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INTRODUCTION: This study aims to describe the occurrence of postoperative complications related to cholesteatoma surgery and to determine factors influencing the most common complication, i.e. postoperative surgical site infection (SSI) in cases with and without mastoid obliteration.MATERIALS AND METHODS: Retrospective analyses were performed on surgically treated cholesteatomas in our hospital between 2013 and 2019. Patient characteristics, peri- and postoperative management and complications were reviewed. The cases were divided into two groups based on whether mastoid obliteration was performed or not.RESULTS: A total of 336 cholesteatoma operations were performed, of which 248 cases received mastoid obliteration. In total 21 complications were observed, of which SSI was the most common (15/21). No difference in occurrence of any postoperative complication was seen between the obliteration and no-obliteration group (p = 0.798), especially not in the number of SSI (p = 0.520). Perioperative and/or postoperative prophylactic antibiotics were not associated to the development of an SSI in both groups. In the no-obliteration group a younger age (p = 0.015), as well as primary surgery (p = 0.022) increased the risk for SSI. In the obliteration group the use of bioactive glass (BAG) S53P4 was identified as independent predictor of SSI (p = 0.008, OR 5.940).DISCUSSION: SSI is the most common postoperative complication in cholesteatoma surgery. The causes of SSI are multifactorial, therefore further prospective research is needed to answer which factors can prevent the development of an SSI in cholesteatoma surgery.

Objective To evaluate and compare the STAM classification, STAMCO classification and the EAONO/JOS staging system as predictors for cholesteatoma recidivism and postoperative hearing, using a large patient cohort in our tertiary referral center. Method Two hundred thirty-one patients who underwent surgery for primary cholesteatoma between 2003 and December 2012 were included and retrospectively classified and staged according to the STAM classification, STAMCO classification, and EAONO/JOS staging system. Data on cholesteatoma recidivism rates and postoperative hearing were collected. The predictive value of the three instruments for recurrent and residual cholesteatoma was compared by using receiver operating characteristic curves. Results For predicting recurrent cholesteatoma, the STAMCO classification was significantly superior compared to the other two instruments. For predicting residual cholesteatoma, the STAMCO classification was superior to the EANO/JOS Staging system. The postoperative hearing shows a significant increase in ABG with increasing extension of cholesteatoma in the CWU group and a significant decrease in AC threshold level with increasing stage and a significant increase in AC with increasing ossicular chain status in the CWD group. Conclusion Based on our study, the STAMCO classification represents the best available predictor for recurrent cholesteatoma and holds most promise for predicting residual cholesteatoma. Extension of cholesteatoma seems to be linked to postoperative hearing and thus the classifications and staging systems may be able to predict postoperative hearing. More studies are needed to assess the validation of these classifications.

Purpose Prolonged endotracheal intubation may lead to laryngeal damage, with stridor being the most relevant clinical symptom. Our objective was to determine the incidence of post-extubation stridor and their clinical consequences in children within a tertiary referral center and to identify contributing factors. Methods 150 children, aged 0–16 years, intubated for more than 24 h were prospectively enrolled until discharge of the hospital. Potential relevant factors, thought to mediate the risk of laryngeal damage, were recorded and analyzed. Results The median duration of intubation was 4 days, ranging from 1 to 31 days. Stridor following extubation occurred in 28 patients (18.7%); 3 of them required reintubation due to respiratory distress and in 1 child stridor persisted for which a surgical intervention was necessary. In multivariate analyses, we found the following independent predictors of stridor: intubation on the scene, the use of cuffed tubes and lower age. Conclusion Despite a high incidence for post-extubation stridor, only few children need reintubation or surgical intervention as a result of post-extubation lesions. Intubation on the scene, the use of cuffed tubes and young age are associated with a significant increased risk of post-extubation stridor. Awareness of these factors gives the possibility to anticipate on the situation and to minimize laryngeal injury and its possible future consequences.

The aim of this systematic review was to review the literature on hearing impairment and ear anomalies in patients with craniofacial microsomia and to determine their prevalence. Sixty-two records including 5122 patients were included. Ear anomalies were present in 52–100% of patients. The most reported external ear malformations were microtia, pre-auricular tags, and atresia of the external auditory canal. Ossicular anomalies were the most reported middle ear malformations, whereas the most reported inner ear malformations included oval window anomalies, cochlear anomalies, and anomalies of the semicircular canals. Hearing loss in general was reported in 29–100% of patients, which comprised conductive hearing loss, mixed hearing loss, and sensorineural hearing loss. Between 21% and 51% of patients used hearing aids, and 58% underwent a surgical intervention to improve hearing. The relationship between different phenotypes of craniofacial microsomia and the type and severity of hearing loss is mostly unclear. In conclusion, the high prevalence of ear and hearing anomalies in patients with craniofacial microsomia underlines the importance of audiological screening in order to facilitate individual treatment.

Highlights • Brain (micro-)structural differences were found in pre-adolescents who stutter. • Persistency was associated with marginally smaller left frontal gray matter volume. • Recovery was associated with higher mean diffusivity in white matter tracts. • Distinct brain structures implicated in persistence and recovery of stuttering., Background Stuttering is a complex speech fluency disorder occurring in childhood. In young children, stuttering has been associated with speech-related auditory and motor areas of the brain. During transition into adolescence, the majority of children who stutter (75–80%) will experience remission of their symptoms. The current study evaluated brain (micro-)structural differences between pre-adolescents who persisted in stuttering, those who recovered, and fluently speaking controls. Methods This study was embedded in the Generation R Study, a population-based cohort in the Netherlands of children followed from pregnancy onwards. Neuroimaging was performed in 2211 children (mean age: 10 years, range 8–12), of whom 20 persisted in and 77 recovered from stuttering. Brain structure (e.g., gray matter) and microstructure (e.g., diffusion tensor imaging) differences between groups were tested using multiple linear regression. Results Pre-adolescents who persisted in stuttering had marginally lower left superior frontal gray matter volume compared to those with no history of stuttering (β −1344, 95%CI −2407;-280), and those who recovered (β −1825, 95%CI −2999;-650). Pre-adolescents who recovered, compared to those with no history of stuttering, had higher mean diffusivity in the forceps major (β 0.002, 95%CI 0.001;0.004), bilateral superior longitudinal fasciculi (β 0.001, 95%CI 0.000;0.001), left corticospinal tract (β 0.003, 95%CI 0.002;0.004), and right inferior longitudinal fasciculus (β 0.001, 95%CI 0.000;0.001). Conclusion Findings suggest that relatively small difference in prefrontal gray matter volume is associated with persistent stuttering, and alterations in white matter tracts are apparent in individuals who recovered. The findings further strengthen the potential relevance of brain (micro-)structure in persistence and recovery from stuttering in pre-adolescents.

A retrospective cohort study was set up to analyse the prevalence and treatment of obstructive sleep apnoea (OSA) in relation to the severity of the deformity in patients with craniofacial microsomia (CFM). This study included a population of 755 patients with CFM from three craniofacial centres. Medical charts were reviewed for severity of the deformity, types of breathing difficulty, age at which breathing difficulty first presented, treatment for OSA, and treatment outcome. In total, 133 patients (17.6%) were diagnosed with OSA. Patients with Pruzansky IIB/III classification or bilateral craniofacial microsomia were significantly more often diagnosed with OSA than unilaterally affected patients of Pruzansky I/IIA classification. The initial treatment of OSA consisted of adenotonsillectomy, tracheotomy, or non-invasive positive pressure ventilation. Thirty-seven patients received more than one treatment (range 1-3). In this study, the prevalence of OSA in patients with CFM was higher than the prevalence in the healthy population described in the literature. Although several treatment modalities are available for the treatment of OSA in patients with CFM, treatment should be individualized and based on clinical symptoms, the severity of the deformity, and comorbidities.

DOOFNL Consortium., In a Dutch consanguineous family with recessively inherited nonsyndromic hearing impairment (HI), homozygosity mapping combined with whole-exome sequencing revealed a MPZL2 homozygous truncating variant, c.72del (p.Ile24Metfs∗22). By screening a cohort of phenotype-matched subjects and a cohort of HI subjects in whom WES had been performed previously, we identified two additional families with biallelic truncating variants of MPZL2. Affected individuals demonstrated symmetric, progressive, mild to moderate sensorineural HI. Onset of HI was in the first decade, and high-frequency hearing was more severely affected. There was no vestibular involvement. MPZL2 encodes myelin protein zero-like 2, an adhesion molecule that mediates epithelial cell-cell interactions in several (developing) tissues. Involvement of MPZL2 in hearing was confirmed by audiometric evaluation of Mpzl2-mutant mice. These displayed early-onset progressive sensorineural HI that was more pronounced in the high frequencies. Histological analysis of adult mutant mice demonstrated an altered organization of outer hair cells and supporting cells and degeneration of the organ of Corti. In addition, we observed mild degeneration of spiral ganglion neurons, and this degeneration was most pronounced at the cochlear base. Although MPZL2 is known to function in cell adhesion in several tissues, no phenotypes other than HI were found to be associated with MPZL2 defects. This indicates that MPZL2 has a unique function in the inner ear. The present study suggests that deleterious variants of Mplz2/MPZL2 affect adhesion of the inner-ear epithelium and result in loss of structural integrity of the organ of Corti and progressive degeneration of hair cells, supporting cells, and spiral ganglion neurons., This work was supported by a grant from the Heinsius Houbolt Foundation (to H.K., R.J.E.P., and H.P.M.K.) and partially by grants from the Spanish Ministry of Economy and Competitiveness and the European Regional Development Fund (Fonds Europeen de Developpement Economique et Regional, [FEDER]: SAF2014-53979-R) and from FEDER, CIBERER, and Instituto de Salud Carlos III (ISCIII) (to I.V.N.), and a grant from ISCIII to I.d.C. (PI14/01162; Plan Estatal de IþDþI 2013-2016, with co-funding from the European Regional Development Fund). S.M., A.M.C., and E.G.R. hold CIBERER ISCIII researcher contracts.

Children with craniofacial microsomia (CFM) are at risk of obstructive sleep apnoea (OSA). This systematic review provides an overview of the literature on the prevalence of OSA in children with CFM. A search was performed in PubMed, Embase, Cochrane Library, and Web of Science for articles on CFM and OSA. The following data were extracted from the articles: number of patients, patient characteristics, presence of OSA, polysomnography outcomes, and the treatments and outcomes of OSA. We included 16 articles on CFM and OSA, four of which reported the prevalence of OSA (range 7-67%). Surgical treatment was more often described in these patients than conservative treatment. According to the literature, OSA is related to CFM. However, as there have been no prospective studies and few studies have presented objective measurements, no definitive conclusions can be drawn. Prospective studies are needed to determine the prevalence of OSA in patients with CFM.

Introduction To describe the characteristics and surgical outcome of 98 infants and children treated for an acquired laryngeal stenosis after intubation for respiratory support. Material and methods We retrospectively reviewed our data from the last 18 years (1994–2013) concerning infants and children with an acquired laryngotracheal stenosis who were treated in our hospital with a laryngotracheal reconstruction or a cricotracheal resection. Outcome was defined by decannulation ratio. Results Of the 98 infants and children who were studied, 54% were preterm, 18% neonates, 13% infants and 14% children. Ninety-one SS-LTR’s, two DS-LTR’s and five CTR’s were performed as primary surgery; three revision operations were performed (DS-LTR). Seventy-seven children had a tracheostomy prior to surgery; decannulation ratio was 93% after primary surgery and 95% after inclusion of revision surgery. For SS-LTR, the decannulation ratio was 93%, including grade III stenosis with comorbidities. Male sex and glottic involvement of the stenosis are correlated to failure of decannulation. Intubation in the term neonatal period is correlated to complicated post-operative course after SS-LTR. Conclusions Excellent results of surgery for acquired laryngotracheal stenosis can be obtained with a high decannulation rate. Even for higher grades of stenosis with comorbidities and glottic involvement, an SS-LTR is an effective surgical treatment for acquired laryngeal stenosis.

BACKGROUND: The Reflux Finding Score for Infants (RFS-I) was developed to objectively assess signs of laryngopharyngeal reflux (LPR). Based on review of flexible laryngoscopic videos, only moderate inter- and highly variable intraobserver reliability was found. We hypothesized that examination of the infant larynx with rigid laryngoscopy would provide better agreement.AIM: to assess the validity of the RFS-I in the detection of LPR-related findings using flexible versus rigid laryngoscopy.METHODS: Thirty consecutive infants underwent flexible and rigid laryngoscopy. Based on the recorded videos, RFS-I was scored by 4 otorhinolaryngologists, 2 otorhinolaryngology fellows, and 2 inexperienced observers. Videos were presented in a randomized order, blinded for clinical profile and findings during initial examination. Observers were provided an instruction sheet prior to evaluation. For categorical data, agreement was calculated using Cohen's kappa (2 observers) and Fleiss' kappa (>2 observers). For ordinal data the intraclass correlation coefficient (ICC) was used.RESULTS: Of 30 included patients (17 M; median age 7.5 (0-19.8) months), main reasons for referral were: stridor (n = 15,50%), ALTE/apneas (n = 7,23.3%), follow-up of laryngeal abnormalities (n = 6,20%), aspiration (n = 5,16.7%), and other indications (n = 7,23.3%). Overall interobserver agreement of the RFS-I was moderate for both flexible (ICC = 0.60, [95%CI 0.44-0.76]) and rigid (ICC = 0.42, [95%CI 0.26-0.62] laryngoscopy. There were no significant differences in agreement on overall RFS-I scores and individual RFS-I items for flexible versus rigid laryngoscopy. We did observe higher overall agreement amongst the 2 inexperienced observers for rigid (ICC = 0.40, [95%CI 0.03-0.67]), compared to flexible (ICC = 0.11, [95%CI 0.17-0.41]) laryngoscopy, albeit not significant. Comparing RFS-I results for flexible versus rigid laryngoscopy per observer, agreement ranged from no to substantial agreement (k = -0.16-0.63, mean k = 0.22) and the observed agreement (not adjusted for chance) was 0.08-0.35%.CONCLUSIONS: Interobserver agreement of the RFS-I was only moderate and did not differ between flexible and rigid laryngoscopy. This indicates that the RFS-I should not be used with flexible, nor rigid laryngoscopy to detect signs of LPR.

Patients with craniofacial microsomia are at higher risk of developing obstructive sleep apnoea (OSA), as described in the previous article entitled "Obstructive sleep apnoea in craniofacial microsomia: a systematic review". These patients are also more likely to develop feeding difficulties. The present systematic review provides an overview of the literature on the prevalence, treatment, and follow-up of feeding difficulties in children with craniofacial microsomia (CFM). A search was performed in PubMed, Embase, Cochrane Library, and Web of Science for articles on CFM and feeding difficulties. The following data were extracted from the articles: number of patients, patient characteristics, presence of feeding difficulties, and the treatment and outcomes of feeding difficulties. Eight articles on CFM and feeding difficulties were included, two of which reported the prevalence of feeding difficulties (range 42-83%). Treatment mostly consisted of tube feeding. No information regarding follow-up was found in these articles. According to the literature, feeding difficulties are related to CFM. However, as there have been no prospective studies and few studies have presented objective measurements, no definitive conclusions can be drawn. Prospective studies are needed to determine the prevalence of feeding difficulties in patients with CFM.