The role of vertical semicircular canal function in the vertical component of skull vibration-induced nystagmus.

Generally, vertical component of the skull vibratory nystagmus (VCN) is ignored in the clinical practise. Thus, the relative contribution of the vestibular organs in the presence of VCN remains unknown. To determine the association between vertical semicircular canal (vSCC) function and the presence of VCN.

Valencia's Cathedral Church Bell Acoustics Impact on the Hearing Abilities of Bell Ringers.

Studies on the effect of occupational noise have been widely performed for occupations such as construction workers, workers of factories or even musicians and workers of nightclubs. However, studies on the acoustics of church bells are very scarce and usually reported in languages other than English. In Spain, although the tradition of bell ringers is progressively getting lost, some bell ringers that continue transmitting the tradition remain.

Parameters of skull vibration-induced nystagmus in normal subjects.

Hypothesis: The knowledge of vibration-induced nystagmus test (SVINT) values in the normal population is highly relevant to provide a rapid orientation on the diagnosis attitude in a patient with vertigo.

Background: Although mastoid bone vibration should only induce nystagmus in the presence of vestibular asymmetry, it has also been reported in normal individuals raising doubts as to how to interpret the SVINT. To date, no population studies involving the use of the SVINT and that establish normative values have been published.

Regulation of Fn14 Receptor and NF-κB Underlies Inflammation in Meniere's Disease.

Meniere's disease (MD) is a rare disorder characterized by episodic vertigo, sensorineural hearing loss, tinnitus, and aural fullness. It is associated with a fluid imbalance between the secretion of endolymph in the cochlear duct and its reabsorption into the subarachnoid space, leading to an accumulation of endolymph in the inner ear. Epidemiological evidence, including familial aggregation, indicates a genetic contribution and a consistent association with autoimmune diseases (AD).

Audiological Findings in Charcot-Marie-Tooth Disease Type 4C.

Objective: Charcot-Marie-Tooth disease type 4C (CMT4C) is a hereditary demyelinating early onset neuropathy with prominent unsteadiness and occasional cranial nerve involvement. Vestibulopathy caused by the dysfunction of cranial nerve VIII has been demonstrated in a high percentage of these patients, but the presence and degree of auditory neuropathy are unknown. The aim of the study was to characterize the hearing abnormalities of a series of patients with CMT4C and to determine the presence and severity of auditory neuropathy (AN) in these patients.

Ipsilesional Nystagmus Induced by Vibration in Subjects With Ménière's Disease or Vestibular Schwannoma.

Objective: To analyze the frequency in which vibration-induced nystagmus (VIN) with ipsilesional direction appears in subjects with Ménière's disease (MD) or vestibular schwannoma (VS).

Study Design: Cross-sectional study.

Setting: Tertiary referral center.

Atypical periodic alternating nystagmus responding to high-dose intravenous immunoglobulins: a case report.

Background: Acquired periodic alternating nystagmus (PAN) is a rare but well-defined syndrome that consists of a horizontal nystagmus that cyclically reverses its direction. PAN can be caused by degenerative, neoplastic, or toxic diseases of the cerebellum and, in a few cases, by subacute cerebellar ataxia of immune origin.

Case Presentation: A 44-year-old man came to our attention because of rapidly progressive gait instability and blurred vision.

An Iatrogenic Cholesteatoma of the Squamous Part of the Temporal Bone.

A cholesteatoma is a relatively common condition within the middle ear cavity, whereas a cholesteatoma of the squamous part of the temporal bone is an exceptionally rare entity. A case of an iatrogenic cholesteatoma located in the squamous part of the temporal bone is presented, which was revealed by an intermittent purulent discharge from an opening above the right ear 20 years after retroauricular myringoplasty. The diagnosis of an iatrogenic cholesteatoma is often made after several years of evolution, sometimes even at the stage of complications.

Clinical Subgroups in Bilateral Meniere Disease.

Meniere disease (MD) is a heterogeneous clinical condition characterized by sensorineural hearing loss, episodic vestibular symptoms, and tinnitus associated with several comorbidities, such as migraine or autoimmune disorders (AD). The frequency of bilateral involvement may range from 5 to 50%, and it depends on the duration of the disease. We have performed a two-step cluster analysis in 398 patients with bilateral MD (BMD) to identify the best predictors to define clinical subgroups with a potential different etiology to improve the phenotyping of BMD and to develop new treatments.

Response Over Time of Vertigo Spells to Intratympanic Dexamethasone Treatment in Meniere's Disease Patients.

Objective: To assess the effectiveness and response over time of intratympanic dexamethasone on the symptoms of Meniere's disease.

Materials And Methods: We performed a matched cohort study of 24 patients with Meniere's disease who were unresponsive to initial treatment and underwent 3 sessions of weekly intratympanic dexamethasone injections using a concentration of 16 mg/mL and 24 matched controls with the same characteristics with regard to vertigo spells.

Results: Compared with control subjects, intratympanic dexamethasone injections resulted in a decrease in the frequency of vertigo spells in the first 6-month period.

Increased Velocity Storage in Subjects with Meniere's Disease.

Objective: Velocity storage mechanism is a multisensory rotation estimator; it compensates for errors in the information provided by the peripheral vestibular organs by means of an adjustment in the duration of the vestibular signal. The aim of this study was to determine the activity of the velocity storage mechanism in the presence of a labyrinthine disorder, using galvanic vestibular stimulation to cause direct activation of the vestibular afferent neurons.

Materials And Methods: Forty-one subjects with definite Meniere's disease (MD) and 36 healthy volunteers were evaluated using a 20-s galvanic vestibular stimulation.

Effect of the stringency of conditions on caloric test results in healthy subjects.

The caloric test is widely used to assess vestibular function, but the conditions in which it is performed can vary. Caloric nystagmus obtained in 57 healthy subjects were compared: 24 subjects studied in ideal conditions and 33 subjects in non-ideal conditions. A statistically significant decrease in the slow phase velocity of the 4 irrigations performed on the subjects in non-ideal conditions was observed.

Vestibular impairment in Charcot-Marie-Tooth disease type 4C.

Charcot-Marie-Tooth disease type 4C (CMT4C) is a hereditary neuropathy with prominent unsteadiness. The objective of the current study is to determine whether the imbalance in CMT4C is caused only by reduced proprioceptive input or if vestibular nerve involvement is an additional factor. We selected 10 CMT4C patients and 10 age-matched and sex-matched controls.

Allelic variants in TLR10 gene may influence bilateral affectation and clinical course of Meniere's disease.

Toll-like receptors trigger the innate immune response by activating various cell types such us macrophages and lymphocytes. We genotyped SNV of TLR3, TRL7, TLR8 and TLR10 in 863 Spanish and 150 Italian patients with Meniere's disease (MD) and 1,013 controls by using Taqman assays. Real-Time qPCR was used to measure the expression level of TLR10 in peripheral blood leukocytes.

Functional variants of MIF, INFG and TFNA genes are not associated with disease susceptibility or hearing loss progression in patients with Ménière's disease.

Variability in acute immune response genes could determine susceptibility or prognosis for Ménière's disease (MD). The cytokines tumor necrosis factor α (TNFα), macrophage migration inhibitory factor (MIF) and interferon γ (INFγ) are proinflammatory cytokines of the innate immune response. These cytokines mediate inflammation and have been previously associated with the inflammatory process in several autoimmune diseases.

MICA-STR A.4 is associated with slower hearing loss progression in patients with Ménière's disease.

Hypothesis: Immune response may influence hearing outcome in Ménière's disease (MD).

Background: Major histocompatibility complex class I chain-related A (MICA) encodes a highly polymorphic stress-inducible protein, which interacts with NKGD2 receptor on the surface of NK, γδ T cells and T CD8 lymphocytes. We investigated the association of MICA gene with hearing outcome in MD and its linkage disequilibrium (LD) with human leukocyte antigen (HLA)-B.

[Peripheral vertigo classification of the Otoneurology Committee of the Spanish Otorhinolaryngology Society: diagnostic agreement and update (version 2-2011)].

Introduction: In 2008, the Otoneurology committee of the SEORL-PCF published a classification of peripheral vertigo, based on clinical criteria. The objective of this study was to validate this classification through analysing the diagnostic agreement among several medical assessors.

Methods: Seven medical assessors, all with clinical experience, from 6 different hospitals, participated in the study.

High prevalence of systemic autoimmune diseases in patients with Menière's disease.

Background: Autoimmunity appears to be associated with the pathophysiology of Meniere's disease (MD), an inner ear disorder characterized by episodes of vertigo associated with hearing loss and tinnitus. However, the prevalence of autoimmune diseases (AD) in patients with MD has not been studied in individuals with uni or bilateral sensorineural hearing loss (SNHL).

Methods And Findings: We estimated the prevalence of AD in 690 outpatients with MD with uni or bilateral SNHL from otoneurology clinics at six tertiary referral hospitals by using clinica criteria and an immune panel (lymphocyte populations, antinuclear antibodies, C3, C4 and proinflammatory cytokines TNFα, INFγ).

Evolution of symptoms in Ménière's disease.

Objective: To investigate the sequence and correlation of symptoms of Ménière's disease (MD) depending on their order of manifestation.

Methods: Descriptive, longitudinal study of the symptoms in 237 tertiary hospital patients who had been diagnosed with definite MD according to the criteria of the American Academy of Otolaryngology. Patients were followed for 1-31 years.

Functional variants in NOS1 and NOS2A are not associated with progressive hearing loss in Ménière's disease in a European Caucasian population.

Hearing loss in Ménière's disease (MD) is associated with loss of spiral ganglion neurons and hair cells. In a guinea pig model of endolymphatic hydrops, nitric oxide synthases (NOS) and oxidative stress mediate loss of spiral ganglion neurons. To test the hypothesis that functional variants of NOS1 and NOS2A are associated with MD, we genotyped three functional variants of NOS1 (rs41279104, rs2682826, and a cytosine-adenosine microsatellite repeat in exon 1f) and the CCTTT repeat in the promoter of NOS2A gene (rs3833912) in two independent MD sets (273 patients in total) and 550 controls.

Hearing assessment in Menière's disease.

Objectives: To investigate the level of hearing loss and the configuration of the mean audiometric curve over the course of Menière's disease, correcting the data according to patient age.

Study Design: A retrospective study of 3,963 hearing tests.

Methods: Descriptive, longitudinal study of pure-tone audiometries of 237 patients at a tertiary hospital who had been diagnosed with definitive Menière's disease according to the American Academy of Otorhinolaryngology criteria.

Polymorphisms of CD16A and CD32 Fcγ receptors and circulating immune complexes in Ménière's disease: a case-control study.

Background: Autoimmune diseases with elevated circulating autoantibodies drive tissue damage and the onset of disease. The Fcγ receptors bind IgG subtypes modulating the clearance of circulating immune complexes (CIC). The inner ear damage in Ménière's disease (MD) could be mediated by an immune response driven by CIC.