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Δευτέρα 30 Νοεμβρίου 2020

The Swedish hearing in noise test for children, HINT-C.

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The Swedish hearing in noise test for children, HINT-C.

Int J Pediatr Otorhinolaryngol. 2020 Nov 19;:110509

Authors: Hjertman H, Hällgren M, Mäki-Torkko E, Stenfelt S

Abstract
OBJECTIVE: The aim of this study was to develop and evaluate a Swedish version of the Hearing In Noise Test for Children (HINT-C).
DESIGN: In the first part, the Swedish HINT lists for adults was evaluated by children at three signal to noise ratios (SNRs), -4, -1 and +2 dB. Lists including sentences not reaching 50% recognition at +2 dB SNR were excluded and the rest constituted the HINT-C. In the second part, HINT-C was evaluated in children and adults using an adaptive procedure to determine the SNR for 50% correctly repeated sentences. Study Sample In the first part, 112 children aged 6-11 years participated while another 28 children and 9 adults participated in the second part.
RESULTS: Eight out of 24 tested adult HINT lists did not reach the inclusion criteria. The remaining 16 lists formed the Swedish HINT-C which was evaluated in children 6-11 years old. A regression analysis showed that the predicted SNR threshold (dB) was 0.495-0.365*age (years + months/12) and the children reached the mean adult score at an age of 10.5 years.
CONCLUSIONS: A Swedish version of HINT-C was developed and evaluated in children six years and older.

PMID: 33234330 [PubMed - as supplied by publisher]

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Novel variants in EDNRB gene in waardenburg syndrome type II and SOX10 gene in PCWH syndrome.

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Novel variants in EDNRB gene in waardenburg syndrome type II and SOX10 gene in PCWH syndrome.

Int J Pediatr Otorhinolaryngol. 2020 Nov 13;:110499

Authors: Varga L, Danis D, Drsata J, Masindova I, Skopkova M, Slobodova Z, Chrobok V, Profant M, Gasperikova D

Abstract
Waardenburg syndrome (WS) is a clinically and genetically heterogeneous group of inherited disorders manifesting with sensorineural hearing loss and pigmentary anomalies. Here we present two Caucasian families with novel variants in EDNRB and SOX10 representing both sides of phenotype spectrum in WS. The c.521G>A variant in EDNRB identified in Family 1 leads to disruption of the cysteine disulfide bridge between extracellular segments of endothelin receptor type B and causes relatively mild phenotype of WS type II with low penetrance. The novel nonsense variant c.900C>A in SOX10 detected in Family 2 leads to PCWH syndrome and was found to be lethal.

PMID: 33234331 [PubMed - as supplied by publisher]

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Acute isolated sphenoid sinusitis in children: A case series and systematic review of the literature.

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Acute isolated sphenoid sinusitis in children: A case series and systematic review of the literature.

Int J Pediatr Otorhinolaryngol. 2020 Nov 11;:110492

Authors: Clement WA, Sooby P, Doherty C, Qayyum N, Irwin G

Abstract
OBJECTIVE: This study aims to present a case series and systematic review of acute isolated sphenoid sinusitis (AISS) in children in order to better characterize clinical presentation, diagnosis, treatment, and outcomes of this condition.
DATA SOURCES: Ovid MEDLINE, Pubmed, Embase, Cochrane Library, and Google Scholar.
STUDY SELECTION: Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Full-text, peer-reviewed journal publications from 1994 to 2020 in English; focus on acute sphenoid sinusitis; pediatric patients (<18 years of age); series with two or more children. Studies were assessed for data including demographics, presenting symptoms and signs, radiological investigations, treatment, outcomes and complications.
RESULTS: Ten studies identifying 71 patients were included. Average age at presentation was 12.0 years (range 5-17 years). M:F ratio 1:1. The most common presenting symptoms were headache (98.6%), fever (50.7%), nasal symptoms (22.5%) ocular symptoms (19.7%) and decreased level of consciousness (12.7%). Twenty patients (28.1%) had neurological signs. Twenty-three patients (32.4%) presented with headache in isolation. Unsuspected diagnosis at presentation was noted in 54.0%. Average time to initial presentation was 14.0 days (median = 5.5 days, range 1-90 days). The majority of children were treated with antibiotics (98.6%) with 31.0%, 2.8% and 2.8% also undergoing sinus surgery, revision sinus surgery and neurosurgery, respectively. Intracranial complications occurred in 16.9% of patients. Significant long term sequelae occurred in 2 children (2.8%) and one death (1.4%) was also reported.
LIMITATIONS: All studies were retrospective case note reviews.
CONCLUSIONS: Acute sphenoid sinusitis is a rare and difficult condition to diagnose in children. The majority of patients make a full recovery with appropriate treatment. If treatment is delayed however consequences can be life-threatening.

PMID: 33234332 [PubMed - as supplied by publisher]

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A homozygous MPZL2 deletion is associated with non syndromic hearing loss in a moroccan family.

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A homozygous MPZL2 deletion is associated with non syndromic hearing loss in a moroccan family.

Int J Pediatr Otorhinolaryngol. 2020 Nov 02;:110481

Authors: Amalou G, Bonnet C, Riahi Z, Bouzidi A, Elrharchi S, Bousfiha A, Charif M, Kandil M, Lenaers G, Petit C, Barakat A

Abstract
Adhesion glycoproteins are implicated in the pathophysiology of hearing loss, the most frequent inherited sensory disorder, affecting 1 in 1000 new-borns. Exome sequencing of a consanguineous Moroccan patient with mild hearing loss identified for the first time in a North African family a single homozygous mutation c.72delA in MPZL2 gene, encoding the Myelin Protein Zero-Like 2, reported as causing deafness in two other populations. Variable tandem repeat genotyping of this family revealed that the c.72delA MPZL2 allele shared a common haplotype with Turkish and Dutch families. These results confirm the pathogenicity of this MPZL2 mutation in recessive mild to moderate non-syndromic deafness.

PMID: 33234333 [PubMed - as supplied by publisher]

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Acute mastoiditis complicated by cerebral venous sinus thrombosis in children.

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Acute mastoiditis complicated by cerebral venous sinus thrombosis in children.

Int J Pediatr Otorhinolaryngol. 2020 Nov 17;:110508

Authors: Vergadi E, Velegrakis S, Raissaki M, Bitsori M, Karatzanis A, Galanakis E

Abstract
AIM: Diagnosis and management of complicated mastoiditis in childhood are still controversial. We investigated the clinical manifestations, evaluation and management of children with mastoiditis complicated with cerebral venous sinus thrombosis.
METHODS: Retrospective cohort study that included all children admitted for acute mastoiditis over the last 5 years. Children were divided in two groups based on the presence or not of venous sinus thrombosis. Clinical, laboratory, imaging and management data were retrieved and compared.
RESULTS: Overall, 20 children with acute mastoiditis were included, of whom 5 had magnetic resonance imaging-confirmed cerebral venous sinus thrombosis and elevated intracranial pressure (ICP). In all complicated cases, neurological signs rather than mastoiditis signs, prevailed. The more prominent neurologic signs observed were lethargy (60%), nuchal rigidity (60%), abducens nerve palsy (60%) and ataxic gait (20%). Treatment consisted of intravenous antibiotics combined with anticoagulation. Surgery was performed in four children (4/5). Complicated cases had prolonged symptoms prior to admission (p 0.002), presented with neurologic signs and symptoms (p < 0.001), underwent more often lumbar puncture (p < 0.001) and brain imaging (p < 0.001), and were treated with prolonged courses of antibiotics and surgery (<0.001), compared to children with uncomplicated mastoiditis.
CONCLUSION: Neurological signs and symptoms and elevated ICP dominate in children with mastoiditis complicated with thrombosis. Brain imaging is essential for early diagnosis of cerebral venous sinus complications and appropriate management.

PMID: 33234334 [PubMed - as supplied by publisher]

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Bipolar loop device versus bipolar diathermy for tonsillectomy: A pilot randomized controlled trial.

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Bipolar loop device versus bipolar diathermy for tonsillectomy: A pilot randomized controlled trial.

Int J Pediatr Otorhinolaryngol. 2020 Nov 19;:110505

Authors: Fakhrealizadeh F, Hoveidaei AH, Eslami J, Hashemi SB, Hoveidaei A, Kazemi T, Nazarpour Z

Abstract
OBJECTIVE: Tonsillectomy is one of the most common surgeries performed in the pediatric population. Although different forms of instruments and various methods are used to perform tonsillectomy, none of them is still recognized as the best global technique. This study aimed to compare the outcomes of the new Bipolar Loop tonsillectomy versus bipolar diathermy technique.
METHODS: This study is a pilot randomized clinical trial and was conducted on 40 pediatric patients who were the candidate of tonsillectomy. Patients were divided into two groups of Bipolar Loop or bipolar diathermy. Operation time, intraoperative bleeding, tonsillar fossa wound, postoperative complications, and duration of return to normal diet were evaluated in the current study.
RESULTS: In both of the Bipolar Loop and bipolar diathermy groups, no significant difference was found in terms of sex, age, and weight. The average amount of the operative time, intra-operative blood loss, and postoperative pain loss were significantly less in the Bipolar Loop group (P < .001). In addition, the tonsillar fossa wound healing scores were significantly better (on the 14th day, P = 0.009). However, there was no significant difference between the two groups in terms of postoperative bleeding, duration of return to a normal diet, and postoperative symptoms of fever, otalgia, or voice change between the groups.
CONCLUSION: The study showed that blood loss and postoperative pain through day 7 were significantly less in the Bipolar Loop group. Tonsillectomy with Bipolar Loop can be recommended as one of the methods for tonsillectomy, especially in pediatric patients.

PMID: 33239197 [PubMed - as supplied by publisher]

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Self-concept of children and adolescents with cochlear implants.

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Self-concept of children and adolescents with cochlear implants.

Int J Pediatr Otorhinolaryngol. 2020 Nov 17;:110506

Authors: Boerrigter M, Vermeulen A, Marres H, Mylanus E, Langereis M

Abstract
OBJECTIVES: Communicative disorders can complicate social interactions and may be detrimental for one's self-concept. This study aims to assess the self-concept of children with Cochlear Implants (CI). Results of educational peer groups (special needs or typical) were compared. Correlations amongst speech perception, language comprehension, self-concept and other study variables are determined.
METHODS: This retrospectively patient file study contained 53 CI participants with a mean age of 14.2 (SD = 2.8). Self-concept was measured with the Dutch language version of the Self-Perception Profile for Children and Adolescents. Proportions of low, normal and high competence scores were compared to a normative sample. Outcomes were analyzed for the total CI group and for the two educational peer groups.
RESULTS: In the Scholastic Competence, Athletic Competence, Physical Appearance and Behavioral Conduct domains larger proportions of high perceived competence levels were found in the CI Total group compared to the hearing normative sample. Children with CIs in the Mainstream educational subgroup were found to have larger proportions of high levels on these domains. Remarkably, children with CI in the Special hearing impaired educational subgroup reported comparable self-concept scores as their hearing peers. Speech perception and language comprehension were positively correlated to Scholastic Competence.
CONCLUSION: This study has shown that self-concept levels of profoundly hearing impaired children with CI are comparable to those of hearing peers. They are generally satisfied with their functioning in various domains. Better speech perception and language comprehension levels are related to higher outcomes in the Scholastic Competence domain.

PMID: 33243619 [PubMed - as supplied by publisher]

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Airway management protocol for conjoined twins delivery.

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Airway management protocol for conjoined twins delivery.

Int J Pediatr Otorhinolaryngol. 2020 Nov 04;:110477

Authors: Snoap A, Varadarajan VV, Mowitz ME, Islam S, Collins WO

Abstract
OBJECTIVE: Conjoined twin deliveries require collaborative preparation by multiple specialties for successful airway management. Literature regarding neonatal airway management after conjoined twin delivery is limited to case reports. We present a case series of conjoined twins and introduce an airway management protocol for conjoined twin delivery.
METHODS: The medical records of conjoined twins and their mothers at a tertiary care center were reviewed from April 2016 to December 2018. The NCBI database was queried for literature regarding preparation for neonatal airway management after conjoined twins delivery.
RESULTS: Five sets of conjoined twins were delivered. Of 10 neonates, all required bag valve mask ventilation. Other airway interventions included continuous positive airway pressure (7), endotracheal intubation (6), and direct laryngoscopy with telescopic video evaluation (1). No patients required ex-utero intrapartum treatment or emergent tracheostomy. A protocol for airway management is described and special considerations are discussed, including anatomic variations, equipment list, operating room staffing and layout, multidisciplinary prenatal conference, and airway imaging review.
CONCLUSION: Conjoined twin deliveries have significant implications for the otolaryngologist and require multidisciplinary collaboration. An airway management protocol allows for a standardized process to secure the neonatal airway and optimize patient outcomes.

PMID: 33243620 [PubMed - as supplied by publisher]

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Feasibility of a hearing screening programme using DPOAEs in 3-year-old children in South Auckland.

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Feasibility of a hearing screening programme using DPOAEs in 3-year-old children in South Auckland.

Int J Pediatr Otorhinolaryngol. 2020 Nov 18;:110510

Authors: Su E, Leung JH, Morton RP, Dickinson LJ, Vandal AC, Balisa NB, Purdy SC

Abstract
OBJECTIVES: In New Zealand (NZ), permanent hearing loss is associated with higher levels of socioeconomic deprivation, and is more prevalent amongst Māori and Pacific than NZ European children. Many of these hearing losses are detected through newborn hearing screening, however there is a need to screen children again later, to look for childhood hearing losses that are either late-onset, progressive, or acquired. This study evaluated the feasibility of implementing an objective screening protocol that includes otoscopy, distortion product otoacoustic emission screening (DPOAEs), and tympanometry. It also evaluated the feasibility of using Early Learning Centres (ELCs) to contact families, recruit, and test 3-year-old children from an area of high socioeconomic deprivation in Auckland, New Zealand.
METHODS: Sixty-one 3-year-old children were recruited from ELCs within the Counties Manukau District Health Board (CMDHB) region which services the geographical area of South Auckland. The first part of the screening protocol consisted of otoscopy, DPOAEs, and tympanometry. Children identified with hearing loss and/or middle ear problems were either referred directly to Otolaryngology/Audiology at the local hospital or invited back for a re-screen 4-8 weeks later. Children who were referred from the screening were followed up to track and document their subsequent clinical pathway through the public health system.
RESULTS: Mean overall time for the screening protocol was 4.1 minutes. The combination of otoscopy, DPOAEs, and tympanometry was well accepted by the 3-year-old children. DPOAE amplitude and signal-to-noise ratio results significantly differentiated between different tympanometry results, providing support for this combination of measures to accurately screen for hearing loss and/or middle ear disease. Thirty-eight of the 61 children (62%) passed the screening protocol. Of the remaining 23 children, five were referred to the hospital after not passing the screening, but following more in-depth audiological testing, were discharged with normal hearing. Six children referred to the hospital were diagnosed with varying degrees of conductive hearing loss, and two of the six received grommet insertion surgery. The remaining 12 children who were referred to the hospital were lost to follow-up, highlighting challenges for the families to successfully navigate the current public health system.
CONCLUSION: This study demonstrates that identifying hearing loss and ear disease in 3-year-old children in the pre-school setting is feasible. A number of barriers were identified in the current health system that contribute to a large proportion of children referred with suspected hearing loss and ear disease being unsuccessful in accessing Otolaryngology/Audiology clinical care through the local hospital.

PMID: 33248714 [PubMed - as supplied by publisher]

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Nasal dorsum reconstruction after pediatric nasal dermoid excision.

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Nasal dorsum reconstruction after pediatric nasal dermoid excision.

Int J Pediatr Otorhinolaryngol. 2020 Nov 16;:110502

Authors: Carroll WW, Farhood Z, White DR, Patel KG

Abstract
BACKGROUND: To review our experience with pediatric nasal dermoids, and discuss reconstructive options for the nasal dorsum after pediatric nasal dermoid removal.
METHODS: Retrospective review of pediatric nasal dermoid cases from January 1 2005 through October 1 2016.
RESULTS: Twenty-five cases (12 males, 13 females) were identified. Median age at time of surgery was 24 months (7-144). Ten nasal dermoids were superficial; eleven, intraosseous; one, intracranial extradural; three, intracranial intradural. Seven were located on the glabella; fifteen, dorsum; three, nasal tip. Twelve underwent vertical midline incision; ten underwent external rhinoplasty; and three combined approach with craniotomy. There was one recurrence four years postoperatively; which was secondarily resected completely via external rhinoplasty approach. Seven cases utilized endoscopic assistance. Conchal cartilage grafting was utilized in nine cases for dorsal reconstruction. A temporoparietal fascial graft was utilized to reconstruct the soft tissue defect in three patients. Median follow-up was 1.17 years (1 month-10 years).
CONCLUSIONS: Nasal dermoid is a rare congenital pathology. Recurrence rate is generally low provided that complete surgical excision is achieved. Achieving complete surgical excision means sometimes compromising the upper lateral cartilages and nasal bones. Conchal cartilage grafting is useful in reconstruction for lesions that significantly disrupt the nasal cartilages and/or nasal bones, wherein the defect is significant and osteotomies may not be sufficient. Temporoparietal fascia is a favorable adjunct for reconstructing soft tissue deficits when the skin is thin. Further studies and longer follow up are needed to adequately assess functional and cosmetic outcomes.

PMID: 33248715 [PubMed - as supplied by publisher]

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Tumor spread through air spaces (STAS): prognostic significance of grading in non-small cell lung cancer

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Abstract
Tumor spread through air spaces (STAS) is an invasive pattern of lung cancer that was recently described. In this study, we investigated the association between the extent of STAS and clinicopathological characteristics and patient outcomes in resected non-small cell lung cancers (NSCLCs). STAS has been prospectively described from 2008 and graded its extent with a two-tiered system (STAS I: <2500 μm [one field of ×10 objective lens] from the edge of tumor and STAS II: ≥2500 μm from the edge of tumor) from 2011 in Seoul National University Bundang Hospital. We retrospectively analyzed the correlations between the extent of STAS and clinicopathologic characteristics and prognostic significance in 1869 resected NSCLCs. STAS was observed in 765 cases (40.9%) with 456 STAS I (24.4%) and 309 STAS II (16.5%). STAS was more frequently found in patients with adenocarcinoma (ADC) (than squamous cell carcinoma), pleural invasion, lymphovascular invasion, and/or higher pathologic stage . In ADC, there were significant differences in recurrence free survival (RFS), overall survival (OS), and lung cancer specific survival (LCSS) according to the extent of STAS. In stage IA non-mucinous ADC, multivariate analysis revealed that STAS II was significantly associated with shorter RFS and LCSS (p < 0.001 and p = 0.006, respectively). In addition, STAS II was an independent poor prognostic factor for recurrence in both limited and radical resection groups (p = 0.001 and p = 0.023, respectively). In conclusion, presence of STAS II was an independent poor prognostic factor in stage IA non-mucinous ADC regardless of the extent of resection.

Modern Pathology, Published online: 16 November 2020; doi:10.1038/s41379-020-00709-2

Tumor spread through air spaces (STAS): prognostic significance of grading in non-small cell lung cancer
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