Validation of a French version of the Sunnybrook facial grading system.

BACKGROUND: The aim of this study was to translate the Sunnybrook Facial Grading System and its specific criteria into French and validate its use by French-speaking physicians for facial palsy evaluation. MATERIAL AND METHODS: The original English version of the Sunnybrook Facial Grading System and its specific criteria was translated into French according to international standards. Twenty videos of patients with a wide range of facial palsy in terms of duration and severity were independently rated, twice each, by 6 physicians with varied experience in facial palsy care. Internal consistency and intra- and inter-rater reliability were analyzed. RESULTS: The French version of Sunnybrook Facial Grading System and its specific criteria both showed good internal consistency, with Cronbach alpha of 0.84 and 0.86 respectively. Inter-rater reliability was excellent in both sessions for the composite score, the score of symmetry at rest and during voluntary movement and synkinesis: intraclass correlation coefficient (ICC) between 0.77 and 0.98. Intra-rater reproducibility on the composite score and subscores was also excellent and comparable for expert, experienced and novice physicians, with an average ICC of 0.95. CONCLUSION: The French version of the Sunnybrook Facial Grading System and its specific criteria is reliable, reproducible and easy to use by French-speaking teams for facial palsy evaluation.

Musical Ear Syndrome: Prevalence and characteristics in cochlear implant bearers.

INTRODUCTION: Musical Ear Syndrome (MES) is an uncommon phenomenon described as the perception of auditory musical sensations not corresponding to any external stimulus. It seems to be more frequent in case of profound hearing loss. Our objective was to evaluate prevalence, characteristics and risk factors in a population of cochlear implant patients. METHODS: A retrospective study was conducted in cochlear implant patients, who were adult (>18 years) in 2020 and underwent cochlear implantation between 1993 and 2019. We analyzed the presence and characteristics of MES. RESULTS: 118 of the 358 patients (33%) perceived or had perceived auditory musical sensations: 71 (19.8%) before, 100 (28%) after, and 53 (14.8%) both before and after implantation. The musical auditory sensations were usually short and well-tolerated, resembling instrumental music, and occurring several times a day. Thirteen patients (11%) considered them intolerable. Fatigue was a triggering factor in 40 patients (33.9%). Personal and medical characteristics, type of implantation, make of implant, etiology and tinnitus did not emerge as risk factors. On the other hand, MES+ patients were significatively younger (56±17.4 years versus 61.9±17.9 years; P=0.0009). Despite the phenomenon, patients were satisfied with implant functioning and subjective auditory performance was not affected. CONCLUSION: Prevalence of Musical Ear Syndrome was high in cochlear implant patients, and especially in younger subjects. It is essential to improve knowledge of this phenomenon.

Sarco(endo)plasmic reticulum Ca(2+)-ATPase (SERCA2) gene products are regulated post-transcriptionally during rat cardiac development.

OBJECTIVE: The Sarco(endo)plasmic reticulum Ca(2+)-ATPase (SERCA2) plays a major role in the contraction-relaxation cycle and is responsible for transporting calcium into the lumen of the sarcoplasmic reticulum. This study was performed to determine if the increase in SERCA2 messenger RNA (mRNA) abundance during the perinatal period is regulated transcriptionally. METHODS: Transcriptional activity was determined by nuclear run-on assays and mRNA and protein abundances were determined during late fetal and early neonatal cardiac development in rat. RESULTS: From nuclear run-on assays, SERCA2 gene transcription at 17/18 embryonic days (139 +/- 41 parts per million (ppm), n = 7) did not differ from that at 20 neonatal days (139 +/- 37 ppm, n = 6) after birth. No increase in transcriptional activity could be demonstrated during the time frame examined. In contrast, both alpha and beta myosin heavy chains showed significant changes in measured transcriptional activity. SERCA2 mRNA normalized to 18S RNA levels are very low in the fetus (9.8 +/- 1.9 to 13.4 +/- 4.9 arbitrary units (A.U.) from 17/18 to 19/20 embryonic days) and significantly increase from birth (15 +/- 3.8 A.U.) to reach a maximum at 20 days of age (29.1 +/- 9.5 to 48.3 +/- 7.0 in 15 to 20 neonatal days rats respectively). Similarly, SR Ca(2+)-ATPase protein levels are less abundant in the fetus (0.82 +/- 0.08 to 1.13 +/- 0.13 A.U./microgram total protein) and reach a maximum at 15-20 neonatal days (3.08 +/- 0.58 to 2.98 +/- 0.17). Ca2+ uptake in the fetal heart is about one sixth the level seen in the adult, reaches the highest observed value at 5 days after birth (6.05 +/- 0.77 pmole Ca2+ per microgram/min) and remains relatively constant over the next 15 days. The activity increases even though phospholamban protein increases in abundance. CONCLUSIONS: Since the transcriptional activity of this gene is unchanged whereas the mRNA, protein abundance and activity increase, we conclude that the abundance of SERCA2 gene products is regulated primarily through post-transcriptional mechanisms during the perinatal period.

Angiotensin I-converting enzyme genotype influences arterial response to injury in normotensive rats.

Two normotensive strains of rat, the Lou and Brown Norway (BN) strains, have contrasting levels of plasma angiotensin-converting enzyme (ACE). To investigate the degree of genetic determination of ACE expression, a polymorphic marker of the ACE gene was analyzed in inbred rats of the two strains. The two inbred strains were shown to bear different alleles for a polymorphic marker at the ACE gene. The segregation of the alleles of this marker and the plasma ACE levels were studied in a group of F2 rats issued from a cross between Lou and BN rats. The degree of genetic determination of plasma ACE activity was estimated to be 94% in the F2 cohort. The ACE locus accounts for 74% of total plasma ACE variance. ACE activity and mRNA expression in lungs were also genetically determined. The difference observed in ACE mRNA accumulation in the lungs between the two strains was due to a difference in the transcriptional rate of the ACE gene, as shown in nuclear run-on experiments. No differences were observed in arterial blood pressure of homozygous F2 progeny. In these animals, ACE genotype did not interfere with the pressor or the depressor responses to ACE-dependent vasoactive peptides. There was a significant effect of strain on constitutive or inducible membrane or soluble ACE activity in primary cultures of vascular cells. Neointima formation in the carotid artery 14 days after balloon injury was also influenced by the genotype in F2 homozygous progeny, whereas the medial area was not. These results demonstrate that there is a close relationship between the genetically determined ACE expression and the inducibility of the ACE gene. The degree of genetic determination of ACE expression in inbred rat strains offers a unique opportunity to study the interaction between genetic and environmental determinants of ACE expression and its involvement in response to experimental cardiovascular and renal injury.

The sarco(endo)plasmic reticulum Ca(2+)-ATPase gene is regulated at the transcriptional level during compensated left ventricular hypertrophy in the rat.

In mammalian myocardium, relaxation is mainly triggered by the reuptake of calcium from the cytosol to the lumen of the sarcoplasmic reticulum (SR) through the cardiac isoform of the sarco(endo)plasmic reticulum calcium ATPase, SERCA2a. Relaxation abnormalities related to deficient SR Ca(2+)-uptake have been identified in human heart failure and in animal models of cardiac hypertrophy and failure. These alterations have been associated with a reduction in SERCA2a activity and in steady-state SERCA2a protein and mRNA levels. As a first step in the analysis of the mechanisms responsible for this reduction, we have studied a possible down-regulation of the SERCA2 gene transcription during left ventricular hypertrophy (LVH) induced by constriction of the ascending aorta in the rat. Quantifications of the mRNA levels demonstrated no alteration, compared to sham-operated rats, at 5 d after imposition of the pressure overload, whereas a significant decrease was observed at 11 d. Transcription in-vitro experiments (cardiac nuclear run-on assays) performed in isolated cardiomyocytes nuclei showed no changes at 5 d and a 37% reduction of the SERCA2 gene transcription at 11 d. These results strongly suggest that SERCA2 gene expression down-regulation during cardiac hypertrophy occurs, at least in part, at the level of the transcription.