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). We conducted a case–control study in two phases using an immune genotyping array in a total of 420 patients with bilateral MD and 1,630 controls. We have identified the first locus, at 6p21.33, suggesting an association with bilateral MD [meta-analysis leading signal rs4947296, OR = 2.089 (1.661–2.627); p = 1.39 × 10−09]. Gene expression profiles of homozygous genotype-selected peripheral blood mononuclear cells (PBMCs) demonstrated that this region is a trans-expression quantitative trait locus (eQTL) in PBMCs. Signaling analysis predicted several tumor necrosis factor-related pathways, the TWEAK/Fn14 pathway being the top candidate (p = 2.42 × 10−11). This pathway is involved in the modulation of inflammation in several human AD, including multiple sclerosis, systemic lupus erythematosus, or rheumatoid arthritis. In vitro studies with genotype-selected lymphoblastoid cells from patients with MD suggest that this trans-eQTL may regulate cellular proliferation in lymphoid cells through the TWEAK/Fn14 pathway by increasing the translation of NF-κB. Taken together; these findings suggest that the carriers of the risk genotype may develop an NF-κB-mediated inflammatory response in MD

Sudden idiopathic deafness. Review of 58 cases

Introduccion y Objetivo: La sordera súbita (SS) se define como la hipoacusia neurosensorial de inicio brusco, en menos de 72 horas, con pérdida de 30 o más decibelios (dB) en tres frecuencias consecutivas en la audiometría tonal. En el 90% de los casos no es posible determinar su etiología dando lugar a lo que se conoce como sordera súbita idiopática (SSI). El objetivo de este estudio es describir las características de los pacientes con SSI diagnosticados en los últimos 20 años, identificar factores pronósticos y mostrar los resultados en función del tratamiento. Método: Estudio descriptivo retrospectivo de 58 pacientes diagnosticados de SSI entre 1995-2016. Se determinaron el nivel de pérdida auditiva y el grado de recuperación posterior. Se estudiaron los factores de riesgo cardiovascular, asociación con otros síntomas, inicio del tratamiento, severidad de la hipoacusia y morfología de la curva auditiva. Resultados: No se encontró relación entre la recuperación auditiva y los diferentes factores de riesgo cardiovascular. Presentaron una mejoría completa un 24,1%, parcial un 17,2% y leve el 17,2%. El 41,5% no obtuvo ninguna mejoría con un peor pronóstico auditivo en los sujetos con hipoacusia profunda. Se obtuvo un 100% de recuperaciones completas en los sujetos con morfología inclinación ascendente. El síntoma asociado más frecuente fue el acúfeno. Los síntomas vestibulares al inicio del cuadro, determinaron un peor pronóstico auditivo. El tiempo de inicio desde la aparición de la hipoacusia hasta la instauración del tratamiento intravenoso no se ha relacionado con una mejor evolución. El porcentaje “sin mejoría” es del 54% (DIT y tratamiento sistémico) frente al 75% en los tratados exclusivamente con tratamiento intravenoso, pero esta mejoría no tiene significación estadística. Conclusiones: La afectación vestibular, severidad de la hipoacusia y la morfología de la curva auditiva son determinantes como factor pronóstico. Aunque hay mejores resultados utilizando la DIT como tratamiento de rescate, nuestra experiencia no determino un beneficio evidente de su uso en sujetos con SSI.Objective: Sudden deafness is defined as sudden-onset sensorineural hearing loss in less than 72 hours, with loss of 30 or more decibels (dB) at three consecutive frequencies in tonal audiometry. In 90% of the cases it is not possible to determine its etiology giving rise to what is known as idiopathic sudden deafness. Our objective is To describe patient’s characteristics with diagnosed ISHL in the last twenty years, to identify prognostic factors and show the results in regards of treatment. Method: Retrospective descriptive study of 58 patients, diagnosed with ISHL between 1995-2016. Level of hearing loss was determined as well as the degree of posterior recovery. Cardiovascular risk factors were studied, in addition to association with other symptoms, the onset of treatment, severity of hearing loss and morphology of the audiometric curve. Results: No association was found between hearing recovery and different cardiovascular risk factors. 24.1% of the patients presented a complete improvement. Partial improvement and mild improvement were obtained in 17.2% each. 41.5% did not obtain any improvement with a worse hearing prognosis in those patients with profound hearing loss. 100% of recovery was obtained in subjects with ascending audiometric morphology. The most common associated symptom was tinnitus. Accompanying vestibular symptoms as an initial manifestation determine a worse hearing prognosis. Starting time from the emergence of hypoacusis until the instauration of systemic (IV) treatment was not related to a better prognosis. 54% of the patients did not show any improvements with ITD and systemic treatment compared to a 75% in those treated exclusively with systemic treatment. This was not statistically significant. Conclusions: Vestibular involvement, hypoacusis severity and hearing curve morphology are determinant prognostic factors. Although there are good outcomes published using ITD as recovery treatment, our experience does not determine an evident benefit of its use on subjects with ISHL

Sordera súbita idiopática: revisión de 58 casos

Objective: Sudden deafness is defined as sudden-onset sensorineural hearing loss in less than 72 hours, with loss of 30 or more decibels (dB) at three consecutive frequencies in tonal audiometry. In 90% of the cases it is not possible to determine its etiology giving rise to what is known as idiopathic sudden deafness. Our objective is To describe patient’s characteristics with diagnosed ISHL in the last twenty years, to identify prognostic factors and show the results in regards of treatment. Method: Retrospective descriptive study of 58 patients, diagnosed with ISHL between 1995-2016. Level of hearing loss was determined as well as the degree of posterior recovery. Cardiovascular risk factors were studied, in addition to association with other symptoms, the onset of treatment, severity of hearing loss and morphology of the audiometric curve. Results: No association was found between hearing recovery and different cardiovascular risk factors. 24.1% of the patients presented a complete improvement. Partial improvement and mild improvement were obtained in 17.2% each. 41.5% did not obtain any improvement with a worse hearing prognosis in those patients with profound hearing loss. 100% of recovery was obtained in subjects with ascending audiometric morphology. The most common associated symptom was tinnitus. Accompanying vestibular symptoms as an initial manifestation determine a worse hearing prognosis. Starting time from the emergence of hypoacusis until the instauration of systemic (IV) treatment was not related to a better prognosis. 54% of the patients did not show any improvements with ITD and systemic treatment compared to a 75% in those treated exclusively with systemic treatment. This was not statistically significant. Conclusions: Vestibular involvement, hypoacusis severity and hearing curve morphology are determinant prognostic factors. Although there are good outcomes published using ITD as recovery treatment, our experience does not determine an evident benefit of its use on subjects with ISHL.Introduccion y Objetivo: La sordera súbita (SS) se define como la hipoacusia neurosensorial de inicio brusco, en menos de 72 horas, con pérdida de 30 o más decibelios (dB) en tres frecuencias consecutivas en la audiometría tonal. En el 90% de los casos no es posible determinar su etiología dando lugar a lo que se conoce como sordera súbita idiopática (SSI). El objetivo de este estudio es describir las características de los pacientes con SSI diagnosticados en los últimos 20 años, identificar factores pronósticos y mostrar los resultados en función del tratamiento. Método: Estudio descriptivo retrospectivo de 58 pacientes diagnosticados de SSI entre 1995-2016. Se determinaron el nivel de pérdida auditiva y el grado de recuperación posterior. Se estudiaron los factores de riesgo cardiovascular, asociación con otros síntomas, inicio del tratamiento, severidad de la hipoacusia y morfología de la curva auditiva. Resultados: No se encontró relación entre la recuperación auditiva y los diferentes factores de riesgo cardiovascular. Presentaron una mejoría completa un 24,1%, parcial un 17,2% y leve el 17,2%. El 41,5% no obtuvo ninguna mejoría con un peor pronóstico auditivo en los sujetos con hipoacusia profunda. Se obtuvo un 100% de recuperaciones completas en los sujetos con morfología inclinación ascendente. El síntoma asociado más frecuente fue el acúfeno. Los síntomas vestibulares al inicio del cuadro, determinaron un peor pronóstico auditivo. El tiempo de inicio desde la aparición de la hipoacusia hasta la instauración del tratamiento intravenoso no se ha relacionado con una mejor evolución. El porcentaje «sin mejoría» es del 54% (DIT y tratamiento sistémico) frente al 75% en los tratados exclusivamente con tratamiento intravenoso, pero esta mejoría no tiene significación estadística. Conclusiones: La afectación vestibular, severidad de la hipoacusia y la morfología de la curva auditiva son determinantes como factor pronóstico. Aunque hay mejores resultados utilizando la DIT como tratamiento de rescate, nuestra experiencia no determino un beneficio evidente de su uso en sujetos con SSI

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. Church bells create sound with a large sound pressure level that can be heard from a great distance. However, despite the characteristics of the sound of church bells, bell ringers do not present symptoms of occupational hearing loss unlike musicians and construction workers. To determine the effects of the sound of the church bells on bell ringers, in this paper, an acoustic study of the church bells and a physiological study of the hearing abilities of bell ringers. Results show sound pressure levels reaching 120 dB inside the bell tower. The resulting hearing loss in bell ringers is small considering the great intensity of the sound produced by the bells. This is likely due to the short amount of time that bell ringers are exposed to the sound even if it reaches high sound pressure levels

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

Abstract 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. Clinical examination showed PAN and a mild pancerebellar syndrome. Eye movement recordings disclosed a short cycle PAN with significant slow-phase velocity only in darkness. Under the effect of a γ-aminobutyric acid type B (GABAB) agonist, PAN was not modified. Right after treatment with intravenous immunoglobulin (IVIg) was started, PAN was essentially eliminated. Three months after last dose of IVIg, this nystagmus reappeared. Conclusions IVIg resolved PAN in this patient. This finding may point to an autoimmune mechanism underlying this patient’s nystagmus. This case suggests that the usefulness of IVIg at treating PAN might be worth a consideration in similar clinical settings