a heterogeneous group of diseases with transient or permanent loss of vestibular function. Vestibulopathy is in most cases associated with migraine, Ménière disease, hereditary ataxias, or sensorineural hearing loss. We identified two brothers and their first cousin affected by hearing loss and episodic vertigo. The brothers were homozygous STRC nonsense variant [c.4027 C > T, p.(Q1343*)], whereas their first cousin was compound heterozygous for the STRC nonsense variant and a 97 kb deletion spanning the entire STRC gene. Clinical investigations confirmed pathological vestibular responses in addition to a characteristic DFNB16 hearing loss. The STRC gene encodes Stereocilin in the cochlea and in the vestibular organ where it ensheathes the kinocilium of the otolithic membranes. Stereocilin is associated with the gel overlaying the vestibular kinocilia, suggesting a role for the protein in sensing balance and spatial orientation. Our findings support such a function for Stereocilin in the vestibular organ and expand the phenotype associated with DFNB16.

Vestibular findings in patients with persistent geotropic positional nystagmus: the 'light cupula' phenomenon.

CONCLUSIONS: Persistent geotropic nystagmus indicates a condition of a light cupula, which is accompanied by vestibular disability and a high incidence of pathological findings in the vestibular tests. The prevalence of migraine is high. OBJECTIVES: To examine subjective symptoms and characteristics of nystagmus in patients with persistent geotropic nystagmus using vestibular tests, as well as possible correlations to migraine in this group. METHODS: We enrolled 20 patients with a mean age of 53 years. The slow phase velocity (SPV) of the geotropic nystagmus and the nystagmus with the patient's head in the supine (S) and prone (P) positions was recorded. All patients completed caloric tests, subjective visual horizontal (SVH), and vestibular evoked myogenic potential (VEMP). All tests were repeated at follow-up (FU). RESULTS: SPV of the geotropic nystagmus directed to the left was 5.5°/s and that to the right was 3.5°/s. In 72% of patients, nystagmus in the P position was opposite to that in the S position. The vestibular tests were pathologic in about 60% of patients. At FU geotropic nystagmus was found in 40% of patients, but was significantly less intense. The vestibular test results remained at the same level at FU. Recurrent vertigo was reported in 78% of the patients. In all, 40% of the patients suffered from migraine.

Is the nystagmus pattern in hemi-labyrinthectomized subjects during positional alcohol nystagmus 2 similar to that found in patients with cupulolithiasis in the lateral semicircular canal?

CONCLUSION: A positional nystagmus pattern compatible with a condition of a heavy cupula (cupulolithiasis) in the lateral semicircular canal could be reproduced in hemi-labyrinthectomized subjects during positional alcohol nystagmus 2 (PAN 2). The nystagmus pattern was opposite to that found in the same subjects during PAN 1. The affected side could not be judged by applying Ewald's second law. OBJECTIVES: To mimic the condition of a heavy cupula in the lateral semicircular canal by using unilaterally deafferented subjects during PAN 2 and compare (a) results reported in the literature with those of patients with cupulolithiasis, and (b) the nystagmus findings in the same subjects during PAN 1. METHODS: Five hemi-labyrinthectomized subjects were studied during PAN 2 when they kept their heads pointed straight forward or turned sideways in the prone and supine positions, respectively. RESULTS: When the subjects were examined with their heads turned in the supine or prone positions, the alcohol-induced nystagmus pattern was compatible with that of cupulolithiasis. When the head was pointed straight forward in the prone and supine positions, the nystagmus directions were opposite to those found during PAN 1. Directional preponderance was not seen for the apogeotropic nystagmus for either ampullofugal or ampullopetal deviation of the cupula.

Can the nystagmus pattern in patients with a 'light cupula' be reproduced in hemi-labyrinthectomized subjects during positional alcohol nystagmus 1?

CONCLUSION: A positional nystagmus pattern compatible with a condition of a light cupula in the lateral semicircular canal seen in clinical patients could be reproduced only partially in hemi-labyrinthectomized subjects during the stage of positional alcohol nystagmus 1 (PAN 1). OBJECTIVES: To mimic the condition of a light cupula in the lateral semicircular canal by using unilaterally deafferented subjects during the stage of PAN 1 and compare the results with those of patients with a light cupula. METHODS: Five hemi-labyrinthectomized subjects were studied during PAN 1 with videonystagmography when they kept their heads straight forward or turned sideways in the prone and supine positions, respectively. A zero zone, indicating a cupula dysfunction, in which the geotropic nystagmus changed direction during slow head turn in the supine position, was also looked for. RESULTS: When the subjects were examined with their heads turned left or right in the supine or prone positions, the alcohol-induced nystagmus pattern was compatible with that of a light cupula. However, the nystagmus directions at the head straight forward in the prone and supine positions, as well as localization of the zero zones, deviated from the pattern seen in patients with a light cupula.

Different types of dizziness in patients with peripheral vestibular diseases--their prevalence and relation to migraine.

CONCLUSIONS: Besides spontaneous attacks of vertigo or unsteadiness, other symptoms, i.e. drop attacks, lateropulsion, illusions that the room or body is tilted, 'walking on pillows' or 'stepping into a hole', occur without precipitating head movement in almost 50% of patients with peripheral vestibular dysfunctions. The sensation of static tilt was closely connected to migraine and Meniere's disease (MD). OBJECTIVES: To record the prevalence of the different symptoms with respect to vestibular diagnosis and its relation to migraine. METHODS: Data from 100 patients with MD, benign paroxysmal positional vertigo (BPPV), or unilateral peripheral vestibular impairment (UPVI) were analyzed with respect to vestibular diagnosis and migraine as a secondary diagnosis. RESULTS: Spontaneous attacks of vertigo or unsteadiness occurred in 74% and 48% of patients, respectively. Vertigo was significantly more often reported in patients with MD and BPPV. In patients with BPPV, the duration of spontaneous vertigo was shorter than in patients with MD. The relative incidence of other symptoms were: unsteadiness, 48%; 'stepping into a hole', 46%; lateropulsion, 35%; 'walking on pillows', 21%; and drop attacks, 19%. Only the sensation of static tilt, which occurred in 8% of patients, was significantly correlated to MD or to migraine.

Persistent geotropic nystagmus--a different kind of cupular pathology and its localizing signs.

CONCLUSION: A persistent geotropic positional nystagmus indicates a dysfunction in the lateral semicircular canal with a cupula of less specific weight than the surrounding endolymph. It is possible to determine the side of the affected cupula by recording the nystagmus pattern in yaw and pitch plane. OBJECTIVES: To identify the clinical features in patients with a persistent geotropic positional nystagmus, establish lateralizing signs and relate the findings to a pathophysiologic mechanism. PATIENTS AND METHODS: Six patients with acute onset vertigo of a peripheral origin and persistent geotropic nystagmus were examined with videonystagmoscopy and the nystagmus characteristics in different positions of the head in yaw and pitch plane were studied. RESULTS: Besides the persistent geotropic nystagmus, a zero zone was found with no nystagmus, beyond which the nystagmus changed direction when the head of the patient in supine position was gradually rotated from side to side. The zero zone was present when the head was turned slightly towards one side and is thought to represent a position where the affected cupula is aligned with the gravitational vertical. With the head bent forwards the nystagmus direction was to the non-affected side and when the head was bent backwards to the affected side.