Dominant negative variants in IKZF2 cause ICHAD syndrome, a new disorder characterised by immunodysregulation, craniofacial anomalies, hearing impairment, athelia and developmental delay.

BACKGROUND: Helios (encoded by IKZF2), a member of the Ikaros family of transcription factors, is a zinc finger protein involved in embryogenesis and immune function. Although predominantly recognised for its role in the development and function of T lymphocytes, particularly the CD4+ regulatory T cells (Tregs), the expression and function of Helios extends beyond the immune system. During embryogenesis, Helios is expressed in a wide range of tissues, making genetic variants that disrupt the function of Helios strong candidates for causing widespread immune-related and developmental abnormalities in humans. METHODS: We performed detailed phenotypic, genomic and functional investigations on two unrelated individuals with a phenotype of immune dysregulation combined with syndromic features including craniofacial differences, sensorineural hearing loss and congenital abnormalities. RESULTS: Genome sequencing revealed de novo heterozygous variants that alter the critical DNA-binding zinc fingers (ZFs) of Helios. Proband 1 had a tandem duplication of ZFs 2 and 3 in the DNA-binding domain of Helios (p.Gly136_Ser191dup) and Proband 2 had a missense variant impacting one of the key residues for specific base recognition and DNA interaction in ZF2 of Helios (p.Gly153Arg). Functional studies confirmed that both these variant proteins are expressed and that they interfere with the ability of the wild-type Helios protein to perform its canonical function-repressing IL2 transcription activity-in a dominant negative manner. CONCLUSION: This study is the first to describe dominant negative IKZF2 variants. These variants cause a novel genetic syndrome characterised by immunodysregulation, craniofacial anomalies, hearing impairment, athelia and developmental delay.

Spontaneous Resolution of Ear Lidding in Newborns: A Prospective Observational Cohort Study.

ABSTRACT: Ear lidding is a cosmetic outer ear shape deformity commonly observed in newborns. Although lidding is considered a benign condition, psychological concerns such as bullying and depression have been observed in older children supporting correction of the condition. Nonsurgical correction of lidding using molding and splinting techniques has become increasingly popular, achieving successful outcomes in the majority of cases. Spontaneous resolution of the condition has also been reported in the literature however there is minimal prospective data available on the natural progression of ear lidding. In our case series of 11 closely followed newborns, we aimed to characterize the natural progression and resolution of lidding. Ten consecutive newborns participated in the observation plan and all 10 had complete spontaneous resolution of lidding within an average of 40 days. One other newborn's parents self-selected to have molding and splinting treatment. These results suggest that cosmetic treatment for less severe cases of ear lidding may be unnecessary as they have the potential to resolve on their own. Future research in this area could include controlled study designs and more work is needed to identify, which infants will require treatment. Our study may provide helpful reassurance to families and physicians that many newborns may see complete resolution of lidding without intervention.

Health-Related Quality of Life Among Young Children With Cochlear Implants and Developmental Disabilities.

OBJECTIVE: The present study examined differences in health-related quality of life (HRQoL) between deaf children with cochlear implants (CI) with and without developmental disabilities (DD) and differences across HRQoL domains within both groups of children. METHODS: Ninety-two parents of children with CI aged 3-7 years participated in this cross-sectional study. Of these children, 43 had DD (i.e., CI-DD group) and 49 had no DD or chronic illness, demonstrating overall typical development (i.e., CI-TD group). Parents of children in both groups completed the KINDL, a generic HRQoL questionnaire. Parents also provided anecdotal comments to open-ended questions, and parent comments were evaluated on a CI benefits scale to assess parent-perceived benefits of CI for the deaf children with and without disabilities. RESULTS: Children in the CI-DD group had significantly lower HRQoL compared to children in the CI-TD group, including lower scores on the self-esteem, friend, school, and family HRQoL subscales. No significant differences among groups were found on the physical well-being and emotional well-being subscales. For the CI-TD group, age at implantation correlated negatively with self-esteem and school HRQoL subscales. In the CI-DD group, children's current age correlated negatively with family and with the total HRQoL scores. Parent anecdotal comments and scores on the CI-benefits scale indicated strong parent perceptions of benefits of implantation for children in both groups. CONCLUSION: Based on parents' proxy report, findings suggest that having DD affects multiple domains of HRQoL among young children with CIs above and beyond that of the CI itself. Parents of deaf children with DD may need greater support through the CI process and follow-up than parents of deaf children without DD.

Symptomatic Congenital Cytomegalovirus Infection Is Underdiagnosed in British Columbia.

Records were reviewed from all infants tested for congenital cytomegalovirus infection in British Columbia, Canada from 2006 to June 2014. Fourteen of 701 infants, or approximately 4.2 per 100,000 live births, had a positive test, indicating that >90% of expected symptomatic congenital cytomegalovirus infection cases were not diagnosed using clinician-initiated testing.

Type IV congenital laryngeal web: Case report and 15 year follow up.

A five day old patient with mild VACTERL syndrome had repair of a type IV congenital laryngeal web with successful decannulation 76 days later. Voice and respiratory outcome is good with follow up 15 years later. This case presents a rare clinical finding of a type IV laryngeal web successfully repaired with a keel and subsequent long term follow up during an era when it was suggested that repair be delayed until 18 months of age at the earliest.

Innate immune control of EBV-infected B cells by invariant natural killer T cells.

Individuals with X-linked lymphoproliferative disease lack invariant natural killer T (iNKT) cells and are exquisitely susceptible to Epstein-Barr virus (EBV) infection. To determine whether iNKT cells recognize or regulate EBV, resting B cells were infected with EBV in the presence or absence of iNKT cells. The depletion of iNKT cells increased both viral titers and the frequency of EBV-infected B cells. However, EBV-infected B cells rapidly lost expression of the iNKT cell receptor ligand CD1d, abrogating iNKT cell recognition. To determine whether induced CD1d expression could restore iNKT recognition in EBV-infected cells, lymphoblastoid cell lines (LCL) were treated with AM580, a synthetic retinoic acid receptor-α agonist that upregulates CD1d expression via the nuclear protein, lymphoid enhancer-binding factor 1 (LEF-1). AM580 significantly reduced LEF-1 association at the CD1d promoter region, induced CD1d expression on LCL, and restored iNKT recognition of LCL. CD1d-expressing LCL elicited interferon γ secretion and cytotoxicity by iNKT cells even in the absence of exogenous antigen, suggesting an endogenous iNKT antigen is expressed during EBV infection. These data indicate that iNKT cells may be important for early, innate control of B cell infection by EBV and that downregulation of CD1d may allow EBV to circumvent iNKT cell-mediated immune recognition.

Esophageal wishbone extraction: not for the chicken-hearted.

OBJECTIVE: Ingestion of foreign bodies in the pediatric population is common and in the majority of cases involves spontaneous passage through the esophagus; however, they can become lodged in spaces of anatomical narrowing. Sharp foreign bodies are of particular concern due to a higher chance of perforation and other complications. The goal of this case report is to describe the safe removal of a chicken wishbone and 3 alternate options in the event that the initial choice was unsuccessful. METHODS: We report the case of a 2-year-old boy who presented to our pediatric tertiary center after unsuccessful endoscopic removal of a chicken wishbone from the esophagus. RESULTS: Radiologically, the wishbone was oriented with the tines pointing up. Endoscopic examination revealed the tips of both tines to be embedded deeply into the lateral walls of the esophageal mucosa. Esophagoscopy and protecting the sharp points of the wishbone were used to successfully extract the intact wishbone. CONCLUSION: Previous techniques have involved cutting the bone; however, in this case, tension was so high that it was felt that cutting the bone would result in perforation. Proper management of such cases requires planning and often multiple strategies.

Cochlear implantation among deaf children with additional disabilities: parental perceptions of benefits, challenges, and service provision.

Although increasing numbers of children with additional disabilities are receiving cochlear implants (CIs), little is known about family perspectives of the benefits and the challenges of cochlear implantation in this pediatric population. This study examines perceptions among parents of deaf children with additional disabilities regarding satisfaction with service provision, benefits, and challenges of the CI process. This was a mixed-methods study, which included a survey and interviews. Twenty-three families of deaf children with additional disabilities participated in this study, and 17 of these parents participated in in-depth interviews regarding their child's experience with the CI, including benefits and challenges. Interviews were analyzed through inductive thematic analysis. Parent-perceived benefits of cochlear implantation included children's improved sound awareness, communication skills, and greater well-being compared to preimplantation status. However, the majority of families felt that they and their children were not receiving enough services. Major challenges included managing funding; coping with limited availability of specialized services, particularly in rural areas; and continuing concerns about the child's communication, social skills, and academic performance. Results suggest that children with additional disabilities benefit from CIs, but they and their families also face unique challenges that professionals should consider when working with these families.

Cranial fasciitis: a rare pediatric non-neoplastic lesion with 14-year follow up.

Cranial fasciitis is a fibroblastic lesion found in the cranium of children three weeks to six years of age. It most commonly manifests as a solitary, rapid growing mass on the scalp with frequent involvement of underlying bone and occasional intracranial expansion. Patients with cranial fasciitis may present with a wide range of associated symptoms. Otologic symptoms such as otalgia, otorrhea, hearing loss and middle ear effusion are not frequently encountered. We present a case of cranial fasciitis with intracranial involvement and associated otologic symptoms in a four year old boy with subsequent follow up 14 years later.

Two-Year Outcomes After Pediatric In-Office Tympanostomy Using Lidocaine/Epinephrine Iontophoresis and an Automated Tube Delivery System.

OBJECTIVE: Evaluate 2-year outcomes after lidocaine/epinephrine iontophoresis and tympanostomy using an automated tube delivery system for pediatric tube placement in-office. STUDY DESIGN: Prospective, single-arm. SETTING: Eighteen otolaryngology practices. METHODS: Children age 6 months to 12 years indicated for tympanostomy were enrolled between October 2017 and February 2019. Local anesthesia of the tympanic membrane was achieved via lidocaine/epinephrine iontophoresis and tympanostomy was completed using an automated tube delivery system (the Tula® System). An additional Lead-In cohort of patients underwent tube placement in the operating room (OR) under general anesthesia using only the tube delivery system. Patients were followed for 2 years or until tube extrusion, whichever occurred first. Otoscopy and tympanometry were performed at 3 weeks, and 6, 12, 18, and 24 months. Tube retention, patency, and safety were evaluated. RESULTS: Tubes were placed in-office for 269 patients (449 ears) and in the OR for 68 patients (131 ears) (mean age, 4.5 years). The median and mean times to tube extrusion for the combined OR and In-Office cohorts were 15.82 (95% confidence interval [CI]: 15.41-19.05) and 16.79 (95% CI: 16.16-17.42) months, respectively. Sequelae included ongoing perforation for 1.9% of ears (11/580) and medial tube displacement for 0.2% (1/580) observed at 18 months. Over a mean follow-up of 14.3 months, 30.3% (176/580) of ears had otorrhea and 14.3% (83/580) had occluded tubes. CONCLUSION: In-office pediatric tympanostomy using lidocaine/epinephrine iontophoresis and automated tube delivery results in tube retention within the ranges described for similar grommet-type tubes and complication rates consistent with traditional tube placement in the OR.

High frequency of otolaryngology/ENT encounters in Canadian primary care despite low medical undergraduate experiences.

Otolaryngology involves the treatment of patients with diseases and disorders of the ear, nose, throat (ENT), and related structures of the head and neck. Many medical students in Canada have limited experiences in ENT and a vast majority of these students go on to pursue a career as primary care physicians. Physicians at a primary care facility classified patient's visits as either being "ENT" related or not, to assess the amount of ENT related concerns they typically encounter. The data was collected separately in the summer and winter months to assess any seasonal variability. One in eight patient encounters presented with an ENT related concern. The percentage of ENT related symptom presentation visits in the pediatric population for both data collection periods (29%) was more than three times that of the adult population (9%). The rate of ENT symptom presentation in both adult and pediatric populations was not affected by seasonality. Primary care physicians will encounter new patients presenting with ENT related concerns quite frequently. This is especially true in the pediatric patient population. Increased ENT medical education is both necessary and essential for undergraduate medical students, residents, and primary care physicians.

L'oto-rhino-laryngologie (ORL) concerne les maladies et les troubles de l'oreille, du nez, de la gorge et des structures connexes de la tête et du cou. De nombreux étudiants au Canada n'ont qu'une expérience limitée de cette spécialité alors que la grande majorité d'entre eux poursuivent une carrière de médecin de soins primaires. Les médecins d'un établissement de soins primaires ont classé les visites des patients afin de déterminer le volume de consultations en lien avec l'ORL. Les données ont été recueillies séparément pendant les mois d'été et d'hiver pour évaluer la variabilité saisonnière. D'après les données, une consultation sur huit était liée à la présence de symptômes ORL. Le pourcentage de consultations chez la population pédiatrique pour les deux périodes de collecte de données (29 %) était plus de trois fois supérieur à celui de la population adulte (9 %). La survenance de symptômes ORL n'était pas affectée par la saisonnalité, ni chez l'une ni chez l'autre. Les médecins de soins primaires voient assez souvent de nouveaux patients présentant des problèmes ORL, particulièrement des enfants. Un renforcement de l'enseignement de la médecine ORL est à la fois nécessaire et essentiel pour les étudiants en médecine de premier cycle, les résidents et les médecins de soins primaires.

Apolipoprotein-mediated lipid antigen presentation in B cells provides a pathway for innate help by NKT cells.

Natural killer T (NKT) cells are innate-like lymphocytes that recognize lipid antigens and have been shown to enhance B-cell activation and antibody production. B cells typically recruit T-cell help by presenting internalized antigens recognized by their surface antigen receptor. Here, we demonstrate a highly efficient means whereby human B cells present lipid antigens to NKT cells, capturing the antigen using apolipoprotein E (apoE) and the low-density lipoprotein receptor (LDL-R). ApoE dramatically enhances B-cell presentation of alpha-galactosylceramide (alphaGalCer), an exogenous CD1d presented antigen, inducing activation of NKT cells and the subsequent activation of B cells. B cells express the LDL-R on activation, and the activation of NKT cells by B cells is completely LDL-R dependent, as shown by blocking experiments and the complete lack of presentation when using apoE2, an isoform of apoE incapable of LDL-R binding. The dependence on apoE and the LDL-R is much more pronounced in B cells than we had previously seen in dendritic cells, which can apparently use alternate pathways of lipid antigen uptake. Thus, B cells use an apolipoprotein-mediated pathway of lipid antigen presentation, which constitutes a form of innate help for B cells by NKT cells.

Mastoid pressure dressing - Current practice amongst Canadian pediatric cochlear implant surgeons.

OBJECTIVES: Mastoid pressure dressing (MPD) has routinely been used following major ear surgery, such as cochlear implant (CI) surgery, to prevent postoperative wound complications. To date, controlled studies have suggested no difference in the incidence of wound complications following MPD use. However, there is a variation in the practice of MPD usage across pediatric CI surgeons. In this study, we aimed to identify the most common type of postoperative dressing management after pediatric cochlear implantation and the factors in the decision-making process for post-surgical care amongst Canadian pediatric CI surgeons. METHODS: Canadian Otolaryngologists who perform pediatric CI surgery were identified (n = 18) and contacted via email to complete a short online questionnaire regarding current post-operative head dressing practice following CI surgery. Descriptive statistics were used to analyze the response data. RESULTS: The participants provided an approximate number of CI's they performed in 2016. 100% of the recipients completed the survey. Approximately 376 CI's were completed in 2016 with an average of 21 CI's per surgeon. 61% of participants routinely used MPDs following surgery justified by reasons such as wound protection, institutional standard of care, and physician's original training practice. CONCLUSION: There is no clear consensus on the use of MPDs amongst Canadian pediatric CI surgeons. Since the current evidence in the literature suggests no difference in wound complication incidence post-surgery with MPD use, a change in postoperative dressing management to non-use in those that employ this practice may be justified. Eliminating the usage of a MPD may also have potential economic benefits. Further prospective controlled studies may be warranted.

Intranasal Dexmedetomidine increases the successful sedation of children with autism for out-patient auditory brainstem response hearing tests.

INTRODUCTION: The auditory brainstem response (ABR) hearing test can be challenging in children with autism spectrum disorder (ASD) due to the inherent behavioral challenges associated with this condition. To attempt to increase our success in sedating ASD patients, we added the use of intranasal Dexmedetomidine (Dexmed) to be used alone or with oral Chloral Hydrate (CH) in an ambulatory care setting, with monitoring by a specialist nurse. OBJECTIVES: To determine the success and safety of a protocol for ABR testing performed under sedation with intranasal Dexmed and oral chloral hydrate in ASD patients. To compare the success rate, the occurrence of adverse events and time needed to initiate ABR between Dexmed-CH protocol and previous CH-alone protocol in ASD patients. METHODS: Retrospective review. ASD patients in Dexmed-CH sedation protocol were age- and sex-matched to ASD patients who underwent CH-alone sedation protocol, for comparison. RESULTS: 74 ABR records in ASD children were included, 37 patients using Dex-CH protocol and 37 patients using CH-alone protocol. In the Dexmed-CH protocol group, Dexmed was used in 2 different ways: alone as a first choice in patients who refused to swallow CH (9/37), or combined with CH as a rescue (28/37). Under this sedation protocol, 89% of the attempted ABRs were completed successfully with no major adverse effects. In comparison, in ASD patients sedated using the protocol of CH-alone, the success rate significantly lower (69% vs. 89%). The time needed to initiate the test was not significantly different. CONCLUSION: The use of intranasal Dexmed by itself or in combination with CH was a safe and reliable method of performing sedated ABR in ASD patients. Modifying our previous oral CH protocol to include intranasal Dexmed, substantially improved our success rate of sedation in ASD patients in an ambulatory setting. This study may be of significant value to centers worldwide exploring alternatives to general anesthesia for ABR testing in ASD patients.

A national study of choanal atresia in tertiary care centers in Canada - part II: clinical management.

BACKGROUND: To evaluate the clinical management of choanal atresia (CA) in tertiary centers across Canada. METHODS: Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with choanal atresia to a participating center. RESULTS: The health charts of 215 patients (59.6% female) with choanal atresia (CA) were reviewed. Mean age of initial surgical repair was 0.8 months for bilateral CA, and 48.6 months for unilateral CA. Approaches of surgical repair consisted of endoscopic transnasal (31.7%), non-endoscopic transnasal (42.6%), and transpalatal (25.2%). Stents were used on 70.7% of patients. Forty-nine percent of patients were brought back to the OR for a planned second look; stent removal being the most common reason (86.4%). Surgical success rate of initial surgeries was 54.1%. Surgical technique was not associated with rate of restenosis [χ2 (2) = 1.6, p = .46]. CONCLUSIONS: The present study is the first national multi-institutional study exploring the surgical outcomes of CA over a 30-year period. The surgical repair of CA presents a challenge to otolaryngologists, as the rate of surgical failure is high. The optimal surgical approach, age at surgical repair, use of stents, surgical adjuncts, and need for planned second look warrant further investigation.

A national study of choanal atresia in tertiary care centers in Canada - part I: clinical presentation.

BACKGROUND: To evaluate the clinical presentation of choanal atresia (CA) in tertiary centers across Canada. METHODS: Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with CA at a participating center. RESULTS: The health charts of 215 patients (59.6% female) with CA were reviewed and included in this study. The mean age of patients at time of CA presentation was 0.4 months (range 0.1 to 7.2 months) for bilateral CA and 37.8 months (range 0.1 to 164.1 months) for unilateral cases. The most common presenting symptoms for bilateral CA in decreasing order were respiratory distress (96.4%), feeding difficulties (68.2%), and rhinorrhea (65.5%), and for unilateral cases in decreasing order were rhinorrhea (92.0%), feeding difficulties (24.7%), and respiratory distress (18.0%). For the majority of patients (73.2%), the obstruction comprised mixed bony and membranous tissue, with only 10.5% presenting with a purely membranous obstruction. Familial history of CA was confirmed in only 3.3% of cases. One half of patients with CA presented with one or more associated anomalies and 30.6% had a syndrome. CONCLUSIONS: The present investigation is the first national multi-institutional study evaluating the clinical presentation of CA over three decades. The present cohort of CA patients presented with a breadth of co-morbidities with highly variable presentations, with bilateral cases being more severely affected than unilateral cases. Further investigation into hereditary linkages to CA development is warranted.

Wideband reflectance in normal Caucasian and Chinese school-aged children and in children with otitis media with effusion.

OBJECTIVES: Wideband reflectance (WBR) is a middle ear analysis technique that quantifies frequency-specific sound conduction over a wide range of frequencies. One shortcoming of WBR is that there is limited normative data, particularly for pediatric populations and children with middle ear pathology. The goals of this study were to establish normative WBR data for early school-aged children; to determine whether WBR differs significantly between Caucasian and Chinese children, male and female children, and children and adults (experiment 1); and to compare the normative pediatric WBR data with the WBR data obtained from children with abnormal middle ear conditions (experiment 2). DESIGN: WBR was measured from 78 children with normal middle ear status with an average age of 6.15 yrs and 64 children with abnormal middle ear status with an average age of 6.34 yrs. Control group subjects and subjects without previously diagnosed middle ear pathology were recruited from eight elementary schools in the Greater Vancouver Area. Subjects with known middle ear pathology were recruited through the British Columbia Children's Hospital Otolaryngology department. Middle ear effusion (MEE) was identified in one of the two ways. In the British Columbia Children's Hospital group, MEE was diagnosed by a pediatric otolaryngologist (OTL) using pneumatic otoscopy and video otomicroscopy. These cases (21 ears) were classified as OTL confirmed. Subjects who were assessed through screenings at their elementary schools and suspected to have MEE based on audiological test battery results including elevated air conduction thresholds, flat low- and high-frequency tympanograms, and absent transient-evoked otoacoustic emissions were classified as not OTL confirmed (21 ears). Data were statistically analyzed for effects of gender, ethnicity (Caucasian versus Chinese), age (child versus adult), and middle ear condition. WBR equipment used for this study was from Mimosa Acoustics (RMS-system, version 4.03). Data were averaged in one-third octave bands collected from 248 frequencies ranging from 211 to 6000 Hz. RESULTS: Control group subject data (experiment 1) revealed no effects of gender or ear, and their interactions with frequency were not significant. There was a significant interaction between ethnicity (Caucasian versus Chinese) and frequency. Chinese children had lower energy reflectance (ER) values over the mid-frequency range. ER was significantly different between pediatric data and previously collected adult data. Diseased group ER was significantly different among all four middle ear conditions (normal, mild negative middle ear pressure, severe negative middle ear pressure, and MEE) (experiment 2). The overall test performance of ER was objectively evaluated using receiver operating characteristic (ROC) curve analyses; it was compared across frequencies averaged in one-third octave bands. Statistical comparison of the area under ROC (AUROC) plots revealed that ER above 800 Hz (except for ER at 6300 Hz) had better test performance in distinguishing normal middle ear status from MEE compared with ER at 630 and 800 Hz. Although not statistically different from other frequencies between 800 and 5000 Hz, ER at 1250 Hz had the largest AUROC curve (sensitivity of 96% and specificity of 95%) and was selected for further analysis. Comparison of AUROC curves between WBR at 1250 Hz and static admittance at 226-Hz probe tone frequency revealed significantly better test performance for WBR in distinguishing between healthy ears and MEE. CONCLUSIONS: A preliminary set of normative ER data have been generated for a pediatric population between the ages of 5 and 7 yrs, which were significantly different from previously gathered normative adult ER data. In this study, pediatric normative data were warranted for testing children, but ethnic-specific norms were not required to detect middle ear pathology and changes in middle ear status. WBR shows promise as a clinical diagnostic tool for measuring the mechanoacoustic properties of the middle ear and the changes that result in the presence of negative middle ear pressure or MEE.

In-Office Tympanostomy Tube Placement in Children Using Iontophoresis and Automated Tube Delivery.

OBJECTIVES/HYPOTHESIS: Evaluate technical success, tolerability, and safety of lidocaine iontophoresis and tympanostomy tube placement for children in an office setting. STUDY DESIGN: Prospective individual cohort study. METHODS: This prospective multicenter study evaluated in-office tube placement in children ages 6 months through 12 years of age. Anesthesia was achieved via lidocaine/epinephrine iontophoresis. Tube placement was conducted using an integrated and automated myringotomy and tube delivery system. Anxiolytics, sedation, and papoose board were not used. Technical success and safety were evaluated. Patients 5 to 12 years old self-reported tube placement pain using the Faces Pain Scale-Revised (FPS-R) instrument, which ranges from 0 (no pain) to 10 (very much pain). RESULTS: Children were enrolled into three cohorts with 68, 47, and 222 children in the Operating Room (OR) Lead-In, Office Lead-In, and Pivotal cohorts, respectively. In the Pivotal cohort, there were 120 and 102 children in the <5 and 5- to 12-year-old age groups, respectively, with a mean age of 2.3 and 7.6 years, respectively. Bilateral tube placement was indicated for 94.2% of children <5 and 88.2% of children 5 to 12 years old. Tubes were successfully placed in all indicated ears in 85.8% (103/120) of children <5 and 89.2% (91/102) of children 5 to 12 years old. Mean FPS-R score was 3.30 (standard deviation [SD] = 3.39) for tube placement and 1.69 (SD = 2.43) at 5 minutes postprocedure. There were no serious adverse events. Nonserious adverse events occurred at rates similar to standard tympanostomy procedures. CONCLUSIONS: In-office tube placement in selected patients can be successfully achieved without requiring sedatives, anxiolytics, or papoose restraints via lidocaine iontophoresis local anesthesia and an automated myringotomy and tube delivery system. LEVEL OF EVIDENCE: 2b Laryngoscope, 130:S1-S9, 2020.

Subjective and objective vestibular changes that occur following paediatric cochlear implantation: systematic review and meta-analysis.

OBJECTIVE: Cochlear implantation can result in post-operative vestibular dysfunction of unknown clinical significance. The objective of this study was to characterize the presence, magnitude, and clinical significance of vestibular dysfunction that occurs after pediatric cochlear implantation. DATA SOURCES: The databases Embase, Medline (OvidSP), and PubMed were used. Only articles published in English were included. Grey literature and unpublished sources were also reviewed. STUDY SELECTION: Articles published from 1980 until the present which documented pre-operative and post-operative vestibular testing on children under the age of 18 were used. DATA EXTRACTION: Parameters that were assessed included number of patients, pre- and post-operative vestibular-evoked myogenic potentials (VEMPs), head impulse testing (HIT), calorics, and posturography, timing of pre- and post-operative testing, symptomatology, and other demographic data such as etiology of the hearing loss. DATA SYNTHESIS: Ten articles were included. Relative risk values evaluating the effect of cochlear implantation on vestibular function were calculated for VEMPs and caloric testing due to the availability of published data. I2 values were calculated and 95% confidence intervals were reported. Separate analyses were conducted for each individual study and a pooled analysis was conducted to yield an overall relative risk. Assessment on risk of bias in individual studies and overall was performed. CONCLUSION: Pediatric cochlear implantation is associated with a statistically significant decrease in VEMP responses post-operatively (RR 1.8, p < 0.001, I2 91.86, 95%CI 1.57-2.02). Similar results are not seen in caloric testing. Insufficient data is available for analysis of HIT and posturography. Further studies are necessary to determine the effect of cochlear implantation on objective vestibular measures post-operatively and whether any changes seen are clinically relevant in this population.