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Congenital cytomegalovirus (CMV) infection leads to olfactory bulb lesions in the fetus, yet little is known about its impact on olfaction after birth. Here, we have assessed in a prospective study conducted on children in two French hospitals from 2016 to 2019, infection severity and olfactory performance after congenital CMV infection. Children with congenital CMV infection aged 3 to 10 years and healthy controls (CTL) matched for age and sex to CMV children symptomatic at birth (sCMV) were enrolled. Olfactory discrimination was assessed using mono-odorants and binary mixtures. Data were analyzed for 54 children with PCR-confirmed congenital CMV infection, including 34 sCMV (median [IQR] age, 6 [5–8] years; 19 [55.9%] male), and 20 CMV asymptomatic at birth (aCMV, median [IQR] age, 4 [3–6] years; 12 [60.0%] male). sCMV were compared to 34 CTL children. Olfactory scores in CMV-infected children were independent from vestibular deficit and hearing loss. The olfactory score was efficient to discriminate between CTL and sCMV for children > 6 years (area under the receiver-operating characteristic curve (AUC, 0.85; P = 0.0006), but not for children 6 years, the proportion of children with total olfactory score

International audience; Primary ciliary dyskinesia (PCD) is a rare genetical disease characterized by an abnormal structure or function of the cilia, causing sinusitis, otitis, and bronchiectasis. Hearing loss affects 60% of PCD patients, but data are lacking concerning hearing and temporal bone imaging in adults. Our aim was to describe clinical and radiological ear disease in adults with genetically confirmed PCD. Data were recorded from January 2018 to December 2019. PCD patients were compared with controls with bronchiectasis without PCD. Clinical examination included otomicroscopy and auditory tests. A temporal bone CT scan (TBCT) was systematically performed. Seventeen patients (34 ears) were included in each group. The eardrums were abnormal in 25 (74%) PCD ears versus 8 (24%) ears in the controls (p < 0.05). Conductive hearing loss was more frequent in the PCD group (24% vs. 12% in controls). TBCT were abnormal in 94% PCD patients vs. 32% in the controls (p < 0.05). The Main CT-scan images in PCD were middle ear inflammation (65%), mastoid condensation (62%), or ossicular anomalies (35%). With its excellent sensitivity, TBCT gives typical arguments for PCD diagnosis, adding otological signs to the usual sinus CT signs (hypoplasia, aplasia). Systematic TBCT could be useful in the initial evaluation of patients with suspicion of PCD.

Purpose To examine the MRI diagnostic performance in the assessment of therapeutic response to burosumab in children with X-linked hypophosphatemia (XLH). Design Prospective longitudinal open cohort. Patients Seventeen children with XLH, average age of 10.2 ± 2.7 years, had a knee MRI at baseline and after 1 year of burosumab. Intervention Children received burosumab at an average dose of 1.4 ± 0.5 mg/kg during 1 year for the treatment of severe rickets (the target serum phosphate ≥ 1.2 mmol/L (≥3.7 mg/dL). The primary endpoint was the change from baseline to 12 months in rachitic lesions on knee MRI. Secondary endpoints were changes in biochemical parameters of phosphate and alkaline phosphatase (ALP). Results One year of treatment with burosumab significantly reduced radiological disease activity on knee MRI (by 44 ± 29% in the transverse extent of widening) which was accompanied by a significant reduction in biochemical activity, namely in serum ALP activity, by 28 ± 17%. Additionally, MRI parameters after 1 year of treatment with burosumab (the maximum width of medial physis at 12 months and the change from baseline in the maximum width of lateral physis) were associated with ALP activity at 12 months. Conclusion We suggest that MRI is a valuable and quantitative tool to evaluate the therapeutic response to burosumab. MRI could be an excellent alternative to standard bone radiographs for evaluation of the rachitic lesions in a clinical setting avoiding repeated exposition to ionizing radiation.

Gitelman syndrome (GS) is a rare salt-losing tubulopathy caused by an inactivating mutation in the SLC12A3 gene, encoding the thiazide-sensitive sodium chloride cotransporter (NCC). Patients with GS frequently complain of vertigo, usually attributed to hypovolemia. Because NCC is also located in the endolymphatic sac, we hypothesized that patients with GS might have vestibular dysfunction. Between April 2013 and September 2016, 20 (22%) out of 90 patients followed at the reference center complained of vertigo in the absence of orthostatic hypotension. Sixteen of them were referred to an otology department for investigation of vestibular function. The vertigo was of short duration and triggered in half of them by head rotation. Seven patients (44%) had a vestibular syndrome. Vestibular syndrome was defined: (1) clinically, as nystagmus triggered by the head shaking test (n = 5), and/or (2) paraclinically, as an abnormal video head impulse test (n = 0), abnormal kinetic test (n = 4) and/or abnormal bithermal caloric test (n = 3). Five patients had associated auditory signs (tinnitus, aural fullness or hearing loss). In conclusion, we found a high frequency of vestibular disorder in GS patients suffering from vertigo, suggesting a role of NCC in the inner ear. Referent physicians of these patients should be aware of this extrarenal manifestation that requires specific investigations and treatment.

Lipoblastoma is a rare, benign tumor of embryonic fat occurring in infants and young children. Generally found in the trunk or extremities, it is rarely located in other areas such as the head and neck, where it may cause respiratory symptoms. A median, pretracheal location is extremely atypical and, to our knowledge, has never been described. Here we describe such a tumor in an 11-month-old infant. The heterogeneous, fatty content of the tumor is very suggestive of lipoblastoma, whatever its location, particularly before age 3 years.

HIGHLIGHTS • Isolated loss of smell without nasal obstruction is an early red-flag of COVID-19 • These patients should adopt all the preventive measures and a lockdown • Olfactory/gustative dysfunction had high predictive value to identify COVID-19 • Olfactory/gustative dysfunction had high specificity to identify COVID-19 • Self-reported loss of smell, among other symptoms, could help to screen COVID-19, Objectives To determine the frequency of SARS-CoV-2 positive samples in a subset of patients consulting for primarily isolated acute (

Limited data is available concerning the safety of active middle ear implants (AMEI) during Magnetic Resonance Imaging (MRI). Measurements in temporal bones are the gold standard for preclinical assessment of device safety. In this study the coupling stability of an actuator as used in a fully implantable AMEI was determined in temporal bones. Eleven temporal bones were implanted with the actuator according to the manufacturer’s surgical guidelines. The actuator was coupled on the incus short process as recommended for sensorineural hearing loss. Temporal bones were exposed 10 times to the MRI magnetic field by entering the MRI suite in a clinically relevant way. Computed Tomography (CT) images were acquired before and after the experiment to investigate the risk of actuator dislocation. Based on the electrical impedance of the actuator, the loading of the actuator to the incus was confirmed. Relative actuator displacement was determined on the CT images by comparing the initial with the final actuator position in 3D space. Impedance curves were analyzed after each exposure to check the loading of the actuator to the ossicles. Analysis of CT images with a 0.30.6 mm in-plane resolution indicate no actuator displacement. The maximum detected change in impedance for all actuators was 8.43 Ω at the actuator’s resonance frequency. Impedance curves measured when the actuator was retracted from the short process after the experiment still indicate the presence of a clear resonance peak. No actuator displacement or dislocation could be detected in the analysis of CT images and the measured impedance curves. Impedance curves obtained when the actuator was retracted from the incus short process still show a clear resonance peak, indicating the device is still functional after the MRI exposures. ispartof: Plos One vol:15 issue:4 ispartof: location:United States status: Published online

Screening and early treatment of deafness regardless of age is essential. Deafness leads to social isolation, depression, and decreased cognitive function. The diagnosis requires an otoscopy and a confirmation of the type and degree of deafness by audiometry. Sudden deafness and meningitis are neuro-sensorial emergencies. Deafness may be the mode of disclosure of an autoimmune disease or part of the evolutionary profile. Hearing complaints with a normal classical audiogram may be the manifestation of a so-called "hidden" hearing loss and must be explored more carefully.

Points essentiels Devant un tableau d’otite externe trainante malgre un traitement bien conduit, en particulier sur un terrain immunodeprime, il faut toujours rechercher une osteomyelite de la base du crâne qui necessite un traitement antibiotique parenteral urgent de plusieurs semaines. L’otite moyenne aigue (OMA) est la plus frequente des infections bacteriennes de l’enfant. Chez l’enfant de moins de 2 ans presentant une OMA purulente, l’antibiotherapie par amoxicilline est systematique pour une duree de 8–10 jours. Apres 2 ans d’âge et si les symptomes d’OMA sont peu bruyants, un traitement symptomatique peut se justifier en premiere intention. L’otite seromuqueuse (OSM) est frequente apres un episode d’OMA et ne devient chronique qu’apres 3 mois d’evolution. La pose d’aerateurs trans-tympaniques reduit la frequence des episodes d’OMA. Toute OMA compliquee d’une meningite impose une surveillance par audiogramme et IRM de l’oreille.

The management of deafness has become a major public health issue as their lack of detection has a deleterious effect in children and increases the risk factors for aggravation of other pathologies in adults. The detection of deafness remains a real challenge: in the newborn, systematic screening at birth is a good strategy, in adults, much remains to be done. The functional rehabilitation of deafness is based on the use of hearing aids by aerial or bone conduction or of auditory implants. There are three types of auditory implants available: bone anchored hearing implants, middle ear implants and cochlear implants. Many actors, in particular social organizations, can intervene in the financial management of these medical devices.

The interrogation is essential to trace the genesis of the tinnitus and to appreciate its repercussion. Clinical examination should look for a local, vascular or cervical cause. The ENT consultation with audiogram and tinnitus evaluation is essential to know the characteristics of the tinnitus and to consider the treatment. If tinnitus is accompanied by a decrease in hearing, then wearing hearing aids can correct deafness and decrease the tinnitus. The psychological impact of tinnitus must always be taken into account and the use of a multidisciplinary team is an interesting solution. Medical treatments are not very effective; on the other hand psychotherapies (CBT, TRT and sophrology) bring a real improvement. The doctor's speech must always be supportive and provide therapeutic hope.

Introduction/objectif L’hyperparathyroidie est une manifestation tres frequente dans le cadre de l’hypophosphatemie liee a l’X (XLH). Bien que le mecanisme patho-physiologique ne soit pas bien connu, on suppose que la supplementation en phosphate peut stimuler la secretion de l’hormone parathyroidienne (PTH). Un niveau eleve de FGF23, caracteristique d’XLH, favorise la synthese de PTH et contribue ainsi au developpement de l’hyperparathyroidie. Nous avons etudie l’effet du traitement anti-FGF23 par burosumab sur l’hyperparathyroidie chez les enfants atteints d’XLH. Materiels/methodes 62 enfants atteints d’XLH (40 filles/22 garcons ; 9,1 ± 3,6 ans) traites par burosumab. Les parametres comme le phosphore/calcium sanguins, TmP/eGFR, ALP, 1,25(OH)2D, PTH ont ete controles au debut (M0) et apres 3-6-12-18 mois de traitement (M3-M6-M12-M18). Le diagnostic d’hyperparathyroidie a ete retenu si les taux de PTH etaient superieurs a la limite superieure de la norme (n : 18,5-88 ng/l). Resultats Avant traitement par burosumab, 11 patients (21,5 %) avaient une hyperparathyroidie secondaire. Ils presentaient des taux eleves de PTH (88 ± 39 ng/l) et des taux normaux de calcium sanguin (2,44 ± 0,17 mmol/l). Les enfants atteints d’hyperparathyroidie etaient plus âges que ceux sans hyperparathyroidie (12,0 ± 3,6 vs 8,5 ± 3,3 ans, p = 0,002) et ont recu des supplements de phosphore pendant plus longtemps (10,7 ± 3,9 vs 6,2 ± 3,3 ans, p = 0,000). Sous burosumab les taux de PTH ont diminue chez les enfants atteints d’hyperparathyroidie (M0-M3-M6-M12-M18 : PTH 88 ± 39-76 ± 24-71 ± 16-84 ± 27-75 ± 12 ng/l, p = 0,7). Aucun patient n’a developpe d’hypercalcemie. Conclusion Nos resultats suggerent que le traitement anti-FGF23 semble limiter, voire controler, l’hyperparathyroidie chez les enfants atteints de XLH. Neanmoins, un suivi plus long est indispensable afin de mieux comprendre ce phenomene.

BACKGROUND Bone conduction implants based on abutment-driven acoustic transmission result in good hearing outcomes; however, skin complications impact the quality of life (QOL) and possibly the viability of the device for many patients. The transcutaneous magnetic Baha® Attract technology was developed with the goal of minimising skin complications. OBJECTIVES To analyse surgical, auditory and QOL outcomes for patients implanted with the Baha® Attract. DESIGN Prospective multicentre cohort study. SETTING Four French tertiary referral centres. PARTICIPANTS Thirty-two patients implanted with the Baha® Attract, including 25 with conductive and mixed hearing loss and 7 with single-sided deafness. MAIN OUTCOME MEASURES Postoperative follow-up involved the visual analysis of soft tissue adaptation and sound processor magnet strength measurement. The audiometric outcomes were evaluated in quiet and noise, and the QOL was assessed using three different questionnaires. RESULTS After 12 months of use, soft tissue was thinner, and mean magnet strength was significantly decreased (3.7-3.1, P

International audience; Polycystic kidney diseases (PKDs) are genetic disorders that can cause renal failure and death in children and adults. Lowering cAMP in cystic tissues through the inhibition of the type-2 vasopressin receptor (V2R) constitutes a validated strategy to reduce disease progression. We identified a peptide from green mamba venom that exhibits nanomolar affinity for the V2R without any activity on 155 other G-protein-coupled receptors or on 15 ionic channels. Mambaquaretin-1 is a full antagonist of the V2R activation pathways studied: cAMP production, beta-arrestin interaction, and MAP kinase activity. This peptide adopts the Kunitz fold known to mostly act on potassium channels and serine proteases. Mambaquaretin-1 interacts selectively with the V2R through its first loop, in the same manner that aprotinin inhibits trypsin. Injected in mice, mambaquaretin-1 increases in a dose-dependent manner urine outflow with concomitant reduction of urine osmolality, indicating a purely aquaretic effect associated with the in vivo blockade of V2R. CD1-pcy/pcy mice, a juvenile model of PKD, daily treated with 13 [Formula: see text]g of mambaquaretin-1 for 99 d, developed less abundant (by 33%) and smaller (by 47%) cysts than control mice. Neither tachyphylaxis nor apparent toxicity has been noted. Mambaquaretin-1 represents a promising therapeutic agent against PKDs.

In front of external otitis in spite of a well-conducted treatment, especially in immunodeficient patient, it is always necessary to look for an osteomyelitis of the skull base that requires an urgent parenteral antibiotic treatment of several weeks. Acute otitis media (AOM) is the most common bacterial infection of the child. In children under 2 years with purulent AOM, antibiotic therapy with amoxicilline is systematic for a period of 8-10 days. After 2 years of age and with mild symptoms of AOM, symptomatic treatment may be justified as first-line treatment. Chronic otitis media is frequent after an episode of AOM and becomes chronic only after 3 months of evolution. Grommets reduce the frequency of AOM episodes. All AOM complicated with meningitis requires monitoring by audiogram and MRI of the ear.

The management of deafness has become a major public health issue as their lack of detection has a deleterious effect in children and increases the risk factors for aggravation of other pathologies in adults. The detection of deafness remains a real challenge: in the newborn, systematic screening at birth is a good strategy, in adults, much remains to be done. The functional rehabilitation of deafness is based on the use of hearing aids by aerial or bone conduction or of auditory implants. There are three types of auditory implants available: bone anchored hearing implants, middle ear implants and cochlear implants. Many actors, in particular social organizations, can intervene in the financial management of these medical devices.

Screening and early treatment of deafness regardless of age is essential. Deafness leads to social isolation, depression, and decreased cognitive function. The diagnosis requires an otoscopy and a confirmation of the type and degree of deafness by audiometry. Sudden deafness and meningitis are neuro-sensorial emergencies. Deafness may be the mode of disclosure of an autoimmune disease or part of the evolutionary profile. Hearing complaints with a normal classical audiogram may be the manifestation of a so-called "hidden" hearing loss and must be explored more carefully.

Deafness is the most common sensory disability in developed countries affecting more than 2 births in 1000. Eighty percent of congenital deafness is genetic. Universal newborn hearing screening has been in place since 2012 in France. All genetic hearing losses are not congenital and all congenital hearing losses are not genetic. Genetic hearing loss may be syndromic (associated with other symptoms) (10 %) or non-syndromic (isolated) (90 %). Hearing loss may initially be the only symptom of syndromic deafness. A genetic origin can be diagnosed and must therefore be evoked systematically even in the adult.

BackgroundDirect punctures of intracranial tumors have rarely been described in the literature.ObjectiveTo assess the feasibility, safety, efficacy, and advantages of using 3D DSA-guided direct puncture rather than the traditional transarterial route to preoperatively devascularize intracranial lesions in particular clinical situations, paying special attention to any correlation with surgical observations; we present the largest series to date.MethodsBetween July 2015 and July 2016, data from all presurgical embolizations performed in our institution were prospectively collected. Information on tumor type, location, size, eventual bone erosion, complications, devascularization percentage, and estimated blood loss was analyzed.ResultsTumors of four patients (two meningioma, two endolymphatic sac tumor) were embolized using direct puncture. 3D XperGuide planning software was used in all procedures. Embolization was feasible in all cases. In one case, a small craniotomy was specifically performed to allow needle positioning. In all cases n-butyl cyanoacrylate was used. No ischemic or hemorrhagic complications related to embolization occurred. Complete or near complete devascularization was obtained in all cases. In one case, surgery was not performed and the patient was monitored. Resection was complete without significant blood loss in two cases, and resection was incomplete but satisfactory in one case.ConclusionsIn selected cases, 3D-guided direct puncture of intracranial tumors appears safe, feasible, and efficient for preoperative embolization.

Menière's disease, clinically characterized by fluctuating, recurrent, and invalidating vertigo, hearing loss, and tinnitus, is linked to an increase in endolymph volume, the so-called endolymphatic hydrops. Since dysregulation of water transport could account for the generation of this hydrops, we investigated the role of aquaporin 3 (AQP3) in water transport into endolymph, the K-rich, hyperosmotic fluid that bathes the apical ciliated membrane of sensory cells, and we studied the regulatory effect of dexamethasone upon AQP3 expression and water fluxes. The different AQP subtypes were identified in inner ear by RT-PCR. AQP3 was localized in human utricle and mouse inner ear by immunohistochemistry and confocal microscopy. Unidirectional transepithelial water fluxes were studied by means of (3)H2O transport in murine EC5v vestibular cells cultured on filters, treated or not with dexamethasone (10(-7) M). The stimulatory effect of dexamethasone upon AQP3 expression was assessed in EC5v cells and in vivo in mice. AQP3 was unambiguously detected in human utricle and was highly expressed in both endolymph secretory structures of the mouse inner ear, and EC5v cells. We demonstrated that water reabsorption, from the apical (endolymphatic) to the basolateral (perilymphatic) compartments, was stimulated by dexamethasone in EC5v cells. This was accompanied by a glucocorticoid-dependent increase in AQP3 expression at both messenger RNA (mRNA) and protein level, presumably through glucocorticoid receptor-mediated AQP3 transcriptional activation. We show that glucocorticoids enhance AQP3 expression in human inner ear and stimulate endolymphatic water reabsorption. These findings should encourage further clinical trials evaluating glucocorticoids efficacy in Menière's disease.

To analyze the difference between the endolymphatic sac tumors (ELSTs) in sporadic cases and in von Hippel-Lindau (VHL) disease.Retrospective case review in a tertiary referral center.Fourteen cases of ELST, occurring since 1998, were reviewed. We analyzed the initial symptoms, characteristics of the tumor, treatment, sequelae, and follow-up for each group.The ELSTs were sporadic in 6 cases and associated with VHL disease in 8 cases. The mean age at the time of the first surgery was 26 years (range, 12-41). All except two of the patients presented with a unilateral tumor. The initial symptoms were hearing loss (n = 9), tinnitus (n = 7), and/or vertigo (n = 5). Hearing loss was more prevalent in the sporadic cases. Preoperative arteriography was performed for 4 patients, with embolization performed for 1 patient. The size of the tumor was significantly larger in the sporadic cases (31.7 mm) than in the cases of VHL disease (19.3 mm). The surgical approach was more extensive in the sporadic cases. The surgeons found 2 types of tumors. Cystic tumors with massive bleeding invading the surrounding structures (the dura mater or jugular bulb) were more common in the sporadic cases. Fibrous tumors that infiltrate the bone and have moderate bleeding were more common in the cases associated with VHL disease.Two patients with small lesions were not operated on but were followed for 6 years without tumor growth. They died of metastasis from gastric and kidney cancer. Four recurrences occurred during the 14 years of follow-up. Four facial palsies and 8 cases of profound deafness were encountered postoperatively.Sporadic tumors are more aggressive than those associated with VHL disease. Complete surgical resection should be the goal of treatment. Preoperative angiography with embolization is recommended. In some cases, embolization may be impossible, and preoperative or postoperative radiotherapy should be discussed.

In children, hypophosphatemic rickets (HR) is revealed by delayed walking, waddling gait, leg bowing, enlarged cartilages, bone pain, craniostenosis, spontaneous dental abscesses, and growth failure. If undiagnosed during childhood, patients with hypophosphatemia present with bone and/or joint pain, fractures, mineralization defects such as osteomalacia, entesopathy, severe dental anomalies, hearing loss, and fatigue. Healing rickets is the initial endpoint of treatment in children. Therapy aims at counteracting consequences of FGF23 excess, i.e. oral phosphorus supplementation with multiple daily intakes to compensate for renal phosphate wasting and active vitamin D analogs (alfacalcidol or calcitriol) to counter the 1,25-diOH-vitamin D deficiency. Corrective surgeries for residual leg bowing at the end of growth are occasionally performed. In absence of consensus regarding indications of the treatment in adults, it is generally accepted that medical treatment should be reinitiated (or maintained) in symptomatic patients to reduce pain, which may be due to bone microfractures and/or osteomalacia. In addition to the conventional treatment, optimal care of symptomatic patients requires pharmacological and non-pharmacological management of pain and joint stiffness, through appropriated rehabilitation. Much attention should be given to the dental and periodontal manifestations of HR. Besides vitamin D analogs and phosphate supplements that improve tooth mineralization, rigorous oral hygiene, active endodontic treatment of root abscesses and preventive protection of teeth surfaces are recommended. Current outcomes of this therapy are still not optimal, and therapies targeting the pathophysiology of the disease, i.e. FGF23 excess, are desirable. In this review, medical, dental, surgical, and contributions of various expertises to the treatment of HR are described, with an effort to highlight the importance of coordinated care.

Background and objectives Hypocitraturia has been associated with metabolic acidosis and mineral disorders. The aim of this study was to investigate the occurrence of urinary acidification defects underlying hypocitraturia. Materials and methods This retrospective observational study included 67 patients (32 men), aged 40.7±15.1 years with hypocitraturia (

Objective To evaluate the perceptive and linguistic results in 2 predefined genetic population of children with implants. Study design Retrospective cohort study. Setting Otolaryngology department of a tertiary referral hospital. Patients Among 336 children with prelingual deafness who underwent implantation in our department between 1997 and 2007, 85 with GJB2 gene-related (Cx) deafness and 30 patients with Waardenburg syndrome (WaardS) were included. Mean age at implantation was 4.7 years (range, 15 mo to 16.5 yr) in the Cx group and 4.8 years (range, 16 mo to 16 yr) in the WaardS group. The mean follow-up period was 6.5 years (range, 19 mo to 12 yr) in the Cx group and 7.1 years (range, 27 mo to 13.5 yr) in the WaardS group. More than 75% of the families in both groups scored 4 and 5 when using the Mary Pat Moeller rating (p > 0.05). A psychoneurological evaluation was performed in one third of the patients in both groups. Main outcome measures Patients underwent linguistic and perceptive evaluations at 12, 24, 36, and 48 months: speech perception with closed-set and open-set words, speech production (Level 1 = vocalizations to Level 5 = complex sentences), and lexical comprehension with EVIP/GaelP (Peabody Picture Vocabulary Test) tests that ranged in 5 levels (-2 SD to +2 SD). Results Score results for open-set words were 38.5%, 57.5%, 69%, and 75% in the Cx group and 30.5%, 59%, 67.5%, and 78% in the WaardS group (p > 0.05) at 12, 24, 36, and 48 months. The proportion of children in Levels 4 and 5 of speech production was 27%, 42%, 57.5%, and 58% in the Cx group and 23%, 33%, 55%, and 66% in the WaardS group (p > 0.05). Lexical result at Level -2 SD at 1 year was 90% in the Cx group and 85% in the WaardS group and that at 4 years was 70% in the Cx group and 65% in the WaardS group (p > 0.05). Conclusion Perceptive and linguistic evolutions for both populations were of good quality, but lexical evaluation showed residual language difficulties in both groups.

Objectives To assess the diagnostic strategy, treatment and outcome of acquired subglottic cysts. Materials and methods Retrospective, monocentric, tertiary referential center study of 172 preterm neonates assessed by endoscopic examination over a 10 years period. Identification of patients presenting with subglottic cysts. Results 17 children were diagnosed with subglottic cysts. Among them, 98% were prematurates (28 ± 4 weeks of gestation), and 76% had a history of hyaline membrane disease or a bronchopulmonary dysplasia. All patients were intubated during the neonatal period, for a mean duration of 14 days. Mean age at diagnosis was 8 months. An associated laryngotracheal anomaly was diagnosed in 30% of cases. Six procedures, including flexible controls, were needed to achieve full recovery. We used cold steel microinstruments, CO 2 or Thulium LASER. Mean follow up was 3 years. Conclusions Acquired subglottic cysts concern early preterm infants. Children treated for subglottic cysts should undergo a long term follow up, as there is a trend for cysts to recur, as well as a risk of secondary subglottic stenosis.

International audience; A strict control of endolymph composition (high potassium, low sodium fluid) and volume is instrumental for a proper functioning of the inner ear. Alteration of endolymph homeostasis is proposed in the pathogenesis of Menière's disease. However, the mechanisms controlling endolymph secretion remain elusive. By using the vestibular EC5v cells, we provide evidence for the presence of vasopressin, catecholamine and purinergic signaling pathways, coupled to adenylate cyclase, phosphoinositidase C and Ca(2+) activation. We demonstrate that vasopressin and catecholamines stimulate while ATP inhibits apical potassium secretion by EC5v cells. These results open new interesting perspectives for the management of inner ear diseases.

International audience; OBJECTIVE: To assess the efficacy of partial ossicular chain reconstruction using autologous cartilage. DESIGN: Prospective study (April 1, 1997, through January 1, 2008). SETTING: Tertiary academic children's hospital. PATIENTS: Two hundred forty-eight children (268 ears) underwent partial ossicular chain reconstruction using a shaped block of tragal cartilage interposed between the head of the stapes and an underlay tympanic membrane reconstruction along with tragal cartilage and its perichondrium. MAIN OUTCOME MEASURES: Anatomical and audiologic results were evaluated according to the American Academy of Otolaryngology-Head and Neck Surgery guidelines. χ² Tests and multivariate analysis were used for statistical evaluation. RESULTS: Mean age at surgery was 10.9 years. Single-stage surgery was performed in 124 ears (46.3%) (62.9% for cholesteatomas and 32.3% for retraction pockets). Second-look patients (53.7%) included 93.8% of staged surgery. Audiometric results were available for 222 ears at 1 year and for 78 ears at 5 years. Closure of the average air-bone gap (ABG) to within 20 dB was achieved in 62.2% of ears at 1 year. The mean (SD) preoperative and 1-year postoperative ABGs were 25 (11.8) dB and 18.9 (10.3) dB, respectively. Anatomical results were satisfactory in 87.3%. No cases of extrusion, resorption, or displacement of the cartilage were encountered. No statistically significant difference was found between audiometric results at 1 and 5 years. Multivariate analysis showed a significant negative correlation between preoperative and postoperative ABGs and between postoperative otitis media with effusion and postoperative ABG (P < .05). CONCLUSIONS: Cartilage ossiculoplasty is a reliable technique for partial ossicular replacement. Long-term hearing outcomes remain stable and satisfactory. Preoperative ABG and postoperative otitis media are the predictive factors of the hearing outcome.

Objectives To determine the presentation of third or fourth branchial pouch anomalies in various age groups of children and evaluate endoscopic cauterization as a treatment technique. Design Retrospective study of patients treated from 2000 to 2009. Setting Tertiary care children's hospital. Patients Pediatric patients aged 0 to 18 years (mean age, 5.5 years), including 5 neonates. Interventions Endoscopic and/or open surgical management of third and fourth branchial pouch anomalies; clinical and endoscopic follow-up. Main Outcome Measures Absence of clinical recurrence; closure of the sinus tract. Results Two forms of presentation were identified: a neonatal form, characterized by a voluminous and compressive cervical mass (5 of 20 [25%]) and a childhood form, presenting as a cervical abscess (15 of 20 [75%]). The vast majority of our patients regardless of presentation were treated endoscopically (n = 19), with a success rate of 68% (13 of 19) after 1 procedure, 79% (15 of 19) after 2 procedures, and 89% (17 of 19) after 3 procedures. Neonatal and adult presentations require slightly different therapeutic approaches. Conclusions Third and fourth branchial pouch anomalies can present in 2 distinct forms: a neonatal form and a childhood form. The endoscopic technique should be the favored approach for both forms: whenever possible, in view of its simplicity, rapidity, and the lack of serious postoperative complications. Recurrences can be treated by repeated cauterization using the same technique, with good long-term outcomes. An age-based management algorithm has been developed.

Objective To present the indications, techniques, results, and limitations of endoscopic surgical treatment of laryngotracheal cleft. Design Retrospective case note study (2005-2009). Setting Department of Otolaryngology–Head and Neck Surgery, Armand Trousseau Children's Hospital, Paris, France. Patients Eleven patients who underwent endoscopic cleft closure as a primary (n = 8) or secondary (n = 3) procedure among 22 patients treated for laryngotracheal clefts. We report patients' demographics, symptoms leading to the diagnosis, endoscopic evaluation method, medical examination results, and surgical techniques. Intervention Endoscopic closure of the cleft under spontaneous ventilation via 2-layer interrupted sutures after excision of the mucosal edge using a carbon dioxide laser in 10 patients and a thulium laser in 1. Main Outcome Measures Analysis of postoperative complications, revision surgery, need for intensive care unit admission, closure of the cleft, and long-term symptom results. Results Successful closure of the 11 clefts (with revision surgery in 3 patients) without the need for intubation or intensive care unit admission enabled the elimination of aspiration in 10 patients and significant improvement in 1 patient with bilateral vocal cord paralysis. There were no significant postoperative complications. Conclusions Endoscopic closure of laryngotracheal clefts is a reliable technique that significantly reduces perioperative and postoperative morbidity. The results of this technique are entirely satisfactory, and we suggest that it is suitable as a primary procedure for the treatment of type I, II, and III clefts extending to the cervical trachea, including in neonates.

Objective To evaluate the long-term results and predictive factors of a good outcome with the use of a total ossicular replacement prosthesis in children. Design Retrospective case review. Setting Tertiary referral center. Patients The study included 114 children (116 ears). Interventions A total of 116 ears underwent total ossicular chain reconstruction with a titanium prosthesis. Cartilage was always used for tympanic membrane reconstruction. Main Outcome Measures Audiological results were evaluated according to the guidelines of the American Academy of Otolaryngology–Head and Neck Surgery. Predictive factors of audiological results were determined. Logistic regression and χ2 tests were used for statistical analysis. Results The mean age at surgery was 9.8 years. Ossiculoplasty was performed during second-look surgery in 91 ears (78.4%) and during another stage in 25 ears (21.6%). The first-stage procedure was always performed for cholesteatoma. Audiometric results were available for 116 ears at 1 year, for 89 ears (76.7%) at 2 years, and for 42 ears (36.2%) at 5 years. Closure of the average air-bone gap (ABG) to within 20 dB was achieved in 65 ears (56%) at 1 year. The mean (SD) preoperative and postoperative (at 1 year) ABGs were 41.0 (9.5) dB and 22.4 (12.6) dB, respectively. There were no cases of extrusion, but 17 luxations of the prosthesis were confirmed by computed tomography. Luxation occurred on average at 31.4 months. Only three 4000-Hz degradations of bone conduction were reported, with no dead ears. We examined 3 predictive factors of auditory results: preoperative ABG, footplate status, and postoperative otoscopic findings. Conclusions Total ossiculoplasty is a reliable technique in children. Long-term hearing outcomes are stable and satisfactory, but luxation can occur at any time. Preoperative ABG and footplate status are negative predictive factors of auditory results.