Cost-effectiveness of screening and valacyclovir-based treatment strategies for first-trimester cytomegalovirus primary infection in pregnant women in France.

OBJECTIVE: To assess the effectiveness, cost and cost-effectiveness of four screening strategies for first-trimester (T1) cytomegalovirus (CMV) primary infection (PI) in pregnant women in France. METHODS: In a simulated pregnant population of 800 000 (approximate number of pregnancies each year in France), using costs based on the year 2022, we compared four CMV maternal screening strategies: Strategy S1, no systematic screening (current public health recommendations in France); Strategy S2, screening of 25-50% of the pregnant population (current screening practice in France); Strategy S3, universal screening (current medical recommendations in France); Strategy S4, universal screening (as in Strategy S3) in conjunction with valacyclovir in case of T1 PI. Outcomes were total cost, effectiveness (number of congenital infections, number of diagnosed infections) and incremental cost-effectiveness ratio (ICER). Two ICERs were calculated, comparing Strategies S1, S2 and S3 in terms of euros (€) per additional diagnosis, and comparing Strategies S1 and S4 in € per avoided congenital infection. RESULTS: Compared with Strategy S1, Strategy S3 enabled diagnosis of 536 more infected fetuses and Strategy S4 prevented 375 congenital infections. Strategy S1 was the least expensive strategy (€98.3m total lifetime cost), followed by Strategy S4 (€98.6m), Strategy S2 (€106.0m) and Strategy S3 (€118.9m). In the first analysis, Strategy S2 was dominated and Strategy S3 led to an additional €38 552 per additional in-utero diagnosis, compared with Strategy S1. In the second analysis, Strategy S4 led to an additional €893 per avoided congenital infection compared with Strategy S1, and was cost-saving compared with Strategy S2. CONCLUSIONS: In France, current screening practice for CMV PI during pregnancy is no longer acceptable in terms of cost-effectiveness because this strategy was dominated by universal screening. Moreover, universal screening in conjunction with valacyclovir treatment would be cost-effective compared with current recommendations and is cost-saving compared with current practice. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Impact of the COVID-19 epidemic on ENT surgical volume.

PURPOSE: The purpose of this study was to assess the impact of the COVID-19 pandemic on the surgical volume of three ENT departments in Ile-de-France, a region severely affected by the epidemic. MATERIALS AND METHODS: The number and nature of surgeries was collected from three university hospital ENT departments from 17/03/2020 to 17/04/2020 and from 18/03/2019 to 18/04/2019. Centre 1 is a general adult ENT department specialized in otology, centre 2 is a general adult ENT department specialized in cancer and centre 3 is a paediatric ENT department. Comparative analysis of the decreased surgical volume was conducted between 2019 and 2020. OBJECTIVE: To analyse the reduction of ENT surgical volume. RESULTS: The three centres operated on 540 patients in 2019, versus 89 in 2020, i.e. an 84% decrease: 89% in Centre 1, 61% in Centre 2, and 95% in the paediatric centre. Otological surgery decreased by 97%, endonasal surgery decreased by 91%, head and neck surgery decreased by 54%, plastic surgery decreased by 82%, and transoral surgery decreased by 85%. The number of surgical operations for skin cancer decreased (24 vs. 9), while the total number of head and neck cancer surgeries remained stable (18 vs. 22). The number of planned tracheostomies increased from 8 to 22. CONCLUSION: The number of ENT surgeries decreased by 84% during the first month of the COVID-19 epidemic. This decreased surgical volume mainly concerned functional surgery, while the level of cancer surgery remained stable. Hospital units will need to absorb a marked excess surgical volume after the epidemic.

Recurrent laryngeal nerve monitoring in children using cricothyroid membrane needle electrodes.

AIMS: Thyroid pathology is rare in children and the rate of malignancy is higher than in adults. Thyroid surgery in children is therefore particularly at risk of causing recurrent laryngeal nerve (RLN) palsies. The classical technique for monitoring the RLN is not always adapted to children due to the large size of the dedicated endotracheal tubes. MATERIAL AND METHODS: Double-needle electrodes (NIM 3.0) were placed medially or paramedially through the cricothyroid membrane and carefully kept submucosal just below the level of the vocal folds. Before identification of the RLN, the vagal nerve was dissected on the side of the concerned lobe and stimulated. The thyroid surgery was then performed with the routine identification of the RLN. The response of the RLN is periodically checked using a stimulating probe. The main outcomes were the identification and stimulation of the RLN, quality of the voice in post-operative time. OBJECTIVE: The main objective of our study is to present a simple and efficient method, available for children of all ages, in order to perform monitoring of the recurrent laryngeal nerves during thyroid surgery. RESULTS: We present the results of our retrospective series, in a tertiary-care university pediatric hospital. We included nine children, corresponding to 15 nerves. In all cases, the RLN was identified, stimulated and a positive response was obtained via monitoring. CONCLUSIONS: This technique of monitoring is safe, feasible at any age, even in neonates, and, as the electrode stays in the operation field, its position is easily controlled.

Vestibular impact of Friedreich ataxia in early onset patients.

BACKGROUND: Friedreich ataxia (FRDA) is the most frequent form of inherited ataxias. Vestibular and auditory assessments are not commonly part of the check up for these patients despite hearing and balance complaints. Screening of vestibular and auditory function was performed in a large group of young patients with genetically confirmed FRDA. METHODS: Our study included 43 patients (7-24 years of age). A complete vestibular assessment was performed including the canals function evaluation at 3 head velocities (bithermal caloric test, earth vertical axis rotation (EVAR) and head impulse test (HIT)) and otolith function evaluation (cervical vestibular evoked myogenic potentials). Information regarding the hearing evaluation of the patients were also retrieved including impedance tympanometry, distortion product otoacoustic emissions (DPOAEs), air and bone conduction audiometry and auditory brainstem response (ABR). RESULTS: Vestibular responses were impaired for canal responses (only at high and middle head velocities) and vestibulospinal otolithic responses. Abnormal neural conduction in the central auditory pathways was frequently observed. Oculomotor abnormalities were frequent, mostly hypermetric saccades and gaze instability. Inhibition of the vestibulo-ocular reflex by fixation was normal. CONCLUSIONS: We show that Friedreich ataxia, even at onset, frequently associate saccadic intrusions, abnormal ABRs and decreased vestibulo-ocular and vestibulospinal responses progressing over time. These sensory impairments combined with ataxia further impair patient's autonomy. These vestibular, auditory and visual impairments could be used as markers of the severity and progression of the disease. Adding vestibular and auditory testing to Friedreich patient's evaluation may help physicians improve patient's management.

COVID-19 and ENT Pediatric otolaryngology during the COVID-19 pandemic. Guidelines of the French Association of Pediatric Otorhinolaryngology (AFOP) and French Society of Otorhinolaryngology (SFORL).

OBJECTIVE: Joint guidelines of the French Pediatric Otolaryngology Society (AFOP) and of the French Society of otorhinolaryngology-head and neck surgery (SFORL) on the management of paediatric otolaryngology patients in the context of the COVID-19 pandemic. METHODS: A nation-wide workgroup drew guidelines based on clinical experience, national and local recommendations and scientific literature. Proposals may have to be updated on a day-to-day basis. RESULTS: In children, incidence of symptomatic COVID-19 (1-5%) is low and of good prognosis. The indications for nasal flexible endoscopy should be drastically limited. If undertaken, full Personal Protective Equipment (PPE) including FFP2 masks are required, as well as use of a sheath. Saline nose wash done by caregivers other than parents at home should require PPE. Unless foreign body tracheobronchial aspiration is clinically obvious, CT-scan should be performed to confirm indication of endoscopy. Surgical indications should be limited to emergencies and to cases that cannot be delayed beyond 2 months (especially endonasal, endopharyngeal laryngo-tracheobronchial procedures). Postponement should ideally be a group decision and recorded as such in the medical file. Surgical techniques should be adapted to limit the risk of viral dissemination in the air, avoiding the use of drills, microdebriders, monopolar cautery or lasers. Continuous suction should be placed near the operating field. In case of confirmed Covid-19 cases, or suspected cases (or in some centres systematically), PPE with FFP2 mask should be worn by all staff members present in the operating room.

Recurrent laryngeal nerve monitoring during thyroidectomy and parathyroidectomy in children.

OBJECTIVE: Injury of the recurrent laryngeal nerve (RLN) is the most frequent complication of thyroid and parathyroid (TP) surgery. Monitoring of the RLN in children is not widely studied as this is not a common disease in the pediatric population. The aim of our study was to evaluate the reliability, feasibility and benefits of RLN monitoring during TP surgery in children. METHODS: We analyzed all children who underwent TP surgery in our department between January 2009 and March 2018. Patients were classified into three groups: without monitoring (group 1), monitoring with an endotracheal tube (group 2) and monitoring with a double-needle electrode placed through the cricothyroid ligament (group 3). RESULTS: We performed 53 surgeries (77 RLNs at risk) on 47 patients aged between 6 months and 18 years. Fourteen RLNs were in group 1, 47 in group 2 and 16 in group 3. We found one transient lesion of the RLN in group 1, three in group 2 and two in group 3. Seven patients in group 3 could not undergo monitoring with the tube because they need an orotracheal tube smaller than the smallest monitoring tube size available. The sensitivity and specificity values of the monitoring methods were 33% and 97% in group 2 and 67% and 100% in group 3, respectively. The positive and negative predictive values were 50% and 95% in group 2 and 100% and 92% in group 3, respectively. No complications arose during insertion of the electrode through the cricothyroid ligament. CONCLUSION: Monitoring of the RLN with a double-needle electrode inserted through the cricothyroid ligament is a reliable method with no associated complication, which is achievable at any age.

Guidelines (short version) of the French Society of Otorhinolaryngology (SFORL) on pediatric cochlear implant indications.

OBJECTIVES: The authors present the guidelines of the French Society of Otorhinolaryngology - Head and Neck Surgery (Société française d'oto-rhino-laryngologie et de chirurgie de la face et du cou - SFORL) on the indications for cochlear implantation in children. METHODS: A multidisciplinary work group was entrusted with a review of the scientific literature on the above topic. Guidelines were drawn up, based on the articles retrieved and the group members' individual experience. They were then read over by an editorial group independent of the work group. The guidelines were graded as A, B, C or expert opinion, by decreasing level of evidence. RESULTS: The SFORL recommends that children with bilateral severe/profound hearing loss be offered bilateral cochlear implantation, with surgery before 12months of age. In sequential bilateral cochlear implantation in children with severe/profound hearing loss, it is recommended to reduce the interval between the two implants, preferably to less than 18months. The SFORL recommends encouraging children with unilateral cochlear implants to wear contralateral hearing aids when residual hearing is present, and recommends assessing perception with hearing-in-noise tests. It is recommended that the surgical technique should try to preserve the residual functional structures of the inner ear as much as possible.

French Society of ENT (SFORL) guidelines. Indications for cochlear implantation in adults.

The authors present the guidelines of the French Society of ENT and Head and Neck Surgery (SFORL) regarding indications for cochlear implantation in adults. After a literature review by a multidisciplinary workgroup, guidelines were drawn up based on retrieved articles and group-members' experience, then read over by an independent reading group to edit the final version. Guidelines were graded A, B, C or "expert opinion" according to decreasing level of evidence. There is no upper age limit to cochlear implantation in the absence of proven dementia and if autonomy is at least partial. Bilateral implantation may be proposed if unilateral implantation fails to provide sufficiently good spatial localization, speech perception in noise and quality of life, and should be preceded by binaural hearing assessment. Rehabilitation by acoustic and electrical stimulation may be proposed when low-frequency hearing persists. Quality of life should be assessed before and after implantation.

Barriers to research among residents in Otolaryngology - Head & Neck Surgery around the world.

OBJECTIVES: To document the challenges faced by residents in Otolaryngology - Head & Neck Surgery (OTL-HNS) around the world to successfully complete research projects. The second objective is to assess if the challenges are uniform worldwide. METHODS: A survey was sent to all OTL-HNS under 45 years old from the 2017 IFOS meeting. This survey was conducted by the YO-IFOS group (Young Otolaryngologists of the International Federation of Otolaryngological Societies). Data was collected for a period of 1 month. Demographic characteristics, information regarding research projects conducted and data concerning perceived barriers to completion of research projects were collected. RESULTS: Among the 2787 attendees, 928 responded to the survey (response rate=33.3%). Of these 928 answers, 267 responses were from residents/interns in OTL-HNS, while 635 responses were from certified otolaryngologists. The three most frequent obstacles to conducting research projects for trainees were limited dedicated time (64%), insufficient financial resources (55%) and lack of education in research (45%). There was no statistical difference in these barriers among the different countries (P>0.05). CONCLUSION: This is the first international study that provides insight on trainee's challenges to conduct research projects during residency. Despite the notion that research is essential for generating new knowledge to guide patient care, many residents fail to successfully incorporate research in their surgical curriculum. These obstacles must be addressed by Otolaryngology - Head & Neck Surgery programs in order to facilitate and support resident's research.

EFNB2 haploinsufficiency causes a syndromic neurodevelopmental disorder.

Ephrin B2, one of the ligand of the EphB receptors, is involved in a complex signaling pathway regulating the development of the nervous system, neuronal migration, erythropoiesis and vasculogenesis. We report a patient with a de novo variant in EFNB2 and a family in which segregates a 610-kb deletion at chromosome 13q33 encompassing only ARGLU1 and EFNB2 genes. The de novo variant was observed in a patient with anal stenosis, hypoplastic left ventricle and mild developmental delay. The deletion was identified in 2 sibs with congenital heart defect and mild developmental delay. One of the affected sibs further had myoclonic epilepsy and bilateral sensorineural hearing loss. The carrier mother was apparently asymptomatic. Because EFNB2 is located in the subtelomeric region of 13q chromosome, we reviewed the previous reports of terminal 13q deletion. We suggest that haploinsufficiency of the EFNB2 could be at the origin of several clinical features reported in 13qter deletions, including intellectual disability, seizures, congenital heart defects, anorectal malformation and hearing loss.

International consensus (ICON) on management of otitis media with effusion in children.

Otitis media with effusion (OME) is a common childhood disease defined as the presence of liquid in the middle ear without signs or symptoms of acute ear infection. Children can be impacted mainly with hearing impairment and/or co-occurring recurrent acute otitis media (AOM) thus requiring treatment. Although many meta-analyses and national guidelines have been issued, management remains difficult to standardize, and use of surgical and medical treatments continue to vary. We convened an international consensus conference as part of the 2017 International Federation of Oto-rhino-laryngological Societies Congress, to identify best practices in OME management. Overall, regional differences were minor and consensual management was obtained on several important issues. At initial assessment, although a thorough medical examination is necessary to seek reflux, allergy or nasal obstruction symptoms; an age-appropriate auditory test is the only assessment required in children without abnormal history. Non-surgical treatments poorly address the underlying problem of an age-dependent dysfunctional Eustachian tube; auto-inflation seems to be the only beneficial, low-risk and low-cost non-surgical therapy. There was a clear international recommendation against using steroids, antibiotics, decongestants or antihistamines to treat OME, because of side-effects, cost issues and no convincing evidence of long-term effectiveness. Decisions to insert tympanostomy ventilation tubes should be based on an auditory test but also take into account the child's context and overall hearing difficulties. Tubes significantly improve hearing and reduce the number of recurrent AOM with effusion while in place. Adjuvant adenoidectomy should be considered in children over four years of age, and in those with significant nasal obstruction or infection.

Otologic disorders in Turner syndrome.

INTRODUCTION: Patients with Turner syndrome (TS) have craniofacial malformations, such as Eustachian tube hypoplasia and dysfunction and velar dysfunction, which foster acute otitis media. The aim of this study was to inventory pediatric otologic disorders in patients with TS at their first ENT consultation in our center. PATIENTS AND METHODS: We reviewed the ENT consultation data of pediatric TS patients followed in our center between 2005 and 2015: otoscopy, hearing threshold, and history of acute otitis media or ENT surgery. Data were compared according to karyotype: X monosomy (45,X), mosaic (45,X/46,XX), isochromosome (46,Xi [Xq]), X ring chromosome X (XrX), with Y material, and "other". RESULTS: Ninety patients, with mean age 11.9years (±4.8years) at first ENT consultation, were included: 29% showed tympanic abnormality on otoscopy, 21% had hearing loss, 24% had history of recurrent acute otitis media; 18% had undergone adenoidectomy, 24% T-tube insertion, and 5.6% tympanoplasty. No particular karyotype was associated with higher risk of hearing loss or acute otitis media. CONCLUSION: Patients with TS showed high prevalence of pediatric otologic disorders; they therefore require close and prolonged ENT follow-up.

Emergency Department care of childhood epistaxis.

OBJECTIVE: The aim of this review is to determine an efficient and safe primary strategy care for paediatric epistaxis. DATA SOURCES: We searched PubMed and Cochrane databases for studies referenced with key words 'epistaxis AND childhood'. This search yielded 32 research articles about primary care in childhood epistaxis (from 1989 to 2015). Bibliographic references found in these articles were also examined to identify pertinent literature. We compared our results to the specific management of adult epistaxis classically described in the literature. RESULTS: Epistaxis is one of the most common reasons for referral of children to a hospital ENT outpatient department. The bleeding usually originates from the anterior septum, as opposed to adults. Crusting, digital trauma, foreign bodies and nasal colonisation with Staphylococcus aureus have been suggested as specific nosebleed factors in children. Rare aetiologies as juvenile nasopharyngeal angiofibroma appear later during adolescence. There are different modes of management of mild epistaxis, which begin with clearing out blood clots and bidigital compression. An intranasal topical local anaesthetic and decongestant can be used over 6 years of age. In case of active bleeding, chemical cauterisation is preferred to anterior packing and electric cauterisation but is only feasible if the bleeding site is clearly visible. In case of non-active bleeding in children, and in those with recurrent idiopathic epistaxis, antiseptic cream is easy to apply and can avoid 'acrobatic' cauterisation liable to cause further nasal cavity trauma. CONCLUSIONS: Aetiologies and treatment vary with patient age and the existence or not of active bleeding at the time of the examination. Local treatments are usually easy to perform, but physicians have to ponder their indications depending on the possible complications in order to inform parents and to know paediatric epistaxis specificities.

Audiovestibular consequences of congenital cytomegalovirus infection.

Congenital cytomegalovirus (CMV) infection is the second most frequent cause of mental retardation and sensorineural hearing loss, after genetic factors. Recently, pediatric forensic and fetopathological studies have led to progress in understanding the pathophysiological mechanisms underlying the various neurosensory sequelae. Thanks to the identification of certain prognostic factors of hearing loss, therapeutic protocols based on antiviral molecules are now proposed for target populations. This treatment has shown efficacy in limiting hearing threshold deterioration and even, in some cases, seems to provide partial recovery of hearing in symptomatic congenitally infected CMV neonates. However, optimal treatment duration and administration modalities are not clearly defined. This article reviews recent data concerning audiovestibular sequelae and their management in children congenitally infected by CMV.

Detailed in utero ultrasound description of 30 cases of congenital cytomegalovirus infection.

OBJECTIVES: The aim of this research was to describe precisely prenatal ultrasound (US) features in congenital cytomegalovirus (CMV) infection. METHODS: We retrospectively evaluated the US descriptions of cases of congenital CMV infection between 2004 and 2013. RESULTS: In 69 congenital CMV infections, related US abnormalities were reported in 30 cases (43.5%). There were both extracerebral and cerebral abnormalities in 16 cases, purely abnormal brain features in ten, and purely extracerebral features in two. About 19/30 cases presented extracerebral features of 11 different sorts of abnormalities, mainly hyperechogenic bowel (ten cases) and intrauterine growth retardation (nine cases). About 24/30 cases presented cerebral features of 13 different sorts, mainly brain calcifications (12 cases) and occipital horn cavity (11 cases). The main US findings in our series are not specific to CMV infection. However, a frequent finding attracted our attention: the anechogenic cavity located on the extremity of the occipital horn, a region which contains numerous proliferating and differentiating germinal cells. CONCLUSIONS: By improving knowledge of US findings linked to CMV infection, US sensitivity may be improved. Understanding why CMV leads to lesions of the occipital horn may help clarify the pathophysiology of congenital infection.