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. We have defined five clinical variants in BMD. Group 1 is the most frequently found, includes 46% of patients, and is defined by metachronic hearing loss without migraine and without AD. Group 2 is found in 17% of patients, and it is defined by synchronic hearing loss without migraine or AD. Group 3, with 13% of patients, is characterized by familial MD, while group 4, that includes 12% of patients, is associated by the presence of migraine in all cases. Group 5 is found in 11% of patients and is defined by AD. This approach can be helpful in selecting patients for genetic and clinical research. However, further studies will be required to improve the phenotyping in these clinical variants for a better understanding of the diverse etiological factors contributing to BMD

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

<p>Abstract</p> <p>Background</p> <p>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. We examined single-nucleotide polymorphism (SNPs) in the CD16A and CD32 genes in patients with MD which may determine a Fcγ receptor with lower binding to CIC.</p> <p>Methods</p> <p>The functional CD16A (FcγRIIIa*559A > C, rs396991) and CD32A (FcγRIIa*519A > G, rs1801274) SNPs were analyzed using PCR-based TaqMan Genotyping Assay in two cohorts of 156 mediterranean and 112 Galicia patients in a case-control study. Data were analyzed by χ²with Fisher's exact test and Cochran-Armitage trend test (CATT). CIC were measured by ELISA for C1q-binding CIC.</p> <p>Results</p> <p>Elevated CIC were found in 7% of patients with MD during the intercrisis period. No differences were found in the allelic frequency for rs396991 or rs1801274 in controls subjects when they were compared with patients with MD from the same geographic area. However, the frequency of AA and AC genotypes of CD16A (rs396991) differed among mediterranean and Galicia controls (Fisher's test, corrected p = 6.9 × 10^-4for AA; corrected p = 0.02 for AC). Although genotype AC of the CD16A receptor was significantly more frequent in mediterranean controls than in patients, [Fisher's test corrected p = 0.02; OR = 0.63 (0.44-0.91)], a genetic additive effect for the allele C was not observed (CATT, p = 0.23). Moreover, no differences were found in genotype frequencies for rs396991 between patients with MD and controls from Galicia (CATT, p = 0.14). The allelic frequency of CD32 (rs1801274) was not different between patients and controls either in mediterranean (p = 0.51) or Galicia population (p = 0.11).</p> <p>Conclusions</p> <p>Elevated CIC are not found in most of patients with MD. Functional polymorphisms of CD16A and CD32 genes are not associated with onset of MD.</p

Alternate Splicing of Interleukin-1 Receptor Type II (IL1R2) In Vitro Correlates with Clinical Glucocorticoid Responsiveness in Patients with AIED

Autoimmune Inner Ear Disease (AIED) is poorly characterized clinically, with no definitive laboratory test. All patients suspected of having AIED are given glucocorticoids during periods of acute hearing loss, however, only half initially respond, and still fewer respond over time

Utilidad de la videonistagmografía en el diagnóstico y tratamiento del VPPB

Presentamos ejemplos de nistagmus registrados mediante VNG (Videonistagmografía), tanto en la maniobra de Dix-Hallpike como en la maniobra liberadora de Epley. Discutimos su fisiopatología y confirmamos la utilidad de la VNG en el diagnóstico VPPB, en su tratamiento y control evolutivo, concluimos que la VNG es la prueba de elección en el estudio del CSP (canal semicircular posterior)

Cofosis bilateral secundaria a infección por Estreptococo suis

We report a medical case of a 38-year-old man admitted to our hospital for severe deafness and fever for 5 days with a history of pig handling. A B-haemolytic streptococcus was grown from both blood and CSF identified as Streptococcus suis. Streptococcus suis infection is rare in humans and often presents as meningitis with the sequela of permanent deafness.Presentamos un caso de un varón de 38 años que ingresa con fiebre e hipoacusia severa de 5 dias de evolución con antecedentes de manipulación de ganado porcino. Un Estreptococo B-hemolitico creció en el hemocultivo y cultivo de LCR identificado como Estreptococo suis. La infección por Estreptococo suis es rara en humanos y habitualmente se presenta como meningitis con secuela de hipoacusia permanent

Osteomielitis del seno frontal. Caso clínico.

Se presenta un caso de osteomielitis del seno frontal con destrucción de tabla interna. Revisamos la etiología, vías de propagación, diagnóstico y tratamient

Lamin A/C Ablation Restricted to Vascular Smooth Muscle Cells, Cardiomyocytes, and Cardiac Fibroblasts Causes Cardiac and Vascular Dysfunction.

Mutations in the LMNA gene (encoding lamin A/C proteins) cause several human cardiac diseases, including dilated cardiomyopathies (LMNA-DCM). The main clinical risks in LMNA-DCM patients are sudden cardiac death and progressive left ventricular ejection fraction deterioration, and therefore most human and animal studies have sought to define the mechanisms through which LMNA mutations provoke cardiac alterations, with a particular focus on cardiomyocytes. To investigate if LMNA mutations also cause vascular alterations that might contribute to the etiopathogenesis of LMNA-DCM, we generated and characterized Lmnaflox/floxSM22αCre mice, which constitutively lack lamin A/C in vascular smooth muscle cells (VSMCs), cardiac fibroblasts, and cardiomyocytes. Like mice with whole body or cardiomyocyte-specific lamin A/C ablation, Lmnaflox/floxSM22αCre mice recapitulated the main hallmarks of human LMNA-DCM, including ventricular systolic dysfunction, cardiac conduction defects, cardiac fibrosis, and premature death. These alterations were associated with elevated expression of total and phosphorylated (active) Smad3 and cleaved (active) caspase 3 in the heart. Lmnaflox/floxSM22αCre mice also exhibited perivascular fibrosis in the coronary arteries and a switch of aortic VSMCs from the 'contractile' to the 'synthetic' phenotype. Ex vivo wire myography in isolated aortic rings revealed impaired maximum contraction capacity and an altered response to vasoconstrictor and vasodilator agents in Lmnaflox/floxSM22αCre mice. To our knowledge, our results provide the first evidence of phenotypic alterations in VSMCs that might contribute significantly to the pathophysiology of some forms of LMNA-DCM. Future work addressing the mechanisms underlying vascular defects in LMNA-DCM may open new therapeutic avenues for these diseases.This study was supported by grants SAF2016-79490-R and PID2019-108489RB-I00 from the Spanish Ministerio de Ciencia e Innovación (MCIN)/Agencia Estatal de Investigación (AEI)/10.13039/ 501100011033, with co-funding from the European Social Fund (“The ESF invests in your future”). Microscopy was conducted at the Microscopy and Dynamic Imaging Unit, CNIC, ICTS-ReDib, co-funded by MCIN/AEI/10.13039/501100011033. A.D.M.-M. was supported by the MCIN (predoctoral contract BES-2014-067791), C.E.-E. and V.F. by the Fundación “la Caixa” (predoctoral contracts LCF/BQ/DR19/1170012 and LCF/BQ/DE14/10320024, respectively), Í.R.-P.d.L. by MCIN/AEI/ 10.13039/501100011033 and the European Social Fund (“The ESF invests in your future”) (predoctoral contract PRE2020-092264), and M.G.-A. by MCIN (post-doctoral contract FJC 2021-047576-I). The CNIC is supported by the MCIN, the Instituto de Salud Carlos III, and the Pro-CNIC Foundation and is a Severo Ochoa Center of Excellence (grant number CEX2020-001041-S funded by MCIN/AEI/10.13039/501100011033).S

Late holocene environments in Las Tablas de Daimiel (south central Iberian peninsula, Spain)

The use of a high resolution pollen record in combination with geochemical data from sediments composed mainly of layers of charophytes alternating with layers of vegetal remains plus some detrital beds permits the reconstruction of the environmental evolution of the last 3000 years in an inland wetland of the Mediterranean domain, thus introducing a new climatic dataset for the Late Holocene. Hydrological fluctuations, reflected in the relationship between emerged and aquatic vegetation and inorganic and organic C and N changes, can be related to aridity or humid phases, while relations among arboreal taxa (Quercus and Pinus) and Artemisia are used as temperature indicators. Five climatic periods have been identified: a Subatlantic Cold Period (<150 b.c.), cold and arid; the RomanWarm Period (150 b.c.–a.d. 270), warmer and wetter; the Dark Ages (a.d. 270–a.d. 950), colder and drier; the Medieval Warm Period (a.d. 950–a.d. 1400), warmer and wetter; and the Little Ice Age (>a.d. 1400) indicated by a cooling and drying trend. Despite the lack of any direct evidence of human action, there are some episodes related to deforestation during the Reconquista (Middle Ages) that mask the real climatic signal