Nicole L. Alexander, Zipei Feng, Rodrigo C. Silva, Yi-Chun Carol Liu
Publication date 20-12-2020
To characterize the adherence of bone conduction hearing devices (BCHDs) for hearing management in children with unilateral congenital aural atresia (UCAA) in relation to the age of offer and fitting. BCHD Soft Bands help predict amplification benefits before surgery can be performed beginning around five years when both hearing and parental compliance reach stability. We hypothesized device compliance might decrease with age of fitting from lack of early acclimatization. Retrospective case series of patients less than five years old at a tertiary pediatric center's microtia clinic database, born between 2014 and 2018 with UCAA. Adherence was assessed through electronic health record note documentation at less than 1 year, 1-2 years, and greater than 2 years from fitting. The ages at offer and fitting, along with the time from offer to fitting, were recorded. One hundred and eight patients with UCAA were identified, including 46 patients fit with a BCHD used for further analysis. Adherence rates at 1 year, 1-2 years, and greater than 2 years were 47.8%, 30%, and 43.5%, respectively. However, there was no significant association between age offered, age fit, or time from offer to fit and adherence at all time points. Also, there was no significant difference between ages at the time of BCHD offer for those who chose not to proceed with fitting (20.9 months) compared to the age of offer in patients that were subsequently fit (13.9 months). BCHD adherence in patients less than five years old may not be affected by the age offered or fit. The time between offer and fitting was also not associated with usage. BCHDs should be offered to UCAA patients regardless of age. Further investigation in this younger age group would help expand these findings.Pubmed PDF Web
Haisheng Zeng, Mingyu Xie, Jianbo Li, Haoqiang Xie, Xiaomei Lu
Publication date 21-12-2020
The purpose of this study is that analyze the clinical characters of Treacher Collins syndrome (TCS) with the de nove TCOF1 mutation and emphasize the genetic research result.
Genomic DNA from the proband and his parents were extracted from 200 to 400 μl of peripheral blood samples. A 4000 pathgenic genes diagnostic screening panel developed by our laboratory group was used for gene mutation screening. The panel covered the TCOF1 (NM_001135243.1), POLR1C (NM_203,290) and POLR1D (NM_015,972) genes associating with TCS.
We reported a case of typical, complete syndrome with a nonsense mutation c.1622G > A (p.
W541*) in exon 11 of TCOF1, who presents bilateral external ears abnormalities, atresia of external auditory canals, antimongoloid slant of the eyes, bilateral partial coloboma of the lateral part of the lower lids, a large and protruding nose, macrostomia, cleft palate and hair displacement anterior to the auricle. Our report expands the spectrum of known pathogenic TCOF1 variants associated with TCS in humans.
TCOF1 deficiency may cause a severe neonatal presentation with birth defects.
Małgorzata Ganc, Joanna Kobosko, W. Wiktor Jedrzejczak, Bartosz Kochański, Henryk Skarzynski
Publication date 22-12-2020
Hearing is one of our most important senses, and hearing ability has an enormous impact on a child's psychomotor development. Children with auditory perception disorders may show abnormal development in terms of speech, language, and communication skills, as well as other disorders involving the cognitive sphere, social-emotional interactions, and motor development. This paper describes different paths of psychomotor development in three 4-year-old children. All were born with bilateral profound sensorineural hearing loss and were implanted early on with a cochlear implant. A longitudinal study of the children's psychomotor development was done for 3 years from the time of cochlear implantation.
We present three children with congenital, profound bilateral sensorineural hearing loss. Psychomotor development was evaluated using the Children Development Scale (CDS) and the Psychomotor Development Evaluation Cards (PDEC).
The three children were: Girl A (4 years 2 months 17 days) - a user of one CI, last assessment of psychomotor development (PDEC) was 37 months after CI activation; Boy B (4 years 3 months 21 days) - a user of two CIs, last assessment of psychomotor development (PDEC) was 39 months after activation of first CI; Boy C (4 years 1 month 5 days) - user of two CIs, last assessment of psychomotor development (PDEC) was 36 months after activation of the first CI. Analysis of the results from Girl A showed very poor dynamics of development from the age of 12 months, when the first CDS evaluation was performed, up to the PDEC evaluation performed at age 4 years. The CDS score of Boy B showed a very high level of psychomotor development. The PDEC evaluation performed after 39 months of using the first CI showed that the psychomotor development of Boy B was consistent with his chronological age. Boy C presented an average level of psychomotor development (compared to typically developing children) in his CDS scores in the perioperative period and then at 4, 9, and 14 months after cochlear implantation. After 24 months, his CDS scores showed a high level of psychomotor development. After 3 years of CI use, the PDEC evaluation showed that Boy C had an average score in five tested areas, a high score in the area of fine motor skills and lateralization, and a low score in knowledge and learning competencies. Children with bilateral profound hearing loss can present different paths of psychomotor development. Children who receive a CI may show an age-appropriate level of psychomotor development compared to typically developing children. However, a hearing, speech, and language rehabilitation specialist responsible for the child still needs to perform regular checkups to monitor all areas of psychomotor development. Also, the effects of the deaf child's environmental determinants on psychomotor development should be studied in detail. These determinants include the parents' emotional state, the parents' involvement in the child's rehabilitation, the family's quality of life, and the presence of deaf siblings. In particular, the difficulties encountered by the mother (or father) in being the parent of a deaf child may hinder the family from receiving adequate support.
Ela Cömert, Ziya Şencan, Furkan Melih Koçak, Gökçe Şimşek, Nuray Bayar Muluk
Publication date 21-12-2020
The objectives of the present study were to evaluate the vestibular impairment in children with acute otitis media (AOM) and dizziness by using video head impulse test (vHIT) and to compare their results with healthy children. The study included two groups of subjects. The patient group consisted of 34 pediatric patients with AOM and dizziness and the control group consisted of 35 healthy children, age between 4 and 15. The age, gender, mean vHIT gains and gain asymmetry values were compared between groups for each canal. In both groups, mean vHIT gains were compared between the right and left sides. Additionally, mean vHIT gains and the presence of saccades according to AOM stages were analyzed. The comparison of vHIT gains between affected and unaffected sides in patients revealed a significant decrease only in the anterior canal plane on the affected side. Covert saccades were observed in 32% of the patients. When comparing the AOM stages and the presence of saccades in patients, no difference was detected between stages. vHIT is a useful vestibular test for the evaluation of vestibular impairment in children with an applicability rate of 92% in healthy children and 70% in patients with AOM and dizziness. The patients with AOM and dizziness are presented with a decrease in vHIT gains and the presence of cover saccades only in vertical canal planes, supporting that slight vestibular impairment in these patients may represent pathologic vHIT results only in vertical canal planes.Pubmed PDF Web
P. Reynard, E. Ionescu, C.A. Joly, A. Ltaief-Boudrigua, A. Coudert, H. Thai-Van
Publication date 21-12-2020
Enlarged vestibular aqueduct (EVA) is a common finding in tomodensitometry. When cranial MRI is performed, enlarged endolymphatic sac (EES) can also be found. Profound hearing loss is a common finding in these patients but a few studies have investigated vestibular function after cochlear implantation (CI) in EVA and EES patients. Our main objective was to find out whether in EVA children candidates to CI, a higher endolymphatic sac (ES) volume was predictive for higher rates of postsurgical vestibular complications. We retrospectively included EVA children who benefited from CI, during the last 2 years. Two groups were constituted according to the presence or not of a vestibular impairment (decrease in the VOR gain on the VHIT test on one of the semicircular canals and/or a loss of cVEMPs) 6 months after CI. Endolymphatic volume of both VA and ES was measured for each patient. Fifteen patients were included. The mean endolymph volume was significantly higher in the impaired group (0.40 cm In EVA children, a combined EES appears to increase the risk of severe post CI vestibular impairment. To minimize this risk prior CI surgery, besides tomodensitometry, MRI measurement of the ES volume should be systematically performed.Pubmed PDF Web
Allison Keane, Robert A. Saadi, Einat Slonimsky, Meghan Wilson, Jason May
Publication date 22-12-2020
To compare tracheoscopy and chest radiograph measurements of tracheostomy tube position in infants. Retrospective chart review. Otolaryngology Department at Penn State Milton S. Hershey Medical Center. All cases of pediatric patients who underwent tracheotomy at less than 1 year of age from 2014 to 2019 were reviewed. Patients were included if they had both intraoperative measurement of tracheostomy tube position relative to the carina by tracheoscopy and postoperative chest radiograph. Documented intraoperative findings were compared to measurements made on chest radiograph by an attending radiologist blinded to the intraoperative measurements. The study included 66 patients; 30 patients (14:16, M:F) had available data. The mean distance from the distal tracheostomy tube to the carina measured by tracheoscopy was 8.88 mm (range, 3.5-20 mm) and measured radiographically was 11.71 mm (range, 2.4-23.3 mm). The mean difference between the measurements was 2.82 mm (p-value = 0.016). Ninety percent (n = 27) of patients had measurements that differed by greater than 2 mm; 53% (n = 16) had measurements that differed by 5 mm and 1% (n = 3) had measurements differing by greater than 10 mm. In the infant population, significant discrepancy was found between direct tracheoscopy and chest radiograph measurements of the tracheostomy tube position. Measurements obtained by chest radiographs tend to overestimate the relative distance of the distal tracheostomy tube to the carina as compared to that of tracheoscopy. Clinical decisions regarding changes to tracheostomy tube sizes should mostly rely on tracheoscopy performed with the patient supine.Pubmed PDF Web
Aimee Kennedy, Catherine K. Hart, Alessandro de Alarcon, Karthik Balakrishnan, An Bowudewyns, Robert Chun, Pierre Fayoux, Steven L. Goudy, Christopher Hartnick, Wei-Chung Hsu, Romaine F. Johnson, Michael Kuo, Shazia Peer, Seth M. Pransky, Reza Rahbar, Scott Rickert, Soham Roy, John Russell, Kishore Sandu, Douglas R. Sidell
Publication date 21-12-2020
To provide recommendations to otolaryngologists, pulmonologists, and allied clinicians for tracheostomy decannulation in pediatric patients. An iterative questionnaire was used to establish expert recommendations by the members of the International Pediatric Otolaryngology Group. Twenty-six members completed the survey. Recommendations address patient criteria for decannulation readiness, airway evaluation prior to decannulation, decannulation protocol, and follow-up after both successful and failed decannulation. Tracheostomy decannulation recommendations are aimed at improving patient-centered care, quality and safety in children with tracheostomies.Pubmed PDF Web
Chengetai Mahomva, Anne Kim, Judith E.C. Lieu, Donald M. Goldberg, Samantha Anne
Publication date 20-12-2020
The impact of mild-moderate unilateral sensorineural hearing loss (USNHL) on speech and language delay (SLD) is not well established. Objectives included (1) determining SLD prevalence in patients with mild-moderate USNHL in comparison to prevalence in the general population and severe-profound USNHL patients and (2) examining speech, language, and auditory function testing (SLAT) results in USNHL patients. A retrospective chart review of pediatric patients with USNHL, classified using pure tone averages (PTA) into mild-moderate (PTA 21-60) and severe-profound (PTA ≥ 61) USNHL groups was conducted. Abnormal SLAT values defined SLD. Prevalence and association of SLD based on USNHL severity was calculated. Onesample binomial tests compared observed frequencies of SLD to reported values. Forty-nine patients were identified with USNHL; 34 patients underwent SLAT. SLD frequency for mild-moderate USNHL was 25% (95% CI, 9-49%), higher than the general population rate (5.95%). No statistically significant difference was noted between SLD frequency in mild-moderate versus severe-profound USNHL. There were no significant correlations between SLAT measures and PTA thresholds. There was a statistically significant increase in SLD in mild-moderate USNHL compared to the general population. There were no correlations between SLAT measures and PTA thresholds. Children with USNHL need close monitoring of speech, language and auditory development and functioning. Studies with larger sample sizes will help delineate if these findings truly reflect results in children with USNHL.Pubmed PDF Web
Silvia Palma, Maria Federica Roversi, Maria Paola Alberici, Maurizio Negri, Claudio Chiossi, Alberto Berardi, Elisabetta Genovese
Publication date 21-12-2020
Since January 2012, babies born in the province of Modena, Italy, have routinely undergone hearing testing as part of a two-stage screening programme. Newborn hearing screening (NHS) has been based on an integrated hospital and community care system and this study aims to assess screening coverage, referral rates, the prevalence, type and extent of hearing loss several years into the programme. Data were collected from January 1, 2012 to December 31, 2015. Coverage was over 99% in all five facilities of the province. The ratio of "fails of the screening" to the total number of infants tested varied over the period from 1.2% to 0.9% in the third level facility, and from 0.8% to 0.4% in the other four. Although hearing loss was mainly associated with dysmorphic\syndromic diseases or a family history of hearing loss, some 23% of cases were identified with no known risk factors. We highlight the importance of the NHSP and the need for strong support from healthcare administrators to ensure high coverage. This is especially true since although the prevalence of hearing loss was higher among infants with audiological risk factors, several cases of hearing loss were found in newborns with no known risk factors.Pubmed PDF Web
Weronika Swierniak, Elzbieta Gos, Piotr Henryk Skarzynski, Natalia Czajka, Henryk Skarzynski
Publication date 21-12-2020
Parental suspicion of hearing impairment in their children is generally inaccurate. Parents tend to underestimate hearing problems in their children. The aim of the study was to assess the accuracy of parental suspicion of hearing loss in their children. This was a population-based, epidemiological study conducted in elementary schools in villages and small towns in Poland. The study sample was 64,750 children aged 6-13 years old. The children underwent hearing screening with pure-tone audiometry. The parents answered a question about hearing problems in their children. The outcome parameters were sensitivity, specificity, and predictive value of parental perception of hearing problems in their children. Parental suspicion of hearing problems was assessed by a questionnaire. Pure-tone air-conduction hearing thresholds were obtained from 0.5 to 8 k Hz. Hearing loss was defined as a pure-tone average higher than 20 dB in one or both ears in at least one of the three following pure-tone averages: four-frequency pure-tone average, high-frequency pure-tone average, and low-frequency pure-tone average. Positive results of hearing screening were obtained in 16.3% of children. Hearing loss was detected in 6025 children (9.3%), of whom 1074 (17.8%) were correctly perceived by parents as having hearing problems. The degree of hearing loss was a significant factor which influenced the sensitivity of parental suspicion of HL in their children. Sensitivity of detecting hearing loss by parents reached about 20% for mild hearing loss and above 31% for moderate or worse hearing loss. Parents underestimate hearing problems in their children, but they are more able to accurately detect hearing loss if it involves speech-related frequencies and is at least a moderate hearing loss or bilateral loss.Pubmed PDF Web
Burcu Deniz, Eyyup Kara, Zahra Polat, Rışvan Deniz, Ahmet Ataş
Publication date 18-12-2020
The primary aim of this study is to investigate whether the electrical stimulation of the second ear causes a difference in electrically evoked auditory brainstem responses (eABR) between two ears over time. The study included thirteen subjects under the age of five who used cochlear implants for at least six months in the first ear prior to the sequential cochlear implantation. Postoperative eABRs were conducted on the 1st (first fitting of the second speech processor), 3rd, and 6th months of the second implantation in the basal, medial, and apical electrode positions. The recording was started with the second cochlear implant (CI2), and then the first cochlear implant (CI1) was tested. Sound field audiometry and receptive/expressive language assessments were also performed at 1 and 6 months after the second cochlear implantation. eABR results indicate that when eV wave latencies are examined for all electrodes, CI2 is significantly longer than CI1 (p < 0.05). When eV wave amplitudes are examined for all electrodes, CI1 is significantly higher than the CI2 (p < 0.05). eV latency and amplitude changes between both implants were examined up to six months after implantation. Statistically significant changes were observed in the basal, medial, apical electrode for eV wave latencies, and only in the medial electrode for eV wave amplitudes (p < 0.05). Average sound field thresholds and receptive/expressive language scores improved statistically significantly for all subjects at the end of the study (p < 0.05). The postoperative eABR test is a valuable test battery that provides the clinician with important ideas about the estimated threshold, comfortable and audible sound level, CI performance, and auditory pathways up to the brainstem. Since the maturation is still ongoing, an extended period longer than six months is needed to evaluate interaural differences.Pubmed PDF Web
Mateus Morais Aires, Camila Barbosa Marinho, Silvio José de Vasconcelos
Publication date 18-12-2020
To evaluate outcomes of injection laryngoplasty (IL) and laryngeal reinnervation for the treatment of pediatric Unilateral Vocal Fold Paralysis (UVFP), especially on swallowing and quality of voice. A literature review was performed in Medline/Pub Med and Cochrane Library, following PRISMA guidelines, with no constraints on publication date. We included studies in English, Portuguese or Spanish about surgical treatment for UVFP on the pediatric population (0-21 years) that documented outcomes for one of the following techniques: IL or laryngeal reinnervation. Study characteristics, patient demographics, technical aspects of each procedure, complications, and outcomes for voice and swallowing were extracted. A meta-analysis with inverse variance, random-effects model was performed. The PRISMA approach yielded 22 studies, totaling 267 patients. Seven reinnervation articles were included in meta-analysis for maximum phonation time (MPT) and quality of voice measured by Grade, Roughness, Breathiness, Asthenia and Strain (GRBAS) scale. Cardiac surgery had caused UVFP in 62.8% (142/226) of the cases. The main indication for IL was aspiration and for reinnervation was dysphonia. For IL, there was an improvement of 84.5% (confidence interval [CI] 82.6-88.4%) in swallowing and 81.4% (CI 74.6-88.1%) in voice. For reinnervation, there was an improvement of 91.6% (CI 88.2-94.9%) in swallowing and 96.8% (CI 95.5-98.0%) in voice. We found an increase of 6.19 s (CI 1.00 to 11.38) in MPT and a mean difference in GRBAS sum of -3.53 points (CI -6.15 to -0.91) after reinnervation. Retrospective cohort studies suggest that injection laryngoplasty and reinnervation are both effective in improving swallowing and voice in children with UVFP. There was clinical evidence of improvement in the MPT and GRBAS scale meta-analysis in patients undergoing reinnervation.Pubmed PDF Web
Saied Ghadersohi, Salina Haville, Megan Hedman, Kirsten Adkisson, Emily Cooper, Alexander Kaizer, Sarah A. Gitomer, Peggy E. Kelley
Publication date 19-12-2020
Patients with microtia and aural atresia have multiple options for treatment of conductive hearing loss (CHL) and auricle reconstruction; however, little is known about the factors influencing treatment selection. This study aims to review the socioeconomic and clinical data of microtia/atresia patients to evaluate congruency with national data and whether these factors affect treatment decisions.
Retrospective review of patients evaluated in the microtia and atresia multidisciplinary clinic (MDC) at a tertiary academic children's hospital between 2008 and 2018. Outcomes included demographic, socioeconomic and clinical factors associated with hearing surgery and framework surgery.
373 patients were seen in the Microtia MDC: 193 (51.7%) were male, 187 (50.1%) identified as Hispanic and 23 (6.2%) identified as Asian. 267 (75.6%) patients received a nonsurgical bone conduction hearing device (BCHD); fitting at a younger age was associated with better nonsurgical BCHD compliance. Multivariate analysis was performed on the patients that were eligible for surgery based on age and appropriate follow-up. 70 (18.8%) patients had placement of an osseointegrated BCHD; inconsistent compliance with nonsurgical BCHD decreased the odds of proceeding with osseointegrated BCHD placement. 60 (16.1%) patients underwent framework surgery for external reconstruction. Placement of osseointegrated BCHD was the only factor that was associated with proceeding with framework surgery. Other assessed demographic and socioeconomic factors were statistically not associated with selection of surgical intervention. Fitting a nonsurgical BCHD at a younger age is associated with higher likelihood of nonsurgical BCHD compliance, that is in turn associated with patients and families proceeding with osseointegrated BCHD and framework surgery.
Cynthia Schwartz, Stacy Philip, Winslo Idicula, Joshua Demke
Publication date 15-12-2020
Tessier 7 clefts are a rare congenital anomaly, usually surgically repaired with Z-plasty or other reconstructive methods, although undesirable scars may result. We present a review of the literature and a case of unilateral Tessier 7 cleft repaired with a novel reconstruction technique using a combined Z-plasty and geometric broken line closure (GBLC) to camouflage and irregularize the otherwise linear scar. We present this case to expand the armamentarium of surgical options to address Tessier 7 clefts and to review techniques for repair.Pubmed PDF Web
Rhea Jain, Renée L. Betancourt, Austin S. Rose
Publication date 10-12-2020
Lipoblastomatosis is an extremely rare condition and is often not recognized prior to final pathologic analysis. In this case, a two-year-old female child presented with a non-tender, submental midline neck mass which had enlarged very slowly over the course of five months. The differential diagnosis included lipoma, lymphatic malformation, and thyroglossal duct cycst and a surgical excision was planned. The child was consented for a possible Sistrunk procedure if necessary. Intraoperatively, the mass was adherent to the hyoid bone and clear mucoid drainage was noted during resection leading the team to further weight the possibility of thyroglossal duct cyst and proceed with a Sistrunk procedure. Ultimately, final pathology revealed the diagnosis of lipoblastomatosis.Pubmed PDF Web
Michelle White, Jennifer L. Harb, Malgorzata Dymerska, Sylvia H. Yoo, Pasquale Eckert, David Chang, Alexander P. Marston
Publication date 04-12-2020
Neonatal orbital infections are quite rare, and are most often attributed to ethmoid sinusitis. This report describes a case of subperiosteal orbital abscess in a neonate secondary to an infected neonatal tooth. Although there have been two cases reported in the literature describing odontogenic infection resulting in orbital abscess in neonates, these cases were due to infected tooth buds rather than an infected neonatal tooth. We discuss workup and surgical management of this patient, including tooth extraction and intraoral approach to the orbit for abscess drainage.Pubmed PDF Web
Christina Dorismond, Zainab Farzal, Nicholas J. Thompson, Saangyoung E. Lee, Carlton J. Zdanski
Publication date 15-12-2020
(s): Patient-reported outcome measures (PROMs) are tools that allow patients to directly share information about their health with their healthcare provider. Health literacy experts recommend that health information, such as PROMs, be written at a 6th grade level to ensure patients can read and comprehend it. As the readability of PROMs used in pediatric otolaryngology has yet to be studied, our goal was to analyze the readability of these PROMs and assess their compliance with readability recommendations. The Gunning Fog, the Simple Measure of Gobbledygook (SMOG), and the FORCAST readability formulas were used to determine the readability of disease-specific PROMs for pediatric otolaryngology. Fourteen PROMs were reviewed in the study. Most were intended for caregiver completion (n = 13, 92.9%). Ten PROMs when measured by Gunning Fog (71.4%), 2 PROMs when measured by SMOG (14.3%), and 0 PROMs when measured by FORCAST (0.0%) were at or below the 6th grade reading level. Most PROMs available for use in pediatric otolaryngology are above the recommended 6th grade reading level when measured by FORCAST, the most appropriate metric for assessing questionnaires. The high reading grade level needed to complete these PROMs can contribute to health disparities among underserved and vulnerable populations, such as children. Pediatric otolaryngology PROMs developed in the future should take readability into account in order to ensure equity in the delivery of care.Pubmed PDF Web
Hanaa Knaneh-Monem, Beth Osterbauer, Christian Hochstim
Publication date 11-12-2020
In the present study, tracheal epithelial biopsy samples between intubated children, children with tracheostomy and a control group of non-intubated children are compared with respect to their degree of normal differentiation versus the presence of squamous metaplasia. Tracheal epithelial biopsies were obtained from intubated neonates undergoing tracheostomy, children with tracheostomy undergoing suprastomal granuloma excision and non-intubated control children undergoing laryngoscopy and bronchoscopy. Paraffin tissue blocks were sectioned at 5 μm thickness and subjected to both routine Hematoxylin and Eosin (H&E) staining and immunostained with the relevant antibodies for markers of epithelial differentiation including B-tubulin, CC10, Muc5ac, P63, keratin5 and keratin14. Squamous metaplasia was seen in 3/3 infants, all intubated and in 3/3 children with tracheostomy tubes in place undergoing excision of suprastomal granuloma. No metaplasia was observed in control tracheal epithelial biopsies in 7/7 non-intubated children. Our results demonstrate a clear association between intubation or tracheostomy and the presence of squamous metaplasia which is not otherwise encountered in control pediatric tracheal biopsies.Pubmed PDF Web
Tomomi Yamamoto-Fukuda, Naotaro Akiyama, Norifumi Tatsumi, Masataka Okabe, Hiromi Kojima
Publication date 11-12-2020
Cholesteatoma is an epithelial lesion that expands into the middle ear, resulting in bone destruction. The acceleration of the proliferative activity of epithelial stem/progenitor cells is involved in the pathogenesis of cholesteatoma. Recently, the use of a menin-mixed lineage leukemia 1 (MLL1) inhibitor, MI503, in experiments has resulted in inhibition of the growth of tumors under histone modification. In this study, we investigated the effects of the menin-MLL inhibitor against cholesteatoma growth in an in vivo model. We first correlated the expression level of histone H3 trimethylation at lysine 4 (H3K4me3) among cholesteatoma cases, chronic otitis media cases and normal skin tissues. Based on the role of keratinocyte growth factor (KGF) in the development of cholesteatoma, KGF-expression vector was transfected into the ear and we analyzed the expression level of H3K4me3. After cholesteatoma was induced, MI503 was administered daily into the ear for 14 days. We detected the highest labeling index of H3K4me3 in the cholesteatoma specimens. After KGF-expression vector transfection in the mouse ear, a high expression level of H3K4me3 was observed in the epithelial layers. The use of MI503 reduced cholesteatoma in the in vivo model and decreased the proliferation of epithelial stem/progenitor cells in a dose-dependent manner. We demonstrated that inhibition of the menin-MLL interaction may be a potentially useful strategy in the conservative treatment of cholesteatoma.Pubmed PDF Web
Sarah M. Sussman, Daniel D. Sharbel, Arsh Momin, John D. Prosser, William W. Carroll
Publication date 11-12-2020
There remains variation in management of orbital complications of acute bacterial rhinosinusitis (ABRS); specifically, those subperiosteal abscesses that present without immediate surgical indication. Recent systematic reviews on management and proposed treatment algorithms are helpful but do not consider the financial implications for healthcare systems and patients.
A retrospective chart review of pediatric patients from a tertiary care children's hospital between 2002 and 2020 was performed, identifying patients via ICD coding corresponding to acute bacterial sinusitis and orbital involvement classified as Chandler 3 confirmed by contrasted computed tomography (CT).
Two groups of patients were identified: intravenous (IV) antibiotics alone and IV antibiotics plus surgery. Billing records of total hospital charges and physician fees were recorded for financial analysis. Demographic, length of stay, and charges were analyzed. 58 patients with ABRS and Chandler 3 orbital involvement were confirmed by CT imaging. Twenty-nine (50%) were treated with IV antibiotics alone, and twenty-nine (50%) underwent surgery in addition to IV antibiotics. There were no significant differences in patient demographics. The average total hospital charges for the medically managed group were $9262 ± 4831 compared to $30,830 ± 11,397 for the surgical group (p < 0.0001). In the medically managed group, the average hospital fees were $7305 ± 4048 and the average physician fees were $1543 ± 799. In the surgical group, the average hospital and physician fees were also significantly higher at $23,071 ± 7305 (p < 0.0001) and $7763 ± 3335 (p < 0.0001), respectively. Patients who were treated medically and had a longer than average LOS still had significantly fewer charges than those treated with antibiotics plus surgery and a shorter than average length of stay [$15,311 and $27,723, respectively (p = 0.02)]. Pediatric ABRS with orbital involvement requires prompt attention and management. Controversy persists over subperiosteal abscess management that present without overt surgical indications. Surgical intervention is expensive. Our subgroup analysis demonstrates the magnitude of this cost difference. Specifically, longer inpatient stays with IV antibiotics alone appear to be significantly cheaper than shorter ones that include surgery. Not all Chandler 3 patients are candidates for non-surgical management; however, clinicians are encouraged to keep these data in mind for those patients where further medical management is safe and may yield less expensive clinical resolution.
Cynthia Wang, Romaine Johnson, Sami Horani, Clarice Brown, Ron Mitchell, Gopi Shah
Publication date 11-12-2020
Non-surgical management of chronic rhinosinusitis (CRS) in children with cystic fibrosis (CF) has been increasing over the last decade. This study examines inpatient children with pulmonary exacerbation of CF who were also diagnosed with CRS and underwent endoscopic sinus surgery (ESS). We used the 2003 to 2016 Kids Inpatient Database to perform a cross-sectional analysis of inpatients (ages 0-21 years) diagnosed with CF and CRS in the United States from 2003 to 2016. Demographics and CF-associated comorbidities were recorded and rates of CRS and ESS in children with CF were examined. 49,110 children were included in the study. A total of 9334 (19%) were diagnosed with CRS. The average age was 13 (SD 5.9) years; the majority were female (56%), and White (67%). The prevalence of CRS increased from 2003 to 2016 (14%-23%, p < 0.001) while the rate of ESS decreased (20%-11%, p < .001). Patients with CRS that underwent ESS more commonly had CF-associated comorbidities including GI manifestations (15%-25%, p < .001) and liver disease (15%-30%, p < .001). The diagnosis of CRS in children with CF hospitalized for pulmonary exacerbation has increased while ESS has decreased in the last decade. Patients with CRS that underwent ESS more commonly had CF-associated comorbidities. Studies to determine whether children with CF-associated comorbidities are more likely to benefit from ESS are needed.Pubmed PDF Web
Sara C. Gallant, Amy R. Danehy, Greg R. Licameli
Publication date 15-12-2020
To investigate the prevalence and nature of adverse events in magnetic resonance imaging (MRI) of pediatric cochlear implant (CI) patients. Retrospective chart review at a tertiary pediatric hospital. CI patients who underwent MRI from 2004 through 2019 were identified via our internal radiology database. Comorbidities, CI model, age at MRI, number of MRIs, type of MRIs, indication for MRIs, precautions taken for MRIs, quality of MRIs, anesthesia during MRIs, patient language abilities, and adverse events were recorded from the electronic medical record. The literature was reviewed, and our results were compared to those of previous similar series. From 2004 to 2019, 12 pediatric patients (17 ears) with CIs underwent 22 MRIs. 12 MRIs were performed in CI patients with retained internal magnet. 4/22 MRIs resulted in morbidity; 2 patients experienced pain requiring MRI abortion, 1 experienced magnet rotation requiring surgical replacement, and 1 underwent operative removal of the magnet prior to the scan with surgical replacement thereafter. 19/22 MRIs were performed to evaluate the brain; 17/22 of the radiologic reports noted limitation of evaluation due to artifact. 18/22 MRIs required the administration of anesthesia. 9 of the 22 MRI events involved 2 patients whose CIs had been without internal magnet in anticipation of future MRI requirement. Adverse events affecting pediatric patients with CI can occur as a result of MRI, despite appropriate precautions. Safety requires consideration of factors unique to a pediatric hearing-impaired population. Clinicians must remain informed on best practices and manufacturer recommendations.Pubmed PDF Web
Khaloud Al Bahri, C. Carrie Liu
Publication date 10-12-2020
To systematically review the literature on the yield of surveillance airway endoscopy in pediatric patients with tracheostomies.
A systematic search was performed according to PRISMA guidelines of the MEDLINE/Pubmed and Embase databases.
Data were collected on the following outcomes of interest: abnormal airway findings in surveillance endoscopy performed in pediatric tracheostomy patients, frequency and nature of interventions performed during endoscopy, and predictive factors associated with abnormal airway findings. Seven studies were included in the review. The timing of endoscopy post-tracheostomy placement was variable and ranged from 1 to 24 months. All studies reported abnormal airway findings on initial endoscopic examination, with rates varying from 20 to 87%. Airway granulomas/granulation tissue was the most common finding, followed by airway stenosis and suprastomal collapse. Interventions performed to improve airway safety occurred in 18%-64% of patients undergoing surveillance endoscopy. The most commonly reported interventions were debridement of granulation tissue and dilation of subglottic stenosis. No endoscopy-related complications were reported across the studies. The presence of tracheostomy-related symptoms was the most consistently reported predictor of abnormal airway findings and airway interventions. Pediatric tracheostomy patients undergoing surveillance airway endoscopy have a high rate of abnormal airway findings and interventions. However, additional studies are needed before routine endoscopy can be recommended in asymptomatic patients.
Salman F Alhabib, Yassin Abdelsamad, Medhat Yousef, Farid Alzhrani, Abdulrahman Hagr
Publication date 11-12-2020
To assess the evolution of electrode impedance after the early fitting of audio processors (activation after one-day) and classical fitting (activation after one-month) over an up-to-one year after cochlear implant (CI) surgery. A retrospective cohort study on Fifty-two CI recipients divided into two groups. The study group included 24 recipients (40 ears) who underwent early fitting, whereas the control group contained 28 recipients (40 ears) who underwent classical fitting. The electrode impedance was recorded during the surgery, switch-on session and at one, three, six, and twelve-months after the surgery. In the study group, electrode impedance values obtained intraoperatively and at switch-on and one, three, six, and twelve-months were 4.89, 3.69, 6.52, 6.24, 6.05, and 5.81 KΩ, respectively, and only the switch-on and one-month values were significantly different (p < 0.0001). In the control group, electrode impedance values obtained intraoperatively and at switch-on and one, three, six, and twelve-months were 4.71, 7.19, 6.40, 6.05, and 5.73 KΩ, respectively. Thus, the electrode impedance value at switch-on was 52.65% (p < 0.001) greater than it intraoperatively. For both groups, the electrode impedance value at twelve-months was significantly higher than the respective intraoperative values (study group: 18.6% higher, P = 0.04; control group: 21.65% higher, P = 0.0001). Electrode impedance was significantly lower in the study group compared to the control group until one month after the surgery. However, the electrode impedance at twelve-months after the CI was similar in both groups.Pubmed PDF Web
Jordyn P. Lucas, Meredith Allen, Bianca Siegel, Nathan Gonik
Publication date 10-12-2020
Determine the utility of preoperative imaging and the optimal course of management for congenital floor of mouth (FOM) cysts in infants. A systematic review of the literature was performed conforming to PRISMA guidelines. Pubmed, Embase and Cochrane Library databases were queried to identify cases of infants with congenital floor of mouth masses. Patient demographics, presenting findings, imaging, management, complications, and outcomes were determined. 85 patients were evaluated. 98% of patients presented at 16 months of age or younger. The most common presenting symptom was submental mass or swelling, 31.3%. Among the patients that underwent imaging, the suspected diagnosis obtained from imaging findings was consistent with the final pathologic diagnosis 59% of the time reported and inaccurate 34% of the time. There were multiple definitive treatment modalities described in the literature review including surgical excision, 82.3%, marsupialization, 12.9%, chemical injection 2.3%, sclerotherapy 1.2%,% and radiation, 1.2%. Recurrence rate after initial definitive treatment was as follows, surgical excision, 8.8%, marsupialization, 80%, sclerotherapy, 100%, chemical injection, 50%, and radiation, 100%. Preoperative imaging studies should not be relied upon alone to determine suspected pathology and subsequent management in pediatric patients with FOM masses. It may be beneficial for these patients to undergo primary surgical excision regardless of imaging studies or suspected pathology. Needle aspiration offers limited addition to pathologic diagnosis and should only be performed in the setting of acute symptomatic management. Surgical excision should be considered as definitive treatment modality in all patients with FOM masses, regardless of the suspected diagnosis of ranula. Further multi-institutional cohort studies could be invaluable to elucidate definitive treatment guidelines in this patient population.Pubmed PDF Web
Ana Concheiro-Guisan, Antía Fiel-Ozores, Reyes Novoa-Carballal, María Luisa González-Duran, Mar Portugués de la Red, Cristina Martínez-Reglero, Isabel Fernández-Pinilla, Isabel González-Guijarro
Publication date 12-12-2020
Anosmia/hyposomia have been described as early signs of COVID-19 infection in adults, including young asymptomatic patients who commonly refer olfactory disfunction as their only clinical manifestation. Very few studies involving paediatric age patients have been published until now. This study aims to determine the presence of olfactory dysfunction in children with COVID-19 infection through the use of a self-reported questionnaire and a new olfactory screening tool. Nested case-control study. All paediatric patients screened by reverse transcription polymerase chain reaction (RT-PCR) and Anti-SARS-CoV-2 antibodies for COVID-19 infection, during the study period (March-May 2020), were asked to respond to a questionnaire about symptoms of olfactory disfunction. Patients above six years old also performed an odor identification test based on seven odorants (Kradeo®). This test was designed based on our cultural context and eating habits. 126 patients were recruited, including 33 with COVID-19 infection. 15% of the infected children referred anosmia and/or dysgeusia on the questionnaire, all of them were older than eleven years. The results of the odor test (69 patients) revealed subtle disturbances in the infected group (mostly misrecognition of odorants). Median odorant recognition was 3 odors [Interquartile range (IQR) 2-4] in case group and 4 [IQR 3-5] in controls. Male patients showed significantly larger disturbances than girls in both groups (p = 0.03). Self-referred prevalence of olfactory disfunction in our sample of infected children is lower than that described in adults, especially among the youngest ones, maybe due to immature development of angiotensin-converting enzyme 2 (ACE2) receptors expressed in nasal mucosa. Nevertheless, one month after infection, subtle disturbances (misrecognition of odors) were identified among the infected children. This screening olfactory test provides a hygienic, user-friendly tool, suitable for screening children older than six years of age.Pubmed PDF Web
Maayan Gruber, Danny Damry, Nur Ibrahim, Daniel Glikman, Ohad Ronen
Publication date 09-12-2020
Acute otitis externa (AOE), is a common infectious disease affecting children and adults. Its peak prevalence is around the summer months, it involves the external auditory canal and in most cases is due to bacterial agents.
This is a retrospective observational analytical case-based study involving all consecutive patients under the age of 18 years old presenting with AOE to a pediatric emergency department.
We collected data from 337 patients under 18 years of age with 344 visits to the Emergency Department, between the years 2011-2018. Nearly half of the visits presented during the summer months.
Children were divided into two subgroups: hospitalized and non-hospitalized. Median hospitalization time was 3 days. The hospitalized sub-group had higher rates of failed treatment, as well as higher rates of external ear canal edema, systemic fever, canal discharge and auricular edema.
On multivariable analysis the following variables had the strongest correlation for hospital admission: auricular edema (OR 27.98), otorrhea (OR 1.82), narrowing of the ear canal by more than 50% (OR 1.91), fever (OR 2.92), and previous systemic treatment (OR 2.53). Pseudomonas aeruginosa (PA) was isolated in 78% of cultures in the hospitalized sub-group. All PA strains were sensitive to ciprofloxacin. This study highlights the main clinical variables which may predict hospitalization among children with AOE as well as the dominant role of sensitive strains of PA in the pathogenesis of this condition in children.
Glenn E. Mann, Steven Z. Flamer, Singh Nair, James N. Maher, Brandon Cowan, Agathe Streiff, David Adams, Naum Shaparin
Publication date 09-12-2020
Opioids are administered during the intraoperative and postoperative periods in pediatric adenotonsillectomy and tonsillectomy. Non-opioid analgesics are often used as an analgesic during pediatric adenotonsillectomy and tonsillectomy. In this hypothesis generating study, we are evaluating safety and efficacy of stand-alone opioid analgesia for adenotonsillectomy and tonsillectomy.
This is a single-center retrospective chart review of patients ages 2 to 13 who underwent elective adenotonsillectomy and tonsillectomy. We used a convenience sampling method to select patients who received intraoperative intravenous fentanyl, acetaminophen, ibuprofen, or any combination thereof.
The following outcomes were analyzed in this study: (i) the length of Post Anesthesia Care Unit stay, (ii) administration of postoperative opioids; (iii) postoperative opioid equivalents required; (iv) administration of postoperative non-opioid analgesics; and (v) inpatient admission from ED within 30 days. We used univariate analysis to compare the data points. We analyzed data from 323 patients who underwent adenotonsillectomy and tonsillectomy. The Post Anesthesia Care Unit length stay was similar for the intraoperative opioid-free and intraoperative opioid groups, 146.68 (±67.35) and 143.18 (±37.85) minutes, respectively (p = 0.586). Additionally, 102 patients (73.4%) in the intraoperative opioid-free group and 184 patients (83.2%) in the intraoperative opioid group did not receive any postoperative opioids (p = 0.033). The incidence of adverse events was similar between the intraoperative opioid-free and intraoperative opioid groups 3 (2.2%) and 5 (2.7%) respectively, p-value 0.749. A subgroup analysis comparing extracapsular 235 (72.8%) versus intracapsular 88 (27.2%) tonsillectomy yielded similar results. In this study, our data indicates that American Society of Anesthesiologists I- II pediatric patients undergoing adenotonsillectomy and tonsillectomy can be efficiently and safely managed with an opioid-free intraoperative and postoperative analgesic regimen. Due to the explained limitations, our study results should be interpreted cautiously.
Chelsea S. Hamill, Meghan M. Tracy, Vincent S. Staggs, Winston M. Manimtim, Laura L. Neff, Daniel R. Jensen
Publication date 09-12-2020
Tracheostomy in children is often performed to alleviate airway obstruction (AO) or to facilitate long-term ventilator support due to respiratory failure of various etiologies, such as heart failure, and postoperative respiratory failure. Although many of these pathologies are common among trisomy 21 patients, tracheostomy rates among this population have not previously been reported. The aim of our study was to determine the incidence of trisomy 21 patients undergoing tracheostomy. Secondary objectives include decannulation rates and mortality associated with tracheostomy. A retrospective cohort study was conducted on pediatric trisomy 21 patients undergoing tracheostomy between 2004 and 2013. Twenty patients underwent tracheostomy at a median age of 7.1 months (interquartile range [IQR] = 3.5,21.3). The estimated incidence of tracheostomy in trisomy 21 patients among our tracheostomy population was 1.7% (20/1173) over 10 years. The most common indications were airway obstruction (AO) (55%), cardiac/pulmonary respiratory failure (CRF) (25%), or both (20%). Overall mortality was 30%, much lower among AO patients (9%) than CRF (40%) or both (60%), (P = 0.029). Nine patients (45%) were successfully decannulated, with median duration of cannulation of 2.2 years (IQR = 1.7,3). This study suggests a rate of tracheostomy in the pediatric trisomy 21 population approximately 3 times that of the general pediatric population. Over half in this cohort underwent tracheostomy for isolated AO, while the general pediatric tracheostomy population demonstrates a much higher prevalence of prematurity-related CRF. Overall mortality rate and decannulation rate approximated that of the general pediatric tracheostomy population, although outcomes were significantly poorer among patients trisomy 21 patients undergoing tracheostomy for CRF.Pubmed PDF Web
Kevin Bachrach, Ashank Bains, Jennifer N. Shehan, Jessica R. Levi
Publication date 10-12-2020
Trisomy 21 is the most common chromosomal abnormality in the United States with an incidence of 1 in 700 in live-born children. Children with trisomy 21 are at increased risk for chronic ear disease, as well as other medical issues that may complicate healthcare access. In this study, we investigate whether children with trisomy 21 experience delays in tympanostomy tube insertion, and if there are any factors that contribute to a delay in care. We performed a retrospective analysis on patients with trisomy 21 undergoing tympanostomy tube insertion at Boston Medical Center between 2005 and 2019 by analyzing the electronic medical record. This cohort was matched to patients without trisomy 21 by age, gender, surgery date and surgeon. The primary outcome was time elapsed between surgery decision date and date of tympanostomy tube insertion. A total of 20 patients with trisomy 21 underwent tympanostomy tube insertion at Boston Medical Center between 2005 and 2019. The trisomy 21 cohort was matched to 39 patients in the general population. The average time elapsed from decision to undergo surgery to tympanostomy tube insertion was 43.1 days in the general population and 119.0 days in the trisomy 21 cohort (p < .01). Patients with trisomy 21 experience greater delays in tympanostomy tube insertion than the general population, which may be due to the increased burden of comorbid conditions in people with trisomy 21.Pubmed PDF Web
Alexandra M. Forsyth, P. Ryan Camilon, Lauren Tracy, Jessica R. Levi
Publication date 07-12-2020
To evaluate the prevalence and features of pediatric laryngeal malignancies and to review the demographics, management, and survival of pediatric patients with laryngeal squamous cell carcinoma as compared to adult patients. Patients aged 0 (younger than 1) to 18 with laryngeal malignancy identified in the Surveillance, Epidemiology, and End Results (SEER) database were included from 1973 to 2016 published April 2019. Diagnosis of malignant laryngeal tumor was made using the 3rd edition of the International Classification of Diseases for Oncology (ICDO-3) code: C32.0 Glottis, C32.1 Supraglottis, C32.2Subglottis, andC32.9 Larynx primary site. 23 cases of pediatric laryngeal malignancies were identified. 16 cases were squamous cell carcinomas and 7 were other histologic types. Pediatric laryngeal SCC tended to be diagnosed in adolescence (mean age 14.8 years, range younger than 1 to 18, 82.6% of cases were age 12 and above). Management of laryngeal SCC included no recorded treatment (18.8%), primary radiation (18.8%), primary surgery (12.5%), radiation and surgery (25%), radiation and chemotherapy combined (18.8%), and surgery with radiation and chemotherapy (12.5%). Surgeries were local excision, excision and lymphnode dissection, as well as total laryngectomy, and not otherwise specified. The 2-year and 5-year overall and disease-specific survivals were 78.6%. Pediatric laryngeal cancer is rare. Squamous cell carcinoma is the most commonly diagnosed malignant laryngeal histology affecting pediatric patients. Despite different risk factors, survival rates remain similar for pediatric patients with laryngeal squamous cell carcinoma as compared to adults. Physicians should include laryngeal cancer in the differential for pediatric patients with hoarseness, dysphagia, and progressive airway obstruction to avoid a late diagnosis.Pubmed PDF Web
Robert Monfort, Robert Hill, James Sipp
Publication date 05-12-2020
To analyze a case series of adenoidectomy patients who received interoperative ketorolac. To also analyze a case series of adenoidectomy patients who did not receive ketorolac. This is a retrospective chart review, analyzing 429 patients aged 2-18 years of age who underwent adenoidectomy without tonsillectomy. Data collected included patient age at surgery, gender, secondary procedures, medication, post-operative care, post-operative bleeding, and bleeding, and medical history. Statistical analysis was performed using JMP ® Pro, Version 14.0.0. SAS Institute Inc., Cary, NC, 1989-2019. The primary study outcomes were returning to the operating room prior to discharge from hospital or any return to the emergency department within 4 weeks of the surgery with active bleeding. The average age on the day of surgery for those that received ketorolac was 3.35 years and 62% (n = 61) were male. Of the 98 patients who received interoperative ketorolac, none had a bleeding episode. The remaining 331 adenoidectomy patients who did not receive ketorolac also had 0 bleeds. The numbers of patients are insufficient to support an equivalence study. Bleeds after adenoidectomy are scarce, with or without ketorolac, and thus it is difficult to detect an increase in complications. A much larger study would be necessary to generate enough statistical power. There are no findings in this study that pre-empt further investigation into whether ketorolac truly increases perioperative adenoidectomy bleed rates in medically eligible patients.Pubmed PDF Web
Maya Stevens, Christopher J. Mayerl, Laura Bond, Rebecca Z. German, Julie M. Barkmeier-Kraemer
Publication date 15-12-2020
The purpose of this study was to elucidate the pathophysiology of aspiration in previously studied female infant piglets after a unilateral superior laryngeal nerve (uSLN) lesion.
Videofluoroscopic swallow studies (VFSS) were acquired from 15 female piglets ages 2-3 weeks (9 with uSLN lesion and 6 controls). VFSS were analyzed at 30 frames/second sampling rate. Quantitative measures were conducted and compared between groups using published methodologies for VFSS assessment in adult and infant humans.
Measures included the: 1) number of lingual-palatal contacts (LPC) (i.e. pre-swallow), 2) total pharyngeal transit time (TPT), 3) offset of swallow (offP), as well as onset of: 4) pharyngeal stage (onP), 5) pharyngoesophageal segment opening (oPES), 6) maximum PES opening (maxPES), 7) airway closure onset (oAC), and 8) maximum airway closure (maxAC). Measures 5-7 were determined relative to onP. Bolus residue was rated by severity (0 (none) to 3 (severe)). A gamma regression was used to compare continuous measures between lesioned and control groups. The number of LPC (p = .006), TPT (p = .023) and timing of maxAC (p = .041) were significantly greater in the uSLN lesion than the control group. Outcomes of this study replicated prior published findings and elucidated that piglets with right uSLN lesions exhibited delayed maxAC. Noteworthy was the use of clinically relevant quantitative videofluoroscopic measures in piglets for comparison to future studies in human pediatric populations.
Sydney L. Moseley, Christopher Heine, Thomas Valente, Drew Stone, Dylan A. Levy, Jaye Bea Downs, Shaun A. Nguyen, Phayvanh P. Pecha, Clarice Clemmens
Publication date 05-12-2020
To evaluate the overall perioperative times among pediatric patients undergoing adenotonsillectomy (AT) who, in the perioperative period, received either parental presence at induction of anesthesia (PPIA), midazolam, both, or neither.
A retrospective chart review of patients under the age of twelve years who underwent AT during 2018 was performed at a tertiary children's hospital.
Exclusion criteria were: patients with concomitant procedures done at the time of AT, American Society of Anesthesiologists (ASA) score greater than 3, or unclear documentation of PPIA participation.
Patients were categorized into one of four groups: those who received PPIA, those who received midazolam, and those who received both or neither. Time spent in holding, the operating room, and in recovery were recorded. For comparison of continuous variables, either a One-Way ANOVA or a Kruskal-Wallis test was used as appropriate with post-hoc comparisons using a Dunn's Test. A total of 274 patient charts were reviewed. After application of exclusion criteria, 152 charts were included for analysis. A total of 69 patients had PPIA alone, 18 received midazolam alone, 57 received both, and 8 had neither. The median age of the patients was 5.34 years (range 0.62-11.97). There was no significant difference in median time in holding between groups, but there was a significant difference in both OR and recovery time (p = 0.005 and p = 0.021 respectively). On further analysis with post-hoc pairwise comparisons, the only significant difference was between the midazolam only group and the group who received both PPIA and midazolam (p < 0.05), with patients receiving both having a shorter OR duration. Patients who received both PPIA and midazolam had a shorter OR duration than patients in the midazolam only group. There were no significant differences found in holding time or recovery time between groups. This supports the conclusion that PPIA can be implemented when deemed appropriate without increasing time spent in the operating room and may even have a beneficial effect when compounded with midazolam.
Levent Sennaroglu, Munir Demir Bajin
Publication date 03-12-2020
Certain inner ear malformations have stapes footplate fistula which may cause meningitis during otitis media. This may result in fatality. It is the responsibility of the otolaryngologist to diagnose and treat the condition to prevent further attacks of meningitis. Surgical findings of the 17 patients who have inner ear malformations with oval window fistula were retrospectively analyzed. Inner ear malformations were classified according to Sennaroglu classification. Different stages of stapes footplate fistula are classified. Seventeen patients had spontaneous stapes footplate fistula at the oval window. No patient had spontaneous leakage at the round window site. Proper sealing of the leakage area with fascia in a dumbbell fashion is mandatory. Keeping the stapes in place and lumbar drainage are the two most important factors in the successful management of fistula. Particularly important is the simultaneous fistula repair and cochlear implantation where combined postauricular-transcanal approach provides the best method. Vaccination is important but not sufficient to prevent meningitis in inner ear malformations unless repair of the fistula is performed. If the patient has a history of meningitis in the presence of inner ear malformation, particular attention should be given to oval window area to look for an opacity, cyst or a leaking lesion at the stapes footplate. Immediate surgical exploration and repair of the leak is mandatory to prevent further attacks of meningitis. Surgeon should not leave the operation without fully controlling the leak.Pubmed PDF Web
Cher X. Zhao, Natasha D. Dombrowski, Antonio R. Perez-Atayde, Caroline D. Robson, Salim Afshar, Katherine A. Janeway, Reza Rahbar
Publication date 04-12-2020
Pediatric head and neck desmoid tumors are rare neoplasms that can cause significant morbidity due to infiltration of vital anatomic structures. The goal of this study is to review presentation, evaluation, and management of these tumors. Retrospective study of children with head and neck desmoid tumors treated from 1999 to 2018 and literature review. 11 patients (5 boys, 6 girls) were included. Presentation included firm neck mass (n = 8), trismus (n = 2) and tongue lesion (n = 1). All patients had preoperative imaging with CT (n = 2), MRI (n = 1) or both (n = 8). Five patients underwent needle biopsy, five had open biopsy and one was diagnosed on pathology from primary excision. Seven patients were treated by primary surgical resection, with positive surgical margins in six cases due to proximity to vital neurovascular structures. None needed chemotherapy, had disease recurrence or progression. Three patients with unresectable disease were treated with chemotherapy. One patient was monitored with imaging without any treatment and did not have disease progression. Follow-up ranged from 6 months to 6 years (median 21 months). Ten patients (7 surgical, 2 chemotherapy, 1 observation) were either disease-free or had stable disease at last follow-up. Pediatric head and neck desmoid tumors, though rare and histologically benign, are locally infiltrative and aggressive. When feasible, surgical treatment results in good disease control despite positive margins. A balance between achieving negative margins and minimizing functional deficits should be considered. Chemotherapy can be successfully utilized in patients where surgery entails a high risk of morbidity and mortality.Pubmed PDF Web
K. Amraei, S. Amirsalari, M. Ajalloueyan
Publication date 20-12-2020
Davide Soloperto, Federica Spinnato, Stefano Di Gioia, Flavia Di Maro, Patrick Pinter, Alfonso Bisceglia, Daniele Marchioni
Publication date 03-12-2020
Subglottic cysts (SGCs) are a rare cause of laryngeal stenosis that mainly seem to affect preterm infants with an intubation history. To review the related literature and compare different management protocols for patients presenting SGCs, as well as briefly report our clinical case treated at Verona University Hospital by Pediatric Airways Team. The articles resulting from a Pub Med and MEDLINE search were analysed and selected using previously established criteria. A systematic review of the selected papers was conducted following PRISMA guidelines. The search yielded 571 related articles; cross-checking of articles led to the identification and exclusion of 239 duplicates. The remaining 332 papers were screened according to previously established eligibility criteria. The final number of selected articles was 13. Well-planned teamwork, with active collaboration between the ENT specialists, pediatricians and anesthesiologists, is the key to achieve multidisciplinary management of patients diagnosed with SGCs. Long-term follow-up is crucial considering the high recurrence rate of this disease.Pubmed PDF Web
Cecilia Nakeva von Mentzer
Publication date 01-12-2020
Mahitab Mohamed Rashad Ghoneim, Mohamed Rashad Ghonim, Ahmed Ali Mohamed Badawy, Ahmed Abdel Khalek Abdel Razek, Montaser Abdel Salam Hafez, Mostafa Sayed Hamad, Mohammed Abdelbadie Salem
Publication date 02-12-2020
To evaluate the add value of combined analysis of high resolution computed tomography (HRCT) temporal bone parameters in accurate prediction of round window (RW) visibility through posterior tympanotomy. a retrospective observational study was held in a tertiary center, conducted on 45 cochlear implant pediatric candidates between January 2017 and September 2019. Candidates were referred for CT temporal bone. They were classified into fully visible RW cases and partially or non-visible RW cases. Radiological measurements were compared between the two groups for prediction of RW visibility separate and in combination. 45 patients were included in the study (26 males (57.8%) and 19 females (42.2%)). Their median age was 4 years (2-12 years). There were 38 (84.4%) fully visible and 7 (15.6%) partially or non-visible RW cases. Kashio posterior line (n:32/3), fascial recess width (FRW) (mean: 4.9 (3.5-7.5)/4.2 (4-4.7) mm) and round window location (RWL) (3 (1-4)/2.8 (1-3)mm) measurements significantly differentiated between the two groups; (P value 0.034, 0.012 & 0.025 respectively). Posterior kashio line and cut off values of ≥4.75 mm of FRW & 2.95 mm of RWL were showed sensitivity 84.2%, 63.2% & 65.8%, and accuracy 80%, 68.9% &68.9% in prediction of RW visibility respectively. Combination of Kashio prediction line with cut off value ≥ 7.45 mm (sum of FRW & RWL) showed P value 0.003 with further improve in the sensitivity and overall accuracy in prediction of RW visibility from 84.2% to 80% up to 91.4% and 88.6% respectively. combined parameters using Kashio line with FRW and RWL increases sensitivity and overall accuracy in prediction of RW visibility rather than single parameter.Pubmed PDF Web
Lifeng Li, Ricardo L. Carrau, Daniel M. Prevedello, Bentao Yang, Nicholas Rowan, Demin Han, Nyall R. London Jr.
Publication date 03-12-2020
Comprehensive quantitative evaluation of the intercarotid artery distance (ICD) in the pediatric population has not been sufficiently explored. This study aims to measure the minimal ICDs at multiple levels of the skull base to assess changes in the ICD during development. Measurement of the ICDs between the paired paraclival, parasellar, and paraclinoid segments of the internal carotid artery (ICA) was performed on coronal MRI from 540 patients ranging from 0 to 17 years old (n = 30 for each age). Comparison of these indices in the very young (0-5 years, Group 1) and young (6-17 years, Group 2) patients, and assessment of the degree of sphenoid sinus pneumatization was employed. The narrowest ICD was located at the paraclinoid ICAs in the vast majority of cases (89.44%). When comparing the ICDs in very young age patients with the ICDs of 17 years old subjects, a statistically significant difference was found at the paraclival (ages 0-5), parasellar (ages 0-2), and paraclinoid (ages 0-4) ICDs (p < 0.05). Comparison of the ICDs between the intergroups (Group 1 and 2) also demonstrated a statistically significant difference (p < 0.0001). Pneumatization of the sphenoid sinus was initially noted to start at 3 years of age, and there were no patients with a non-pneumatized sphenoid sinus identified after 7 years of age in our cohort. Measurement of ICDs at multiple levels provides a valuable reference for EEA procedures in the pediatric population. While the ICD may be largely stable in the pediatric population after 5 years of age, additional anatomic factors may restrict transsphenoidal access in very young patients (0-5 years).Pubmed PDF Web
Joanna Kobosko, Małgorzata Ganc, Paulina Paluch, W. Wiktor Jedrzejczak, Małgorzata Fludra, Henryk Skarzynski
Publication date 04-12-2020
There is little research into the relationship between a deaf child's developmental outcomes and their mother's self-perceived parental role. The aim of this study was to find out whether the different levels of global psychomotor development in young deaf children who had a cochlear implant (CI), or were candidates for a CI, were related to particular family factors: the self-perceived parental role and the family quality of life (FQoL) as gauged by their hearing mothers, as well as the child's sociodemographic and deafness-related factors and the sociodemographic characteristics of the mothers. The study was conducted on a group of 64 children with bilateral severe or profound sensorineural hearing loss (SNHL) who were CI users (36 children) or qualified for cochlear implantation (28 children) and their hearing mothers. The age of the children ranged from 6.5 to 47 months (M = 23.6; SD = 10.1), among whom 55% were girls (n = 35). The mothers of the children were aged 24-48 years (M = 32.7; SD = 5.3). Information was collected via the Children Development Scale (CDS), the Self-Perception of Parental Role (S-PPR), the Family Quality of Life Survey (FQOLS-2006), and an additional information questionnaire which included questions about sociodemographic variables of the child and the mother, as well as questions related to deafness, the CI, and the child's rehabilitation. Based on results of the CDS, the deaf children were divided into two groups: those who had a low level of global psychomotor development (Low global psychomotor development subgroup - LGPD) and others with medium or high scores (Medium/high global psychomotor development subgroup - MHGPD). The mothers of deaf children in the LGPD group assessed their investment in motherhood lower than did mothers from the MHGPD group. The mothers of the LGPD group rated their satisfaction with the child's rehabilitation lower than did mothers from the MHGPD group. The two subgroups did not differ in FQOLS-2006 domains except for community interactions, which were significantly higher in the MHGPD families. Among the determinants of the global psychomotor development of a young deaf child, the most important ones relate to the use of a CI, but maternal investment in parenting is also important. Community interactions of the family with a deaf child are also crucial, as they create a framework for social network and support for the deaf child's optimal development. Any psychological diagnosis should include, apart from psychomotor development of the deaf child, an assessment of how the mother is coping with their parenting. Mothers of deaf children, especially those with LPGD, need additional support in parenting.Pubmed PDF Web
Elisabet Engström, Petter Kallioinen, Cecilia Nakeva von Mentzer, Magnus Lindgren, Birgitta Sahlén, Björn Lyxell, Marianne Ors, Inger Uhlén
Publication date 04-12-2020
The primary aim was to examine how event-related potentials (ERPs) and mismatch negativity (MMN) change and develop over time among children with hearing loss (HL) using hearing aids (HAs) or cochlear implants (CIs). Children with normal hearing (NH) were tested as a reference group. This three-year follow-up study included 13 children with sensorineural HL (SNHL); 7 children using bilateral HAs and 6 children using CIs; and 10 children with NH as a reference group. ERPs were recorded at baseline and after three years. At time for the original study the children were approximately 5-8 years old and at the follow-up study 8-11 years old. ERP recordings and data processing were identical in both sessions. A standard stimulus alternated with five different deviants (gap, intensity, pitch, location and duration), presented in a pseudorandom sequence, thus following the multi-feature paradigm, Optimum-1. MMN was calculated from the average ERP of each deviant minus the standard stimuli. Repeated measures ANOVA was used for the statistical analyses and the results were based on samples within a specific time interval; 80-224 ms. There was a statistically significant difference in the obligatory responses between the NH and HA groups at baseline, but this difference disappeared after three years in our follow-up study. The children with HA also showed a significant difference in mean ERP at baseline compared to follow-up, and significant differences between the deviants at follow-up but not at baseline. This suggests an improvement over time among the children with HAs. On the other hand, the children with CIs did not differ from the NH children at baseline, but after three years their mean ERP was significantly lower compared to both the children with HA and NH, indicating a reduced development of the central auditory system in this age span among the children with CIs. Regarding MMN, there was an interaction between the duration deviant and time for the children with HA, also indicating a possible improvement over time among the HA children. This three-year follow-up study shows neurophysiological differences between children with HL and children with NH. The results suggest a delay in the central auditory processing among the HA children compared to children with NH, but a possible catch-up, over time, and this potential may be worth to be utilized. Regarding the CI children, similar improvement in this age span is missing, meaning there are differences between the subgroups of children with HL, i.e. the children with HAs vs. CIs. The results highlight the importance of distinguishing between subgroups of children with HL in further research.Pubmed PDF Web
Susan Nittrouer, Joanna H. Lowenstein
Publication date 04-12-2020
In spite of recent gains in language development made by children with hearing loss (HL) as a result of improved auditory prostheses and earlier starts to intervention, these children continue to struggle academically at higher grade levels. We hypothesize that one reason for these incongruent outcomes for language and academics may be that the language demands of school escalate as grade level increases, outstripping the language abilities of children with HL. We tested that hypothesis by examining a higher level skill that is essential for success with academic language, the ability to access multiple interpretations for a sentence.
122 children participated at the end of middle school: 56 with normal hearing (NH), 15 with moderate HL who used hearing aids (HAs), and 51 with severe-to-profound HL who used cochlear implants (CIs). Children's abilities to provide more than one interpretation for an ambiguous sentence were assessed. These sentences were ambiguous due either to words having multiple meanings or to syntactic structure that could evoke more than one interpretation. Potential predictors of those abilities were evaluated, including expressive vocabulary, comprehension of syntactic structures, grammaticality judgments, forward digit span, and several audiologic factors. Children with NH performed best, children with CIs performed poorest, and children with HAs performed intermediately to those groups. Children in all groups achieved higher scores on the multiple meanings than on the syntactic structure items. The variables that were associated with performance varied across groups. Audiologic factors did not explain any variability in performance on the ambiguous sentences task for children with HL. The kind of linguistic flexibility needed to consider more than one interpretation for sentences lacking immediate, real-world context is essential to processing academic language. Children with HL - especially those with severe-to-profound HL who required CIs - showed deficits in this skill, which could contribute to their ongoing academic struggles. Continued language support is needed for these children to allow them to acquire the higher level language skills necessary for success through all of their years in school.
Fatemeh Fakhrealizadeh, Amir Human Hoveidaei, Jamshid Eslami, Seyed Basir Hashemi, Armin Hoveidaei, Tayebeh Kazemi, Zohre Nazarpour
Publication date 27-11-2020
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. 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. 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. 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.Pubmed PDF Web
Anniina J. Sakki, Laura K. Mäkinen, Mervi Kanerva, Johanna Nokso-Koivisto
Publication date 26-11-2020
To compare postoperative self-reported recovery results with monopolar tonsillotomy and cold dissection tonsillectomy in children. To evaluate the feasibility of the monopolar technique in tonsillotomy. Children <12 years undergoing tonsillotomy or tonsillectomy between April 2018 and March 2020 who (with a caregiver) were willing to participate in a two-week follow-up formed the study group. They filled in a questionnaire about pain-related outcomes, return to normal activities, weight changes, complications, and length of home care. Altogether 166 patients were recruited; 103 (62%) returned the questionnaire. The first pain-free day with tonsillotomy was day 5 and with tonsillectomy day 11. After tonsillotomy, patients returned to normal activities faster, e.g. they were able to eat normally 6.5 days earlier than tonsillectomy patients. During the first postoperative week weight dropped after tonsillectomy, but not after tonsillotomy. The length of home care was 6 days with tonsillotomy and 10 days with tonsillectomy. The incidence of postoperative hemorrhage (including minor bleedings at home) was 14% after tonsillotomy and 32% after tonsillectomy. Hemorrhages needing interventions were 0% with tonsillotomy and 2% with tonsillectomy. Children operated on with monopolar tonsillotomy recovered faster and had less postoperative hemorrhage than those undergoing tonsillectomy. They were able to return earlier to daycare/school and their caregivers back to work. Recovery results with monopolar tonsillotomy were equal to other tonsillotomy techniques reported in the literature, hence the monopolar technique can be considered an alternative method to perform tonsillotomy.Pubmed PDF Web
Heléne Hjertman, Mathias Hällgren, Elina Mäki-Torkko, Stefan Stenfelt
Publication date 26-11-2020
The aim of this study was to develop and evaluate a Swedish version of the Hearing In Noise Test for Children (HINT-C). 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. 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. A Swedish version of HINT-C was developed and evaluated in children six years and older.Pubmed PDF Web
Emily Su, Joan H. Leung, Randall P. Morton, Louise J. Dickinson, Alain C. Vandal, Nelson B. Balisa, Suzanne C. Purdy
Publication date 30-11-2020
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. 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. 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. 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.Pubmed PDF Web
Merle Boerrigter, Anneke Vermeulen, Henri Marres, Emmanuel Mylanus, Margreet Langereis
Publication date 28-11-2020
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. 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. 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. 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.Pubmed PDF Web
Urara Funakoshi, Syuji Yonekura, Tomohisa Iinuma, Yukiko Arimoto, Atsuko Nakano, Akiko Yamaide, Minako Tomiita, Akira Hoshioka, Daiju Sakurai, Yoshitaka Okamoto
Publication date 24-11-2020
The influence of tonsillectomy on allergic airway diseases is not well known. In the present study, the influence of tonsillectomy on perennial allergic rhinitis (PAR) and bronchial asthma (BA) among pediatric subjects was prospectively investigated. The tonsillectomy (surgery group) and the age-matched non-surgical subjects (control group) were examined and followed prospectively. In addition, immunological analysis was conducted. After in vitro allergen stimulation, the production of a small number of allergen-specific Th2 cells was induced in the tonsillar cells, even in sensitized subjects. Flow cytometry analysis detected more effector regulatory T cells (Tregs) in the tonsils than in peripheral blood. Clinically, after surgery, the PAR and BA symptoms improved in the surgery group but not in the control group. The total IgE in the surgery group was significantly lower than in the control group; after surgery, IgE levels slightly increased but remained lower. The postoperative Dermatophagoides farina (Der f)-specific IgE level increased in the sensitized subjects but not in the non-sensitized subjects. Tonsillectomy did not improve the underlying mechanisms of the allergy, however the decreased risk of infection and upper airway obstruction could lead to improved symptoms of allergic airway diseases.Pubmed PDF Web
Kevin Chorath, Luis Garza, Aina Tarriela, Neil Luu, Karthik Rajasekaran, Alvaro Moreira
Publication date 25-11-2020
Several guidelines and consensus statements have been produced and disseminated for the detection and management of newborn hearing loss. However, to date, the quality and methodologic rigor of these screening and management protocols have not been appraised. To identify and evaluate existing guidelines and consensus statements for the detection and management of neonatal hearing loss. A comprehensive search of EMBASE, MEDLINE/Pub Med, SCOPUS and grey literature sources was conducted until August 2020. The quality of these guidelines was assessed by four independent reviewers using the Appraisal of Guidelines for Research and Evaluation, 2nd edition (AGREE II). Domain scores were considered satisfactory quality if they scored >60%, and intraclass correlation coefficients (ICC) were calculated to assess agreement among the appraisers. Twelve guidelines were assessed for critical evaluation. Only two guidelines were classified as 'high quality', and the remaining were 'average' or 'low quality'. The 'Scope and Purpose' domain achieved the highest mean score (91.3% ± 5.8%), and lowest was 'Rigor of Development' (35.8% ± 19.1%). ICC analysis showed good to very good agreement across all domains (0.63-0.95). These findings highlight the variability in methodologic quality of guidelines and consensus statement for the detection and management of neonatal hearing loss. These results may help to improve the reporting of future guidelines and guide the selection and use of these guidelines in clinical practice.Pubmed PDF Web
Weston L. Niermeyer, David Z. Allen, Charlemagne Kern, Charles Elmaraghy
Publication date 22-11-2020
Orbital abscess and subperiosteal abscess are pathologies which may require surgical treatment in the pediatric patient. Though rare, orbital cerebrospinal fluid (CSF) leak is a serious complication of abscess drainage. This paper presents a unique 5-month-old male with transorbital abscess drainage complicated by CSF leak. An endoscope was used to repair the defect through the orbitotomy incision. There was no evidence of persistent leak at follow-up. The surgical approach likely contributed to the complication, and the otolaryngologist played a key role in the leak repair. This case should serve to raise the awareness regarding considerations to avoid orbital CSF leak.Pubmed PDF Web
Eleni Vergadi, Stylianos Velegrakis, Maria Raissaki, Maria Bitsori, Alexander Karatzanis, Emmanouil Galanakis
Publication date 26-11-2020
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. 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. 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. 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.Pubmed PDF Web
William W. Carroll, Zachary Farhood, David R. White, Krishna G. Patel
Publication date 30-11-2020
To review our experience with pediatric nasal dermoids, and discuss reconstructive options for the nasal dorsum after pediatric nasal dermoid removal. Retrospective review of pediatric nasal dermoid cases from January 1 2005 through October 1 2016. 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). 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.Pubmed PDF Web
Jia Liang, Lin Xiao, Xue Yao Sun, Bin Zou
Publication date 25-11-2020
This study aimed to investigate the characteristics of the wideband absorbance (WBA) of acoustic energy in children (3-7 years old) with otitis media with effusion (OME) under environmental ear-canal air pressure and tympanometric peak pressure and to determine the diagnostic value of the acoustic energy absorption rate (AR) in OME at different frequencies.
A total of 178 children aged 3-7 years were enrolled in the study between April and October 2018.
They were separated into two groups: those with middle ear effusion were placed in the OME group (n = 80; 136 ears), while those without OME (n = 98; 182 ears) were placed in the control group. A WBA test was performed on the children in both groups under environmental ear-canal pressure and tympanometric peak pressure to analyze the changing characteristics of the AR value within the 0.226-8 k Hz range. Under ambient ear-canal air pressure, the AR of the OME group in each frequency band was significantly lower than that of the control group (P < 0.01). Under tympanometric peak pressure, the AR of the OME group in most frequencies was also significantly lower than those of the control group (P < 0.01) except in the 2-2.5 k Hz range. The area under the receiver operating characteristic curve (AUROC) was highest at 0.47-1.
03 k Hz: 0.96 and 0.94 at ambient ear-canal pressure and tympanometric peak pressure, respectively. Of the single frequency points, those at 0.65, 0.67, 0.69, 0.71, and 0.73 k Hz had a higher AUROC value (0.96-0.97) under both ambient ear-canal air pressure and tympanometric peak pressure. The difference in the AUROC values of the two pressure conditions was not statistically significant (P < 0.01). Overall, WBA is an effective method of diagnosing OME in children. The frequency band with the most predictive value of AR for OME is 0.47-1.03 k Hz. Middle ear effusion can be quickly identified by observing AR values in this frequency range, which provides a diagnostic basis for OME.
Hanqing Shang, Mica Glaun, Julina Ongkasuwan
Publication date 22-11-2020
Acute macroglossia and laryngeal edema are rare adverse side effects that can cause life-threatening airway obstruction. We report a case of acute macroglossia that began after initiation of ethosuximide in a 15-year-old female with severe medically refractory epilepsy. Macroglossia worsened over the next two weeks of ethosuximide administration, preventing extubation. Macroglossia and laryngeal edema improved upon ethosuximide wean, and completely resolved after discontinuation. The patient was extubated successfully, with precautionary nasal trumpet placement and dexamethasone administration prior to extubation. In medically complex patients on multiple pharmacologic agents, anti-epileptic drugs should be suspected as a possible cause of acute macroglossia.Pubmed PDF Web
Chang Gun Cho, Hee-Bok Kim, Chul-Kyu Kim, Bo Hae Kim, Yun-Sung Lim, Seok-Won Park, Joo Hyun Park
Publication date 22-11-2020
The objective of this study was to evaluate the effects of allergic rhinitis (AR) on the development, progression, and recovery of acute otitis media (OM) in an animal model and investigate the secondary effects of bacterial infection. BALB/c mice were divided into four groups: AR + OM, AR, OM, and control groups. AR + OM and AR groups were sensitized with ovalbumin (OVA) and alum and then challenged intranasally with OVA. Phosphate-buffered saline (PBS) was administered to the OM and control groups the same number of times. After AR induction, OM was induced by surgical inoculation of non-typeable Haemophilus influenza (NTHi) into the middle ear (ME) cavity of the mice in the AR + OM and OM groups. PBS was injected into the bulla in the AR and control groups. Each group was subdivided into sets of six mice, one for each of the four time points (0, 2, 7, and 10 days post-bacterial inoculation), at which point the mice were euthanized and ME and nasal cavity mucosa were obtained and evaluated. The occurrence of OM and the ME mucosa thickness were evaluated and compared among the four groups. Tissue expression of interleukin (IL)-1β, IL-6, and tumor necrosis factor-α (TNF-α) in infected ME mucosa was assessed by immunohistochemical staining. We also investigated IgE, IL-4, and IL-5 in the nasal mucosa. Most of the ears showed OM on post-inoculation day 2 in both AR + OM and OM groups. In the AR + OM group, 58.3% of ears still had OM on post-inoculation day 10, while only 16.7% of the OM group had OM. The ME mucosa of all groups increased, and the AR + OM group exhibited the thickest mucosa. The OM group showed peak thickness on post-inoculation day 2 and then decreased, whereas the ME mucosa thickness of the AR + OM group continued to increase to day 7. In the OM group, the expression of IL-1β, IL-6, and TNF-α in the ME also increased significantly, peaking on post-inoculation day 2, and then gradually decreased. In the AR + OM group, the expression of these proteins increased until day 7 and then decreased. The IgE and Th2 response (IL-4 and IL-5) cytokines were expressed at higher levels in the AR + OM and AR groups than in the OM and control groups. The inflammatory reaction to NTHi was more intense and lasted longer in the allergic group, which indicates that AR affects the progression and subsequent recovery of acute bacterial OM.Pubmed PDF Web
Lukas Varga, Daniel Danis, Jakub Drsata, Ivica Masindova, Martina Skopkova, Zuzana Slobodova, Viktor Chrobok, Milan Profant, Daniela Gasperikova
Publication date 26-11-2020
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.Pubmed PDF Web
Krupa R. Patel, Evelyn Zablah, Phoebe H. Yager, Christopher J. Hartnick
Publication date 21-11-2020
This paper outlines the use of a global telehealth program to leverage the potential of telehealth to not only 1) preserve the previous progress of our pediatric surgical airway global teaching mission, but also: 2) to provide rapid, international dissemination of information related to care of pediatric COVID-19 patients; 3) to virtually support the attainment of self-sufficiency of our host countries in relation to our teaching mission; and 4) to inspire host countries to be local champions for each other during the COVID-19 crisis.Pubmed PDF Web
Sreeraj Konadath, Revathi Raveendran, Krishna Yeshoda
Publication date 23-11-2020
The study aimed to test effect of training on the perception of speech stress in children with severe to profound hearing impairment. The first objective was to compare the speech stress perception scores of children with hearing impairment and language age-matched children with normal hearing sensitivity after short term prosody training and the second objective was to correlate between the speech stress perception scores and the language age of the children with hearing impairment.
Thirty children with normal hearing (CWNH) in the age range of 3-7 years and thirty children with hearing impairment (CWHI) who had a language age of 3-7 years participated in the study. The authors formulated 24 concrete Malayalam sentences and their picture cognate pairs (stressed and unstressed) for each sentence. The stressed and unstressed version for each sentence was audio recorded separately.
The study was carried out in two phases; phase 1 included the training on acoustic cues of speech stress followed by testing in phase 2. Both the phases were attended by all the participants. There was a significant difference between the CWNH and CWHI wherein, children with hearing impairment had lower speech stress perception scores. However, improvements in speech stress perception were observed in children with hearing impairment after the training phase. Also, a positive correlation between the language age and the perception of speech stress scores in CWHI was found indicating that as the language age increased there was an increase in the speech stress perception scores. The pre and post training speech stress perception scores were significantly different, with higher scores on the post-training test, indicating that the short term speech stress training for the children with hearing impairment was beneficial. The positive strong correlation between stress perception scores and language age shows that, prosody perception and language learning facilitate each other. Thereby, the conventional speech-language and auditory training should expand and include prosody training from an early age for children with hearing impairment.