Background: Choanal atresia (CA), a congenital malformation of the posterior nasal apertures, requires a surgical approach to manufacture a patent nasal airway. The study aim was to review outcomes from a 20-year single-centre experience, focusing on the shift toward mucosal flap surgery, including a novel bi-directional mucosal flap technique (BMFT). Methods: A retrospective review was conducted on patients who underwent endoscopic transnasal CA surgery at The Children's Hospital at Westmead between 2000 and 2021. Outcomes of interest included restenosis rates, number of revision operations, and respiratory support in the pre-operative and post-operative setting. Results: Fifty-five patients were included, comprising 24 patients with bilateral CA and 31 patients with unilateral CA, including 18 children with either coloboma, heart disease, atresia of the choanae, retarded growth and mental development, genital anomalies, and ear malformations and hearing loss (CHARGE) (n=10), Treacher Collins (n=3), 22q11.2 deletion syndrome (n=2), Trisomy 21 (n=2) or Prader Willi (n=1) syndromes. Newborns with bilateral CA, who were intubated pre-operatively or had an oral stent in situ, were successfully extubated to room air (n=22) or oxygen via nasal prongs (n=2) post-operatively. The overall restenosis rate was 44% (n=24), although this was higher for bilateral CA (54%, n=13/24) than unilateral CA (36%, n=11/31). The fashioning of mucosal flaps was associated with an odds ratio of 0.29 [95% confidence interval (CI): 0.08-1.09] for developing restenosis, and was associated with a lower mean [+/- standard deviation (SD)] number of revision surgeries (mean 0.5 +/- 1.0 revision procedures, P<0.01). Nasal stenting was associated with the highest number of revision surgeries (mean 2.8 +/- 2.2 revision procedures, P<0.01), followed by patients with bilateral CA (mean 2.3 +/- 2.1 revision procedures, P<0.01). A subset of four patients underwent BMFT, with no reported restenosis or revision procedures. Conclusions: Over two decades, surgical management of CA at The Children's Hospital at Westmead has shifted toward mucosal preservation techniques. The use of mucosal flaps, including the novel BMFT, is associated with promising reductions in restenosis and revision rates. While these findings are encouraging, further prospective studies with larger cohorts are needed to validate the long-term effectiveness of BMFT.

Objectives: Real-time monitoring of cochlear function to predict the loss of residual hearing after cochlear implantation is now possible. Current approaches monitor the cochlear microphonic (CM) during implantation from the electrode at the tip of the implant. A drop in CM response of >30% is associated with poorer hearing outcomes. However, there is prior evidence that CM amplitude can fluctuate in a manner unrelated to hearing trauma, leading to false positives. By monitoring another cochlear response, the auditory nerve neurophonic (ANN), a differentiation between CM drops that result in reduced cochlear output from false positives may be possible. The hypothesis tested in the present work was that ANN/CM ratios measured during a CM drop will increase during drops not associated with postoperative hearing loss. Design: Twenty-eight adult participants with known CM drops during implantation were taken from a larger data set. This contains adult cochlear implant candidates scheduled to receive a Cochlear Nucleus cochlear implant with either the slim-straight or slim-modiolar electrode array with preoperative audiometric low-frequency average thresholds of ≤80 dB HL at 500, 750, and 1000 Hz in the ear to be implanted. Patients were recruited from eight international implant sites. Pure-tone audiometry was measured postoperatively and 4 to 6 weeks after implantation. Electrocochleography was measured during and immediately after the implantation of the array in response to a 500-Hz, 6-msec pure-tone pip at 110 dB HL. Results: The ANN/CM ratio rose during CM drops in 19 of these patients and decreased in 9. At the follow-up timepoint, patients with a decreasing ANN/CM ratio had a median hearing loss of 29.0 dB, significantly worse than the group with increasing ratio at 13.3 dB (p = 0.004). Considering only the change in ANN amplitude during a CM drop led to smaller groups (ANN drop during CM drop N = 17, ANN increasing during CM drop N = 6) due to 5 patients having undetectable ANN during the CM drop. Using the ANN alone also led to as poorer prediction of hearing preservation, with median hearing preservation in the ANN increasing group of 12.9 dB, significantly better than the ANN decreasing group of 25 dB (p = 0.02). The group with a decreasing ANN/CM ratio had maximum CM amplitude immediately after insertion lower than the maximum amplitude reached during insertion (mean maximum postinsertion amplitude of 98% of during-insertion amplitude). In comparison, the ANN/CM ratio increasing group tended to have a larger CM amplitude immediately after insertion (mean maximum CM amplitude postinsertion of 164% of the maximum during-insertion amplitude). Conclusions: These data show that the ANN/CM ratio is a measure that can differentiate between patients with CM drops that lead to a loss of residual hearing and those that do not. The ANN/CM ratio is easily measured and responds rapidly during a CM drop, showing clinical promise for improving current and developing approaches to intraoperative monitoring.

Objective: Timely diagnosis and management of pediatric sensorineural hearing loss (SNHL) is essential for best outcomes. This study evaluated the differences of health access among cochlear implant (CI) centers of countries with different health care systems. Study Design: Retrospective. Setting: Multicenter, tertiary pediatric CI referral center in four different countries: United States (USA), France (FR), Australia (AUS), and Brazil (BR). Patients: Pediatric (age 0-21 years (0-16 years AUS)) patients receiving CI between January 2017 and December 2019. Intervention(s): CI. Main Outcome Measure(s): Primary outcome measures included age at milestones in CI process and age at implantation. Results: There were 1,673 patients who underwent CI surgery at four multinational designated institutions were identified (n = 143 AUS; 153 BRA; 1,158 FR; and 219 USA). Median age at time of hearing loss diagnosis (p < 0.0001), median age at hearing aid (HA) fitting (p < 0.0001), and CI evaluation (p < 0.0001) differed between countries. However, age at CI surgery was not significantly different between countries. Time from diagnosis to HA fitting was impacted by country (p < 0.0001) and language (p = 0.01) but not by private compared with public insurance. There were 512 children with available data regarding onset of hearing loss as prelingual n = 358 (70%) and postlingual n = 154 (30%). In patients with prelingual severe to profound hearing loss, country and race/ethnic origin affected age at diagnosis (p < 0.0001, p = 0.001), HA fitting (p < 0.0001, p < 0.001), candidacy evaluation (p = 0.0001, p = 0.02), and CI surgery (p = 0.0027, p = 0.001). Conclusions: Age at pediatric CI was comparable across the CI centers compared in the four countries. This is encouraging given the effect of neuroplasticity on pediatric CI outcomes, despite different health care expenditures and delivery systems in the four countries. Improvement in care access for certain populations may decrease disparities in pediatric CI access worldwide.

Mycotic aneurysms are rare but potentially catastrophic. We report a case of an innominate artery pseudoaneurysm in a 4-year-old patient that caused a tracheoinnominate fistula requiring tracheoplasty with a costal cartilage graft and a homograft iliac artery replacement of the diseased innominate artery, with a successful outcome.

Viral vectors and lipofection-based gene therapies have dispersion-dependent transduction/transfection profiles that thwart precise targeting. The study describes the development of focused close-field gene electrotransfer (GET) technology, refining spatial control of gene expression. Integration of fluidics for precise delivery of "naked" plasmid deoxyribonucleic acid (DNA) in sucrose carrier within the focused electric field enables negative biasing of near-field conductivity ("conductivity-clamping"-CC), increasing the efficiency of plasma membrane molecular translocation. This enables titratable gene delivery with unprecedently low charge transfer. The clinic-ready bionics-derived CC-GET device achieved neurotrophin-encoding miniplasmid DNA delivery to the cochlea to promote auditory nerve regeneration; validated in deafened guinea pig and cat models, leading to improved central auditory tuning with bionics-based hearing. The performance of CC-GET is evaluated in the brain, an organ problematic for pulsed electric field-based plasmid DNA delivery, due to high required currents causing Joule-heating and damaging electroporation. Here CC-GET enables safe precision targeting of gene expression. In the guinea pig, reporter expression is enabled in physiologically critical brainstem regions, and in the striatum (globus pallidus region) delivery of a red-shifted channelrhodopsin and a genetically-encoded Ca2+ sensor, achieved photoactivated neuromodulation relevant to the treatment of Parkinson's Disease and other focal brain disorders.

INTRODUCTION:Congenital unilateral sensorineural hearing loss (cuSNHL) carries potentially significant social, educational, and developmental consequences. Early diagnosis enables investigation, and consideration of options for management and early intervention, helping to mitigate the effects of hearing loss. Cochlear nerve dysplasia (CND) is a prominent cause of cuSNHL and may affect candidacy for cochlear implantation. Socioeconomic disadvantage may impact on a patient's family's capacity to participate in necessary intervention and follow-up. METHODS:Infants with severe-profound cuSNHL referred to a large Australian quaternary pediatric center between October 2004 and December 2020 were retrospectively included. Audiometric and clinical data, and the presence of hearing loss risk factors were obtained from a prospectively collated database. In Australia MRI scans are provided free-of-charge to citizens and residents. MRI scans were reviewed to determine the status of the nerves within the internal acoustic meatus (IAM grade) along with attendance rates. Travel distance to the hospital was also calculated. Reasons for non-attendance at MRI were obtained from patient medical records and correspondence. Socioeconomic, educational, and occupational indices, and travel distances were obtained using patient residential postcodes with reference to Australian Bureau of Statistics data. RESULTS:A total of 98 patients were reviewed, 64.3% (n = 63) of whom underwent MRI. The median age at diagnosis was 40 days (IQR 27). The prevalence of CND was 75% (n = 47). Importantly, there was no significant difference in the degree of hearing loss between IAM grades (F(4,57) = 1.029, p = 0.405). Socioeconomic indices were significantly lower in patients not attending MRI investigations compared with patients who did attend. Travel distance was not significantly different between the two groups. CONCLUSION:CND is a prominent cause of cuSNHL in Australian infants. MRI at a young age allows parent education regarding management options and timely intervention where indicated. Socioeconomic disadvantage significantly impacts on participation in further routine assessment of cuSNHL, potentially limiting management options for these children long term.

Objective To assess the distance burden for access to cochlear implant (CI)–related services and to assess whether socioeconomic disadvantage or level of education and occupation influenced uptake of CIs. Study Design Retrospective case review. Setting A CI services provider operating across multiple centers. Patients All patients undergoing CI surgery in a 2-year period between March 2018 and February 2020. Intervention(s) Diagnosis of hearing loss, CI surgery, and subsequent habilitation and mapping. Main Outcome Measure(s) Distance traveled by patients to their audiological diagnostic, CI surgery hospital, and habilitation sites; subjects' index of relative socioeconomic advantage and disadvantage (IRSAD) and index of education and occupation (IEO). Results n = 201 children and n = 623 adults. There was a significant difference across IRSAD domains for children ( p < 0.0001) and adults ( p < 0.0001), and IEO in children ( p = 0.015) and adults ( p < 0.0001) when tested for equal proportions. The median driving distance from home to the diagnostic audiological site for children was 20 km (mean, 69 km; range, 1–1184 km; upper quartile, 79 km; lower quartile, 8 km). There was no significant difference between the driving distances from home to the CI surgery hospital site, or the mapping/habilitation sites between children and adults. There was no correlation for age at first surgery and either IRSAD/IEO. Conclusions The burden of distance for access to CI in Australia is significant for the upper quartile who may not live within the large city centers. Greater consideration needs to be given regarding barriers to CI for those in lower socioeconomic and educational groups to ensure equity of access across different socioeconomic and educational level backgrounds.

Purpose To investigate 2-year post-operative hearing performance, safety, and patient-reported outcomes of hearing-impaired adults treated with the Osia ® 2 System, an active osseointegrated bone-conduction hearing implant that uses piezoelectric technology. Methods A prospective, multicenter, open-label, single-arm, within-subject clinical study conducted at three tertiary referral clinical centers located in Melbourne, Sydney and Hong Kong. Twenty adult recipients of the Osia 2 System were enrolled and followed up between 12 and 24 months post-implantation: 17 with mixed or conductive hearing loss and 3 with single-sided sensorineural deafness. Safety data, audiological thresholds, speech recognition thresholds in noise, and patient-reported outcomes were collected and evaluated. In addition, pre-and 6-month post-implantation data were collected retrospectively for this recipient cohort enrolled into the earlier study (ClinicalTrials.gov NCT04041700). Results Between 6- and 24-month follow-up, there was no statistically significant change in free-field hearing thresholds or speech reception thresholds in noise ( p = > 0.05), indicating that aided improvements were maintained up to 24 months of follow-up. Furthermore, improvements in health-related quality of life and daily hearing ability, as well as clinical and subjective measures of hearing benefit remained stable over the 24-month period. No serious adverse events were reported during extended follow-up. Conclusions These study results provide further evidence to support the longer term clinical safety, hearing performance, and patient-related benefits of the Osia 2 System in patients with either a conductive hearing loss, mixed hearing loss, or single-sided sensorineural deafness. Trial Registration ClinicalTrials.gov Identifier: NCT04754477. First posted: February 15, 2021.

Background: This paper presents a cohort study of pinna perichondritis presentations at a rural hospital over a 10-year period, as well as describing a novel approach to the management of pinna abscesses and suppurative perichondritis to prevent cosmetic deformity. A review of the literature with a focus on piercing-associated perichondritis is also presented.Methods: A retrospective analysis of perichondritis presentations to Dubbo Base Hospital between January 2012 and January 2022 was performed and variables of interest collected including demographics, cause of perichondritis, pre-hospital and inpatient management and organism cultured. Statistical analyses were performed using Fisher's exact test for categorical variables and student's t-test for continuous variables.Results: There were 30 cases of perichondritis identified, with a mean age of 35.7 years and 60% were male. Cartilaginous piercing was the most common cause, followed by otitis externa. All cases of piercing-associated pinna abscess grew Pseudomonas aeruginosa (P.aeruginosa) on culture. Indigenous patients had increased rates of P.aeruginosa infection and need for surgical management compared to non-Indigenous patients. Patients were less likely to receive anti-pseudomonal antibiotics in hospital if an abscess was present, despite being more likely to culture P.aeruginosa than other organisms. Older patients were more likely to receive timely and appropriate anti-pseudomonal cover with ciprofloxacin in the community. A novel method of post-drainage primary moulding to minimise cosmetic deformity is described using a finger-cot splint in two patients from this cohort, presented as a case series. Conclusions: This study highlights that in the rural and remote setting cartilaginous piercing is a common risk factor for pinna perichondritis, and P.aeruginosa is frequently implicated in these infections and their subsequent abscess formation. Despite recent recommendations outlining safe use in paediatric patients, ciprofloxacin is not routinely prescribed to patients with this presentation in the community. Our novel method of managing these abscesses is the first of its kind and has been demonstrated to be a cost-effective, readily available and well-tolerated option for the prevention of cosmetic deformity following piercing-associated pinna abscess, with potential application in other causes of pinna collection.

ObjectiveTo investigate ictal nystagmus and audiovestibular characteristics in episodic spontaneous vertigo after cochlear implantation (CI).Study DesignRetrospective and prospective case series.PatientsTwenty-one CI patients with episodic spontaneous vertigo after implantation were recruited.InterventionsPatient-initiated home video-oculography recordings were performed during one or more attacks of vertigo, using miniature portable home video-glasses. To assess canal and otolith function, video head-impulse tests (vHITs) and vestibular-evoked myogenic potential tests were conducted.Main Outcome MeasuresNystagmus slow-phase velocities (SPVs), the presence of horizontal direction-changing nystagmus, and post-CI audiovestibular tests.ResultsMain final diagnoses were post-CI secondary endolymphatic hydrops (48%) and exacerbation of existing Ménière's disease (29%). Symptomatic patients demonstrated high-velocity horizontal ictal-nystagmus (SPV, 44.2°/s and 68.2°/s in post-CI secondary endolymphatic hydrop and Ménière's disease). Direction-changing nystagmus was observed in 80 and 75%. Two were diagnosed with presumed autoimmune inner ear disease (SPV, 6.6°/s and 172.9°/s). One patient was diagnosed with probable vestibular migraine (15.1°/s).VHIT gains were 0.80 ± 0.20 (lateral), 0.70 ± 0.17 (anterior), and 0.62 ± 0.27 (posterior) in the implanted ear, with abnormal values in 33, 35, and 35% of each canal. Bone-conducted cervical and ocular vestibular-evoked myogenic potentials were asymmetric in 52 and 29% of patients (all lateralized to the implanted ear) with mean asymmetry ratios of 51.2 and 35.7%. Reversible reduction in vHIT gain was recorded in three acutely symptomatic patients.ConclusionHigh-velocity, direction-changing nystagmus time-locked with vertigo attacks may be observed in post-CI implant vertigo and may indicate endolymphatic hydrops. Fluctuating vHIT gain may be an additional marker of a recurrent peripheral vestibulopathy.

Objective The primary aim was to analyze the speech perception outcomes of patients with cochlear implants 65 years and older, compared with those younger than 65 years. The secondary aim was to analyze if preoperative hearing levels, severe compared with profound, had an effect on speech perception outcomes in senior citizens. Study Design Retrospective case review of 785 patients, between 2009 and 2016. Setting A large cochlear implant program. Patients Cochlear implant adult recipients younger than 65 years and 65 years and older at the time of surgery. Intervention(s) Therapeutic—cochlear implant. Main Outcome Measure(s) Speech perception outcomes, using City University of New York (CUNY) sentences and Consonant-Nucleus-Consonant (CNC) words. Outcomes were measured preoperatively and postoperatively at 3, 6and 12 months for cohorts younger than 65 years and 65 years and older. Results Adult recipients younger than 65 years compared with those 65 years and older had comparable outcomes for CUNY sentence scores outcomes (p = 0.11) and CNC word scores (p = 0.69). The preoperative four-frequency average severe hearing loss (HL) cohort was significantly better compared with the profound HL cohort, for both the CUNY sentence scores (p < 0.001) and CNC word scores (p < 0.0001). The four-frequency average severe HL cohort had better outcomes irrespective of age. Conclusions Senior citizens have similarly good speech perception outcomes as adults younger than 65 years. Those with preoperative severe HL have better outcomes than profound loss. These finds are reassuring and can be used when counseling older cochlear implant candidates.

Objectives: Electrocochleography (ECochG) is emerging as a tool for monitoring cochlear function during cochlear implant (CI) surgery. ECochG may be recorded directly from electrodes on the implant array intraoperatively. For low-frequency stimulation, its amplitude tends to rise or may plateau as the electrode is inserted. The aim of this study was to explore whether compromise of the ECochG signal, defined as a fall in its amplitude of 30% or more during insertion, whether transient or permanent, is associated with poorer postoperative acoustic hearing, and to examine how preoperative hearing levels may influence the ability to record ECochG. The specific hypotheses tested were threefold: (a) deterioration in the pure-tone average of low-frequency hearing at the first postoperative follow-up interval (follow-up visit 1 [FUV1], 4 to 6 weeks) will be associated with compromise of the cochlear microphonic (CM) amplitude during electrode insertion (primary hypothesis); (b) an association is observed at the second postoperative follow-up interval (FUV2, 3 months) (secondary hypothesis 1); and (c) the CM response will be recorded earlier during electrode array insertion when the preoperative high-frequency hearing is better (secondary hypothesis 2). Design: International, multi-site prospective, observational, between groups design, targeting 41 adult participants in each of two groups, (compromised CM versus preserved CM). Adult CI candidates who were scheduled to receive a Cochlear Nucleus CI with a Slim Straight or a Slim Modiolar electrode array and had a preoperative audiometric low-frequency average thresholds of ≤80 dB HL at 500, 750, and 1000 Hz in the ear to be implanted, were recruited from eight international implant sites. Pure tone audiometry was measured preoperatively and at postoperative visits (FUV1 and follow-up visit 2 [FUV2]). ECochG was measured during and immediately after the implantation of the array. Results: From a total of 78 enrolled individuals (80 ears), 77 participants (79 ears) underwent surgery. Due to protocol deviations, 18 ears (23%) were excluded. Of the 61 ears with ECochG responses, amplitudes were <1 µV throughout implantation for 18 ears (23%) and deemed “unclear” for classification. EcochG responses >1 µV in 43 ears (55%) were stable throughout implantation for 8 ears and compromised in 35 ears. For the primary endpoint at FUV1, 7/41 ears (17%) with preserved CM had a median hearing loss of 12.6 dB versus 34/41 ears (83%) with compromised CM and a median hearing loss of 26.9 dB (p < 0.014). In assessing the practicalities of measuring intraoperative ECochG, the presence of a measurable CM (>1 µV) during implantation was dependent on preoperative, low-frequency thresholds, particularly at the stimulus frequency (0.5 kHz). High-frequency, preoperative thresholds were also associated with a measurable CM > 1 µV during surgery. Conclusions: Our data shows that CM drops occurring during electrode insertion were correlated with significantly poorer hearing preservation postoperatively compared to CMs that remained stable throughout the electrode insertion. The practicality of measuring ECochG in a large cohort is discussed, regarding the suggested optimal preoperative low-frequency hearing levels (<80 dB HL) considered necessary to obtain a CM signal >1 µV.

OBJECTIVE:To investigate the clinical performance, safety, and patient-reported outcomes of an active osseointegrated steady-state implant system that uses piezoelectric technology.STUDY DESIGN:A prospective, multicenter, open-label, single-arm, within-subject clinical investigation.SETTING:Three tertiary referral clinical centers located in Melbourne, Sydney, and Hong Kong.PATIENTS:Twenty-nine adult subjects, 24 with mixed hearing loss or conductive hearing loss and 5 with single-sided sensorineural deafness.INTERVENTION:Implantation with the Cochlear Osia 2 System.MAIN OUTCOME MEASURES:Audiological threshold evaluation and speech recognition in quiet and in noise. Patient satisfaction and safety.RESULTS:At 6-month follow-up after surgery, a mean improvement in pure-tone average of 26.0 dB hearing level and a mean improvement of 8.8 dB signal-to-noise ratio in speech reception threshold in noise was achieved with the investigational device as compared with the unaided situation. Usability of the investigational device was rated 71.4/100 mm for sound processor retention and 81.4/100 mm for overall comfort using a visual analog scale.CONCLUSION:These outcomes confirm the clinical safety, performance, and benefit of an innovative active transcutaneous bone conduction implant using a piezoelectric transducer design in subjects with conductive hearing loss, mixed hearing loss, or single-sided sensorineural deafness.

Introduction: Intra-operative electrophysiological testing is being increasingly used to determine device functionality. Impedance abnormalities (open or short circuits) measured at time of surgery pose a dilemma: is it likely to resolve or is it a permanent fault? There is little in the literature on how to manage these intraoperative finding and if, at time of surgery, the back-up device should be used. Methods: We routinely undertake impedance testing twice intraoperatively, as well as at switch on, 1 and 3 months postoperatively. Retrospective impedance thresholds were analysed for one surgeon's cases between January 2018 and December 2019. Results: There were 235 cochlear implants performed for 217 patients (5,020 electrode contacts) analysed. Thirty-three electrodes had abnormal impedance thresholds on first intraoperative cycle of testing, 76% resolving with the second testing cycle electrode contacts that demonstrated abnormal impedance during both intraoperative test cycles were 16.54 times (95%CI 2.55–107.13, p = 0.003) more likely to be abnormal at three months. Fifty percent resolved by switch on. The intraoperative abnormalities made up 26% of electrode abnormalities seen at 3 months postoperatively. Discussion: This study demonstrates the utility of 2 cycles of intraoperative impedance testing, with persistently abnormal electrodes having 16 times the likelihood of persistent abnormalities of impedance, and 50% resolution. These persistent intra-operative abnormal electrodes are responsibly for 26% of electrode abnormalities at 3 months. This information is useful for the surgeon when considering use of the backup cochlear implant device.

Although eye abnormalities are reported in fetal alcohol spectrum disorders (FASD), no systematic review based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines has been undertaken. Our aim was to document the range and prevalence of eye abnormalities reported in children with prenatal alcohol exposure (PAE) and/or FASD.Searches of electronic databases and manual searches. Eligible articles were observational studies in children with PAE and/or FASD; peer reviewed journal articles in the English language; and studies reporting quantitative or frequency data on functional/structural eye abnormalities. Pooled prevalence, odds ratio, and mean differences were calculated.Of the 1,068 retrieved articles 36 were eligible, including articles on children with diagnosed fetal alcohol syndrome/FASD (N = 31); PAE (N = 3); and FASD or PAE without FASD (N = 2). Structural and functional eye abnormalities were identified, the most prevalent being short palpebral fissure length (66.1%), visual impairment (55.5%), epicanthus (53.5%), subnormal stereoacuity (53.0%), abnormal retinal tortuosity (50.5%), impaired fixation ability (33.3%), telecanthus (31.7%), optic nerve hypoplasia (30.2%), and small optic discs (27.0%). Compared to non-exposed controls, strabismus, subnormal vision, ptosis, short palpebral fissure length, microphthalmos, smaller optic disc area, and retinal vessel tortuosity were more prevalent in children with FASD.Examination of eyes and vision should be considered in children with PAE and suspected or diagnosed FASD to enable early identification and optimal management. This first comprehensive, systematic literature review demonstrates the variety and frequency of eye abnormalities reported in PAE/FASD.

HYPOTHESIS:It is possible to detect when misplacement and malposition of the cochlear implant (CI) electrode array has occurred intraoperatively through different investigations. We aim to explore the literature surrounding cochlear implant misplacements and share our personal experience with such cases to formulate a quick-reference guide that may be able to help cochlear implant teams detect misplacements early.BACKGROUND:Misplacement and malposition of a cochlear implant array can lead to poor hearing outcomes. Where misplacements go undetected during the primary surgery, patients may undergo further surgery to replace the implant array into the correct intracochlear position.METHODS:Systematic literature review on cochlear implant misplacements and malpositions and a retrospective review of our program's cases in over 6,000 CI procedures.RESULTS:Twenty-nine cases of CI misplacements are reported in the English literature. Sixteen cases of cochlear implant misplacements are reported from our institution with a rate of 0.28%. A further 12 cases of intracochlear malpositions are presented. The electrophysiological (CI electrically evoked auditory brainstem response, transimpedance matrix) and radiological (X-ray and computed tomography scan) findings from our experience are displayed in a tabulated quick-reference guide to show the possible characteristics of misplaced and malpositioned cochlear implant electrode arrays.CONCLUSION:Both intraoperative electrophysiological and radiological tests can show when the array has been misplaced or if there is an intracochlear malposition, to prompt timely intra-operative reinsertion to yield better outcomes for patients.

Objective: To quantify the impact of cochlear implantation (CI) on all five vestibular end-organs and on subjective ratings of post-CI dizziness. Methods: Seventy-two patients undergoing unilateral CI were recruited for the study. All participants completed pre- and post-CI three-dimensional video head-impulse tests (3D vHITs) to assess semicircular-canal (SC) function, air- and bone-conducted (AC and BC) cervical and ocular vestibular-evoked myogenic potentials (cVEMPs and oVEMPs) to assess otolith-function and the dizziness handicap inventory (DHI) to measure self-perceived disability. Results: Nineteen percent of patients reported new or worsened dizziness postsurgery. Post-CI abnormalities (new lesions and significant deteriorations) were seen in the AC cVEMP (48%), AC oVEMP (34%), BC cVEMP (10%), and BC oVEMP (7%); and lateral (L) (17%), posterior (P) (10%), and anterior (A) (13%) SC vHITs. CI surgery was more likely to affect the AC cVEMP compared with the other tests (chi(2) test, p < 0.05). Fifty percent of patients reported no dizziness pre- and postsurgery. In the implanted ear, normal pre-CI vHIT gain was preserved in lateral semicircular canal (LSC) (69%), anterior semicircular canal (ASC) (74%), and posterior semicircular canal (PSC) (67%), and normal reflex amplitudes were found in AC cVEMP (25%), AC oVEMP (20%), BC cVEMP (59%), and BC oVEMP (74%). Statistically significant decreases were observed in LSC vHIT gain, AC cVEMP amplitude, and AC oVEMP amplitude postsurgery (p < 0.05). There was a significant moderate positive correlation between change in DHI scores and the summed vestibular deficit postsurgery (r(51) = 0.38, p < 0.05). Conclusion: CI can impact tests that assess all five vestibular end-organs and subjective ratings of dizziness. These results support pre and post-surgical vestibular testing and assist preoperative counseling and candidate selection.

Objective: To review our experience on post-tonsillectomy and/or adenoidectomy hemorrhage (PTAH) at a tertiary pediatric referral hospital and to evaluate the management and risk factors for recurrent postoperative hemorrhage and for delayed bleeding after day 14. Methods: A retrospective chart review was performed for all pediatric patients admitted to The Children’s Hospital at Westmead for PTAH between July 01, 2014, and June 30, 2019. Patients with recurrent hemorrhage and those with bleeding after day 14 were selected for subanalysis. Results: Of the 291 patients admitted for PTAH, 31 (11%) patients had recurrent postoperative hemorrhage, and 11 (4%) patients had delayed bleeding after day 14. Surgical intervention for cessation of hemorrhage was required in 88 (30%) patients, including 2 patients who required return to the theater more than once. Nine (3%) patients received blood transfusions. The average number of days between bleeding episodes was 4 days. Recurrent postoperative hemorrhage occurred in 8.5% of patients who were managed operatively at their first presentation compared to 11.4% of patients who were managed nonoperatively (odds ratio: 1.1; 95% confidence interval 0.43-2.8). No association was found between abnormal coagulation profile, surgical indication, and risk of delayed postoperative hemorrhage. Conclusions: Recurrent or delayed postoperative hemorrhage represents a small proportion of children with postoperative bleeding and cannot be reliably predicted. Management of first presentations with either a conservative or a surgical approach is reasonable since the risk of recurrent of PTAH may be unrelated to the choice of management at initial presentation. Careful preoperative counseling of patients and their families is important to help set expectations in the event of PTAH.