Paediatric vestibular assessment in French cochlear implant centres: Challenges and improvement areas.

OBJECTIVE: Survey of paediatric vestibular activity in all 30 French paediatric cochlear implant (CI) centres to identify challenges and areas of improvement. METHOD: All 30 French CI centres answered a 29-question questionnaire about their paediatric vestibular activity, equipment, and management in different clinical situations (e.g. vestibular assessment before a cochlear implantation or in cases of vertigo) at different ages. RESULTS: Eighteen CI centres had dedicated paediatric vestibular clinics and 12 did not. Minimum age required for vestibular testing was 3 years in eight centres. Four vestibular tests stood out: caloric tests, video Head Impulse Test (vHIT), rotating chair, vestibular evoked myogenic potentials (VEMP). Depending on the centre's experience, the use of vestibular tests in clinical routine was very heterogeneous. Expert centres mostly used vHIT and cervical VEMP (in bone conduction) for assessments before the first cochlear implantation in 1-year-old children. Dizziness assessment in 4-year children was based on the use of vHIT, cervical VEMP on bone conduction, rotatory test, and caloric test. Ocular VEMP was rarely used. CONCLUSIONS: Paediatric vestibular assessment requires specific expertise compared to adults. Due to a lack of specialised human resources, some centres may be unable to follow French paediatric CI guidelines. International recommendations could help standardise paediatric vestibular management and public health policies should be discussed to improve training and access for children.

Benefits of active listening during 3D sound localization.

In everyday life, sound localization entails more than just the extraction and processing of auditory cues. When determining sound position in three dimensions, the brain also considers the available visual information (e.g., visual cues to sound position) and resolves perceptual ambiguities through active listening behavior (e.g., spontaneous head movements while listening). Here, we examined to what extent spontaneous head movements improve sound localization in 3D-azimuth, elevation, and depth-by comparing static vs. active listening postures. To this aim, we developed a novel approach to sound localization based on sounds delivered in the environment, brought into alignment thanks to a VR system. Our system proved effective for the delivery of sounds at predetermined and repeatable positions in 3D space, without imposing a physically constrained posture, and with minimal training. In addition, it allowed measuring participant behavior (hand, head and eye position) in real time. We report that active listening improved 3D sound localization, primarily by ameliorating accuracy and variability of responses in azimuth and elevation. The more participants made spontaneous head movements, the better was their 3D sound localization performance. Thus, we provide proof of concept of a novel approach to the study of spatial hearing, with potentials for clinical and industrial applications.

Vestibular impairment in cochlear implanted children presenting enlarged vestibular aqueduct and enlarged endolymphatic sac.

Enlarged vestibular aqueduct (EVA) is a common finding in tomodensitometry. When cranial MRI is performed, enlarged endolymphatic sac (EES) can also be found. Profound hearing loss is a common finding in these patients but a few studies have investigated vestibular function after cochlear implantation (CI) in EVA and EES patients. Our main objective was to find out whether in EVA children candidates to CI, a higher endolymphatic sac (ES) volume was predictive for higher rates of postsurgical vestibular complications. METHODS: We retrospectively included EVA children who benefited from CI, during the last 2 years. Two groups were constituted according to the presence or not of a vestibular impairment (decrease in the VOR gain on the VHIT test on one of the semicircular canals and/or a loss of cVEMPs) 6 months after CI. Endolymphatic volume of both VA and ES was measured for each patient. RESULTS: Fifteen patients were included. The mean endolymph volume was significantly higher in the impaired group (0.40 cm3 ± 0.23, range 0.08-0.70) than in the non-impaired group (0.11 cm3 ± 0.07, range 0.04-0.29; p = 0.029). Four children of the impaired group were followed during one year. At the end of vestibular rehabilitation, all children recovered a lateral canal function and a saccular function. CONCLUSION: In EVA children, a combined EES appears to increase the risk of severe post CI vestibular impairment. To minimize this risk prior CI surgery, besides tomodensitometry, MRI measurement of the ES volume should be systematically performed.

The French National Cochlear Implant Registry (EPIIC): Cochlear explantation and reimplantation.

This study aims to determine the frequency and causes of cochlear explants with re-implantation (ERI) after 5 years' follow up of the patients included in the French national EPIIC (étude post-inscription des implants cochléaires) registry tracking patients with cochlear implantation. This multicenter, descriptive prospective study was conducted on 5051 patients enrolled in the EPIIC database between January 2012 and December 2016. Ninety-five patients (1.9%) received a primary implant and an ERI during the study. Of these, four benefitted from two ERIs. The number of ERIs was significantly higher in the pediatric population than among adults. The explantation and reimplantation were performed simultaneously in 86% of cases. The reasons for explantation were: in 46.4% of cases linked to a malfunction of the implant, and in 39.3% of cases for medical or surgical reasons. The number of electrodes inserted was significantly higher after the ERI than after the first implantation. There was just one post-ERI infection for these 95 explanted and re-implanted patients. As well as explantation with reimplantation rarely being necessary, it generally presents no major surgical difficulty and in most cases it allows a better integration than in the first implantation.

[Clinical presentation and performance of urine dipstick for diagnosis of urinary infection in geriatric population]. / Présentation clinique et performance de la bandelette urinaire pour le diagnostic d'infection urinaire en population gériatrique.

BACKGROUND: Urinary tract infections (UTI) are the second cause of community-acquired bacterial infections in the elderly. Distinguishing symptomatic UTI from asymptomatic bacteriuria is problematic, as older adults are less likely to present with localized urinary symptoms. We evaluated characteristics of patients presenting UTI among elderly with sepsis. Moreover, we aimed to evaluate the sensibility and specificity of urine dipstick tests in the diagnosis of UTI in geriatric population. PATIENTS AND METHOD: We led a prospective, monocentric, observational study between April 2017 and January 2018. We included patients hospitalized in geriatric wards, who were prescribed urine culture for UTI symptoms or/and infection without primary sites for which a urine culture was prescribed. Dipstick urinalyses were performed for all patients. Clinical and biological characteristics of all patients were compared according to the final diagnosis of UTI. Moreover, results of dipstick tests were evaluated for the diagnosis of UTI in this population. RESULTS: Among 165 patients, 67 (40.6 %) had a UTI and 98 (59.4 %) had another diagnosis. These two groups were comparable for age and daily-living activities. In the UTI group, the proportion of women was higher than in the other group (P<0.05), and mean MMSE score was lower (P<0.05). Positive urine dipstick test for leukocytes and/or nitrites had high sensitivity (92 %), but low specificity (50 %). Negative predictive value of this test was high (91 %). CONCLUSION: For suspicion of UTI among elderly, few criteria are specific. Negative dipstick tests can suggest an absence of UTI due to its high negative predictive value.

Evolution of sinonasal clinical features in children with cystic fibrosis.

OBJECTIVE: to assess the evolution of sinonasal manifestations in children with cystic fibrosis, since the improvement of their prognosis over the last decades. METHODS: an observational, monocentric study with a retrospective cohort. We included 173 children (from 4 to 18 years old) with cystic fibrosis followed at the pediatric cystic fibrosis center of lyon, france. We collected respiratory, infectious and nutritional data, sinonasal complaints and physical examination at the onset of sinonasal symptoms (t-0), at the most severe of evolution (t-max) and at the end of followup (t-end). RESULTS: sinonasal symptomatology appeared early around 5.4 years old, then rapidly reached the maximum at 6.9 years and finally improved during childhood (p < 0.0001), reaching scores at t-end significantly better than at t-0 (p < 0.0001). This evolution was significant for nasal obstruction, rhinorrhea and snoring. The other symptoms were rarer, with no significant 38,7% at t-max (p < 0.0001), and 29,5% at t-end (p = 0.52). The lildholdt score, turbinate hypertrophy and medial bulging of medial wall of the maxillary sinus followed the same evolution (p < 0.003). There was no association between sinonasal evolution and cystic fibrosis disease at infectious, respiratory or nutritional level. CONCLUSION: it is the only recent study evaluating the evolution of each sinonasal manifestations in children with cystic fibrosis. Rhinosinusitis improved during childhood, reaching better scores than at the beginning of management. This particular improvement may be related to good effectiveness to ent management, but also to a positive effect of nasal cavity growth, independently to extra-ent manifestations.

Otorhinolaryngological manifestations and delayed diagnosis in Kawasaki disease.

OBJECTIVES: Kawasaki disease (KD) is a febrile multisystemic vasculitis of unknown etiology whose coronary prognosis is improved by early diagnosis and management. The objective of this study was to describe ENT manifestations encountered and to look for a delayed diagnosis associated with these manifestations. METHODS: A retrospective descriptive single-center study was conducted in Lyon between January 2009 and December 2017. All children treated for Kawasaki disease were included in the study. Clinical, biological and cardiac ultrasound data were collected. According to the diagnosis made at the first medical visit, children were classified into two groups: diagnosis of ENT spectrum or non-ENT diagnosis. The diagnostic times were compared by a Student test. RESULTS: 142 patients were included: 64 in the ENT diagnostic group, 78 in the non-ENT diagnostic group. When the initial diagnosis was of ENT spectrum, the diagnostic time of KD was significantly longer: 8.51 days vs 5.77 days - (p < 0.01). The total duration of fever was also longer - 10.92 vs 8.32 days - (p = 0.013) - and the frequency of antibiotics intake more important - 92.2% vs 46.2% - (p < 0.01). Four children underwent surgery in the ENT diagnostic group: two retro-pharyngeal abscesses, one paracentesis and one cervicectomy. CONCLUSIONS: ENT manifestations are frequently at the forefront of KD and constitute a misleading clinical picture responsible for delayed diagnosis and potentially inappropriate medico-surgical management. It is necessary to provide more education to practitioners for earlier recognition of Kawasaki disease.

[Congenital auricular anomalies: Early non-surgical correction by splinting]. / Anomalies congénitales de l'oreille externe : traitement néonatal par conformation.

OBJECTIVES: Auricular deformities therapy by external molding is possible. It must be undertaken early, ideally before the sixth week of life. Persistency of maternal hormones impregnation during the first six weeks of life permits to maintain the malleable cartilages. They can thus be molded into a new position. After the end of this hormonal therapy, the cartilages gradually become rigid in a specific position. MATERIAL AND METHODS: All infants under 6 weeks of age with isolated auricular deformities between July 2015 and July 2016 received a neonatal treatment with an external molding device called EarWell© or a custom-made one with silicone. The device was removed when the infant was 6 weeks old and had a treatment duration of at least 4 weeks. A 6 months minimum follow-up was carried out to evaluate the result. RESULTS: From July 2015 to July 2016, 42 deformed ears were treated in 38 newborns. The average age to apply the device was 2 weeks of life with a treatment duration of 5 weeks. The immediate outcome was evaluated as excellent by the parents in 81% of cases, with an ear judged as "normal" and as excellent by the surgeon in 75% of cases. There were no major complications. CONCLUSIONS: External molding therapy for infants with ear deformities has proved to be effective in 75 to 81% of cases. It is a non-invasive treatment that avoids subsequent surgical treatment for the majority of patients.

Patterns of Dental Agenesis Highlight the Nature of the Causative Mutated Genes.

The most common outcome of defective dental morphogenesis in human patients is dental agenesis (absence of teeth). This may affect either the primary or permanent dentition and can range from 5 or fewer missing teeth (hypodontia), 6 or more (oligodontia), to complete absence of teeth (anodontia). Both isolated and syndromic dental agenesis have been reported to be associated with a large number of mutated genes. The aim of this review was to analyze the dental phenotypes of syndromic and nonsyndromic dental agenesis linked to gene mutations. A systematic review of the literature focusing on genes ( MSX1, PAX9, AXIN2, PITX2, WNT10A, NEMO, EDA, EDAR, EDARADD, GREMLIN2, LTBP3, LRP6, and SMOC2) known to be involved in dental agenesis was performed and included 101 articles. A meta-analysis was performed using the dental phenotypes of 522 patients. The total number and type of missing teeth were analyzed for each mutated gene. The percentages of missing teeth for each gene were compared to determine correlations between genotypes and phenotypes. Third molar agenesis was included in the clinical phenotype assessment. The findings show that isolated dental agenesis exists as part of a spectrum of syndromes for all the identified genes except PAX9 and that the pattern of dental agenesis can be useful in clinical diagnosis to identify (or narrow) the causative gene mutations. While third molar agenesis was the most frequent type of dental agenesis, affecting 70% of patients, it was described in only 30% of patients with EDA gene mutations. This study shows that the pattern of dental agenesis gives information about the mutated gene and could guide molecular diagnosis for geneticists.

Microbiology and antibiotic therapy of subperiosteal orbital abscess in children with acute ethmoiditis.

OBJECTIVE: The objective of this study was to investigate the microbiological cultures and the management of acute ethmoiditis complicated by subperiosteal orbital abscess (SPOA) in a pediatric population. METHODS: The medical records of children under 18 years old was performed in a tertiary referral pediatric center from January 2009 to April 2017. Clinical examination, computed tomography scans, medical and surgical treatments were reviewed and compared to other studies in literature. RESULTS: One hundred and twenty-nine children were hospitalized for acute ethmoiditis. Among them, forty eight were complicated by SPOA. The mean age of these children were 7 years (range 10 months-16 years). Thirtyfour underwent surgical drainage; for the others the medical treatment was sufficient. Microbiological samples were obtained during the surgical intervention and were contributive in 91% of cases. Streptococcus spp was the most frequently encountered bacteria (60% of cases). We also found anaerobic bacteria (12%), and Staphylococcus aureus (12%). 94% of children received two intravenous antibiotics (a third-generation cephalosporin and metronidazole) for a mean duration of four days. Then the oral treatment was based on amoxicillin-clavulanate during about 8.5 days. All children were cured without sequelae. CONCLUSIONS: For five years Streptococcus milleri, Staphylococcus spp and anaerobic bacteria are on the rise in acute ethmoiditis complicated by SPOA. That is why antibiotics must be adapted to these bacteria even in children under ten years old.

Evaluation of the efficacy and safety of a CS20® protective barrier gel containing OGT compared with topical aciclovir and placebo on functional and objective symptoms of labial herpes recurrences: a randomized clinical trial.

BACKGROUND: Topical or systemic antiviral drugs reduce the duration of herpes simplex virus 1 (HSV-1) recurrences but may not alleviate functional symptoms. OBJECTIVES: To assess the efficacy and safety of CS20 (Acura 24(®) ) protective barrier gel versus topical aciclovir and placebo in resolving functional symptoms in HSV-1 labial recurrences. METHODS: A prospective, randomized, single-centre, assessor-blinded study of CS20 versus topical aciclovir or placebo. The primary endpoint was the total score of four herpes-related functional symptoms (pain, burning, itching, and tingling sensations), evaluated by visual analogue scale (VAS). Secondary endpoints encompassed objective skin changes (oedema, crusting and erythema), evaluated by specific clinical scores. RESULTS: In a study of 106 patients, compared with placebo, a significant improvement in total functional symptom score was observed after 1 day of treatment in the CS20 group, but only after 7 days of treatment in the topical aciclovir group. Burning sensations were significantly reduced by CS20 compared with aciclovir (Days 1-2) or placebo (Days 1-7). Compared to placebo, CS20 significantly reduced pain intensity on Days 1-6. CS20 induced significant and early improvements in the clinical scores for oedema and crusting compared with placebo. Time to cure was similar for CS20 and aciclovir. The treatments were well tolerated and adverse events were comparable in the three treatment groups. Limitations The single-centre and single-blind design of the study and the preselection of patients. CONCLUSION: CS20 showed superior effectiveness against functional symptoms (pain and burning) associated with HSV-1 labial recurrences and was similar to aciclovir for time to cure.

Does Vitamin D play a role on Msx1 homeoprotein expression involving an endogenous antisense mRNA?

Msx1 homeobox gene, a member of Msx family, has been implicated in numerous organs. Its participation was established in different events, such as morphogenetic field determinism and epithelio-mesenchymal interactions. Most of Msx1 target organs are also known for their sensitivity to Vitamin D: such as bone, tooth germ, and hair follicle. Whereas, the expression of Msx2, another member of Msx family, has been shown to be controlled by Vitamin D, no information is available for Msx1. This study aims to analyze the potential relationships between Vitamin D and Msx1 through: (1) comparative analysis of Vitamin D receptor (VDR) and Msx1 protein expression, (2) investigation of Msx1 expression in VDR null mutant mice, and (3) study of Msx1 overexpression impact on osteocalcin VDR expression in immortalized MO6-G3 odontoblasts. Results show the existence of cross-talks between Vitamin D and Msx1 regulation pathways. In odontoblastic cells, Msx1 overexpression decrease VDR expression, whereas in rickets Msx1 sense transcript expression is decreased. These cross-talks may open a new window in the analysis of rickets mineralized tissues physiopathology. In Vitamin D null mutants, the study of the natural Msx1 antisense transcript which has been recently described should be informative.

Cognitive behavior therapy versus supportive therapy in social phobia: a randomized controlled trial.

BACKGROUND: The efficacy of cognitive behaviour therapy (CBT) in social phobia has been demonstrated in several controlled trials and meta-analyses, but no comparison of CBT with supportive therapy (ST) can be found in the literature. METHOD: The aim of the trial was to study the effectiveness of CBT versus ST carried out 'as usual'. Sixty-seven DSM-4 social phobic patients (89% generalized subtype, most with avoidant personality) were randomly allocated into two groups. Group 1 (CBT) received 8 1-hour sessions of individual cognitive therapy (CT) for 6 weeks, followed by 6 2-hour sessions of social skills training (SST) in group weekly. Group 2 received ST for 12 weeks (6 half-hour sessions), then the patients were switched to CBT. All patients agreed not to take any medication during the whole trial. In group 1, 29 patients reached week 6, 27 reached week 12, and 24 weeks 36 and 60 (endpoint). In group 2, 29 patients reached week 6, 28 reached weeks 12 and 18, 26 week 24, and 23 reached weeks 48 and 72 (endpoint). RESULTS: At week 6, after CT, group 1 was better than group 2 on the main social phobia measure. At week 12, after SST, group 1 was better than group 2 on most of the measures and demonstrated a significantly higher rate of responders. This finding was replicated after switching group 2 to CBT. Sustained improvement was observed in both groups at follow-up. Compliance with abstinence from medication increased over time. CONCLUSIONS: CBT was more effective than ST and demonstrated long-lasting effects. This may suggest that social phobia management requires more than a simple and inexpensive psychological intervention.

[Clozapine and refractory schizophrenia. Long-term prospective study of 20 patients]. / Clozapine et schizophrénies résistantes. Etude prospective à long terme chez vingt patients.

Clozapine, a dibenzodiazepine derivative, has potent antipsychotic activity. But bone marrow suppression resulting in agranulocytosis has been associated with clozapine treatment; thus its clinical development has been delayed and the administration of this drug has been restricted to treatment-resistant schizophrenic patient. This report describes an open prospective study of the effects of clozapine on symptomatology of patients who are refractory to neuroleptics. Authors prospectively followed up until 36 months, 20 DSM III-R schizophrenic patients who had failed to respond to various neuroleptics (7.7 +/- 3.0). When clozapine treatment was initiated, the mean duration of the illness was 17 +/- 10 years. Various scales were used for evaluation: total BPRS, BPRS "positive symptoms", BPRS "negative symptoms", PANSS positive and PANSS negative were realized at days 0 and 15, months 1, 2 and 3 and then every 3 months. Significative improvements in total BPRS, BPRS positive symptoms and PANSS positive were noted at day 15 (p < 0.005, p < 0.026, p < 0.02, respectively); clozapine produced significant improvement on the BPRS negative symptoms and the PANSS negative at 1 month (p < 0.03 and p < 0.008, respectively). Side effects were studied: dry mouth was more prominent in the first month after wash-out (15%), while salivation was more and more prevalent (20% within the first month; 53% beyond). There was no agranulocytosis in this cohort; 2 cases (10%) of eosinophilia occurred during the first month; 20% of the patients experienced an increase in total white blood cell count (> 12.000/mm3). Weight gain (> 5 kg) affected 32% of patients.(ABSTRACT TRUNCATED AT 250 WORDS)

[Prevalence of anxiety and depressive symptoms in patients with type 1 and 2 diabetes]. / Prévalences des troubles anxieux et dépressifs chez les patients diabétiques de types 1 et 2.

The authors studied data on psychiatric disorders in 34 insulin-dependent and 27 non-insulin dependent diabetic patients by comparison with 25 patients suffering from hypertension. Analysis was performed following clinical diagnosis (according to DSM III-R criteria), Hamilton Anxiety Inventory, Hopkins Symptom Check List 58 and Beck Anxiety Inventory. In all groups the two major psychiatric clinical diagnoses were anxiety disorders (respectively, 53, 59 and 60%, NS) and depressive disorders (respectively 21, 22 and 20%, NS). These disorders were more common in women. The influence of degenerative complications was studied. Insulin-dependent diabetics with objective nephropathy had significantly higher anxiety scores. In non-insulin-dependent diabetic patients, microangiopathy, diabetic foot and poor control (HbA1c > or = 10%) were also associated with depressive disorders. We conclude that diabetics present with high prevalences of anxiety and depressive disorders and we suggest specific therapeutic approaches.

[When to continue or stop Clozapine therapy?]. / Quand continuer ou arrêter un traitement par la clozapine?

The decision whether or not to stop clozapine therapy in schizophrenic patients depends on a lot of factors involving the benefit/risk ratio. Thus, authors successively analyse various data: the clinical status of the patient is the first one. The evaluation has to take into account short and long-term efficacies; the problem of the minimal duration of clozapine therapy required before concluding to ineffectiveness is still open: from 4 to 12 months; the question of efficacy of the drug according to the type of symptoms is also quite difficult. Efficacy on positive symptoms among schizophrenic patients seems most prominent; negative symptoms also improve but the reasons why are quite difficult to evaluate. It is sometimes difficult to indicate if the improvement in negative symptoms is independent of the improvement in positive symptoms; the patient's request and his feeling (including tolerability) are another decisional factor; because of the lack of dystonia and other extrapyramidal side effects, some patients are more compliant under clozapine therapy; the side effect (hematologic, cardiovascular, hepatic and central nervous systems) lead to discontinuation of clozapine treatment when severe. The most frequent ones are: sedation, EEG alteration, seizures, increase of liver enzymes, hypotension/collapse, hypersalivation, fever (> 38), ECG alteration, tachycardia, gastro-intestinal adverse effects, weight gain, and leucopenia. In the event of a white blood cell count (WBC) below 3,500/mm3, the patient should be evaluated immediately with respect to the WBC and the differential count (DC). Should the results confirm a WBC below 3,500/mm3 and/or reveal an absolute neutrophil granulocyte count of 2,000 to 1,500/mm3, the leucocytes and the granulocytes must be checked at least twice a week.(ABSTRACT TRUNCATED AT 250 WORDS)

[Drug therapies in anxiety disorders of children]. / Les thérapeutiques médicamenteuses des troubles anxieux de l'enfant.

Although childhood anxiety disorders are currently generating new interest, they continue to pose problems in everyday clinical practice. Drug treatment is far from being the only therapeutic approach but does undeniably have a place which should be clearly defined. The main drugs, dosages, indications, and adverse effects of antidepressants, benzodiazepines, antihistamines and neuroleptics which may be used in children with anxiety disorders are discussed.