Audiovestibular Manifestations in Patients with Primary Raynaud's Phenomenon and Raynaud's Phenomenon Secondary to Systemic Sclerosis.

OBJECTIVES: To address the prevalence of audiovestibular disorders in patients with primary Raynaud's Phenomenon (RP). A series of patients with primary RP and secondary RP in the context of systemic sclerosis (SSc) were compared with healthy controls. METHODS: A prospective multicenter observational cross-sectional study was conducted in several Otolaryngology and Rheumatology Divisions of tertiary referral hospitals, recruiting 57 patients with RP and 57 age- and gender-matched controls. Twenty patients were classified as primary RP when unrelated to any other conditions and 37 patients who met the 2013 ACR/EULAR classification criteria for SSc were classified as having secondary RP associated with SSc. Audiometric and vestibular testing (vHIT), clinical sensory integration and balance testing (CTSIB), and Computerized Dynamic Posturography (CDP) were performed. RESULTS: As significant differences were found in the age of the two study groups, primary and secondary RP, no comparisons were made between both groups of RP but only with their control groups. No sensorineural hearing loss (SNHL) was recorded in any of our patients with primary RP and no differences were found in the voice audiometry tests with respect to controls. Four of 37 (10.8%) secondary RP patients presented SNHL. Those with SNHL were 7.03 times more likely to have a secondary RP than controls (p < 0.001). The audiometric curve revealed high-frequency hearing loss in 4 patients with RP secondary to SSc, and statistically significant differences were achieved when RP secondary was compared to controls in vHIT gain, caloric test, CTSIB, and CDP. CONCLUSIONS: Unlike patients with RP secondary to SSc, patients with primary RP do not show audiovestibular abnormalities. Regarding audiovestibular manifestations, primary RP can be considered a different condition than secondary RP.

Estrategias para la práctica de la otorrinolaringología y cirugía de cabeza y cuello durante la fase de control de la COVID-19 / Strategies for the practice of otolaryngology and head and neck surgery during the monitoring phase of COVID-19

La aparición de una nueva enfermedad por coronavirus denominada COVID-19 a finales de 2019 y su expansión pandémica en el mundo ha cambiado la práctica habitual de la especialidad de Otorrinolaringología (ORL). Tras una fase de crecimiento exponencial de los contagios, se ha logrado entrar en una fase de control de la expansión de la enfermedad en la que persiste la posibilidad de contagio, pero la aparición de nuevos casos se considera asumible por el sistema sanitario. El objetivo del presente documento es revisar la evidencia disponible y proponer estrategias y recomendaciones para la práctica médico-quirúrgica de la otorrinolaringología y cirugía de cabeza y cuello, que permitan establecer la actividad habitual, adecuando los estándares de seguridad y eficacia a la situación actual. Se requiere, por lo tanto, identificar y clasificar a los pacientes en función de criterios de estado infeccioso-inmunológico, y establecer las recomendaciones de protección en consultas, hospitalización y quirófano, que eviten la transmisión de la enfermedad a otros usuarios y al personal sanitario, en el contexto específico del desarrollo de nuestra especialidad. El presente documento es fruto de la colaboración de las comisiones científicas y del comité COVID-19 de la SEORLCCC

The appearance of a new coronavirus disease called COVID-19 at the end of 2019 and its pandemic expansion in the world has changed the usual practice of the specialty of Otorhinolaryngology (ENT). After a phase of exponential growth of infections, it has been possible to enter a phase of control of the spread of the disease in which the possibility of infection persists, and the appearance of new cases is considered acceptable by the health system. The aim of this document is to review the available evidence and propose strategies and recommendations for the medical-surgical practice of otorhinolaryngology and head and neck surgery, which allow establishing the usual activity, adapting the safety and efficacy standards to the current situation. Therefore, it is required to identify and classify patients according to criteria of infectious-immunological status, and to establish recommendations for protection in consultations, hospitalization and the operating room, which avoid the transmission of the disease to other users and healthcare personnel, in the specific context of the development of our specialty. This document is the result of the collaboration of all the scientific commissions and the SEORLCCC COVID-19 committee

Burden of Rare Variants in the OTOG Gene in Familial Meniere's Disease.

OBJECTIVES: Meniere's disease (MD) is a rare inner ear disorder characterized by sensorineural hearing loss, episodic vertigo, and tinnitus. Familial MD has been reported in 6 to 9% of sporadic cases, and few genes including FAM136A, DTNA, PRKCB, SEMA3D, and DPT have been involved in single families, suggesting genetic heterogeneity. In this study, the authors recruited 46 families with MD to search for relevant candidate genes for hearing loss in familial MD. DESIGN: Exome sequencing data from MD patients were analyzed to search for rare variants in hearing loss genes in a case-control study. A total of 109 patients with MD (73 familial cases and 36 early-onset sporadic patients) diagnosed according to the diagnostic criteria defined by the Barany Society were recruited in 11 hospitals. The allelic frequencies of rare variants in hearing loss genes were calculated in individuals with familial MD. A single rare variant analysis and a gene burden analysis (GBA) were conducted in the dataset selecting 1 patient from each family. Allelic frequencies from European and Spanish reference datasets were used as controls. RESULTS: A total of 5136 single-nucleotide variants in hearing loss genes were considered for single rare variant analysis in familial MD cases, but only 1 heterozygous likely pathogenic variant in the OTOG gene (rs552304627) was found in 2 unrelated families. The gene burden analysis found an enrichment of rare missense variants in the OTOG gene in familial MD. So, 15 of 46 families (33%) showed at least 1 rare missense variant in the OTOG gene, suggesting a key role in familial MD. CONCLUSIONS: The authors found an enrichment of multiplex rare missense variants in the OTOG gene in familial MD. This finding supports OTOG as a relevant gene in familial MD and set the groundwork for genetic testing in MD.

Strategies for the practice of otolaryngology and head and neck surgery during the monitoring phase of COVID-19. / Estrategias para la práctica de la otorrinolaringología y cirugía de cabeza y cuello durante la fase de control de la COVID-19.

The appearance of a new coronavirus disease called COVID-19 at the end of 2019 and its pandemic expansion in the world has changed the usual practice of the specialty of Otorhinolaryngology (ENT). After a phase of exponential growth of infections, it has been possible to enter a phase of control of the spread of the disease in which the possibility of infection persists, and the appearance of new cases is considered acceptable by the health system. The aim of this document is to review the available evidence and propose strategies and recommendations for the medical-surgical practice of otorhinolaryngology and head and neck surgery, which allow establishing the usual activity, adapting the safety and efficacy standards to the current situation. Therefore, it is required to identify and classify patients according to criteria of infectious-immunological status, and to establish recommendations for protection in consultations, hospitalization and the operating room, which avoid the transmission of the disease to other users and healthcare personnel, in the specific context of the development of our specialty. This document is the result of the collaboration of all the scientific commissions and the SEORLCCC COVID-19 committee.

[Strategies for the practice of otolaryngology and head and neck surgery during the monitoring phase of COVID-19]. / Estrategias para la práctica de la otorrinolaringología y cirugía de cabeza y cuello durante la fase de control de la COVID-19.

The appearance of a new coronavirus disease called COVID-19 at the end of 2019 and its pandemic expansion in the world has changed the usual practice of the specialty of Otorhinolaryngology (ENT). After a phase of exponential growth of infections, it has been possible to enter a phase of control of the spread of the disease in which the possibility of infection persists, and the appearance of new cases is considered acceptable by the health system.The aim of this document is to review the available evidence and propose strategies and recommendations for the medical-surgical practice of otorhinolaryngology and head and neck surgery, which allow establishing the usual activity, adapting the safety and efficacy standards to the current situation. Therefore, it is required to identify and classify patients according to criteria of infectious-immunological status, and to establish recommendations for protection in consultations, hospitalization and the operating room, which avoid the transmission of the disease to other users and healthcare personnel, in the specific context of the development of our specialty. This document is the result of the collaboration of all the scientific commissions and the SEORLCCC COVID-19 committee.

Differential Proinflammatory Signature in Vestibular Migraine and Meniere Disease.

Vestibular Migraine (VM) and Meniere's Disease (MD) are episodic vestibular syndromes defined by a set of associated symptoms such as tinnitus, hearing loss or migraine features during the attacks. Both conditions may show symptom overlap and there is no biological marker to distinguish them. Two subgroups of MD patients have been reported, according to their IL-1ß profile. Therefore, considering the clinical similarity between VM and MD, we aimed to investigate the cytokine profile of MD and VM as a means to distinguish these patients. We have also carried out gene expression microarrays and measured the levels of 14 cytokines and 11 chemokines in 129 MD patients, 82 VM patients, and 66 healthy controls. Gene expression profile in peripheral blood mononuclear cells (PBMC) showed significant differences in MD patients with high and low basal levels of IL- 1ß and VM patients. MD patients with high basal levels of IL- 1ß (MDH) had overall higher levels of cytokines/chemokines when compared to the other subsets. CCL4 levels were significantly different between MDH, MD with low basal levels of IL- 1ß (MDL), VM and controls. Logistic regression identified IL- 1ß, CCL3, CCL22, and CXCL1 levels as capable of differentiating VM patients from MD patients (area under the curve = 0.995), suggesting a high diagnostic value in patients with symptoms overlap.

Guía de Práctica Clínica Para el Diagnóstico y Tratamiento del Vértigo Posicional Paroxístico Benigno. Documento de Consenso de la Comisión de Otoneurología Sociedad Española de Otorrinolaringlogía y Cirugía de Cabeza y Cuello / Practice Guidelines for the Diagnosis and Management of Benign Paroxysmal Positional Vertigo Otoneurology Committee of Spanish Otorhinolaryngology and Head and Neck Surgery Consensus Document

El vértigo posicional paroxístico benigno (VPPB) es la causa más frecuente de vértigo vestibular episódico. EL propósito de esta guía, encomendada por la Comisión de Otoneurología de la SEORL CCC, es disponer de un documento de consenso que sirva de guía práctica para el manejo del VPPB en la clínica diaria. El punto de partida es la clasificación elaborada por la Barany Society, con sus variantes clínicas. Incluye una descripción de las pruebas diagnósticas y de las maniobras terapéuticas para cada una de las variantes establecidas, habiéndose seleccionado aquellas con estudios con nivel adecuado de evidencia o con suficientes series de soporte. Se ha incluido también un capítulo de diagnóstico diferencial, así como un apartado de aspectos generales básicos en el manejo de los pacientes con VPPB

Benign Paroxysmal Positional Vertigo is the most frequent episodic vestibular disorder. The purpose of this guide, requested by the committee on otoneurology of the Spanish Society of Otolaryngology and Head and Neck Surgery, is to supply a consensus document providing practical guidance for the management of BPPV. It is based on the Barany Society criteria for the diagnosis of BPPV. This guideline provides recommendations on each variant of BPPV, with a description of the different diagnostic tests and the therapeutic manoeuvres. For this purpose, we have selected the tests and manoeuvres supported by evidence-based studies or extensive series. Finally, we have also included a chapter on differential diagnosis and a section relating to general aspects in the management of BPPV

Proinflammatory cytokines and response to molds in mononuclear cells of patients with Meniere disease.

Epidemiological studies have found a higher prevalence of allergic symptoms and positive prick tests in patients with Meniere's disease (MD); however the effect of allergenic extracts in MD has not been established. Thus, this study aims to determine the effect of Aspergillus and Penicillium stimulation in cytokine release and gene expression profile in MD. Patients with MD showed higher basal levels of IL-1ß, IL-1RA, IL-6 and TNF-α when compared to healthy controls. We observed that IL-1ß levels had a bimodal distribution suggesting two different subgroups of patients, with low and high basal levels of cytokines. Gene expression profile in peripheral blood mononuclear cells (PBMC) showed significant differences in patients with high and low basal levels of IL-1ß. We found that both mold extracts triggered a significant release of TNF-α in MD patients, which were not found in controls. Moreover, after mold stimulation, MD patients showed a different gene expression profile in PBMC, according to the basal levels of IL-1ß. The results indicate that a subset of MD patients have higher basal levels of proinflammatory cytokines and the exposure to Aspergillus and Penicillium extracts may trigger additional TNF-α release and contribute to exacerbate inflammation.

Practice Guidelines for the Diagnosis and Management of Benign Paroxysmal Positional Vertigo Otoneurology Committee of Spanish Otorhinolaryngology and Head and Neck Surgery Consensus Document. / Guía de Práctica Clínica Para el Diagnóstico y Tratamiento del Vértigo Posicional Paroxístico Benigno. Documento de Consenso de la Comisión de Otoneurología Sociedad Española de Otorrinolaringlogía y Cirugía de Cabeza y Cuello.

Benign Paroxysmal Positional Vertigo is the most frequent episodic vestibular disorder. The purpose of this guide, requested by the committee on otoneurology of the Spanish Society of Otolaryngology and Head and Neck Surgery, is to supply a consensus document providing practical guidance for the management of BPPV. It is based on the Barany Society criteria for the diagnosis of BPPV. This guideline provides recommendations on each variant of BPPV, with a description of the different diagnostic tests and the therapeutic manoeuvres. For this purpose, we have selected the tests and manoeuvres supported by evidence-based studies or extensive series. Finally, we have also included a chapter on differential diagnosis and a section relating to general aspects in the management of BPPV.

Oculographic, Clinical Test of Sensory Integration and Balance and Computerized Dynamic Posturography Findings in Patients With Psoriatic Arthritis.

OBJECTIVE: To assess the frequency and characteristics of the oculographic findings and the usefulness of the clinical test of sensory integration and balance (CTSIB) for the evaluation of balance in patients with psoriatic arthritis (PsA) by comparing this test with the computerized dynamic posturography (CDP). STUDY DESIGN: A series of consecutive patients that fulfilled the Moll and Wright criteria for PsA and matched controls were studied. SETTING: The study was performed at the Otolaryngology Division of a tertiary reference center. INTERVENTION: Vestibular evaluation including CTSIB followed by CDP was performed to all patients and age, sex, and ethnically frequency-matched controls. MAIN OUTCOME MEASURE: Patterns of CTSIB and CDP (gold standard) were assessed and compared. PATIENTS: Sixty PsA patients (63.3% women) and 60 matched controls. RESULTS: PsA patients had higher frequency of abnormal oculocephalic response (13.3%) and abnormal caloric test (26.7%) than controls (0% in both cases) (p = 0.006 and p < 0.001, respectively). Benign paroxysmal positional vertigo (BPPV) was diagnosed in two (3.4%) patients and none of the controls (p = 1). Significantly increased frequency of abnormal CTSIB test with vestibular loss pattern (33.3%) in patients compared with controls (6%) was observed (p = 0.002). Significantly increased frequency of abnormal CDP was also observed in PsA patients (14 [23.3%] versus 0 [0%] of controls) (p < 0.001). CTSIB yielded 100% sensitivity, 73% specificity, 48% positive predictive value, and 100% negative predictive value. CONCLUSIONS: This study indicates that oculographic findings are common in PsA. CTSIB is useful for assessing balance disorder screening in the routine clinical practice in these patients.

Particle repositioning maneuver versus Brandt-Daroff exercise for treatment of unilateral idiopathic BPPV of the posterior semicircular canal: a randomized prospective clinical trial with short- and long-term outcome.

OBJECTIVE: To compare the outcome and probability of recurrence in a series of patients with unilateral idiopathic benign paroxysmal positional vertigo of the posterior canal (PC-BPPV) that were randomly treated by Brandt-Daroff exercise (B-D exercise) or by particle repositioning maneuver (PRM). STUDY DESIGN: Randomized prospective clinical trial. SETTING: Tertiary referral center. PATIENTS: Patients were included in this study if they complained of vertigo and had been diagnosed as having unilateral idiopathic PC-BPPV for at least 1 week before Dix-Hallpike maneuver (DHM), remained for 30 days in the randomly assigned treatment, and had at least 48 months' follow-up. INTERVENTION: Forty-one patients were treated with a single PRM and 40 patients by B-D exercise. MAIN OUTCOME MEASURE: Resolution of benign paroxysmal positional nystagmus on the DHM. The probability of recurrence was also studied. RESULTS: At Day 7, DHM was negative in 80.5% of the PRM-treated patients and in 25% of those treated by B-D exercise (p < 0.001). At Month 1, the differences between both treatment groups remained statistically significant (92.7% in PRM versus 42.5% in the B-D exercise had a negative DHM; p < 0.001). The variable that influenced that DHM became negative was the PRM (RR = 4.8; 95% confidence interval, 2.5-9.2; p < 0.001). The number of recurrences in PRM and B-D exercise were 0.56 ± 0.8 and 0.48 ± 0.8, respectively (p = 0.48). The recurrence rate at 48 months was 35.5% (15/41) in B-D exercise and 36.6% (9/31) in the PRM group (p = 0.62). Although the time interval until the first recurrence was similar (p = 0.44), patients included in the PRM group showed a significantly longer time interval between the first and second recurrence (p = 0.04). CONCLUSION: PRM is more effective treatment and as safe as B-D exercise in the short term for unilateral and idiopathic PC-BPPV, and although it does not reduce the probability of recurrence in the 4-year follow-up period compared with B-D exercise, it may delay the second recurrence's onset in those patients who had already experienced a single recurrence. Our study supports the use of PRM as the treatment of choice in unilateral and idiopathic PC-BPPV, although exercise may be also considered as an alternative treatment in selected cases.

Manifestaciones audiovestibulares en las vasculitis sistémicas / Audiovestibular manifestations in systemic vasculitis

Las vasculitis sistémicas son un grupo heterogéneo de enfermedades con solapamiento de las manifestaciones clínicas y el tamaño de los vasos afectados. Su etiología puede ser primaria o secundaria. La característica común es la inflamación de los vasos sanguíneos que causa los fenómenos isquémicos. En esta revisión se lleva a cabo una descripción de las vasculitis y sus manifestaciones audiovestibulares conocidas hasta la fecha. La clínica audiovestibular puede ser la primera manifestación de una vasculitis sistémica. Entre las manifestaciones auditivas predomina la hipoacusia súbita y la neurosensorial progresiva, mientras que entre las vestibulares predominan vértigo y nistagmo. En la exploración vestibular destacan la hipofunción vestibular en la prueba calórica, las alteraciones en las pruebas de agitación cefálica, oculocefálica y posicionales (vértigo posicional paroxístico benigno). El tratamiento con esteroides suele obtener mejoría de los síntomas y la disfunción audiovestibular en la mayoría de los pacientes con arteritis de células gigantes (AU)

Systemic vasculitides constitute a heterogeneous group of diseases with frequent overlapping in their clinical findings and the size of the vessels involved. They may be primary or secondary to other diseases. Their common characteristic is the inflammation of blood vessels, giving rise to ischaemia. In the present article, we have reviewed the most common audiovestibular features that may be observed in patients with systemic vasculitides. Audiovestibular manifestations may be the first symptom of a systemic vasculitis. Audiological manifestations include sudden hearing loss and progressive sensorineural hearing loss. Vertigo and nystagmus are vestibular symptoms that may be observed in several systemic vasculitides. Vestibular loss in caloric test, abnormal head-shaking nystagmus, head thrust test and positioning test (benign paroxysmal positioning vertigo) may also be found. Improvement of audiovestibular function is frequently observed in patients with giant cell arteritis following corticosteroid therapy (AU)

[Audiovestibular manifestations in systemic vasculitis]. / Manifestaciones audiovestibulares en las vasculitis sistémicas.

Systemic vasculitides constitute a heterogeneous group of diseases with frequent overlapping in their clinical findings and the size of the vessels involved. They may be primary or secondary to other diseases. Their common characteristic is the inflammation of blood vessels, giving rise to ischaemia. In the present article, we have reviewed the most common audiovestibular features that may be observed in patients with systemic vasculitides. Audiovestibular manifestations may be the first symptom of a systemic vasculitis. Audiological manifestations include sudden hearing loss and progressive sensorineural hearing loss. Vertigo and nystagmus are vestibular symptoms that may be observed in several systemic vasculitides. Vestibular loss in caloric test, abnormal head-shaking nystagmus, head thrust test and positioning test (benign paroxysmal positioning vertigo) may also be found. Improvement of audiovestibular function is frequently observed in patients with giant cell arteritis following corticosteroid therapy.