Hearing loss in children with sickle cell disease: A prospective French cohort study.

BACKGROUND: Sickle cell disease (SCD) is the most common genetic disease in France. In developing countries, it is associated with a high incidence of hearing loss. The aim of this study was to determine the prevalence of hearing loss in French children with SCD in order to determine if they need a close audiological follow-up. METHODS: We performed a single-center prospective cross-sectional study of children with SCD. The children, without specific hearing symptom, underwent an ear, nose and throat examination with a hearing assessment between 2015 and 2016. RESULTS: Eighty-nine children were included, aged from 5 to 19 years, with 73% of SS or Sß0 genotype and 27% of SC or Sß+ genotype. Ten children (11.2%) had hearing thresholds higher than 20 dB in at least one ear: one child with subnormal hearing, six otitis media with effusion (OME), and three sensorineural hearing loss. Late age at diagnosis of SCD, a high platelet count and a low hematocrit level were significantly associated with OME; moreover, children with OME had more severe clinical and biological characteristics than children with normal hearing. Furthermore, 12.4% of the children complained of tinnitus. The rate of sudden hearing loss was 2.2%. Finally, 7.1% of patients with normal hearing showed a speech discrimination disorder. CONCLUSIONS: Several causes were identified for hearing loss in children with SCD. They therefore need a close audiological follow-up in order to avoid complications due to curable phenomena and to enable appropriate management for progressive complications.

[Rhinosinusitis in children]. / Rhinosinusites de l'enfant.

Rhinosinusitis in children. Rhinosinusitis is an inflammatory disease of the nasal and sinusal mucosa. The diagnosis of an acute rhinosinusitis in a child is based on the persistence of the symptoms of a febrile upper respiratory tract infection for more than 7 to 10 days. Radiological and microbiological examinations, and blood tests are unnecessary. Treatment is based on anti-pyretic/anti inflammatory drugs associated with nasal treatment, and sometimes but not always oral antibiotics. Antibiotherapy is parenteral in case of acute ethmoïditis. Tomodensitometry is required to measure any intracranial or subperiostal intraorbital abcess and decide of a surgical intervention. Chronic rhinosinusitis is diagnosed on endonasal examination. Tomodensitometry verify the diagnosis but is also important to look for an anatomic abnormality. Treatment is based on intranasal treatments and, in case of allergy or polyposis, intranasal corticotherapy. Surgery is seldom required.

Rhinosinusite de l'enfant. La rhinosinusite est une inflammation de la muqueuse des fosses nasales et d'un ou plusieurs sinus de la face. Le diagnostic des rhinosinusites aiguës simples est purement clinique, sur la persistance pendant plus de 7 à 10 jours des symptômes d'une rhinopharyngite aiguë fébrile. Les examens complémentaires (imagerie, examens microbiologiques et sanguins) sont inutiles. Le traitement fait appel aux antalgiques/ antipyrétiques, aux soins locaux et parfois aux antibiotiques oraux. L'antibiothérapie est parentérale en cas d'ethmoïdite aiguë extériorisée. La tomodensitométrie détermine la taille d'un éventuel abcès intracrânien ou intra-orbitaire sous-périosté et l'indication d'une intervention chirurgicale. Les rhinosinusites chroniques sont diagnostiquées sur l'examen endonasal. L'examen tomodensitométrique vérifie le diagnostic et recherche une cause favorisante. Le traitement est fondé sur les soins locaux, et, en cas de polypose ou d'allergie, les corticoïdes intranasaux. Les indications chirurgicales sont rares.

Clinical and haematological risk factors for cerebral macrovasculopathy in a sickle cell disease newborn cohort: a prospective study.

Children with sickle cell disease (SCD) have a significant vascular morbidity, especially cerebral macrovasculopathy (CV), detectable by transcranial Doppler. This study aimed to identify risk factors for CV using longitudinal biological and clinical data in a SCD newborn cohort followed at the Robert Debre Reference centre (n = 375 SS/Sß(0) ). Median follow-up was 6·8 years (2677 patient-years). Among the 59 children presenting with CV, seven had a stroke. Overall, the incidence of CV was 2·20/100 patient-years [95% confidence interval (95% CI): 1·64-2·76] and the incidence of stroke was 0·26/100 patient-years (95% CI: 0·07-0·46). The cumulative risk of CV by age 14 years was 26·0% (95% CI: 20·0-33·3%). Risk factors for CV were assessed by a Cox model encompassing linear multivariate modelling of longitudinal quantitative variables. Years per upper-airway obstruction [Hazard ratio (HR) = 1·47; 95% CI: 1·05-2·06] or bronchial obstruction (HR = 1·76; 95% CI: 1·49-2·08) and reticulocyte count (HR = 1·82 per 50 × 10(9) /l increase; 95% CI: 1·10-3·01) were independent risk factors whereas fetal haemoglobin level (HR = 0·68 per 5% increase; 95% CI: 0·48-0·96) was protective. Alpha-thalassaemia was not protective in multivariate analysis (ancillary analysis n = 209). Specific treatment for upper or lower-airway obstruction and indirect targeting of fetal haemoglobin and reticulocyte count by hydroxycarbamide could potentially reduce the risk of CV.

Congenital cytomegalovirus is the second most frequent cause of bilateral hearing loss in young French children.

OBJECTIVE: To estimate the prevalence of congenital cytomegalovirus (cCMV) among causes of bilateral hearing loss in young French children. STUDY DESIGN: Children <3 years old with hearing loss were prospectively included at their first visit to a referral center. Cytomegalovirus polymerase chain reaction was performed on dried blood spots from Guthrie cards. Medical records were reviewed. RESULTS: One hundred children with bilateral hearing loss were included at a median age of 15 months; the prevalence of cCMV was 8% (8/100) (95% CI, 2.7%-13.3%) in this population and 15.4% (8/52) in the subpopulation of children with profound bilateral hearing loss. Delayed neurodevelopment and brain abnormalities on computed tomography scan were found more often in children with cCMV than in children with hearing loss without cCMV (P = .027, P = .005). In 6 of 8 cCMV cases, cCMV infection had not been diagnosed before the study. CONCLUSIONS: In a comprehensive study of the causes of bilateral hearing loss in young French children, cCMV is the second most frequent cause of hearing loss after connexin mutations. It underlines that a majority of French children with hearing loss and cCMV are not diagnosed early and therefore may not benefit from early intervention including the possibility of neonatal antiviral treatment. These results make the case for promoting systematic cytomegalovirus screening in neonates with confirmed hearing loss identified through neonatal hearing screening.

Sedation for children 2 to 5 years of age undergoing auditory brainstem response and auditory steady state responses recordings.

OBJECTIVE: To evaluate the feasibility, the duration and results of sedation by intrarectal pentobarbital and oral alimemazine for auditory brain stem responses (ABR) and auditory steady-state responses (ASSR) recordings in children aged 2 to 5 years. DESIGN: Prospective study. STUDY SAMPLE: 180 consecutive children aged 2 to 5 years, referred for language retardation and/or behavioral problems, who could not be tested by behavioral methods, underwent ABR and ASSR recordings. The children who did not spontaneously nap were sedated by intrarectal pentobarbital eventually potentiated by oral alimemazine. RESULTS: A spontaneous nap was obtained in only 23 cases, 72 children received only pentobarbital, and 85 received both pentobarbital and alimemazine. Even so, recording was impossible in 16 cases, and interrupted before completion of the ASSR recordings in 45 cases. Children went to sleep in average 64 min +/- 40. The average recording time for the ABR was 20 minutes, and for the ASSR 25 minutes. CONCLUSION: Sedation by pentobarbital, eventually completed by oral alimemazine, allows ABR and/or ASSR recordings in 89.8% of the children who did not nap in the recording room, and is therefore a good alternative to general anesthesia in these children.

Surgical Treatment for Cerebrospinal Fluid Leaks in Patients With Inner Ear Malformations.

OBJECTIVE: The aim of this study is to present our technique of surgical closure in a series of patients suffering from cerebrospinal fluid (CSF) leak due to inner ear malformations. STUDY DESIGN AND SETTING: We conducted a retrospective study in our tertiary care academic department of pediatric otolaryngology. PATIENTS: We did include all patients who presented a CSF leak or bacterial meningitis (one episode or recurrent) related to a malformation of the inner ear. INTERVENTION(S): Through a retro-auricular or endaural approach we performed a filling of the vestibule cavity with multiple fragments of cartilage with perichondrium introduced through the oval window, after stapedectomy until a near-complete sealing was obtained. After the surgery, all patients received a treatment with acetazolamide during 15 days. MAIN OUTCOME MEASURE(S): We did evaluate our technics with the recurrence of CSF leak. RESULTS: Thirteen patients, from 1 to 14, were operated with our technics. With a follow-up of 4.4 ±â€Š4.7 years, only one patient needed a second intervention. None had a novel episode of meningitis. We observed no complication. The leak was observed in the oval fossa in 11 cases. CONCLUSIONS: Our "minimally invasive" technique of vestibular obliteration with cartilage inserted through the oval window after stapedectomy did demonstrate its safety and reliability.

Positive predictive value for diffusion-weighted magnetic resonance imaging in pediatric cholesteatoma: A retrospective study.

OBJECTIVES: The aims of this study were first to calculate the Positive Predictive Value (PPV) of DW-MRI to detect cholesteatoma and then to analyze false positives. METHODS: All temporal bone MRI with DWI sequences performed in our pediatric university hospital between 2005 and 2015 were included retrospectively. 46 patients with a cholesteatoma diagnosis on the MRI report and who underwent surgery were studied. RESULTS: The number of DW-MRI for identification of cholesteatoma has grown in ten years. We calculated an 89% Positive Predictive Value. DW-MRI sensitivities were 100.0% and 70.7% for respectively keratin and squamous epithelium. CONCLUSION: DW-MRI hypersignal is not synonymous of cholesteatoma diagnosis. Indeed, this diagnosis relies on the importance of a proper otoscopic examination, a suggestive medical history, CT scan data and analysis of other MRI sequences, including T1-weighted sequence, to rule out other etiologies of middle ear DW-MRI hypersignal.

Predictive factors for success after transnasal endoscopic treatment of choanal atresia.

OBJECTIVE: To analyze the different factors affecting the outcome of transnasal endoscopic repair of choanal atresia (CA) in children. DESIGN: Retrospective study. SETTING: Academic tertiary care children's hospital. PATIENTS: Eighty patients (48 girls and 32 boys) aged 3 days to 17 years (mean age, 3 years 8 months) who presented with unilateral (n = 53: 37 right, 16 left) or bilateral (n = 27) CA and underwent surgery between September 1996 and December 2005. INTERVENTION: All patients underwent transnasal endoscopic surgery with telescopes and a microdebrider. Nasal tubes in neonates and nasal packing in older children were removed after 48 hours. Systematic endoscopic revision was performed under local or general anesthesia a week after surgery. Patients were then clinically and endoscopically monitored for nasal obstruction and healing for a mean follow-up of 43 months. RESULTS: A total of 30 patients presented with associated malformations: 9 with CHARGE (coloboma, heart disease, choanal atresia, retardation of postnatal growth()and mental development, genital hypoplasia, and ear anomalies), 1 with Treacher-Collins syndrome, 1 with Kabuki syndrome, 1 with facial cleft, 1 with Down syndrome, 12 with nonsyndromic malformations, and 2 with 22q11 microdeletion. Three children had heart malformations not related to CHARGE association. One child had a congenital nasal piriform aperture stenosis. Twenty-four children had undergone previous surgery; 10 underwent a second procedure with success. Gastroesophageal reflux disease (GERD) was systematically treated in cases of restenosis. Topical mitomycin C was used in 3 patients with relapse. Two patients underwent laser treatment to reduce stenotic scarring. Of the 10 patients who needed revision surgery, 6 had bilateral CA, and 4 had unilateral CA. Age younger than 10 days and presence of GERD increased the chances of restenosis (P = .03). Postoperative stenting negatively affected the outcome. Associated anomalies and previous surgery had no effect on outcome. The bony nature of the CA and bilaterality were not significant (P = .08). However, surgeon learning curve was an important element positively influencing the results (P = .04). CONCLUSIONS: Transnasal endoscopic repair of CA is a safe and successful technique. Predictive factors of restenosis are the presence of GERD, age younger than 10 days at the time of surgery, and insufficient postoperative endoscopic revision. However, previous surgery and associated malformations are not predictive of a poor surgical outcome.

Congenital Midline Cervical Cleft and W-Plasty: Our Experience.

OBJECTIVES: Congenital midline cervical cleft (CMCC) is a very uncommon congenital anomaly of the midline anterior neck, and although it has very pathognomonic features (including nipple-like protuberance), it could be mistaken for other congenital neck lesions, such as thyroglossal duct cyst and branchial apparatus anomalies. Thus, it represents a challenging diagnosis. In this 21-patient series, we discuss the clinical features of CMCC, its pathophysiology characteristics, and its modalities management. MATERIAL AND METHODS: We conducted a retrospective chart review of children presenting with CMCC at our institution, between January 1998 and January 2016. RESULTS: Twenty-one patients were identified with CMCC. Ages ranged between 1 day and 14 years. The length of the lesion varied from 0.5 to 5 cm, and the size of the skin tag varied from 0.2 to 1.5cm. No other significant associated anomalies were found. Surgery was the mainstay treatment, and no recurrence was found. W-plasty was used in most patients to close the defect. CONCLUSION: With a little more than 200 published cases, our series represents the largest series worldwide. The lesion is usually isolated, and no further investigation is required. Surgery is the mainstay of treatment, with complete excision being usually curative. It should be treated at an early age to prevent complications. In our experience, W-plasty was a good alternative to the most commonly used Z-plasty, in skin closure, with respect to both aesthetic and functional results.

Inlay butterfly cartilage tympanoplasty in children.

OBJECTIVE: To assess the results of inlay butterfly cartilage tympanoplasty in children. STUDY DESIGN: Before-and-after trial; follow-up duration, 26.6 +/- 19.9 months (mean +/- standard deviation). SETTING: Tertiary referral center. PATIENTS: Fifty-nine pediatric cases of tympanic membrane perforation. INTERVENTION: Inlay butterfly cartilage tympanoplasty was performed under general anesthesia according to the technique originally described by Eavey and modified by Lubianca-Neto (i.e., without any associated split-thickness skin graft). MAIN OUTCOME MEASURES: Percentage of perforation closures, surgical complications, preoperative and postoperative puretone hearing thresholds; the results of inlay butterfly cartilage tympanoplasty were compared with those obtained in a retrospective series of 29 underlay fascia temporalis myringoplasties. RESULTS: The 71% "take rate" of inlay butterfly cartilage tympanoplasty was not significantly different from the 83% take rate obtained with underlay fascia temporalis tympanoplasty (p = 0.23, chi test). The anatomic results were improved when the graft diameter was at least 2 mm larger than the size of the perforation (81% take rate) (p = 0.009, chi test). No iatrogenic cholesteatoma was observed. Pure-tone hearing thresholds were improved at 0.5, 1, and 2 kHz, and stable at 4 kHz. Hearing levels were not different from those obtained with underlay fascia temporalis tympanoplasty. CONCLUSION: Inlay butterfly cartilage tympanoplasty is a safe, efficient, time-saving, and easy technique of tympanoplasty in children. Anatomic results may be improved by associating a split-thickness skin graft and/or by trimming a tragal graft much larger than the size of the perforation.

Transnasal endoscopic treatment of choanal atresia without prolonged stenting.

OBJECTIVE: To analyze the outcome of transnasal endoscopic repair of choanal atresia (CA) in children without prolonged nasal stenting after surgery. DESIGN: Retrospective study. SETTING: Academic tertiary care children's hospital. PATIENTS: Forty children aged 3 days to 15 years (mean age, 41 months) who presented with unilateral (n = 26) or bilateral (n = 14) CA and underwent surgery between August 1996 and December 1999. INTERVENTION: All children underwent transnasal endoscopy with telescopes, endoscopic instruments, and a microdebrider. Nasal tubes in neonates or infants and nasal packing in older children were always removed after 2 days. Systematic revision endoscopy was performed with the patients under local or general anesthesia on days 6 to 10. All patients were then clinically and endoscopically monitored for nasal obstruction and healing during a mean follow-up period of 18 months. RESULTS: There were 16 patients with associated malformations, including 6 cases of CHARGE association (a malformative syndrome that includes coloboma, heart disease, CA, retarded development, genital hypoplasia, and ear anomalies, including hypoplasia of the external ear and hearing loss), and 14 patients (9 with bilateral CA) with a history of previous choanal surgery (4 transnasal, 4 laser, and 6 transpalatine). Postoperatively, 32 patients (80%) had normal nasal patency and a satisfactory choanal diameter, and 8 (20%) had restenosis or complete choanal closure. Six underwent a second procedure, with success. The results in all children who had been previously treated with laser or transpalatine surgery were successful. The last patient, who presented with severe Treacher Collins syndrome, is still tracheotomized. There were no significant postoperative complications. One patient died of congenital cardiopathy 6 months after surgery. CONCLUSIONS: Transnasal endoscopic repair of CA is a safe and successful technique. The use of powered instrumentation and routine postoperative revision endoscopy seems to avoid prolonged nasal stenting.

Pathogens implicated in acute otitis media failures after 7-valent pneumococcal conjugate vaccine implementation in France: distribution, serotypes, and resistance levels.

BACKGROUND: Before 7-valent pneumococcal conjugate vaccine (PCV7) implementation in France, several studies had described the microbiology of acute otitis media (AOM) treatment failures. The causative pathogens were Streptococcus pneumoniae (Sp) followed by nontypable Haemophilus influenzae (NTHi). The aim of this study was to describe the epidemiology of pathogens involved in AOM treatment failures or recurrences. METHODS: This French multicentric prospective study enrolled 143 children with AOM treatment failure between 2007 and 2009 observed by 8 ear, nose, and throat specialists. Failure was defined as the persistence of AOM symptoms after at least 48 hours of antibiotic therapy or their recurrence within 4 days after the end of treatment. Standardized history and physical examination findings were recorded, and culture of middle ear fluid (MEF) was obtained. RESULTS: Mean age was 16.9 ± 9.9 months (median, 13.7). Eighty-eight percent of children had received more than 1 dose of PCV7, and 70.6% attended day care. The most common antibiotic used at the time of treatment failure or recurrence was a combination of amoxicillin and clavulanate (51.1%). Bacteriologic sampling demonstrated that in 35% of cases (n=50), no otopathogen was cultured at the time of treatment failure or recurrence. Similar proportions of Sp and NTHi were observed in the 86 patients (60.1%) from whom only a single species was recovered from MEF (46.5% for Sp, n=40 and 45.3% for NTHi, n=39). Among Sp strains, 4.4% were penicillin susceptible, 77.8% were penicillin intermediate, and 17.8% were fully penicillin resistant, and serotype 19A represented 84.5% of all serotypes detected. Among NTHi isolates, 15.5% (n=7) were ß-lactamase-producing strains (including 2 strains with only this mechanism of resistance), and strains with reduced susceptibility by changes in protein binding to penicillin (ß-lactamase-negative ampicillin resistant strains) represented 35.5% of cases. Among the 50 sterile MEF samples, polymerase chain reaction was performed in 32, of which 4 were positive for HI, 3 for Sp, and 3 for both. CONCLUSIONS: Among children with AOM treatment failures in France, Sp and NTHi were equally distributed; 19A was the main Sp serotype, and the main resistance mechanism for NTHi was ß-lactamase-negative ampicillin resistance.

Evolving microbiology of complicated acute otitis media before and after introduction of the pneumococcal conjugate vaccine in France.

We compare the microbiology of otopathogens causing recurrent acute otitis media (AOM) or AOM treatment failure in 600 children during 2000 to 2008 before and after the introduction of 7-valent pneumococcal conjugate vaccine (PCV-7). Streptococcus pneumoniae predominated before PCV-7 introduction and during 2007 to 2008, whereas Haemophilus influenzae predominated during 2005 to 2006. S. pneumoniae 19A became the most frequent serotype after PCV-7 introduction.

The health care burden and societal impact of acute otitis media in seven European countries: results of an Internet survey.

This paper estimates medical resource use, direct costs, and productivity losses and costs (indirect costs) during episodes of acute otitis media (AOM) in young children. A 24-item Internet questionnaire was developed for parents in Belgium (Flanders), France, Germany, Italy, The Netherlands, Spain, and the United Kingdom (UK) to report health care resource use and productivity losses during the most recent episode of AOM in their child, younger than 5 years. The percentage who did not seek medical help for AOM was considerable in The Netherlands (28.3%) and the UK (19.7%). Antibiotic use was high, ranging from 60.8% (Germany) to 87.1% (Italy). Total costs per AOM episode ranged from €332.00 (The Netherlands) to €752.49 (UK). Losses in productivity accounted for 61% (France) to 83% (Germany) of the total costs. AOM poses a significant medical and economic burden to society.