bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cochlear Nerve Deficiency: Prevalence and Audiologic Features
Authors: Clemmens Clarice S. , Germiller John A. , Cohn Samuel 
Published: February 2015

ObjectiveCochlear nerve deficiency (CND) is increasingly being recognized as a cause of sensorineural hearing loss. Our objectives were to 1) determine the prevalence of CND in children with unilateral SNHL, and 2) explore the relationship between imaging and audiologic features.MethodIn 115 consecutive children with unilateral SNHL who underwent high resolution MRI, diameter and image intensity of the CN were measured and normalized to the ipsilateral facial nerve (FN). Patients were divided into 2 groups, normal or CND, based on our published criteria (ie, a CN density < FN density, or CN area).ResultsCND was present in 38 of 115 (33%) of patients with unilateral SNHL. In the CND group, a strong correlation was found between PTA and relative CN density (r2 0.55, P <. 0001) and area (r2 0.50, P <. 0001). Cochlear nerve aplasia was always accompanied by severe to profound SNHL. No progression was observed in any patient, except 1 with an ipsilateral EVA. All contralateral ears remained normal. In the normal group (ie, unilateral SNHL without CND), normal variations in nerve size did not correlate with hearing severity.ConclusionCND, once thought rare, is actually a relatively common cause of pediatric unilateral SNHL. Diagnosis is easily made on MRI. A consistent hearing phenotype is observed, whereby hearing levels correlate with nerve size, and remain remarkably stable over years of follow‐up.
Dexmedetomidine and Outcomes after Airway Reconstruction
Authors: McCormick Michael E. , Pena Maria T. , Zalzal George H. , Preciado Diego A. 
Published: February 2015

ObjectiveTo examine the outcomes of children receiving dexmedetomidine (Dex) as a primary sedation agent after single‐stage airway reconstruction.MethodRetrospective analysis of a prospective case cohort protocol using Dex as a primary sedation agent vs ketamine/midazolam/morphine in single‐stage airway reconstruction at a tertiary care children’s hospital between November 2005 and January 2012. Primary outcomes included length of intubation/PICU admission. Secondary outcomes included the need for polypharmacy and complication rates.ResultsForty‐eight patients were included; 28 received Dex sedation. Median age was 18.0 months (2‐131). Age and weight were similar between the groups, as was length of intubation (9 days Dex vs 8 days no‐Dex, P =. 46). There were no statistical differences between lengths of PICU stay after extubation (4 days Dex vs 3.5 days no‐Dex, P =. 83), lengths of stay after extubation (7 days Dex vs 7.5 days no‐Dex, P =. 122), percentage of polypharmacy days (81.6% Dex vs 64.6% no‐Dex, P =. 20), reintubation rates (20% Dex vs 14% no‐Dex, P =. 60), withdrawal rates (46% Dex vs 45% no‐Dex, P =. 92), or rates of airway infection (36% Dex vs 25% no‐Dex, P =. 43).ConclusionDexmedetomidine sedation does not decrease the lengths of intubation or PICU admission, the need for polypharmacy, or the rates of reintubation, withdrawal, and infection after single‐stage airway reconstruction in children. Although surgical success can be expected in greater than 90% of these patients, the optimal postoperative sedation management remains challenging.
Distal Airway Lesions in Mandibular Distraction Patients
Authors: Mulder Corlyne , Rastatter Jeffrey , Patel Pravin , Vicari Frank 
Published: February 2015

Objective1) Determine prevalence and type of synchronous airway lesions (SALs) in patients undergoing mandibular distraction osteogenesis (MDO) at Children’s Memorial Hospital. 2) To determine whether these SALs affect surgical success of MDO in relieving airway obstruction.MethodRestrospective review of patients with micrognathia undergoing MDO and direct laryngoscopy and bronchoscopy (DLB) over a period of 11 years (2001‐2011) at a tertiary care pediatric hospital. Charts were reviewed for demographics, prevalence/type of SALs, success rate for airway improvement for MDO, and coexisting medical and craniofacial conditions.ResultsEighteen patients were found who had undergone both MDO and DLB. The incidence of at least 1 SAL was 77.8%. Types of SALs encountered include laryngeal edema (61.1%), tracheobronchitis (50%), laryngomalacia (38.9%), tracheomalacia (16.7%), bronchomalacia (16.7%), subglottic stenosis (11.1%), pulsatile airway compression (11.1%), posterior laryngeal cleft (0%), and subglottic cysts (0%). 94.4% of MDO patients received tracheostomy, and 88.9% had airway improvement following MDO. Craniofacial anomalies include micrognathia (100%), cleft palate (61.1%), midface hypoplasia (33.3%), craniosynostosis (5.6%), choanal stenosis/atresia (5.6%), pyriform aperture stenosis (5.6%), and macroglossia (0%). Baseline medical characteristics/syndromes include Pierre Robin sequence (61.1%), Nager syndrome (11.1%), Stickler syndrome (11.1%), asthma (11.1%), Goldenhar syndrome (11.1%), arthrogryposis (5.6%), Mobius syndrome (5.6%), and Cornelia de Lange syndrome (5.6%).ConclusionThere is a high rate of SALs in patients undergoing MDO. The most common SALs are laryngeal edema, tracheobronchitis, and laryngomalacia. MDO is highly successful in relieving upper airway obstruction in micrognathic patients despite the high incidence of SALs. DLB should be considered in all patients who require MDO.
DNA Damage in Children with Snoring
Authors: Yoruk Ozgur , Alp Hakan , Yüksel Sancak , Bakan Ebubekir 
Published: February 2015

ObjectiveLearning the levels of oxidative DNA damage in children with obstructive adenotonsillar hypertrophy. We determined the 8‐hydroxy 2‐deoxyguanosine levels in leukocyte DNA and in urine samples as a specific marker of oxidative damage to DNA, and the plasma and urine malondialdehyde levels as an index of lipid peroxidation in children.MethodProspective, randomized controlled study conducted between 2010 and 2011. The study consisted of 30 patients and 25 healthy controls. Patients had snoring and pauses of breathing during sleep. We obtained blood and urine samples from each child to measure 8‐hydroxy 2‐deoxyguanosine and malondialdehyde levels in their initial visit.ResultsA significant difference was observed in leukocyte and urine 8‐hydroxy 2‐deoxyguanosine levels in patients with obstructive adenotonsillar hypertrophy compared to controls (P <. 001 for both). Similarly, a significant difference was observed in plasma and urine malondialdehyde levels in patients with obstructive adenotonsillar hypertrophy compared to controls (P <. 001 for both). In addition, significant correlations were observed between the levels of 8‐hydroxy 2‐deoxyguanosine in leukocyte DNA and plasma malondialdehyde (r = 0.648, P <. 001), and between the levels of urine 8‐hydroxy 2‐deoxyguanosine excretion and urine malondialdehyde (r = 0.588, P <. 001).Conclusion8‐hydroxy 2‐deoxyguanosine in leukocyte DNA and urine levels in children can be useful as an indicator for obstructive adenotonsillar hypertrophy screening.
How to Avoid Malpractice in Forensic ENT Medicine
Authors: Attia Mohamed E. 
Published: February 2015

ObjectiveWith so many cases of medical malpractice referred from police, courts, medico‐legal doctors, and lawyers that need medico‐legal consultation reports from ASCC about their criminal or civil cases, a scientific curriculum or guide in this field is necessary.MethodA forensic ENT specialist who examines 9830 cases over 11 years in the General Department of Criminal Evidence, Arabian Scientific Consultancy Center, in Kuwait and records notes using most recent procedures and apparatuses with total quality means for accurate ENT exam. Cases clinically focus on medico‐legal points, trauma or pathology, the way it was inflicted, and a clinical picture.ResultsForensic ENT medicine studies clinical aspects and medical rules related to ENT. Practice from the medicolegal points of view and preparing to be ready for law application in service of justice and science for the sake of human rights. Differentiation between real or fabricated, recent or old, traumatic, pathological, or malingering reporting about cause, tools, date, cure time and fate, disability and its percentage—clear much new facts in this new specialty practice.ConclusionThe first textbook of its kind in the world, this is a guide for doctors on how to avoid malpractice.
Genetic Assay for Newborn Auditory Screening
Authors: Colby Candice C. , Todd N. Wendell , Lin Xi 
Published: February 2015

Objective1) Perform genetic screening on newborns that fail physiologic hearing screening and determine confirmed positives with hearing loss and false‐positives. 2) Compare physiological screening results to genetic screening results and identify deafness genes. 3) Provide additional data to assist with the development and implementation of a universal protocol.MethodProspective, large‐scale, multi‐institution project examining genetic causes of hearing loss in newborn children at a tertiary‐care academic center and private hospital that has the largest number of births in the country. The primary outcome measure is deafness genes. Data collection began January 2012 and is ongoing.ResultsOur hypothesis is that a majority of newborns confirmed to have hearing impairment based on physiological tests would have identifiable genetic mutations in the currently known 84 deafness genes. This genetic assay technology for identifying genetic abnormalities was tested in patients with known bilateral sensorineural hearing impairment and identified a genetic cause of hearing loss in 26 of 32 (81.3%) patients with usable specimens that were tested. The 2 most commonly identified genetic mutations identified were the SLC26A4 gene, known to encode the pendrin protein, followed by the GJB2 gene, responsible for gap‐junction protein Connexin 26.ConclusionThis DNA‐based genetic hearing screening approach has allowed us to identify genes that are responsible for deafness and may be able to identify children at risk for developing delayed‐onset hearing loss that current screening methods miss. It may have widespread applications in clinical practices beyond hearing screening.
Ibuprofen and Prednisolone after Tonsillectomy in Children
Authors: Rezende Carlos E. , Monteiro Joao T. , Fiorin Luciano , Lima Diego O. , Bertoncello Flavio , Veiga Fernando A. , Rapoport Priscila B. 
Published: February 2015

ObjectiveEvaluate the use of ibuprofen and prednisolone during postoperative of tonsillectomy of pediatric patients.MethodA total of 105 children submitted to tonsillectomy were distributed into 3 groups. The group A used ibuprofen. The group B used prednisolone. The group C didn’t use anti‐inflammatories. The caregivers, during the 5 days after surgery, evaluated descriptively about children pain, alimentation, complications, and the need of symptomatic medication.ResultsThe groups were homogeneous regarding gender and age. There was no difference among the groups in regards to bleeding and the necessity of use of symptomatic medication. There was a faster alimentary progression and pain control among patients who used ibuprofren and prednisolone, and there was no difference between these 2 groups. There was no difference in regard to nausea and vomiting; however, the fever was significantly less present in the group that used ibuprofen.ConclusionThe use of ibuprofen and prednisolone presented safe and effective in controlling postoperative pain, also improving the alimentary acceptance and decreasing the need of rescue medication such as metamizole or dimenidrinate, without raising the complication index.
Is Airway Endoscopy Mandatory in Children Under Three with Obstructive Sleep Apnea?
Authors: Michelson Andrew P. , Hawley Karen A. , Anne Samantha 
Published: February 2015

ObjectiveCure rates for obstructive sleep apnea (OSA) in children <3 range from 0‐35%. Previous studies suggest that these low rates are due to high incidence of synchronous airway lesions (SALs), necessitating intraoperative airway evaluation. This study examines effectiveness of adenotonsillectomy in children <3 and evaluates necessity of laryngoscopy and/or bronchoscopy.MethodRetrospective chart review of children <3 years undergoing tonsillectomy and/or adenoidectomy for OSA from 2005‐2011. Only patients who underwent both pre‐ and postoperative polysomnogram (PSG) were included in analysis. Children with prior airway surgery were excluded. Clinical history, PSG data, intraoperative findings, and SALs were recorded.ResultsA total of 243 charts were reviewed; 48 patients met inclusion criteria. Twenty‐one of 48 were cured (AHI <1 or AHI <5 and minimum SpO2 >92%) of OSA. Patients failing adenotonsillectomy had significantly higher preoperative AHI and lower minimum SpO2 versus cured patients (P <. 001). No significant difference was noted in age, BMI, or incidence of genetic abnormalities between outcome groups. Eight cured and 11 failed patients underwent office laryngoscopy or intraoperative laryngoscopy and/or bronchoscopy. No statistical difference in frequency of airway evaluation or incidences of SALs was found between outcome groups. Flexible laryngoscopy alone could have diagnosed 79% of SALs identified. No surgery was required for lower airway SALs.ConclusionAdenotonsillectomy for OSA in children <3 cured 44% of patients. Failure was associated with higher preoperative AHI and lower minimum SpO2, but not incidence of SALs. No lower airway SALs required surgery and none contributed to failure, suggesting that office laryngoscopy is sufficient when intraoperative evaluation is not otherwise needed.
Management of the Chronically Inflamed Pediatric Airway
Authors: Smith Nicholas , Boesch Paul , Germann Janet , Rutter Michael J. , de Alarcon Alessandro 
Published: February 2015

ObjectiveTo examine our initial experience with the use of azithromycin in children with airway stenosis and chronically inflamed airways.MethodThis retrospective study examined 20 children with airway stenosis and chronically inflamed airways. Patients tested negative for eosinophilic esophagitis and gastroesophageal reflux and took azithromycin (10 mg/kg) 3 times per week for at least a 1‐month period. Outcomes were status of airway inflammation after azithromycin and decision to intervene on airway stenosis.ResultsThe mean age was 3.6 years (range 0.9‐8.0) with 13 females and 7 males. Diagnoses included subglottic stenosis (85%), tracheal stenosis (5%), bronchial stenosis (5%), and bilateral vocal cord paralysis (5%). Mean duration of azithromycin therapy was 116.6 days (range 42‐192). Airway inflammation improved in 14 patients (70%) allowing successful airway intervention in 12 patients. Two did not undergo surgery due to reasons other than airway inflammation. Six patients had persistent inflammation, 5 of whom are currently being observed.ConclusionChildren with airway stenosis and chronic inflammation pose significant challenges for the airway surgeon. Our initial experience suggests that azithromycin can reduce airway inflammation, allowing one to proceed with surgical intervention. Further studies evaluating azithromycin are warranted.
Pharyngeal Dysphagia in Children with Down Syndrome
Authors: O’Neill Ashley C. , Richter Gresham T. 
Published: February 2015

ObjectiveSwallowing problems in children with Down Syndrome (DS) are poorly understood. In particular, longitudinal examination of pharyngeal dysphagia (PD) in this population has not been performed. This study explores PD in a large group of children with DS to assess the incidence, risk factors, and role of intervention on PD.MethodTwenty‐year retrospective chart review; children with DS were identified by ICD‐9 codes. Multidisciplinary charts were longitudinally reviewed for medical and surgical interventions of PD. Patients were excluded if records were incomplete or could not be assessed.ResultsTwo hundred twelve (m = 117, f = 95) children with DS were identified for review. One hundred seventeen (55.2%) patients (m = 67, f = 50) demonstrated PD by video fluoroscopic swallow study (VFSS) with an age range of 0.24 to 24.67 years. The mean age at first diagnosis was 1.69 years. Seventy‐eight patients with PD underwent 1 or more surgeries for upper airway obstruction (UAO). A laryngeal cleft was identified in 3 patients. At the time of the study, PD was unresolved in 97 patients (average age = 6.57 years) and resolved in 20 (average age = 6.72 years). Surgical procedures for UAO had no significant impact on PD.ConclusionThis study suggests that PD is common (greater than 50%) and should be routinely explored in children with DS. Laryngeal clefts and surgical intervention for UAO do not appear to play a significant role in PD for DS patients.
SurgiSIS Myringoplasty: A 10‐Year Study in Children
Authors: D’Eredita Riccardo 
Published: February 2015

ObjectiveSurgiSIS is a bioactive material derived from porcine small intestinal mucosa (SIS). This material promotes early vessel growth, provides scaffolding for the remodeling tissues, and is inexpensive and ready‐to‐use. We evaluated efficacy, safety, and surgery time of SIS myringoplasty, and compared with patients’ temporalis fascia (PTF) repair in children.MethodProspective, 2‐group (SIS and PTF) randomized, blinded study in a tertiary‐care pediatric institution; 404 children with tympanic membrane (TM) repair were randomly assigned to receive SIS or PTF myringoplasty. Primary outcomes were the healing of the TM and surgical time. Ten years’ follow‐up was obtained in all enrolled children.ResultsFour hundred thirty‐two TM (217 in the SIS and 215 in the ATF groups) perforations were treated. There were 212 stable TM closures in the SIS (97.2%) and 204 (94.8%) in the PTF arm. TM closure was higher in the SIS arm. Results were not statistically significant (OR = 0.4, 95% CI 0.12‐1.41). SIS myringoplasty yielded reduced surgical time. No reaction to SIS was encountered.ConclusionSIS myringoplasty is a safe and effective method for TM closure in children with reduced surgical time, as compared to PTF.
The Use of Next‐Generation Sequencing for Hearing Loss
Authors: Black Angela P. , Greinwald John H. , Arumugam Sivakumaran , Kissell Diane , Husami Ammar , Tinkle Brad , Zhang Kejian 
Published: February 2015

ObjectiveRecently, next‐generation sequencing (NGS) technology has emerged as a possible revolutionary force changing the paradigm of molecular diagnosis by offering high‐throughput sequencing. In the present study, we have evaluated the performance of NGS to identify the sequence variants in a panel of 24 genes associated with hereditary hearing loss.MethodThe coding and flanking intronic regions covering 177 kb target sequence were enriched using microdroplet PCR from 8 samples and sequenced in a single lane on Illumina HiSeq 2000 instrument. The quality filtered sequence reads were mapped to the human reference sequence. The sequence variants were evaluated using novel pathogenecity filter.ResultsOf the 1148 variants detected in all 8 samples, 810 (71%) were classified as true variants using our filtering criteria. To validate our strategy, we Sanger sequenced regions of 4/24 genes, accounting for about 25% of all target sequences. Of the 373 variants detected in these 4 genes, we classified 265 variants as potentially true using our filtering criteria. We were able to detect 259 variants found on sequencing, including deletions/insertions up to 22 bp. Our NGS‐based mutation screening strategy showed 99.99% concordance between NGS and Sanger sequencing, resulting in the analytical sensitivity and specificity of 100% and 99.99%, respectively.ConclusionThis study demonstrated that our NGS‐based mutation screening strategy is highly sensitive in detecting sequence variants in the selected hearing loss genes. Therefore, this approach would be a good alternative to current technologies for identifying the multiple genetic causes of hearing loss.
Validity and Responsiveness of VELO: A VPI QoL Instrument
Authors: Skirko Jonathan R. , Weaver Edward M. , Perkins Jonathan A. , Kinter Sara , Eblen Linda E. , Sie Kathleen C. 
Published: February 2015

ObjectiveIn a study of patients with velopharyngeal insufficiency (VPI) diagnosed by pediatric speech pathologists, test the VPI Effects on Life Outcomes (VELO) instrument for criterion validity, construct validity, reliability, and responsiveness (ability to detect change in quality of life).MethodVPI subjects/parents completed the VELO at baseline, 2 weeks later, and 3 months posttreatment. Criterion‐validity tested correlation between VELO score and VPI severity; construct‐validity tested correlation with speech intelligibility. Test‐retest reliability tested intraclass‐correlation between baseline VELO score and 2‐week score. Responsiveness tested mean baseline and posttreatment scores with the paired t test.ResultsParental responses are reported (n = 83); youth responses (obtained in 8+ year‐olds only; n = 24) showed similar results. VPI severity (minimal n = 11, mild n = 31, moderate n = 27, severe n = 14) and speech intelligibility were rated by pediatric speech pathologists. The VELO instrument did not meet criterion validity (Spearman r = −0.21, P =. 07) but did meet construct validity (Spearman r = −0.36, P =. 001). VELO scores demonstrated excellent test‐retest reliability (r = 0.85, P <. 001, n = 40). Treatments included Furlow palatoplasty (n = 20), sphincter pharyngoplasty (n = 14), or an obturator (n = 2) with n = 28 (78%) completing the VELO posttreatment. The VELO showed responsiveness to treatment improving from baseline 54 ± 15 to posttreatment 70 ± 18 (P <. 001).ConclusionVELO provides a VPI specific quality of life instrument that demonstrates construct validity, test‐retest reliability, and responsiveness to change in quality of life with treatment.
Weight Gain Post‐tonsillectomy More Common in Young Children
Authors: Vikani Ami , Ishman Stacey L. , Smith David F. , Boss Emily F. , Benke James R. 
Published: February 2015

ObjectiveAdenotonsillectomy (TA), performed for either obstructive sleep apnea (OSA) or recurrent tonsillitis (RT), has been associated with significant weight gain after surgery. The objective of this study was to look at a large population of children undergoing TA and the demographic factors which may contribute to weight gain.MethodRetrospective chart review of children undergoing TA at an urban tertiary‐academic center between 2008 and 2011. Demographic data (including age, gender, and race), OSA disease severity, pre‐ and 6‐month postoperative normalized body mass index (z scores), and changes in were compared.ResultsOne hundred fifteen children had complete data after TA; 85 of whom underwent surgery for OSA. There were no significant differences between OSA and RT patients in mean age (7.2 ± 4.3 vs 7.3 ± 4.4 years, P =. 955) or gender (38% female vs 50%; P =. 323). However, the OSA patients were more likely to be black (60% vs 33%) than RT patients (P =. 026). Overall, the preoperative BMI z scores increased from 0.98 ± 1.50 to 1.21 ± 1.25 (P =. 0009) with no significant difference by surgical indication (P =. 58). Multiple linear regression analysis found that only age was significantly, and negatively, associated with changes in BMI z scores (P =. 015).ConclusionSimilar to previous studies, children had significant weight gain after TA. This analysis suggests that younger age is a significant predictor of weight gain after surgery. Further investigation of the relationship between weight gain and demographic factors would benefit from prospective evaluation with special attention to the relationship with age.