Iram Ahmad, MD

Abstract

This study aimed to assess the prevalence of cochlear nerve deficiency (CND) in a cohort of pediatric patients with single-sided deafness (SSD). A secondary objective was to investigate trends in intervention and hearing device use in these children.Case series with chart review.Pediatric tertiary care center.Children ages 0 to 21 years with SSD (N=190) who underwent computerized tomography (CT) and/or magnetic resonance imaging (MRI) were included. Diagnostic criteria for SSD included unilateral severe-to-profound sensorineural hearing loss with normal hearing sensitivity in the contralateral ear. Diagnostic criteria for CND included neuroradiologist report of an "aplastic or hypoplastic nerve" on MRI or a "stenotic cochlear aperture" on CT.The prevalence of CND was 42% for children with CT only, 76% for children with MRI only, and 63% for children with both MRI and CT. Of the children with MRI and CT, there was a 90% concordance across imaging modalities. About 36% of children with SSD had hearing devices that routed sound to the normal hearing ear (ie, bone conduction hearing device/contralateral routing of signal), while only 3% received a cochlear implant. Approximately 40% did not have a hearing device. Hearing device wear time averaged 2.9hours per day and did not differ based on cochlear nerve status.There is a high prevalence of CND in children with SSD. Cochlear nerve status should be confirmed via MRI in children with SSD. The limited implementation and use of hearing devices observed for children with SSD reinforce the need for increased support for early and continuous intervention.

View details for DOI 10.1002/ohn.255

View details for PubMedID 36939463

Abstract

OBJECTIVE: To describe national trends in cholesteatoma management.STUDY DESIGN AND SETTING: Retrospective analysis Optum Clinformatics Database from 2003 to 2019.PATIENTS: 16,179 unique adult and pediatric patients who received cholesteatoma surgery.INTERVENTIONS AND MAIN OUTCOME MEASURES: Patients were categorized into three groups by initial surgical modality: canal wall down (CWD), canal wall up (CWU), and tympanoplasty without mastoidectomy (TnoM). Three major comparisons between groups were performed: 1) temporal trends, 2) clinical and sociodemographic determinants, and 3) healthcare use in terms of total costs and incidence of postoperative imaging and subsequent surgery.RESULTS: Overall, 23.2% received initial CWD surgery, 44.3% CWU, and 32.5% TnoM. 1) The incidence of initial CWD surgery decreased (odds ratios [OR]=0.98, 95% confidence intervals [CI] [0.97,0.99]), while CWU increased (OR=1.02, 95% CI [1.01,1.03]), and TnoM remained stable over the study period (OR=0.99, 95% CI [0.98,1.00]). 2) Relative to CWU, TnoM surgery was less likely in adults, patients with prior complications, and non-White patients, while being more likely in patients with higher household income. CWD was more likely than CWU in adults, patients with prior complications, and non-White patients, while income had no effect. 3) Postoperative costs for CWU and CWD were similar. In 2years following initial surgery, postoperative imaging and/or subsequent surgery was performed in 45.48% of CWD, 57.42% of CWU, and 41.62% of TnoM patients.CONCLUSIONS: Incidence of initial CWD surgery decreased and social disparities in cholesteatoma management were observed. Postoperative imaging or second-look surgery were performed in less than 60% of patients with initial CWU surgery and over 40% of patients with initial CWD.

View details for DOI 10.1097/MAO.0000000000003284

View details for PubMedID 34310551

Abstract

Otitis media, a common disease marked by the presence of fluid within the middle ear space, imparts a significant global health and economic burden. Identifying an effusion through the tympanic membrane is critical to diagnostic success but remains challenging due to the inherent limitations of visible light otoscopy and user interpretation. Here we describe a powerful diagnostic approach to otitis media utilizing advancements in otoscopy and machine learning. We developed an otoscope that visualizes middle ear structures and fluid in the shortwave infrared region, holding several advantages over traditional approaches. Images were captured in vivo and then processed by a novel machine learning based algorithm. The model predicts the presence of effusions with greater accuracy than current techniques, offering specificity and sensitivity over 90%. This platform has the potential to reduce costs and resources associated with otitis media, especially as improvements are made in shortwave imaging and machine learning.

View details for DOI 10.1038/s41598-021-91736-9

View details for PubMedID 34131163

Abstract

Lack of ergonomic training and poor ergonomic habits during the operation leads to musculoskeletal pain and affects the surgeon's life outside of work. The objective of the study was to evaluate the severity of ergonomic hazards in the surgical profession across a wide range of surgical subspecialties. We conducted intraoperative observations using Rapid Entire Body Assessment (REBA) score system to identify ergonomic hazards. Additionally, each of the ten surgical subspecialty departments were sent an optional 14 question survey which evaluated ergonomic practice, environmental infrastructure, and prior ergonomic training or education. A total of 91 surgeons received intraoperative observation and were evaluated on the REBA scale with a minimum score of 0 (low ergonomic risk <3) and a maximum score of 10 (high ergonomic risk 8-10). And a total of 389 surgeons received the survey and 167 (43%) surgeons responded. Of the respondents, 69.7% reported suffering from musculoskeletal pain. Furthermore, 54.9% of the surgeons reported suffering from the highest level of pain when standing during surgery, while only 14.4% experienced pain when sitting. Importantly, 47.7% stated the pain impacted their work, while 59.5% reported pain affecting quality of life outside of work. Only 23.8% of surgeons had any prior ergonomic education. Both our subjective and objective data suggest that pain and disability induced by poor ergonomics are widespread among the surgical community and confirm that surgeons rarely receive ergonomic training. Intraoperative observational findings identified that the majority of observed surgeons displayed poor posture, particularly a poor cervical angle and use of ergonomic setups, both of which increase ergonomic risk hazards. This data supports the need for a comprehensive ergonomic interventional program for the surgical team and offers potential targets for future intervention.

View details for DOI 10.1371/journal.pone.0244868

View details for PubMedID 33561117

Abstract

Most of what we know about adaptive immunity has come from inbred mouse studies, using methods that are often difficult or impossible to confirm in humans. In addition, vaccine responses in mice are often poorly predictive of responses to those same vaccines in humans. Here we use human tonsils, readily available lymphoid organs, to develop a functional organotypic system that recapitulates key germinal center features in vitro, including the production of antigen-specific antibodies, somatic hypermutation and affinity maturation, plasmablast differentiation and class-switch recombination. We use this system to define the essential cellular components necessary to produce an influenza vaccine response. We also show that it can be used to evaluate humoral immune responses to two priming antigens, rabies vaccine and an adenovirus-based severe acute respiratory syndrome coronavirus 2 vaccine, and to assess the effects of different adjuvants. This system should prove useful for studying critical mechanisms underlying adaptive immunity in much greater depth than previously possible and to rapidly test vaccine candidates and adjuvants in an entirely human system.

View details for DOI 10.1038/s41591-020-01145-0

View details for PubMedID 33432170

Abstract

Importance: Early detection and intervention of pediatric hearing loss is critical for language development and academic achievement. However, variations in the diagnostic workup and management of pediatric sensorineural hearing loss (SNHL) exist.Objective: To identify patient and clinician factors that are associated with variation in practice on a national level.Design, Setting, and Participants: This cross-sectional study used the Optum claims database to identify 53711 unique children with SNHL between January 1, 2008, and December 31, 2018.Main Outcomes and Measures: National use rates and mean costs for diagnostic modalities (electrocardiogram, cytomegalovirus testing, magnetic resonance imaging, computed tomography, and genetic testing) and interventions (speech-language pathology, billed hearing aid services, and cochlear implant surgery) were reported. The associations of age, sex, SNHL laterality, clinician type, race/ethnicity, and household income with diagnostic workup and intervention use were measured in multivariable analyses.Results: Of 53711 patients, 23735 (44.2%) were girls, 2934 (5.5%) were Asian, 3797 (7.1%) were Black, 5626 (10.5%) were Hispanic, 33441 (62.3%) were White, and the mean (SD) age was 7.3 (5.3) years. Of all patients, 32200 (60.0%) were seen by general otolaryngologists, while 7573 (14.10%) were seen by pediatric otolaryngologists. Diagnostic workup was received by 14647 patients (27.3%), while 13482 (25.1%) received intervention. Use of genetic testing increased (odds ratio, 1.22 per year; 95% CI, 1.20-1.24), whereas use of computed tomography decreased (odds ratio, 0.93 per year; 95% CI, 0.92-0.94) during the study period. After adjusting for relevant covariables, children who were seen by pediatric otolaryngologists and geneticists had the highest odds of receiving workup and intervention. Additionally, racial/ethnic and economic disparities were observed in the use of most modalities of diagnostic workup and intervention for pediatric SNHL.Conclusions and Relevance: This cross-sectional study identified factors associated with disparities in the diagnostic workup and intervention of pediatric SNHL, thus highlighting the need for increased education and standardization in the management of this common sensory disorder.

View details for DOI 10.1001/jamaoto.2020.5030

View details for PubMedID 33377936

Abstract

OBJECTIVE: To introduce a novel intubation technique for difficult pediatric airways.METHODS: This two-provider technique requires a direct laryngoscope and a flexible fiberoptic laryngoscope. One provider performs direct laryngoscopy which allows for introduction of the flexible laryngoscope. The second provider inserts the flexible laryngoscope with the endotracheal tube loaded, through the oropharynx in to the subglottis.RESULTS: We report three pediatric patients that were initially unable to be intubated by conventional methods. We were ultimately able to successfully intubate these patients with difficult airways using our novel technique.CONCLUSIONS: We found that this technique of direct laryngoscopy assisted flexible fiberoptic intubation is a useful adjunct in select pediatric difficult airway patients.

View details for DOI 10.1016/j.ijporl.2020.110232

View details for PubMedID 32896347

Abstract

Sensorineural hearing loss (SNHL) is the most prevalent congenital sensory deficit in children. Information regarding underlying brain microstructure could offer insight into neural development in deaf children and potentially guide therapies that optimize language development. We sought to quantitatively evaluate MRI-based cerebral volume and gray matter microstructure children with SNHL.We conducted a retrospective study of children with SNHL who obtained brain MRI at 3T. The study cohort comprised 63 children with congenital SNHL without known focal brain lesion or structural abnormality (33 males; mean age 5.3years; age range 1 to 11.8years) and 64 age-matched controls without neurological, developmental, or MRI-based brain macrostructure abnormality. An atlas-based analysis was used to extract quantitative volume and median diffusivity (ADC) in the following brain regions: cerebral cortex, thalamus, caudate, putamen, globus pallidus, hippocampus, amygdala, nucleus accumbens, brain stem, and cerebral white matter. SNHL patients were further stratified by severity scores and hearing loss etiology.Children with SNHL showed higher median ADC of the cortex (p=.019), thalamus (p<.001), caudate (p=.005), and brainstem (p=.003) and smaller brainstem volumes (p=.007) compared to controls. Patients with profound bilateral SNHL did not show any significant differences compared to patients with milder bilateral SNHL, but both cohorts independently had smaller brainstem volumes compared to controls. Children with unilateral SNHL showed greater amygdala volumes compared to controls (p=.021), but no differences were found comparing unilateral SNHL to bilateral SNHL. Based on etiology for SNHL, patients with Pendrin mutations showed higher ADC values in the brainstem (p=.029, respectively); patients with Connexin 26 showed higher ADC values in both the thalamus (p<.001) and brainstem (p<.001) compared to controls.SNHL patients showed significant differences in diffusion and volume in brain subregions, with region-specific findings for patients with Connexin 26 and Pendrin mutations. Future longitudinal studies could examine macro- and microstructure changes in children with SNHL over development and potential predictive role for MRI after interventions including cochlear implant outcome.

View details for DOI 10.1016/j.nicl.2020.102328

View details for PubMedID 32622314

Abstract

OBJECTIVE: To systematically review the literature on anti-inflammatory medications for treating pediatric obstructive sleep apnea and perform meta-analysis of the available data.DATA SOURCES: PubMed/MEDLINE and 4 additional databases.REVIEW METHODS: Three authors independently and systematically searched through June 28, 2018, for studies that assessed anti-inflammatory therapy for treatment of pediatric obstructive sleep apnea (OSA). Data were compiled and analyzed using Review Manager 5.3 (Nordic Cochrane Centre).RESULTS: After screening 135 studies, 32 were selected for review with 6 meeting inclusion criteria. In total, 668 patients aged 2 to 5 years met inclusion criteria for meta-analysis. Of these, 5 studies (166 children) that evaluated montelukast alone as treatment for pediatric OSA found a 55% improvement in the apnea-hypopnea index (AHI) (mean [SD] 6.2 [3.1] events/h pretreatment and 2.8 [2.7] events/h posttreatment; mean difference [MD] of -2.7 events/h; 95% confidence interval [CI], -5.6 to 0.3) with improvement in lowest oxygen saturation (LSAT) from 89.5 (6.9) to 92.1 (3.6) (MD, 2.2; 95% CI, 0.5-4.0). Two studies (502 children) observing the effects of montelukast with intranasal corticosteroids on pediatric OSA found a 70% improvement in AHI (4.7 [2.1] events/h pretreatment and 1.4 [1.0] events/h posttreatment; MD of -4.2 events/h; 95% CI, -6.3 to -2.0), with an improvement in LSAT from 87.8 (3.1) to 92.6 (2.2) (MD, 4.8; 95% CI, 4.5-5.1).CONCLUSIONS: Treatment with montelukast and intranasal steroids or montelukast alone is potentially beneficial for short-term management of mild pediatric OSA.

View details for PubMedID 30513051

Abstract

Merlin is the protein product of the NF2 tumor suppressor gene. Germline NF2 mutation leads to neurofibromatosis type 2 (NF2), characterized by multiple intracranial and spinal schwannomas. Patients with NF2 also frequently develop peripheral neuropathies. While the role of merlin in SC neoplasia is well established, its role in SC homeostasis is less defined. Here we explore the role of merlin in SC responses to nerve injury and their ability to support axon regeneration. We performed sciatic nerve crush in wild-type (WT) and in P0Sch39-121 transgenic mice that express a dominant negative Nf2 isoform in SCs. Recovery of nerve function was assessed by measuring mean contact paw area on a pressure pad 7, 21, 60, and 90days following nerve injury and by nerve conduction assays at 90days following injury. After 90days, the nerves were harvested and axon regeneration was quantified stereologically. Myelin ultrastructure was analyzed by electron microscopy. Functional studies showed delayed nerve regeneration in Nf2 mutant mice compared to the WT mice. Delayed neural recovery correlated with a reduced density of regenerated axons and increased endoneurial space in mutants compared to WT mice. Nevertheless, functional and nerve conduction measures ultimately recovered to similar levels in WT and Nf2 mutant mice, while there was a small (17%) reduction in the percent of regenerated axons in the Nf2 mutant mice. The data suggest that merlin function in SCs regulates neural ultrastructure and facilitates neural regeneration, in addition to its role in SC neoplasia.

View details for DOI 10.1016/j.neuroscience.2018.01.054

View details for PubMedID 29408605

View details for PubMedCentralID PMC5841622

Abstract

To share the diagnostic and management challenges created by an extremely rare airway lesion-the subglottic ectopic thymic cyst.Case report and literature review.We review the presentation, management, and clinical course of an infant who presented with a subglottic mass that was histologically confirmed as a thymic cyst. A brief literature review supplements the case presentation Results: We present the third described case of an ectopic thymic cyst presenting as a subglottic mass. The differential diagnosis of subglottic masses in neonates consists primarily of subglottic hemangioma and mucous retention cysts. Otolaryngologists must be prepared for unexpected findings when dealing with critical airways. We compare the presentation and management of our patient with the 2 previously described cases. We propose an embryologic theory for the origin of these rare lesions.An ectopic thymic cyst is a rare and unexpected cause of neonatal stridor. Management of pediatric airway lesions must allow for unexpected findings at the time of diagnostic and therapeutic endoscopy. The appropriate management of subglottic thymic cysts is poorly defined, but close surveillance for recurrence is mandatory.

View details for PubMedID 29291277

Abstract

After nerve injury, Schwann cells (SCs) dedifferentiate, proliferate, and support axon regrowth. If axons fail to regenerate, denervated SCs eventually undergo apoptosis due, in part, to increased expression of the low-affinity neurotrophin receptor, p75(NTR). Merlin is the protein product of the NF2 tumor suppressor gene implicated in SC tumorigenesis. Here we explore the contribution of merlin to SC responses to nerve injury. We find that merlin becomes phosphorylated (growth permissive) in SCs following acute axotomy and following gradual neural degeneration in a deafness model, temporally correlated with increased p75(NTR) expression. p75(NTR) levels are elevated in P0Sch39-121 transgenic mice that harbor an Nf2 mutation in SCs relative to wild-type mice before axotomy and remain elevated for a longer period of time following injury. Replacement of wild-type, but not phospho-mimetic (S518D), merlin isoforms suppresses p75(NTR) expression in primary human schwannoma cultures which otherwise lack functional merlin. Despite elevated levels of p75(NTR), SC apoptosis following axotomy is blunted in P0Sch39-121 mice relative to wild-type mice suggesting that loss of functional merlin contributes to SC resistance to apoptosis. Further, cultured SCs from mice with a tamoxifen-inducible knock-out of Nf2 confirm that SCs lacking functional merlin are less sensitive to p75(NTR)-mediated cell death. Taken together these results point to a model whereby loss of axonal contact following nerve injury results in merlin phosphorylation leading to increased p75(NTR) expression. Further, they demonstrate that merlin facilitates p75(NTR)-mediated apoptosis in SCs helping to explain how neoplastic SCs that lack functional merlin survive long-term in the absence of axonal contact.

View details for DOI 10.1016/j.nbd.2015.05.021

View details for PubMedID 26057084

View details for PubMedCentralID PMC4641051

View details for DOI 10.4300/JGME-07-02-31

View details for PubMedID 26221472

View details for PubMedCentralID PMC4512827

Abstract

Vestibular schwannomas (VSs) arise from Schwann cells (SCs) and result from the loss of function of merlin, the protein product of the NF2 tumor suppressor gene. In contrast to non-neoplastic SCs, VS cells survive long-term in the absence of axons. We find that p75(NTR) is overexpressed in VSs compared with normal nerves, both at the transcript and protein level, similar to the response of non-neoplastic SCs following axotomy. Despite elevated p75(NTR) expression, VS cells are resistant to apoptosis due to treatment with proNGF, a high affinity ligand for p75(NTR) . Furthermore, treatment with proNGF protects VS cells from apoptosis due to c-Jun N-terminal kinase (JNK) inhibition indicating that p75(NTR) promotes VS cell survival. Treatment of VS cells with proNGF activated NF-B while inhibition of JNK with SP600125 or siRNA-mediated knockdown reduced NF-B activity. Significantly, proNGF also activated NF-B in cultures treated with JNK inhibitors. Thus, JNK activity appears to be required for basal levels of NF-B activity but not for proNGF-induced NF-B activity. To confirm that the increase in NF-B activity contributes to the prosurvival effect of proNGF, we infected VS cultures with Ad.IB.SerS32/36A virus, which inhibits NF-B activation. Compared with control virus, Ad.IB.SerS32/36A significantly increased apoptosis including in VS cells treated with proNGF. Thus, in contrast to non-neoplastic SCs, p75(NTR) signaling provides a prosurvival response in VS cells by activating NF-B independent of JNK. Such differences may contribute to the ability of VS cells to survive long-term in the absence of axons.

View details for DOI 10.1002/glia.22709

View details for PubMedID 24976126

View details for PubMedCentralID PMC4150679