Search

Sensorineural hearing loss has been identified in several types of muscular dystrophy, but few studies have investigated any relationship between Duchenne muscular dystrophy and hearing. An animal model of Duchenne muscular dystrophy, the mdx mouse, exhibits the same genetic defect as humans. We performed brainstem auditory evoked responses on mdx and control mice in order to assess sensorineural hearing loss. The amplitude and latency of wave I for each animal were measured at increasing sound pressure levels. A significant increase in threshold and a decrease in wave I amplitude were found in the mdx mice. These results indicate that significant sensorineural hearing loss is associated with muscular dystrophy in the mdx mouse. Possible cellular mechanisms contributing to the hearing deficit are presented. [ABSTRACT FROM AUTHOR]

The article discusses the author's first sighting of a Ross' goose in Connecticut on December 11, 2006. At Long Island Sound in Connecticut, the author saw a single Ross' goose along with a flock of nine Canada geese. He observed the bird in terms of plumage, shape, size, and behavior. On December 16, 2006, a single Ross' goose with a small flock of Canada geese were also seen by Penny Solum and Luke Tiller at the Burying Hill Beach in Westport, Connecticut.

Presents updates on pediatric nursing as of April 2004. Assessment documentation for children with diabetes; Development of a visiting policy for a children's unit in a district general hospital; Information on a parent information leaflet on pain management.

Objective: To identify if the addition of supplementary material, such as video or written resources, to the consent process, can improve a patient's or guardian's health literacy in pediatric otolaryngology., Study Design: Prospective randomized crossover design., Setting: Tertiary Academic Center., Methods: From April 18, 2022 to August 29, 2023, 151 children scheduled to undergo 1 of 6 procedures by the same provider were queried and completed a 6-question baseline test based on the information. They each watched a 2-minute video and read a written summary about the procedure; the order of resources was randomized. They answered the same 6-questions after viewing each resource. All tests were scored based on accuracy using an ordinal scale of 1 to 6. Resource preference was collected. Wilcoxon signed-rank tests were run to analyze differences in scores after the addition of supplementary resources and logistic regression modeling was run to analyze demographic effects on postresource score differences., Results: Of 151 participants, 74.2% were guardians, with 78.8% having completed a high school or greater education. The Wilcoxon signed-rank test indicated that postresource scores were statistically significantly higher (P < .001) than pretest scores. Logistic regression modeling showed that participants were less likely to show score improved if they were younger than 18 and were of white race. A majority (87.4%) preferred the addition of a video to the consent process., Conclusion: The addition of video or written resources significantly improves understanding of elective procedures. The development of procedure-specific resources can supplement the consent process and ensure decision-makers have adequate health literacy for informed decision-making., (© 2024 American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

Background: The International Consortium of Health Outcome Measurements (ICHOM) standard set for cleft care appraisal recommends clinicians assess articulation with percentage consonants correct (PCC) and velopharyngeal function with velopharyngeal competency rating (VPC-R). This study explores the utility and limitations of these generic measures in detecting cleft speech sound disorders by comparing them with two cleft-specific speech-rating systems, cleft audit protocol of speech-augmented Americleft modification (CAPS-A-AM) and Pittsburgh weighted speech scale (PWSS)., Methods: Consecutive children with repaired, nonsyndromic cleft lip/palate, aged 5 years or older (n = 27) underwent prospective speech evaluations conducted at a single academic institution. These evaluations were conducted, recorded, and evaluated by blinded speech-language pathologists experienced with all tools., Results: When comparing measures of articulation, PCC scores correlated better with scores for relevant subcomponents of CAPS-A-AM than PWSS. When comparing measures of velopharyngeal function, VPC-R scores correlated well with relevant components of both scales. Using a "screening test versus diagnostic test" analogy, VPC-R ratings were 87.5% sensitive and 73.7% specific for detecting velopharyngeal dysfunction according to subcomponents of CAPS-A-AM, and 70.6% sensitive and 100% specific according to subcomponents of PWSS., Conclusions: This exploratory study demonstrates that PCC and VPC-R perform moderately well in detecting articulatory and velopharyngeal dysfunction in patients with cleft lip/palate; however, these tools cannot describe nuances of cleft speech sound disorder. Thus, although PCC and VPC-R adequately track basic minimum outcomes, we encourage teams to consider extending the standard set by adopting a cleft-specific measurement system for further evaluation of the tools., Competing Interests: The authors have no financial interest to declare in relation to the content of this article. Del Risco was supported by a National Institutes of Health R25 grant (1R25DC020172-01)., (Copyright © 2024 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons.)

Objective: Children with cleft palate are at increased risk for Eustachian tube dysfunction (ETD) and conductive hearing loss from chronic otitis media. While it has been proposed that the severity of ETD is related to the severity of cleft palate, data are lacking to support this hypothesis. An improved understanding of the relevance of cleft width may have prognostic value that could inform decisions on the timing of tympanostomy tube placement and choice of tympanostomy tube design. The objective of this study was to assess severity of ETD in children with narrow, moderate, and wide cleft palate, with examination of hearing outcomes, number of tympanostomy procedures, and incidence of otologic complications., Methods: Retrospective chart review was conducted on 58 patients with primary palatoplasty performed at a single academic medical center from January 1, 2016-December 31, 2019. The primary outcome was the number of otologic procedures performed after the initial palatoplasty. Secondary outcomes included audiometric findings, number of tympanostomy tube placements, presence of effusion at the time of myringotomy, and occurrence of any postoperative otologic complication. Outcomes were compared for patients with narrow (<10 mm), moderate (10-15 mm), and wide (>15 mm) cleft palate. Analysis included consideration of cleft palatal morphology (Veau I - IV), presence of Robin sequence or syndromes, and risk factors for otitis media., Results: Patients with moderate and wide cleft palate underwent higher mean numbers of otologic procedures [narrow: 1.3 (95% confidence interval [CI] 0.9, 1.7); moderate: 1.6 (95% CI 1.1, 2.1); wide: 1.8 (95% CI 1.2, 2.4)]. Moderate and wide cleft palate were less likely to have normal hearing after their first tympanostomy (narrow: 50%, 10/20; moderate: 25%, 6/24; wide: 36%, 5/14). Patients with a wide cleft palate had a shorter median time between first and second tympanostomy procedures (median, IQR; narrow: 27.0, 20.8-35.7; moderate 20.4, 16.3-25.9; wide 17.3, 11.5-23.4)., Conclusion: Our findings suggest that patients with wider cleft palate may be more susceptible to severe ETD. Further large-scale study may help to allow for more informed and personalized clinical decision making for management of cleft palate, incorporating cleft width for prognosis of risks for persistent middle ear dysfunction., Competing Interests: Declaration of competing interest No conflict., (Copyright © 2023. Published by Elsevier B.V.)

Introduction: Congenital CMV (cCMV) is the leading cause of non-genetic sensorineural hearing loss. Babies with cCMV can present with hearing loss any time but failing the initial hearing screen should trigger cCMV testing. cCMV must be identified within 3 weeks after birth to differentiate congenital from acquired CMV, yet follow-up hearing screens may not occur until after 21 days. A new electronic health record protocol to test cCMV in babies who fail their initial hearing screen was established at our institution in 2013. The purpose of this study is to evaluate adherence and deviations from this protocol., Methods: All term infants born in 2013-2016 who failed initial hearing screen were included. The records were reviewed retrospectively. Demographic data, dates of hearing screens, CMV testing results and follow-up hearing test results were collected., Results: A total of 19,069 newborn babies were screened between 2013 and 2016. Babies who were in the neonatal intensive care unit whether premature or not were excluded as these infants are often in the hospital longer than 3 weeks so audiologic diagnostic testing may be delayed. Among term newborns screened, 1358 failed initial screen and 444 failed subsequent hearing testing prior to discharge. We identified 60 babies who did not follow up and 59 underwent additional audiologic testing. Overall 38 babies were tested for cCMV with 2 positives. We found an increase in cCMV testing over time and a significant decrease between physical distance from birth hospital and outpatient audiologic follow-up testing within 21 days of birth., Discussion: Our results are consistent with a 0.4% rate of cCMV in full-term babies who failed their newborn hearing screen. From 2013 to 2016, more babies received CMV tests, but post-screening follow up was still delayed. Further research is necessary to address factors affecting follow up. Use of electronic health record eased identification of results and improved tracking., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Aims: This study is the first to systematically document histological features of fractures of known age in infants (≦12 months). It has been used to develop a tabulated database specifically to guide histopathologists to age fractures in children considered to have suffered accidental or non-accidental injury (NAI). Currently in the United Kingdom there are insufficient pathologists with experience in histological ageing of fractures to meet the medicolegal need for this examination. This study provides a practical tool that will allow those skilled paediatric and forensic pathologists currently involved in assessing infants for evidence of accidental or non-accidental injury a basis for extending their assessment into this area of unmet need., Methods and Results: One hundred and sixty-nine fractures of known age at death were obtained from 52 anonymised infants over a period of 32 years (1985-2016 inclusive). Sections stained using haematoxylin and eosin (H&E) and Martius scarlet blue (MSB) were used to identify specific histological features and to relate them to fracture age. In 1999 the data were entered into a tabulated database for fractures accumulated between from 1985 to 1998 inclusive. Thereafter cases were added, and at 2-yearly intervals the accumulated data were audited against the previous database and adjustments made., Conclusions: This paper describes the final data set from the 2017 audit. The study was terminated at the end of 2016, as there had been no material changes in the data set for three consecutive audits., (© 2019 Authors. Histopathology Published by John Wiley & Sons Ltd.)

Objective: Approximately 1 to 2 of every 1000 American newborns has hearing loss identified by newborn screening. This study was designed to determine if infant hearing loss is more common in socioeconomically disadvantaged communities., Study Design: In this retrospective study, we analyzed electronic medical record data using geostatistical models., Setting: Infants were residents of Durham County, North Carolina, born in 2 hospitals of the Duke University Health System. This county includes the city of Durham and surrounding suburban and rural communities., Subjects and Methods: Subjects were hearing-screened newborns, born between 2005 and 2016, whose residential address was in Durham County, North Carolina. This was a retrospective study using medical record data. We used Bayesian regression models with smoothing of coordinate date to identify both spatial and nonspatial predictors of infant hearing loss., Results: We identified 19,348 infants from Durham County, of whom 675 had failed initial hearing screening and 191 had hearing loss confirmed on follow-up. Hearing loss was significantly associated with minority race (odds ratio [OR], 2.45; 95% confidence interval, 1.97-3.06), as well as lower gestational age and maternal sexually transmitted infections. We identified significant geographic heterogeneity, with a higher probability of hearing loss in poorer urban neighborhoods (local OR range, 0.59-1.39). Neighborhood disadvantage was a significant predictor of hearing loss, as was high local seroprevalence of cytomegalovirus (CMV) among pregnant women., Conclusions: Urban, low-income neighborhoods have a high prevalence of infant hearing loss compared with more affluent surrounding communities, particularly among minorities. This distribution may be attributable to congenital CMV infection.

Objective: Skeletal survey is a commonly used means of detecting fractures in infants, and is used as a screen in suspected cases of physical abuse. It is recognised that in live infants, a repeat survey some days after a suspected episode of injury will detect more fractures than one taken shortly after the suspected injury, indicating that the latter lacks sensitivity. In infants who die soon after a suspected episode of physical abuse, the managing clinicians do not have the option of a second survey; however there is the opportunity for the microscopic examination of bones removed at autopsy. Increasingly Osteoarticular Pathology at the Manchester University NHS Foundation Trust (MFT) is being sent samples of bones from infants suspected of inflicted injury for histological examination, both from bones with fractures detected at autopsy or skeletal survey and from posterior ribs and long bone metaphyses (sites of significance in assessing for abusive injury) when there is no evidence of fracture on skeletal survey or autopsy. Here we report the results of an audit of the anonymised data from a series of such cases, to establish the sensitivity of skeletal survey (SS) to detect fractures and to define the medico-legal value of submitting bones for histological examination., Methods: This was an audit of skeletal injuries in 38 infants aged <18 months presenting to MFT for specialist histopathological evaluation of suspected non-accidental fractures between January 2011 and June 2017. Histopathological examination was performed on all bones submitted and compared with contact radiography of isolated bones and post-mortem skeletal surveys undertaken by specialist paediatric or musculoskeletal radiologists for the presence of fracture., Results: A total of 318 fractures were detected histologically; of these, 178 (56%) were of the ribs, 119 (37.5%) were of major limb long bones, 10 (3%) were of the skull, and 11 (3.5%) were recorded as 'other'. Excluding refractures, skeletal survey detected 54% of the fractures recorded histologically. No fractures were detected radiologically that were not seen histologically. Generally, for skeletal survey, detection rates improved with the age of the lesion, and rib fractures were more difficult to detect than long bone fractures. Ribs 5-8 were the most frequently fractured ribs, and metaphyses around the knee accounted for most metaphyseal limb long bone fractures undetected by SS., Conclusion: In infants coming to post-mortem, histopathology is more sensitive than SS for the detection of clinically significant fractures. In children suspected of non-accidental injuries but with negative or equivocal SS, sampling of the anterior and posterior end of ribs 5-8 and the bones around the knee for histological examination could reveal clinically unsuspected fractures and significant evidence of physical abuse. 71% of infants showed evidence of old fractures typical of non-accidental injury., (Copyright © 2018. Published by Elsevier Ltd.)

Background: In February 2015, the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNSF) published the Allergic Rhinitis Clinical Practice Guideline (AR-CPG). The objective of this study was to assess otolaryngologists' perception of the accuracy and adherence to the AR-CPG., Methods: A survey was distributed to fellows of the American Academy of Otolaryngic Allergy and members of the American Academy of Otolaryngology-Head and Neck Surgery Foundation. A total of 601 otolaryngologists responded. The survey evaluated otolaryngologists' demographic data, perception of the accuracy of the guideline, and adherence to the guideline action statements., Results: The majority of respondents were actively practicing (544 [90.5%]), for a duration of 11-30 years (308 [51.2%]), in a private practice setting (387 [64.4%]). The cohort was largely fellowship trained (348 [57.9%]) and had reviewed the guideline (428 [71.2%]). Most respondents perceived the guideline as being correct "a great deal" (295 [69.7%]) and deviated from the guideline "only a little" (302 [71.6%]). High rates of adherence to the strong guideline recommendations were observed. Respondents "always/most of the time" recommended intranasal steroids (581 [97.6%]), and oral antihistamines (439 [74%]) as primary therapy. Otolaryngologists in practice for longer were more likely to deviate from the guideline recommendations by obtaining sinonasal imaging (p = 0.007) and recommending oral leukotriene receptor antagonists as primary therapy (p = 0.0001)., Conclusion: Overall perception of the correctness of and adherence to the AR-CPG was high in this cohort. Targeted education resources should be provided to otolaryngologists in practice for longer in efforts to reduce harmful or unnecessary variations in care., (© 2018 ARS-AAOA, LLC.)

Cardiac involvement is common and may be the presenting or predominant manifestation in a variety of neuromuscular disorders, most notably the inherited muscle disorders, or muscular dystrophies. Cardiac manifestations of the neuromuscular disorders result from pathological involvement of the myocardium and the cardiac conduction system, with resulting cardiomyopathy or rhythm disturbances including supraventricular arrhythmias, life-threatening ventricular arrhythmias, and sudden cardiac death. Many of these neuromuscular disorders are rare and may be unrecognized by even experienced specialists in internal and cardiovascular medicine. Furthermore, the initial cardiac manifestations in these patients are often asymptomatic. The goal of this investigation is to review the scope of cardiac conduction defects and rhythm disturbances in these disorders and to propose some practical recommendations for arrhythmia monitoring and management of these patients., (Copyright © 2017. Published by Elsevier Inc.)

Objectives: Pediatric patient caregivers may prefer to avoid a surgical intervention and request a medical management option for eustachian tube dysfunction (ETD). However, there are limited published data evaluating the efficacy of intranasal fluticasone in the medical management of ETD as an alternative to tympanostomy tube placement. The objectives of this study were to: 1) determine if intranasal fluticasone (INF) prevented tympanostomy tube placement in children with ETD, and 2) describe differences in patient response to INF related to cleft lip and/or palate (CLP) and Down syndrome., Methods: Case series with planned chart review at a Tertiary academic hospital. We reviewed pediatric patients treated with INF for ETD. Inclusion criteria included ETD, no prior intranasal or oral steroid therapy, and no prior tympanostomy tube placement. Outcomes included time-to- tympanostomy tube placement with or without INF and therapy compliance. Kaplan-Meier survival analyses with log-rank tests and Fisher's exact tests were used to examine outcome variables., Results: 676 fulfilled inclusion criteria. 393 (58.7%) were male, and 355 (52.5%) Caucasian with mean age of 27.1 months old. 92 (13.6%) had CLP and 46 (6.8%) had Down Syndrome. 266 (39.4%) received INF, and 202 (88.2%) were compliant at their next visit. 474 (70.1%) had tympanostomy tubes placed. Children treated with INF were less likely to have tympanostomy tubes placed than children not treated (52.6% vs. 81.5%; p < 0.0001). Using survival analyses, INF use was associated with significantly longer mean time-to-tympanostomy tube than no INF use (199.4 vs. 133.7 days; p < 0.0001). INF did not reduce time-to-tympanostomy tube in patients with CLP (p = 0.05) or Down Syndrome (p = 0.27)., Conclusion: INF significantly reduces the number of children requiring tympanostomy tube placement for ETD. The CLP and Down Syndrome anatomical variants may attenuate INF efficacy. Further in vivo characterization of INF action on eustachian tube tissues will help further substantiate these observations., (Copyright © 2017 Elsevier B.V. All rights reserved.)

On October 1, 2015, the United States required use of the Clinical Modification of the International Classification of Diseases, 10th Revision (ICD-10-CM) for diagnostic coding. This primer was written to assist the cleft care community with understanding and use of ICD-10-CM for diagnostic coding related to cleft lip and/or palate (CL/P).

Background: The aesthetic aspects of the cleft lip nasal deformity have been appreciated for over a century, but the functional implications have remained largely underappreciated or misunderstood. This study describes the frequency and severity of nasal obstructive symptoms among children with cleft lip and/or cleft palate, addressing the hypotheses that age, cleft type, and severity are associated with the development of nasal obstructive symptoms., Methods: Children with nonsyndromic cleft lip and/or cleft palate and a comparison group of unaffected children born from 1997 to 2003 were identified through the North Carolina Birth Defects Monitoring Program and birth certificates. Nasal airway obstruction was measured using the validated Nasal Obstruction Symptom Evaluation scale., Results: The survey was completed by parental proxy for 176 children with cleft lip and/or cleft palate and 333 unaffected children. Nasal obstructive symptoms were more frequently reported in cleft lip with cleft palate compared with unaffected children (p < 0.0001); children who had isolated cleft lip with or without alveolus and isolated cleft palate were not statistically different from unaffected children. Patients with unilateral cleft lip with cleft palate were found to be more severely affected than bilateral cases. Nasal obstruction was observed in early childhood, although severity worsened in adolescence., Conclusions: This population-based study reports a high prevalence of nasal obstructive symptoms in children with cleft lip and/or cleft palate based on type and severity of the cleft. The authors encourage cleft teams to consider using this or similar screening methods to identify which children may benefit from functional rhinoplasty., Clinical Question/level of Evidence: Risk, I.