Nap polysomnography in infants with laryngomalacia as a tool to predict treatment strategy.

PURPOSE: This study aimed to investigate the role of nap polysomnography (NPSG) in predicting treatment strategies for infants with moderate to severe laryngomalacia and to explore the association between obstructive sleep apnea (OSA) severity, weight gain, and laryngomalacia severity. METHODS: A retrospective analysis was conducted on infants diagnosed with moderate to severe laryngomalacia who underwent NPSG between January 2019 and June 2023. Clinical variables, NPSG parameters, and treatment decisions were collected. Weight gain rate and its correlation with NPSG indices were assessed. Logistic regression analyses were performed to predict treatment strategies based on NPSG findings. RESULTS: Of the 39 infants included (median age: 3.3 months), 77% exhibited OSA, with 69% having moderate to severe OSA [apnea-hypopnea index (AHI) > 5/h]. Weight gain rate correlated negatively with indices of OSA severity, including the hypopnea index (HI) and the AHI. In a multiple logistic regression analysis incorporating the severity of OSA (AHI), weight gain rate, and laryngomalacia severity, only AHI predicted the decision for surgical or non-invasive ventilation treatment (OR = 2.1, CI95 [1.6; 2.8], p ≤ 10-4). The weight gain rate was predicted (r2 = 0.28) by the AHI and the presence of retractions of auxiliary inspiratory muscles. CONCLUSION: This study underscores the importance of NPSG in assessing infants with moderate to severe laryngomalacia. The AHI from NPSG emerged as a potential predictor for treatment decisions and weight gain rate, emphasizing its clinical relevance. These findings advocate incorporating NPSG into the diagnostic and management process for infants with laryngomalacia.

New surgical frontiers for 4K 3D-exoscope in paediatric head and neck surgery.

PURPOSE: To define the interest, advantages, and disadvantages of the use of a 3D-exoscope in paediatric ENT surgery. METHODS: Four surgeons with experience in paediatric surgery completed a questionnaire following each surgery performed under 3D-exoscope to evaluate the contribution of the tool compared to the usual practice (microscope or magnifying loupes). Surgeries were separated into three groups: otology, transoral and cleft palate surgery, and open head and neck surgery. RESULTS: Between June 2021 and June 2022, 151 paediatric surgeries were included in this study. Among them, 93 (62%) otologic surgeries, 35 (23%) transoral surgeries, and 23 (15%) head and neck surgeries were performed. The median age at surgery was 68 months (interquartile range 19-135 months). For otologic surgeries, the mean scores (/100) for the contribution of the exoscope compared to the microscope were 68.4(± 23.2). For transoral and cleft palate surgery, the mean score (/100) for the contribution of the use of the exoscope compared to the magnifying loupes was 92.9 (± 8.6), whereas for open head and neck surgeries, the mean score (/100) was 89.5 (± 7.2). CONCLUSION: 3D-exoscopy appears to be a relevant tool for paediatric head and neck surgery, applicable in otologic, transoral, and cervical fields. It presents educational and ergonomic advantages and improves surgical team communication.

PEANUTS: a national survey on the management of pediatric tracheobronchial foreign bodies.

To assess the different types of management of pediatric foreign body aspirations in order to help define guidelines, depending on clinical presentation. A national survey in France was emailed to all 30 university-affiliated departments of otolaryngology-head and neck surgery and pediatric pulmonologists in France. Data concerning the center and the experience of each participant were collected in order to define an "senior expert" group with experience of extracting more than 10 foreign bodies and a "junior/non-expert" group. Both groups answered questions concerning five clinical cases of foreign body aspiration of different severities. Ninety-eight participants answered this survey (75 otolaryngologists and 23 pulmonologists), representing 28 of the 30 university-affiliated departments in France with a completion rate of 89%. Responses of the senior expert group were similar for clear-cut situations, such as an asymptomatic child with a low probability of foreign body aspiration and for a symptomatic child with respiratory distress. However, for intermediate situations, management varied significantly according to the physician when considering clinical, radiological, and surgical management. In comparison to the senior expert group, the junior non-expert group seemed more precautious in the management of foreign body aspiration for intermediate cases, in particular, concerning the time at which extraction was performed.  Conclusion: The management of foreign body aspiration depends on the physician's experience and the center's habits. In order to optimize patient care for foreign body aspiration, we suggested a management algorithm based on the senior expert group responses. What is Known: • Foreign body aspirations (FBA) are dreaded by pediatricians, pediatric otolaryngologists, and pulmonologists particularly because of the potential fatal outcome. • However, consensus concerning their management is not clearly defined in the literature. What is New: • This study is the first to evaluate the management of foreign body aspirations (FBA) from the clinical assessment by the emergency medicine physician to the extraction of the foreign body. • A management algorithm was designed and secondarily validated by the SE group to help to emergency medicine physician and specialist to manage FBA.

Is CMV PCR of inner ear fluid during cochlear implantation a way to diagnose CMV-related hearing loss?

To evaluate the diagnostic performance of cytomegalovirus (CMV) polymerase chain reaction (PCR) on inner ear fluid collected during cochlear implantation and to assess its interest in current practice. This monocentric prospective study included consecutive children presenting with severe to profound sensorineural hearing loss (SNHL) who were candidates for unilateral and/or bilateral cochlear implantation. The etiology of the SNHL was determined before cochlear implantation when possible. During the surgery, drop-like samples of inner ear fluid and blood were collected. CMV PCR was then performed on both samples. Between January 2017 and September 2021, 113 children with severe to profound SNHL underwent cochlear implantation with inner ear fluid collection. Among these children, 77 of them presented with a known cause of SNHL (68%) and 36 of them had an unknown cause of SNHL at the time of surgery (32%). Sensitivity and specificity of the CMV PCR on inner ear fluid were 60% (95% CI: [49-71]) and 98% (95% CI: [96-100]), respectively. Positive and negative predictive values were 90% (95% CI: [83-97]) and 92% (95% CI: [86-98]), respectively. A sensitivity analysis according to age at cochlear implantation showed a decrease with age. CONCLUSION: Sampling of inner ear fluid during cochlear implant surgery is an interesting, simple and safe way to diagnose CMV-related hearing loss, especially when the diagnosis of congenital infection can no longer be confirmed. However, the sensitivity decreases with age. TRIAL REGISTRATION: NCT04724265 What is Known: • Congenital cytomegalovirus infection is the leading infectious cause of neurological disabilities and sensorineural hearing loss in children. In the absence of systematic screening at birth, many cCMV infections go undetected and are often undiagnosed despite the development of sensorineural sequelae.  • Nearly 40% of indications for cochlear implantation are of unknown etiology. WHAT IS NEW: • Performing CMV PCR on inner ear fluid at the time of cochlear implantation is a safe way with high diagnostic performance (PPV = 90%, NPV = 92%) to detect a CMV-related hearing loss. • This sample may be interesting in cases of unknown cause of hearing loss in order to identify undiagnosed cCMV infections.

Audiovestibular Consequences of Congenital Cytomegalovirus Infection: Greater Vulnerability of the Vestibular Part of the Inner Ear.

OBJECTIVES: To investigate the impact of congenital cytomegalovirus infection on cochlear and vestibular function. DESIGN: This retrospective study conducted between March 2014 and March 2020 included children with confirmed congenital cytomegalovirus infection who underwent a complete audio-vestibular evaluation. It included a bithermal caloric test, a video head impulse test and a cervical vestibular evoked myogenic potential associated with a complete hearing assessment. RESULTS: The cohort of 130 children included in the study had a median age of 21 months (interquartile range: 12 to 37 months). Eighty-three children (64%) showed an inner ear impairment (both cochlear and vestibular). The vestibular part of the inner ear was significantly more frequently impaired than the cochlear part (ρ = 0.003). Sixty-two children (48%) showed confirmed hearing impairment. The severity of hearing loss was variable, with a high proportion of profound hearing loss (30/62, 48%), which was often bilateral (47/62, 76%). The vestibular assessment showed a canal function disorder in 67 children (88%) and an otolith function disorder in 63 children (83%; ρ = 0.36). The video head impulse test was significantly less altered (64%) compared with the bithermal caloric test (80%; ρ = 0.02) and the cervical vestibular evoked myogenic potential (83%; ρ = 0.009). Only seven out of 83 children (8%) showed hearing loss without vestibular dysfunction, of which only one had a normal hearing screening test at birth. For the children who passed the hearing screening test at birth and presented an inner ear impairment [n = 36, median age: 16 (11 to 34) months], vestibular disorders were later found in 35 children (97%) and 17 of them (47%) developed hearing loss secondarily. This underlines the importance of assessing both vestibular and auditory parts of the inner ear. When comparing the agreement of cochlear and vestibular impairment, the severity and the laterality of the impairment were low [Cohen's kappa 0.31 (0.22 to 0.40) and 0.43 (0.32 to 0.55), respectively]. CONCLUSION: In our study, we demonstrated that although both cochlear and vestibular parts of the inner ear can be impaired by congenital cytomegalovirus infection, the vestibular part seems more often impaired compared with the cochlear part. This underlines the importance of vestibular evaluation in the follow-up of cytomegalovirus-infected children associated with hearing assessment.

Predictors of cochleovestibular dysfunction in children with congenital cytomegalovirus infection.

The purpose of this study is to assess the predictive factors of both hearing and vestibular impairment in congenitally cytomegalovirus-infected children (cCMV) through a multivariate analysis of clinical and imaging characteristics collected during pregnancy and at birth. This retrospective study was conducted between March 2014 and March 2020, including confirmed congenitally CMV-infected children with a complete vestibular and hearing assessment. Data concerning pregnancy, date of infection, clinical characteristics, and symptomatology at birth were collected. In total, 130 children were included, with a median age of 21 months. Eighty-three children (64%) presented with an inner ear impairment (both cochlear and vestibular impairment). Sex, modality of maternal infection (seroconversion or reactivation), pregnancy term, weight and head circumference at birth, neonatal clinical signs of infection, and treatment were not significantly correlated with inner ear impairment. However, multivariate analysis confirmed that there are two independent predictive factors of inner ear impairment: antenatal imaging lesions (ORa = 8.02 [1.74; 60.27], p-value = 0.01) and infection during the first trimester (ORa = 4.47 [1.21; 19.22], p-value = 0.03). Conversely, infections occurring during the second trimester were rarely associated with inner ear impairment: 4/13 (31%) in our series, with vestibular impairment alone (4/4) and no hearing loss. None of the children infected during the third trimester developed inner ear dysfunction. CONCLUSION: Besides the symptomatic status of the CMV infection at birth, we found that antenatal imaging brain damage and early infection (mainly during the first trimester) constitute the two best independent predictive factors of inner ear involvement in congenitally CMV-infected children. WHAT IS KNOWN: • Congenital cytomegalovirus infection is the leading infectious cause of neurological disabilities and sensorineural hearing loss in children and responsible of vestibular disorders, which are probably underestimated. • No articles have yet defined the predictive factors of the entire inner ear impairment (vestibule and cochlea). WHAT IS NEW: • The timing of the infection during pregnancy (first and second trimester, ORa=4.47) and antenatal imaging lesions (ORa=8.02) are independently predictive (in a multivariate analysis) of inner ear involvement. • The symptomatic status at birth is a poor predictor of inner ear impairment.

Olfactory function in congenital cytomegalovirus infection: a prospective study.

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 < 7 years. For children > 6 years, the proportion of children with total olfactory score < 4 differed between sCMV and CTL groups (91.2% and 18.7%, P < 0.001), but not between aCMV and age-matched healthy control groups.   Conclusion: Congenital CMV infection is associated with reduced olfactory performance in children with infection symptoms at birth.   Clinical trial registration: NCT02782988 (registration date: May 26, 2016). What is Known: •Congenital cytomegalovirus infection leads to olfactory bulb lesions in the fetus, yet little is known about its impact on olfaction after birth. •Depending on neonatal clinical presentation, children are either categorized as having a symptomatic or asymptomatic infection at birth. What is New: •Congenital cytomegalovirus infection is associated with reduced olfactory performance in children with infection symptoms at birth.

Head and neck tumors in children and adolescents: Impact of a multidisciplinary tumor board.

INTRODUCTION: Cancer treatment in France is based on Multidisciplinary Tumor Board (MTB). In the Ile-de-France region (IDF), which includes 12 million inhabitants from Paris and the surrounding area, pediatric tumors of head and neck are discussed since 2013 in a dedicated Interregional Pediatric Multicentric MTB (IPMTB). The purpose of this study is to analyze the impact of the IDF head and neck IPMTB on the management of these tumors, 5 years after their implementation. MATERIALS AND METHODS: Retrospective study of all patient files presented in the IPMTB for a benign or malignant head-and-neck tumor, between 2013 and 2018. RESULTS: A total of 679 discussions were analyzed representing 428 patients. Median age was 7.5 years (range: 0-31 years). Malignant tumors represented 71% of cases, including 36% of rhabdomyosarcoma. Overall, 12% percent of the cases discussed came from centers outside of IDF. All meetings complied with multidisciplinary criteria required by French law. Proposals made during the IPMTB were followed in 86% of cases. Among the 251 proposals made by the referring teams prior to the IPMTB, 29% were secondarily modified after being discussed in the IPMTB. CONCLUSION: Thanks to their multidisciplinarity, high number of cases discussed and usual respect of their proposals, the IPMTB have made it possible to improve the coordination between all specialties involved in the patient's management, to apply the most recent and scientifically validated protocols, and to share the knowledge of different teams concerning the management of particularly rare tumors.

Right Incus Osteoma in a Child: A Differential Diagnosis of Middle Ear Malformations.

Osteomas of the temporal bone, especially those involving the incus, are rare, unilateral, benign osseous tumors. The clinical presentation is usually isolated conductive hearing loss, and the diagnosis is confirmed by a temporal computed tomography scan. Osteomas of the incus represent a differential diagnosis of congenital middle ear malformations in children, which are the most frequent cause of conductive hearing loss with a normal eardrum in the pediatric population. In case of disabling symptomatology, surgery seems to be a safe way to recover normal hearing.

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.

Bacterial biofilm in adenoids of children with chronic otitis media. Part II: a case-control study of nasopharyngeal microbiota, virulence, and resistance of biofilms in adenoids.

Background: We previously described that adenoid tissue in children with chronic otitis media (COM) contained more mucosal biofilms than adenoid tissue removed for hypertrophy.Aims/objectives: The aim of the second part was to characterize nasopharyngeal microbiota and explore virulence of the most common middle ear pathogens.Material and methods: Bacteriological analysis was performed following a culture-based approach on the samples recovered from 30 patients of COM group (15 biofilm-positive and 15 biofilm-negative) and from 30 patients of a control group (15 biofilm-positive and 15 biofilm-negative). Virulence factors of Streptococcus pneumoniae, Streptococcus pyogenes, and Haemophilus influenzae were investigated.Results: The most frequent species were Firmicutes followed by Proteobacteria and Actinobacteria. The presence of biofilm was statistically associated with an increase of the number of bacterial species and Firmicutes phylum regardless of the condition (case/control). No virulence factors associated with invasive isolates were found for the most common middle ear pathogens.Conclusions and significance: This case-control study demonstrated that the presence of COM plus biofilm was associated with a given microbiota which contained more Firmicutes. Our study allows a better understanding of physiopathological mechanisms involved in chronic otitis media and paves the way for further investigations.

Evaluation of Tonsillotomy Effects on Pharyngeal Volume and Compliance in Children.

Our objective was to assess whether adenotonsillotomy improved pharyngeal compliance, which is a risk factor for sleep-disordered breathing. Otherwise healthy children underwent Obstructive Sleep Apnea (OSA)-18 questionnaire and a pre- and postoperative acoustic pharyngometry in both sitting and supine positions, allowing the measurement of the volume of the palatine tonsil region and pharyngeal compliance. Thirty-five children (median age 5.3 years) were enrolled; they were reevaluated at a median of 18 days (25th-75th percentiles, 15-25) after surgery. Participants were compared according to a normal (n = 18) or an increased (n = 17) preoperative pharyngeal compliance. Surgery was associated with a significant decrease in OSA-18 and Brodsky scores, with a median increase in palatine volume of 0.13 cm3 (25th-75th percentiles, 0.00-0.73). A decrease in pharyngeal compliance was observed in children with increased preoperative compliance. The variation of palatine volume after surgery was positively related to the variation of pharyngeal compliance, suggesting that obstruction relief was associated with muscle relaxation in children with normal preoperative compliance.

Postural Instability in Subjects With Usher Syndrome.

This study investigated postural performances and vestibular impairment in Usher patients. The three groups studied were: 11 patients with Usher type I (with visual and vestibular impairment), 14 patients with Usher type II (with only visual impairment), and 14 healthy control subjects. Postural stability was measured with a Framiral Multitest Equilibre platform with three visual conditions: eyes open (EO), eyes closed (EC), and vision disturbed by optokinetic stimulation (OPT), and two different postural conditions: stable or unstable platform. The surface and mean velocity of the center of pressure displacement (CoP) were measured and a postural instability index (PII) was calculated. Usher type I and II patients were more unstable than control subjects, but only for the unstable platform. Patients with Usher type I (with severe vestibular impairment) were also significantly more unstable than patients with Usher type II (with normal vestibular function) on the unstable platform. The severity of the vestibular impairment was correlated with the surface of the CoP displacement. We suggest that poor postural control of Usher patients is due to the abnormalities in their visual and, when defective, vestibular inputs. Measurements of postural stability on an unstable platform can distinguish type I from type II Usher patients. We emphasize the importance of multisensory evaluation in these patients to guide development of personalized visuo-vestibular rehabilitation techniques to improve their postural stability and improve their quality of life.

Desmoid-type fibromatosis of the head and neck in children: A changing situation.

INTRODUCTION: Desmoid-type fibromatosis (DF) is a rare benign lesion known for its local aggressiveness. The tumor management still remains under debate. Primary head and neck (HN), represents the second most prevalently affected sitein children with DF. This study aims to analyze the specificity of HN-DF in children, focusing on long-term effects of the tumor and therapies. METHODS: This retrospective multicenter study analyzed children treated for a HN-DF between 1993 and 2013. All medical files were reviewed and their outcomes analyzed according to the initial therapies provided. RESULTS: Sixteen children were selected. Mandibular and submandibular areas were the main locations (11 cases). Eight children underwent chemotherapy as first-line therapy with tumor control in 3 cases and 5 cases needing additional treatment. Six children underwent primary surgery: isolated in 3 cases and with additional treatment after tumor progression in 3 cases. A wait-and-see attitude was adopted for 2 children without any additional treatment in 1 case, and followed by additional chemotherapy in the other case. Total burden of treatment to control the disease was a biopsy (1 case), surgery (3 unique cases, 1 multiple case), surgery with chemotherapy (6 cases), and exclusive medical therapies (5 cases). Surgical postoperative sequelae were facial palsy (cases of parotid gland affection), XIth cranial nerve sacrifice or sensory impairment. CONCLUSION: HN-DF is a local and extensive disease that is difficult to control with surgery alone. Sequelae are frequent due to the initial tumor location or therapies. Initial conservative strategies need to be discussed in a multidisciplinary way in order to try to control the disease with the minimal morbidity.

Computed Tomography Description of the Uncinate Process Angulation in Patients With Cystic Fibrosis and Comparison With Primary Ciliary Dyskinesia, Nasal Polyposis, and Controls.

BACKGROUND:: There is a medial bulging of the lateral nasal wall in patients with cystic fibrosis (CF). AIMS:: Uncinate process (UP) angulation measurements in patients and controls to objectify this bulging. MATERIALS AND METHODS:: Thirty CF, 17 primary ciliary dyskinesia (PCD), 13 chronic rhinosinusitis with polyps (CRSwp), and 30 controls were included. Angles were measured bilaterally on computed tomography (CT) scans: A, B, C on coronal sections, D and E on axial sections. Angle A was between the UP and the orbit inner wall, whereas the others were between UP and midline. RESULTS:: There was no significant difference between controls, PCD, and CRSwp. However, CF had 3 statistically different angles with controls, 5 with CRSwp, and 4 with PCD. Angle A average value was 126° (±16°) in patients with CF, 138° (±19°) in controls ( P = .007), 145° (±15°) in PCD ( P = .001), and 138° (±14°) in CRSwp ( P = .001). Angle E average value was 35° (±10°) in patients with CF, 20° (±6°) in controls ( P < .001), 21° (±4°) in PCD ( P < .001), and 22° (±6°) in CRSwp ( P < .001). CONCLUSION:: Uncinate process's anatomy is only modified in CF: Angle between UP and inner wall of orbit is closed, and angles between UP and midline are opened. SIGNIFICANCE:: These measures quantify the medial bulging of lateral nasal wall and support nasofibroscopic observations.

Bacterial biofilm in adenoids of children with chronic otitis media. Part I: a case control study of prevalence of biofilms in adenoids, risk factors and middle ear biofilms.

BACKGROUND: Biofilms are communities of bacteria embedded in a self-produced glycocalyx matrix. Adenoids have been shown to harbor bacterial biofilms. Aim/objectives: To compare the prevalence of biofilms in adenoid of children with chronic otitis media (COM) (group1) versus a control group without any COM (group 2) having adenoids removed because of hypertrophy. MATERIAL AND METHODS: One hundred and three children were prospectively enrolled in this case-control study, group 1 (n = 52) and group 2 (n = 51). The main outcome measurement was the prevalence of biofilm in adenoidectomy specimens analyzed using confocal laser scanning microscopy. Children in group 1 who had middle ear (ME) effusion and requiring the insertion of a tympanostomy tube underwent biopsy of the ME mucosa and effusion sampling. RESULTS: Biofilms were found in adenoids' specimens of both groups and in the ME biopsy and effusion. The biofilm prevalence in adenoids was 63.5% (33/52) in group 1 and 47.1% (24/51) in group 2. Day nursery and previous antibiotics intake were significantly more frequent in group 1 than in group 2. CONCLUSIONS AND SIGNIFICANCE: This case-control study demonstrates that adenoid tissue in children with COM contains more mucosal biofilms than adenoid tissue removed for hypertrophy. Biofilm was seen in ME biopsies and effusion.

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.