EAONO/JOS, STAMCO, ChOLE & Potsic Staging of 271 Pediatric Cholesteatoma: Evidence-Based Mod-Pot Staging System.

OBJECTIVE: The objective was to assess Potsic, EAONO/JOS, STAMCO, and ChOLE cholesteatoma staging systems in a large homogenous pediatric cohort with long-term follow-up and propose an evidence-based improved version. STUDY DESIGN: Cohort study. SETTING: Retrospective study in a tertiary referral center. METHODS: Children with congenital or acquired cholesteatoma who underwent surgery between 2008 and 2015 and had a minimum of five years follow-up were included. ROC areas under curve (AUCs) were performed for the residual disease proportion, total number of surgeries, hearing, and C-index for recurrence proportion at the last follow-up. RESULTS: Data from 271 ears with cholesteatoma were collected. Mean age at diagnosis was 7.9 years and 77 (28%) were congenital. Almost all patients (99%) had a canal wall up approach. The mean follow-up was 97.8 months. The Potsic classification, initially designed for congenital cholesteatoma, had the highest AUCs for residual disease rate and for number of surgeries outcomes (respectively 0.73 [0.67-0.78] and 0.71 [0.66-0.77]). For recurrence rate, all the classifications' C-index were low (<0.7). For postoperative hearing, STAMCO ossicular subscore had the highest AUC (0.73 [0.67-0.79]). The Mod-Pot staging system (modified Potsic), including stapes superstructure status and anterior epitympanum or supratubal involvement, improved the Potsic performance especially for postoperative hearing with AUC 0.73 [0.67-0.80], p < 0.001. CONCLUSION: The Potsic, ChOLE, EAONO/JOS, and STAMCO cholesteatoma classifications may be used in children, but in this specific population, their contribution remains limited to predict outcome. The Mod-Pot classification improved the performance of pediatric cholesteatoma classification while remaining simple and intuitive. LEVEL OF EVIDENCE: 3 Laryngoscope, 2024.

Full circumferential human tracheal replacement: a systematic review.

Full Circumferential Tracheal Replacement (FCTR) is a surgical challenge, indicated in rare cases of extensive tracheal resection, with no consensus on surgical technique or materials. A systematic review according to PRISMA guidelines was carried out from 2000 to 2022 to identify cases of FCTR, to compare surgical indications, the nature of the tracheal substitutes and their immunological characteristics, surgical replacement techniques and vascularization. Thirty-seven patients, including five children, underwent FCTR surgery using 4 different techniques: thyrotracheal complex allograft (n = 2), aorta (n = 12), autologous surgical reconstruction (n = 19), tissue-engineered decellularized trachea (n = 4). The mean follow-up was 4 years. Of the 15 deceased patients, 10 died of the progression of the initial pathology. For the majority of the teams, particular care was given to the vascularization of the substitute, in order to guarantee long-term biointegration. This included either direct vascularization via vascular anastomosis, or an indirect technique involving envelopment of the avascular substitute in a richly vascularized tissue. Stent placement was standard, except for autologous surgical reconstructions where tracheal caliber was stable. Internal stents were frequently complicated by granulation and stenosis. Although epithelial coverage is essential to limit endoluminal proliferation and act as a barrier, fully functional ciliated airway epithelium did not seem to be necessary. In order to facilitate future comparisons, a standardized clinical trial, respecting regulatory constraints, including routine follow-up with tracheal biomechanics assessment and scheduled biopsies could be proposed. It would help collecting information such as dynamics and mechanisms of tracheal bio-integration and regeneration.

Management of sleep-disordered breathing in patients with syndromic hemifacial macrosomia.

PURPOSE: Patients with syndromic hemifacial microsomia (SHFM) are at risk of obstructive sleep apnea (OSA). The aim of the study was to describe the prevalence of OSA and its management, especially in patients with Goldenhar syndrome (GS). METHODS: The respiratory polygraphies and clinical management of 15 patients, aged 2 to 23 years, evaluated at a national reference center, were analyzed. RESULTS: Four (27%) patients had no OSA, 4 (27%) had mild OSA, and 7 (46%), of whom 5 were ≤ 2 years old, had severe OSA. None of the patients had central apneas. Only one patient had alveolar hypoventilation, and another one had nocturnal hypoxemia. Two patients had severe OSA despite prior adenoidectomy or mandibular distraction osteogenesis. Median duration of follow-up was 3.5 years (range 0.5-9 years). None of the patients without OSA or with mild OSA at baseline respiratory polygraphy developed OSA during the follow up. Among the 7 patients with severe OSA, 3 required continuous positive airway pressure or noninvasive ventilation, and one patient required a tracheostomy. CONCLUSION: In conclusion, patients with SHFM are at high risk of severe OSA at any age, underlining the importance of systematic sleep studies to diagnose and evaluate the severity of OSA. Individualized treatment should be privileged, based on a careful examination of the entire upper airway, taking in account potential associated risk factors. All patients with SHFM should be managed by a pediatric expert multidisciplinary medical/surgical team until the end of post pubertal growth.

Pediatric Cholesteatoma Follow-Up: Residual and Recurrence in 239 Cases with Over 5-Year Hindsight.

INTRODUCTION: Pediatric cholesteatoma is an aggressive disease which requires long-term evaluation to assess management strategies. The objective was to determine optimal follow-up duration in pediatric cholesteatoma to detect residual and recurrent diseases. METHODS: This cohort study was set in a tertiary referral center. All consecutive patients with a minimum 5-year follow-up were included. Medical history, initial extension, surgical procedures, and follow-up were collected. The main outcome measure were Kaplan-Meier survival curves of residual and recurrence cumulative incidence. RESULTS: Totally 239 ears with the first tympanoplasty between 2008 and 2014 were studied including 25% congenital. At first surgery (S1), mean age was 8.4 years and mean follow-up time 7.9 years. Mastoidectomy was performed in 69% and stapes was absent in 38% of cases. Notably, 83% (199 ears) had a second procedure (S2) of which 186 were planned. After S1, maximum cumulated incidence of residual was 45% [95%CI, 38%; 52%] at 74 months, with probability of residual of 39% at 3 years. After S2 (n = 199), maximum cumulated residual incidence was 21% [95%CI, 12%; 32%] at 62 months and 16% at 3 years. Concerning recurrence, maximum cumulated incidence after S1 (n = 239) was reached at 98 months with 21% [95%CI, 12%; 32%], 13% at 3 years and 16% at 5 years. Congenital disease had significantly less residuals after S1 (p = 0.02), but similar recurrence rate (p = 0.66) compared with acquired. CONCLUSIONS AND RELEVANCE: We recommend MRI follow-up of at least 5 years after the last surgery for residual disease and clinical follow-up of at least 10 years to detect recurrence. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:4789-4798, 2024.

Educational endoscopic videos improve teaching of middle ear anatomy.

PURPOSE: Videos and images are becoming an educational tool in every domain. The objective was to assess the contribution of short educational endoscopic videos in learning the complex anatomy of the tympanic cavity. METHODS: We conducted a prospective study amongst all the otolaryngology residents in 2021 in the greater Paris area (n = 74), from the first year to fifth year. We divided the residents into two groups who had the same 30-min anatomy class based on anatomical drawings. The second group had a 4-min endoscopic educational video at the end, whereas the first group did not. A video test of 10 surgical situations with each time 5 anatomic structures to identify was created. All the residents took the test just after the class, and 1 month later to assess long-term memory. The MERSQI score (design to assess the quality of educational studies) applied to our study was calculated. RESULTS: The two groups were comparable in terms of training. The "video" group had significantly better results in the first test, mean score 24.40/50 (± 11.7) versus 16.74/50 (± 11.1) (p = 0.005) and also at 1 month, mean score 23.25/50 (± 12.3) versus 18.01/50 (± 11.3) (p = 0.035). The score in each group, and the difference between both groups, increased with resident seniority. The MERSQI score of our study was 14.5/18. CONCLUSION: This study highlights the educational value of videos to help memorise complex 3D anatomy such as in the tympanic cavity.

Performance of Non-EPI DW MRI for Pediatric Cholesteatoma Follow-Up.

OBJECTIVE: To evaluate the accuracy, sensitivity, and specificity of nonecho planar (non-EPI) diffusion-weighted (DW) magnetic resonance imaging (MRI) to detect residual cholesteatoma in children. STUDY DESIGN: Retrospective study. SETTING: Tertiary comprehensive hospital. METHODS: Children operated on for a first-stage cholesteatoma procedure from 2010 to 2019 were included. MRIs were performed with non-EPI DW sequences. Initial reports were collected, indicating the presence or absence of hyperintensity suggestive of cholesteatoma. Three hundred twenty-three MRIs were correlated with the subsequent surgery (66%) or year-later MRI (21%), or were considered accurate if performed 5 years or more after the last surgery (13%). The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of each imaging procedure for the detection of cholesteatoma were calculated. RESULTS: Two hundred twenty-four children with mean age of 9 ± 4 years old presented with cholesteatoma. MRIs were performed 27 ± 24 months after surgery. Residual cholesteatoma was diagnosed in 35%. The sensitivity, specificity, PPV, and NPV of MRI were 62%, 86%, 74%, and 78%, respectively. Accuracy, sensitivity, and specificity increased significantly over time (multivariate analysis). The mean delay after last surgery was of 30 ± 2.0 months for accurate MRI (true positive or negative) versus 17 ± 2.0 months for nonaccurate (false positive or negative) MRIs (p < .001). CONCLUSION: However, long the delay after the last surgery, the sensitivity of non-EPI diffusion sequence MRI in children has limitations for the detection of residual cholesteatoma. Surveillance for residual cholesteatoma should incorporate findings at primary surgery, surgeon experience, a low threshold for second-look procedures, and routine imaging.

What defines a great surgeon? A survey study confronting perspectives.

Background: The definition of a great surgeon is usually reported by surgeons themselves. The objective of the study was to define a multifaceted definition of a great surgeon, by confronting patients', healthcare workers', and surgeons' perspectives. Study design: An online open-ended questionnaire was created to identify three qualities and three shortcomings defining a great surgeon. Age, gender, and profession of respondents were collected. Responses with a similar meaning were combined into word groups and labeled within four themes: human qualities, technical surgical skills (TSS), non-technical skills (NTS), and knowledge. Multivariate analyses were conducted between themes and respondent characteristics. Results: Four thousand seven hundred and sixty qualities and 4,374 shortcomings were obtained from 1,620 respondents including 385 surgeons, 291 patients, 565 operating theater (OT) health professionals, and 379 non-OT health professionals. The main three qualities were dexterity (54% of respondents), meticulousness (18%), and empathy (18%). There was no significant difference between professional categories for TSS. Compared with surgeons, non-OT health professionals and patients put more emphasis on human qualities (29 vs. 39% and 42%, respectively, p < .001). OT health professionals referred more to NTS than surgeons (35 vs. 22%, p < 0.001). Knowledge was more important for surgeons (19%) than for all other professional categories (p < 0.001). Conclusions: This survey illustrates the multifaceted definition of a great surgeon. Even if dexterity is a major quality, human qualities are of paramount importance. Knowledge seems to be underestimated by non-surgeons, although it essential to understand the disease and preparing the patient and OT team for the procedure.

Early Magnetic Resonance Imaging to Diagnose Residual Cholesteatoma in Children and Benefit of Radiological Rereview.

OBJECTIVE: Non-echo-planar diffusion-weighted (DW) magnetic resonance imaging (non-EPI MRI) is the appropriate sequence to detect residual cholesteatoma. In the child, MRI may be clinically useful to determine the timing of the second-look procedure. The aim of this paper was to retrospectively evaluate the performance of early MRI (before the 18th postoperative month) in detecting residual cholesteatoma in children after review by experienced specialized neuroradiologists. STUDY DESIGN: Retrospective study. SETTING: One university center comparative cohort. METHODS: All patients who had a 2-staged procedure for cholesteatoma with an MRI before the second stage from 2010 to 2020 were included and analyzed. Three pediatric neuroradiologists reviewed all the images blinded to the surgical result. RESULTS: N = 141 cholesteatoma events (140 children) were included with a mean age at MRI of 10 (±4) years old. Non-EPI MRIs were performed 10.7 (±3.8) months after the first-stage surgery and 2.2 (±2.6) months before the second-stage procedure. Non-EPI MRI had a 0.57 sensitivity (SE) and 0.83 specificity (SP). MRI was reviewed in 112 cases. The diagnosis was corrected in 17 cases (15.1%) (3 true positives, 7 false negatives, and 7 false positives). SE = 0.63 (p = 0.1) and SP = 0.92 (p = 0.08) after rereading. CONCLUSION: Early MRI's SE is poor but SP is excellent after rereading. Evidence does not support the use of early non-EPI MRI to modify the surgical strategy or to postpone the second look. If performed, early non-EPI MRI should be read by specialized experienced radiologists with all 3 sequences (T1, T2, and non-EPI DW) and apparent diffusion coefficient calculation, especially in cases of otitis media with effusion.

Long-term outcomes of retrospective case series of middle ear implantation with Vibrant Soundbridge in children with congenital aural atresia.

PURPOSE: to assess audiological performance in quiet and noise, quality of life and side effects of Vibrant Soundbridge (VSB) in children with congenital aural atresia (CAA). METHODS: A retrospective study including consecutive patients with unilateral or bilateral CAA implanted with VSB from 2009 to 2020 in a tertiary referral centre. RESULTS: 18 patients with CAA and a present stapes were included (3 simultaneous bilateral VSB implants) and 21 ears: 17 VSB were attached to the short incus process, and four to the stapes. Age at implantation ranged from 4.7 to 15.8 years. Average follow-up was 6.5 years (± 3.7 years). In unilateral VSB, air conduction pure tone average (ACPTA) thresholds increased from 75.3 ± 15.2 to 32.6 ± 8.3 dB post-operatively (VSB activated) (n = 15; p < 0.01). The speech reception threshold (SRT) and the word recognition score (WRS) were significantly improved from 81.5 ± 10.4 to 43.9 ± 7.6 dB and 0% to 84.8 ± 8.5% postoperatively (n = 15; p < 0.01). The signal to noise ratio (SNR) was significantly improved from 2.1 ± 2.9 dB VSB inactivated to 0.3 ± 2.7 dB VSB activated (n = 15; p < 0.01). There was no significant difference in performance according to floating mass transducer (FMT) placement. 5/15 children were non-users at last follow-up in unilateral VSB and 0/3 in bilateral. CONCLUSIONS: CAA ears with VSB activated had a significant improvement of ACPTA, WRS, SRT and SNR. A third of patients with unilateral CAA became non-users at last follow-up. The main challenge is to target the indications for the implantation of the VSB to avoid its discontinuation.

French adaptation and validation of the Chronic Otitis Media Questionnaire-12 (COMQ-12).

OBJECTIVE: Chronic otitis media (COM) can seriously impact quality of life (QOL). Disease-specific questionnaires are essential for an accurate assessment of this impact. There is no questionnaire available for French-speaking patients with COM. This study aims to adapt and validate the French version of the COMQ-12 questionnaire. DESIGN: This is a controlled, prospective study, conducted between May 2020 and December 2021. Translation was performed using a forward-backward technique, and statistical validation was performed with a COM patients and a controls adult cohorts. STUDY SAMPLE: 100 patients (mean age 48 ± 16), and 50 controls (mean age 41 ± 16; p = 0.02) completed the test. RESULTS: Internal consistency, assessed by Cronbach's alpha, was 0.870 for the cases group. The mean COMQ-12 total score was 23.92 ± 11.3 for cases versus 3.70 ± 6.15 for controls (p < 0.0001). Individual items all had high discriminatory performances. The three items concerning ear discharge (Q1, Q2, Q9) had weaker correlation with the other items and the global score, but did not have a negative impact on internal consistency. CONCLUSION: The French version of the COMQ-12 is a short and easy-to-use test, with robust statistical properties, for assessing QOL in patients with COM.

Robot-Assisted Transcanal Endoscopic Ear Surgery for Congenital Cholesteatoma.

Congenital cholesteatoma accounts for 25% of cholesteatoma cases in children. Transcanal Endoscopic Ear Surgery (TEES) is ideal for these patients because it offers a wide endoscopic view of the middle ear and a minimally invasive approach. The two main limitations are the loss of one operative hand and a narrow external auditory canal in younger children. Here, we present the case of a 3-year-old patient with a Potsic stage III congenital cholesteatoma adherent to the incus and branches of the stapes. A robotic-assisted TEES procedure was performed, during which a robotic arm with 6 degrees of freedom held a 0°, 2.9 mm wide endoscope, enabling the surgeon to work in a narrow environment with both hands. The procedure's duration was 2 h and 9 min, including 16 min for the installation and draping of the robotic arm. After a trans-canal approach, the cholesteatoma was dissected from the ossicles using both a needle (or sickle knife) and suction to stabilize the ossicles and limit the risk of hearing trauma. The cholesteatoma was debulked to reduce its size, allowing it to be pushed under the malleus anteriorly and then separated from other adherences before removal. A tragal cartilage graft was used to reinforce the tympanic membrane.

Mild MDPL in a patient with a novel de novo missense variant in the Cys-B region of POLD1.

DNA polymerase δ is one of the three main enzymes responsible for DNA replication. POLD1 heterozygous missense variants in the exonuclease domain result in a cancer predisposition phenotype. In contrast, heterozygous variants in POLD1 polymerase domain have more recently been shown to be the underlying basis of the distinct autosomal dominant multisystem lipodystrophy disorder, MDPL (mandibular hypoplasia, deafness, progeroid features, and lipodystrophy syndrome OMIM # 615381), most commonly a recurrent in-frame deletion of serine at position 604, accounting for 18 of the 21 reported cases of this condition. One patient with an unusually severe phenotype has been reported, caused by a de novo c. 3209 T > A, (p.(Ile1070Asn)) variant in the highly conserved CysB motif in the C-terminal of the POLD1 protein. This region has recently been shown to bind an iron-sulphur cluster of the 4Fe-4S type. This report concerns a novel de novo missense variant in the CysB region, c.3219 G > C, (p.(Ser1073Arg)) in a male child with a milder phenotype. Using in silico analysis in the context of the recently published structure of human Polymerase δ holoenzyme, we compared these and other variants which lie in close proximity but result in differing degrees of severity and varying features. We hypothesise that the c.3219 G > C, (p.(Ser1073Arg)) substitution likely causes reduced binding of the iron-sulphur cluster without significant disruption of protein structure, while the previously reported c.3209 T > A (p.(Ile1070Asn)) variant likely has a more profound impact on structure and folding in the region. Our analysis supports a central role for the CysB region in regulating POLD1 activity in health and disease.

Presentation of dizziness in individuals with chronic otitis media: data from the multinational collaborative COMQ-12 study.

PURPOSE: In chronic otitis media (COM), disease chronicity and severity of middle ear inflammation may influence the development of inner ear deficits, increasing the risk of vestibular impairment. This secondary analysis of the multinational collaborative Chronic Otitis Media Questionnaire-12 (COMQ-12) dataset sought to determine the prevalence of vestibular symptoms in patients with COM and identify associated disease-related characteristics. METHODS: Adult patients with a diagnosis of COM in outpatient settings at nine otology referral centers across eight countries were included. We investigated the presence of vestibular symptoms (dizziness and/or disequilibrium) using participant responses to item 6 of a native version of the COMQ-12. Audiometric data and otoscopic assessment were also recorded. RESULTS: This analysis included 477 participants suffering from COM, with 56.2% (n = 268) reporting at least mild inconvenience related to dizziness or disequilibrium. There was a significant association between air conduction thresholds in the worse hearing ear and presence of dizziness [adjusted odds ratio (AOR), 1.01; 95% CI 1.00-1.02; p = 0.0177]. Study participants in European countries (AOR 1.53; 95% CI 1.03-2.28; p = 0.0344) and Colombia (AOR 2.48; 95% CI 1.25-4.92; p = 0.0096) were more likely to report dizziness than participants in Asian countries. However, ear discharge and cholesteatoma showed no association with dizziness in the adjusted analyses. CONCLUSION: Vestibular symptoms contribute to burden of disease in patients with COM and associates with hearing disability in the worse hearing ear. Geographical variation in presentation of dizziness may reflect financial barriers to treatment or cultural differences in how patients reflect on their health state.

Impact of COVID-19 lockdown on a tertiary center pediatric otolaryngology emergency department.

To assess the quantitative and qualitative impact of the COVID-19 lockdown on pediatric otolaryngology emergency activity. A retrospective study was conducted in a pediatric otolaryngology tertiary care center. Emergency activity during the lockdown period from March 17 to May 11, 2020, was compared to the 2019 and 2018 averages for the same period. Study data included a number of emergency consultations and the number and type of surgical procedures: infection management, endoscopic airway procedure, and post-tonsillectomy hemorrhage. Only 350 children were referred to the pediatric otolaryngology emergencies in our center during the lockdown, compared to 761 on the same period the year before (- 54%); 62 emergency surgeries were performed, compared to 93 (- 33%). The ratio between emergency surgeries and consultations was 18% in 2020, versus 12% previously (p = 0.014). The number of surgical procedures for infectious diseases decreased (- 68%), at 16% of surgical emergencies in 2020 compared to 33% previously (p = 0.017). In 2020, 52 emergency endoscopies were performed, versus 59 previously (- 12%), 27% being performed for suspected tracheobronchial or esophageal foreign bodies, compared to 66% in previous years (p < 0.0001). No post-tonsillectomy hemorrhages were managed in 2020.Conclusion: The COVID-19 lockdown changed pediatric ENT emergency activity quantitatively and also qualitatively. What is Known: • SARS-CoV-2 pandemic impacted pediatric ENT emergency activity quantitatively and qualitatively. What is New: • here was a 54% decrease in pediatric ENT emergency consultation and 33% decrease in emergency ENT surgeries. • Rates of surgery for infection of whatever type decreased by 68%.

Noninvasive Respiratory Support as an Alternative to Tracheostomy in Severe Laryngomalacia.

OBJECTIVES/HYPOTHESIS: To analyze the role of noninvasive respiratory support (NRS) as an alternative to tracheostomy in the management of severe laryngomalacia. STUDY DESIGN: We conducted a monocentric retrospective study in a tertiary pediatric care center. METHODS: All children under the age of 3 years with severe laryngomalacia, treated between January 2014 and December 2019, were included. Patient demographics, medical history, nutrition, surgery, NRS, and outcome were reviewed. Predictors for NRS were analyzed. RESULTS: One hundred and eighty-eight patients were included. Mean age was 4 ± 5 months and mean weight was 4,925 ± 1,933 g. An endoscopic bilateral supraglottoplasty was performed in 183 (97%) patients and successful in 159 (87%). NRS was initiated in 29 (15%) patients at a mean age of 3 ± 2 months (1-11 months): 15 (52%) patients were treated with NRS after surgical failure, 9 (31%) were treated with NRS initiated prior to surgery because of abnormal overnight gas exchange, and 5 (17%) were treated exclusively with NRS due to comorbidities contraindicating an endoscopic procedure. NRS was successfully performed in all patients with a mean duration of 6 ± 11 months. No patient required a tracheostomy. Univariate analysis identified the following predictors of NRS: neonatal respiratory distress (P = .003), neurological comorbidity (P < .001), associated laryngeal abnormality (P < .001), cardiac surgery (P = .039), surgical endoscopic revision (P = .007), and nutritional support (P < .001). CONCLUSION: NRS is a safe procedure, which may avoid a tracheostomy in severe laryngomalacia, in particular, in case of endoscopic surgery failure, respiratory failure before surgery, and/or severe co-morbidity. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:1861-1868, 2022.

Use of IOOG Classification to Categorize Middle Ear Surgery in Children: Evaluation After 5 Months of Use in 119 Consecutive Pediatric Middle Ear Surgeries.

INTRODUCTION: SAMEO-ATO classification is an international consensual tool published in 2018. In order to evaluate the ease of use and reliability of this classification in pediatric otologic surgery, a retrospective study was conducted in our tertiary referral center. METHOD: We began to use IOOG classification in September 2018, after a 15-day testing period. In this monocentric retrospective study, we reviewed the classification and the operative charts of all the pediatric middle ear surgeries after the first 5 months of use. Each classification was carefully re-checked by a junior and senior surgeon. In parallel, all the surgeons involved received a questionnaire to evaluate the ease of use, with a score from 1 (very difficult) to 5 (very easy). RESULTS: From September 2018 to February 2019, the tool was used for 119 pediatric surgeries, involving 13 surgeons. The indications for surgery were tympanic membrane perforations (28/119, 23.5%), retraction pockets (36/119, 30.3%), cholesteatoma first procedures (13/119, 10.9%), cholesteatoma revision procedures (41/119, 34.5%) and temporal bone fracture (1/119, 0.8%). All surgical procedures performed could be classified with the ATO-SAMEO classification. We found misclassification in 29/119 cases (24%), 79% of which concerning ATO items. Of the 8 SAMEO-ATO categories in 119 surgeries, the error rate was 4.5% (49/952). Surgeons reported a mean score of use of 4 (quite easy). CONCLUSION: The easy-to-use SAMEO-ATO classification is well suited for pediatric otology and to the categorization of surgical procedures. However, in a multi-user context, misclassifications were observed in up to a quarter of cases during the first months of use. Carefully anticipated explanations and guidelines given to surgeons should ensure an optimal quality of rating.

Congenital abnormalities associated with microtia: A 10-YEARS retrospective study.

OBJECTIVE: Microtia is a congenital auricular malformation, often part of a syndromic form (35%-55% of cases). The accurate prevalence of associated malformations remains to be determined with regard to the heterogeneous results of the previous studies. This study aims to describe in a large population cohort the abnormalities associated with microtia and to determine the most suitable assessment for these children. METHODS: This is a retrospective and observational cohort study collecting data from the medical records of children affected by microtia, diagnosed or followed-up between 2007 and 2017. Data were collected via a computer database. Clinical data, as well as imaging or genetic results, were noted. RESULTS: Six hundred ninety four children were included, 587 (84.6%) with unilateral and 107 (15.4%) with bilateral microtia. Inner ear malformations were observed in 14.1% of the ears. The main associated anomalies were hemifacial microsomia (29%), velopharyngeal insufficiency (9%), ophthalmologic (6.2%), vertebral (5.9%), cardiac (5.5%) and kidney (3%) abnormalities. Main identified entities were Goldenhar, Treacher-Collins and Guion-Almeida syndromes. CONCLUSION: A comprehensive clinical assessment must be completed when microtia is diagnosed. Besides screening well-known oculo-auriculo-vertebral spectrum malformations, velopharyngeal insufficiency should be systematically sought. Specialized care must be provided to the very frequently associated hemifacial macrosomia. Mild forms of this last malformation may correspond to Guion-Almeida syndrome, especially in cases of learning disability.