Management of Tracheobronchial Stenosis in Chondrodysplasia Punctata.

Chondrodysplasia punctata (CDP) is a rare congenital syndrome characterized by aberrant, punctate deposition of calcium during endochondral bone formation, resulting in the characteristic finding of epiphyseal stippling on radiographs. While otolaryngologic manifestations such as nasomaxillary hypoplasia and mixed hearing loss are common, tracheobronchial calcification occurs rarely in neonates with CDP. The management of CDP-related airway stenosis is complex and there is limited literature pertaining to outcomes of airway interventions. Herein, we describe the clinical course and outcome of tracheal dilation for a newborn patient with CDP. Laryngoscope, 134:1464-1468, 2024.

Radiological Assessment of High Anterior Septal Deviation and Its Impact on Sinus Access.

OBJECTIVES/HYPOTHESIS: High anterior septal deviation (HASD) is an underreported anatomic variant that can affect the decision to perform septoplasty for access during sinus surgery and ease of postoperative debridement. This study aims to 1) describe an objective method of assessing HASD, and 2) explore its prevalence and implications for performing septoplasty. STUDY DESIGN: Retrospective cross-sectional study. METHODS: Computed tomography scans from 2014 to 2020 were retrospectively reviewed. Two independent observers measured the following with respect to midline: distance to septum (SDD), distance to lateral nasal wall (LNW), and septal deviation angle (SDA). RESULTS: A total of 147 patients were included, with excellent interrater reliability across 99 patients (0.8-0.9). Mean measurements across all patients were SDD (2.77 mm ± 1.34), SDD/LNW (0.26 ± 0.12), and SDA (8.9° ± 4.0). Of 102 patients who underwent sinus surgery, 47 received septoplasty. Compared to the non-septoplasty cohort, the septoplasty cohort had a greater mean SDD (3.61 mm ± 1.48 vs. 2.27 mm ± 0.95; d = 1.10 [95% CI 0.67-1.51]), SDD/LNW (0.34 ± 0.13 vs. 0.21 ± 0.09; d = 1.18 [95% CI 0.76-1.60]), and SDA (11.1° ± 4.3 vs. 7.3° ± 3.4; d = 1.00 [95% CI 0.58-1.40]). Receiver operating characteristic cutoffs were SDD ≥2.43 mm, SDD/LNW ≥0.25, and SDA ≥7.6°, corresponding to a 49%-58% prevalence of HASD. CONCLUSION: HASD is relatively common and the methods described herein can reliably assess its dimensions. Measurements of SDD, SDD/LNW, and SDA exceeding cutoffs determined by this study may represent clinically significant deflections prompting consideration of septoplasty. These methods may aid in preoperative planning. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:1166-1171, 2022.

A cancer-unique glycan: de-N-acetyl polysialic acid (dPSA) linked to cell surface nucleolin depends on re-expression of the fetal polysialyltransferase ST8SIA2 gene.

BACKGROUND: Polysialic acid (polySia) modifies six cell surface proteins in humans mainly during fetal development and some blood cells in adults. Two genes in humans, ST8SIA2 and ST8SIA4, code for polysialyltransferases that synthesize polySia. ST8SIA2 is highly expressed during fetal development and in cancer but not in adult normal human cells. ST8SIA4 is expressed in fetal and adult brain, spleen, thymus, and peripheral blood leukocytes and in cancer. We identified a derivative of polySia containing de-N-acetyl neuraminic acid residues (dPSA), which is expressed on the cell surface of human cancer cell lines and tumors but not normal cells. METHODS: dPSA-modified proteins in several human cancer cell lines and normal blood cells were identified using co-immunoprecipitation with anti-dPSA antibodies, mass spectroscopy and Western blot. RNAi and CRISPR were used to knockdown and knockout, respectively, the polysialyltransferase genes in human melanoma SK-MEL-28 and neuroblastoma CHP-134 cell lines, respectively, to determine the effect on production of cell surface dPSA measured by flow cytometry and fluorescence microscopy. RESULTS: We found that dPSA is linked to or associated with nucleolin, a nuclear protein reported to be on the cell surface of cancer but not normal cells. Knocking down expression of ST8SIA2 with RNAi or knocking out each gene individually and in combination using CRISPR showed that cell surface dPSA depended on expression of ST8SIA2. CONCLUSIONS: The presence of dPSA specifically in a broad range of human cancers but not human adult normal cells offers novel possibilities for diagnosis, prevention and treatment targeting the dPSA antigen that appears to be cancer-specific, consistent across not only human cancers but also species, and may be an unrecognized mechanism of immune shielding.

Primary diffuse large B-cell lymphoma of the major salivary glands: Increasing incidence and survival.

BACKGROUND: Diffuse Large B-Cell Lymphoma (DLBCL) of the major salivary glands is a rare high-grade malignancy that often presents with vague symptoms. This study aimed to evaluate its incidence and prognosis on a population level. METHODS: The Surveillance, Epidemiology, and End Results database was queried for cases of major salivary gland DLBCL. RESULTS: 896 patients had DLBCL affecting the parotid gland (78.3%) or submandibular gland (19.8%). The incidence was increasing at 1.5% (P = 0.005) per year from 1973 to 2016. 24.4% of patients underwent parotidectomy, 16% had biopsy, and the remaining provided no details of diagnostic method. Five-year disease-specific survival increased from 59.8% to 84.0%. Late-stage disease (HR = 1.7, P = 0.003) and female sex (HR = 2.1, P = 0.018) were associated with increased risk of mortality. CONCLUSION: Despite the rising incidence of major salivary gland DLBCL, chemotherapy and radiation treatment has resulted in increasingly favorable survival outcomes. The otolaryngologist - head and neck surgeon continues to play a critical role in establishing the diagnosis of salivary gland DLBCL.

Hair Transplantation in Frontal Fibrosing Alopecia and Lichen Planopilaris: A Systematic Review.

OBJECTIVE: Evolving hair transplantation (HT) techniques have offered new possibilities for hair restoration. However, the role of HT in patients with frontal fibrosing alopecia (FFA) and lichen planopilaris (LPP) remains unclear. This study aims to evaluate the outcomes and temporal relationship of HT in this population. METHODS: A literature search of three databases was conducted. We reviewed 1) literature reporting outcomes of patients with LPP or FFA who received HT, and 2) studies reporting the development of LPP or FFA resulting from HT. RESULTS: Thirteen articles included 42 patients that provided data for evaluation. Fifteen patients had previously been diagnosed with FFA or LPP, and the remaining 27 patients developed disease after undergoing HT. Seven patients with FFA and eight patients with LPP received HT, with a mean sustained disease remission of 2.69 years prior to HT. In total, two of seven (29%) patients with FFA and five of eight (75%) patients with LPP experienced positive HT results over a follow-up period of 8-72 months. Interestingly, 27 patients without evidence of previous disease developed FFA or LPP following HT after a median duration of 16 months. CONCLUSIONS: HT for LPP and FFA is feasible but results may be less favorable compared to HT for other causes. Outcomes may be more favorable for LPP than FFA but this was not statistically significant and evidence is very limited. FFA and LPP can also develop following HT in patients without previous evidence of disease. LEVEL OF EVIDENCE: NA Laryngoscope, 131:59-66, 2021.

Drug-Induced Ototoxicity: A Comprehensive Review and Reference Guide.

OBJECTIVE: In an era of increasing polypharmacy, adverse drug effects such as ototoxicity have significant public health implications. Despite the availability of evidence, many health care professionals may not know the risk of ototoxicity in common medications. Therefore, the aim of this review is to provide a comprehensive, easy to use, ototoxic profile of medications with an assessment of supporting evidence. METHODS: Medications of interest were identified through adverse drug reaction reports derived from Micromedex (IBM), Lexicomp (Wolters Kluwer), and the textbook, Drug Induced Diseases: Prevention, Detection, and Management. Additional evidence was identified though a query of PubMed and the Cochrane database. Evidence of causality was graded according to the following: A (randomized, controlled clinical trials), B (nonrandomized clinical trials, prospective observational studies, cohort studies, retrospective studies, case-controlled studies, and/or postmarketing surveillance studies), and C (case reports/case series). RESULTS: A total of 194 systemically administered medications associated with ototoxicity were identified, most commonly antimicrobials (53), psychotropics (21), antihypertensive/antiarrhythmics (19), nonsteroidal antiinflammatory drugs (18), and antineoplastics (16). There was evidence of cochleotoxicity in 165 medications (evidence grading A [22], B [77], C [69]), vestibulotoxicity in 100 medications (evidence grading A [23], B [47], and C [30]), and dizziness in 142 medications (evidence grading A [50], B [76], and C [16]). In addition, a review of the evidence of ototoxicity in ototopical medications is also reviewed. CONCLUSION: The effect and severity of ototoxicity can vary immensely depending on pharmacological and individual patient risk factors. The intent of this comprehensive review was to help health care providers of all sectors obtain a deeper knowledge of drug-induced ototoxicity to make more informed management decisions for their patients.

Emergent and urgent otologic surgeries during the SARS-CoV-2 pandemic: a protocol and review of literature.

OBJECTIVE: To review guidelines for otologic procedures during the SARS-CoV-2 pandemic and to present a protocol for emergent or urgent mastoidectomy at our institution. DATA SOURCES: Guidelines regarding emergent and urgent otologic procedures from otolaryngologic societies are reviewed. We described a protocol for emergent or urgent mastoidectomy at our institution. CONCLUSION: Patients requiring urgent or emergent mastoidectomy during the COVID-19 pandemic require prompt treatment. We make the following recommendations: Emergent or urgent otologic procedures should be performed in a contained environment, such as a tent created by the microscope drape. The surgical team should practice using instruments in the tent setup to prepare for real cases. Otologic procedures should adhere to guidelines set for high-risk procedures.

Using Intraoperative Recordings to Evaluate Surgical Technique and Performance in Mastoidectomy.

Importance: Otolaryngology residency programs currently lack rigorous methods for assessing surgical skill and often rely on biased tools of evaluation. Objectives: To evaluate which techniques used in mastoidectomy can serve as indicators of surgeon level (defined as the level of training) and whether these determinations of technique can be made based solely on the movement of the drill head or suction. Design, Setting, and Participants: In this prospective, observational study conducted from January 1, 2015, to December 31, 2019, at a single tertiary care institution, 3 independent observers made blinded evaluations on 24 intraoperative recordings of surgeons (6 junior residents, 4 senior residents, and 2 attending surgeons) performing mastoidectomies. Main Outcomes and Measures: Observers assessed drill stroke count, drilling efficiency, stroke pattern, use of suction and irrigation, and estimated surgeon level. Assessments were made on both original videos and animated videos that show only the path of the burr head or suction as dots against a white background. Results: Among the 24 recorded mastoidectomies performed by the 12 study surgeons, intraclass correlation was excellent for original video assessment of drill stroke count (0.98 [95% CI, 0.97-1.00]), use of suction (0.75 [95% CI, 0.52-0.89]), use of irrigation (0.83 [95% CI, 0.66-0.92]), and estimated surgeon level (0.82 [95% CI, 0.64-0.92]) and fair for drilling efficiency (0.54 [95% CI, 0.09-0.79]) and stroke pattern (0.49 [95% CI, -0.02 to 0.76]). Intraclass correlation was excellent for animated video assessment of drill stroke count per unit time (0.98 [95% CI, 0.96-0.99]) and drilling efficiency (0.80 [95% CI, 0.60-0.91]), good for stroke pattern (0.68 [95% CI, 0.38-0.85]) and estimated surgeon level (based on path of drill) (0.69 [95% CI, 0.38-0.85]), and fair for use of suction (0.58 [95% CI, 0.16-0.80]) and estimated surgeon level (based on path of suction) (0.58 [95% CI, 0.17-0.80]). On evaluation of original videos, junior residents had lower drill stroke count compared with senior residents and attending surgeons (6.0 [interquartile range (IQR), 3.0-8.0] vs 9.5 [IQR, 5.0-13.0] vs 10.5 [IQR, 5.0-17.8]; η2 = 0.14 [95% CI, 0.01-0.28]). On evaluation of animated videos, junior residents also had lower drill stroke count compared with senior residents and attending surgeons (6.0 [IQR, 4.0-9.0] vs 10.5 [IQR, 10.0-13.8] vs 10.5 [IQR, 4.3-21.0]; η2 = 0.19 [95% CI, 0.04-0.33]). Compared with junior and senior residents, attending surgeons had higher median ratings of drilling efficiency (original videos: junior residents, 4.0 [IQR, 3.0-4.0]; senior residents, 4.0 [IQR, 3.0-4.8]; attending surgeons, 5.0 [IQR, 4.3-5.0]; η2 = 0.23 [95% CI, 0.06-0.37]; animated videos: junior residents, 4.0 [IQR, 3.0-4.0]; senior residents, 3.0 [IQR, 2.0-4.0]; attending surgeons, 5.0 [IQR, 4.0-5.0]; η2 = 0.25 [95% CI, 0.08-0.39]) and stroke pattern (original videos: junior residents, 4.0 [IQR, 3.0-4.0]; senior residents, 4.0 [IQR, 3.0-4.8]; attending surgeons, 5.0 [IQR, 5.0-5.0]; η2 = 0.17 [95% CI, 0.03-0.31]; animated videos: junior residents, 4.0 [IQR, 3.0-4.0]; senior residents, 4.0 [IQR, 2.0-4.0]; attending surgeons, 5.0 [IQR, 5.0-5.0]; η2 = 0.15 [95% CI, 0.02-0.29]). Conclusions and Relevance: This study suggests that observation of intraoperative mastoidectomy recordings is a feasible method of evaluating surgeon level. Reasonable indicators of surgeon level include the drill stroke count, drilling efficiency, stroke pattern, and use of the suction irrigator. Observing the path of the drill alone is sufficient to appreciate differences in drilling technique but not sufficient to accurately determine surgeon level. Intraoperative recordings can serve as a useful addition to resident education and evaluation.

Cochlear Implantation for Treatment of Tinnitus in Single-sided Deafness: A Systematic Review and Meta-analysis.

OBJECTIVE: Quantify the benefit of cochlear implantation (CI) for tinnitus relief among individuals with single-sided deafness (SSD). DATA SOURCES: PubMed, Scopus, and Cochrane databases were searched through July 10, 2019. Search strategies used a combination of subject headings (e.g., MeSH in PubMed) and keywords for the following three concepts: single-sided deafness, cochlear implantation, and tinnitus. STUDY SELECTION: English articles that reported the preintervention (baseline) tinnitus-related patient-reported outcome measures (e.g., Tinnitus Handicap Inventory [THI] and Visual Analog Scale [VAS] for loudness) in patients with SSD that underwent CI were included. DATA EXTRACTION: Number of patients, mean age, etiology of hearing loss, duration of deafness, baseline and follow-up THI and VAS scores. DATA SYNTHESIS: A total of 17 studies met inclusion criteria encompassing 247 patients with SSD receiving a cochlear implant (mean age 50.2 yr, range 23-71). For THI, CI resulted in a mean difference of -35.4 points [95% CI -55.8 to -15.0, p < 0.001]. VAS decreased by -4.6 points [CI -6.0 to -3.3, p < 0.001]. A weighted proportion of 14.9% [CI 6.4-26.1] of patients experienced complete resolution of tinnitus, while 74.5% [CI 63.1-84.5] experienced partial improvement; 7.6% [CI 4.1-12.6] of patients had no change in severity, and 3.0% [CI 1.0-6.7] experienced worsening of their tinnitus. CONCLUSIONS: On both THI and VAS, patients reported significant reduction in their scores, representing an overall improvement in tinnitus severity while wearing the cochlear implant. Most patients with SSD will experience partial improvement or complete resolution of tinnitus with a cochlear implant.

Impact of early gastroesophageal reflux disease on childhood otologic outcomes.

OBJECTIVE: Recent evidence has suggested that children with gastroesophageal reflux disease (GERD) face increased risk for otologic disease. This study aims to evaluate otologic outcomes in children with GERD using a large pediatric hearing database. METHODS: Data pertaining to children with GERD in the first year of life were extracted from the Audiological and Genetic Database. Subgroups included children with uncomplicated GERD, complicated GERD (with esophagitis), and GERD requiring surgery (fundoplication or fundoplasty). Main outcome measures included hearing loss (HL) prevalence, type, severity, and progression. Children without GERD in the first year of life served as controls. RESULTS: 5747 children were diagnosed with GERD in the first year of life. Evidence of HL was present in 40.9% children with uncomplicated GERD, 63.1% in children with complicated GERD, 75% in children with GERD requiring surgical intervention, and 43.3% in controls (p < 0.001). Children with GERD requiring surgery demonstrated the highest odds of developing any HL (aOR 3.02 [1.40-7.26]), moderate-to-profound HL (aOR 3.44 [1.56-7.29]), and mixed HL (aOR 4.58 [2.07-9.65]) relative to children with uncomplicated or complicated GERD. GERD requiring surgery was independently associated with Eustachian tube dysfunction (aOR 2.41 [1.17-5.20]) and tympanostomy tube placement (aOR 6.23 [3.05-13.01]). CONCLUSION: GERD diagnosed within the first year of life is associated with pediatric hearing impairment and otologic issues, including otitis media, Eustachian tube dysfunction, and need for tympanostomy tube placement. GERD requiring surgical intervention is particularly associated with hearing loss in this population. Children with GERD and HL deserve close audiologic follow-up.

Factors affecting complications and comorbidities in children with cholesteatoma.

OBJECTIVE: To evaluate the effects of age, race, payer status, and socioeconomic status on complications and comorbidities in children with cholesteatoma. METHODS: An analysis of the Kids' Inpatient Database was performed on cases of cholesteatoma between 2006 and 2016, along with associated complications or co-morbidities and surgical interventions. RESULTS: 1552 cases of pediatric cholesteatoma represented 5.6 cases per 100,000 total discharges over the study period. The mean age was 9.9 (±5.4) years. Compared to children in the 4th age quartile, those in the 1st age quartile had decreased risk of conductive hearing loss (OR 0.64 [0.42-0.99]), venous thrombosis (OR 0.24 [0.06-0.88]), intracranial abscess (OR 0.35 [0.13-0.96]), and facial nerve palsy (0.44 [0.20-0.97]), but increased risk of chronic otitis media (OR 2.24 [1.23-4.10]). Compared to children identified as Other race, children identified as Black had increased risk of acute otitis media (OR 9.20 [1.35-62.78]). Both children identified as Black (OR 9.90 [1.48-66.35]) or Hispanic (OR 6.24 [1.01-38.51]) had increased risk of facial nerve palsy. Relative to children in the 4th income quartile, children in the 1st income quartile had increased risk of acute mastoiditis (OR 1.87 [1.15-3.03]) and subperiosteal abscess (OR 6.75 [2.22-20.56]). Children in the 2nd income quartile were less likely to receive ossicular chain surgery (OR 0.31 [0.13-0.72]). CONCLUSION: Differences pertaining to age, race, payer status, and socioeconomic status exist in the presentation and surgical management of children hospitalized with cholesteatoma. Older children are at increased risk of intracranial complications. Patients of Black and Hispanic race might have a higher risk of facial nerve palsy. Compared to children of higher income families, those from lower income families more frequently develop acute mastoiditis and subperiosteal abscess. Providers should be mindful of these risk factors when caring for children with cholesteatoma.

Transcutaneous Electrical Nerve Stimulation for Treatment of Tinnitus: A Systematic Review and Meta-analysis.

OBJECTIVE: To evaluate the treatment efficacy of transcutaneous electrical nerve stimulation (TENS) in patients with tinnitus. DATA SOURCES: PubMed, Scopus, Web of Science, and Cochrane Library were searched for the following concepts: "Transcutaneous Electric Nerve Stimulation" and "Tinnitus." STUDY SELECTION: Inclusion: 1) double- or single-blinded randomized controlled trials; 2) double- or single-blinded randomized comparison trials; 3) prospective or retrospective observational studies; and 4) case series. Exclusion: Non-English studies, nonhuman studies, case reports (n ≤ 5), and review articles. DATA EXTRACTION: Tinnitus Handicap Inventory (THI), the Visual Analog Scale (VAS), and perceived tinnitus suppression after treatment. Additional data collected included tinnitus laterality, duration of symptoms, location of electrode placement, time to follow-up, etiology of tinnitus, and treatment side effects. DATA SYNTHESIS: The literature search yielded 2941 unique articles. After reviewing 118 full-text articles, 17 studies reporting on 1,215 patients were included for final analysis. Four studies provided data available for meta-analysis of pre- and posttreatment THI and VAS (Cochrane Review Manager). TENS showed significant overall reduction on THI (-7.55 [-10.93 to -4.18], p < 0.0001) and VAS (-0.65 [-0.99 to -0.30], p < 0.0002). Subjective improvement of tinnitus was pooled across 13 studies using meta-analysis of proportions (MedCalc). Tinnitus suppression occurred in 40.0% [28.9-51.7%] patients. Among those who responded, 22.2% [12.2-29.7%] experienced complete suppression and 10.2% [0.2-31.9%] experienced persistent improvement at 3 months. CONCLUSIONS: TENS represents a safe and feasible treatment option for tinnitus and might be a worthy consideration among the spectrum of interventions developed for tinnitus.

Endonasal Endoscopic Surgery for Pediatric Sellar and Suprasellar Lesions: A Systematic Review and Meta-analysis.

OBJECTIVES: The advent of endonasal endoscopic skull base surgery (ESBS) has redefined the management of pediatric sellar and suprasellar lesions. To date, the outcomes of these procedures have not been systematically reviewed. This study performed a systematic review with meta-analysis of surgical outcomes for pediatric patients undergoing ESBS for sellar and suprasellar lesions. DATA SOURCES: PubMed (National Library of Medicine, National Institutes of Health), Scopus (Elsevier), and Cochrane Library (Wiley). REVIEW METHODS: Articles reporting on pediatric patients undergoing ESBS for craniopharyngiomas, pituitary adenomas, and Rathke's cleft cysts were reviewed. The primary outcome was postoperative cerebrospinal fluid (CSF) leak. Secondary outcomes included endocrine, visual, and other complications. RESULTS: Twenty-five articles reporting on 554 patients were included. Overall postoperative CSF leak rate was 8.6%, with tumor-specific rates of 10.6% in craniopharyngiomas, 6.5% in pituitary adenomas, and 7.2% in Rathke's cleft cysts (P > .05). Older studies demonstrate higher postoperative CSF leak rates as compared with more recent studies (12.5% vs 6.1%, P = .0082). Younger children (8.9-12.6 years old) experienced a higher rate of postoperative CSF leaks as compared with older children (13.0-16.6 years old; 12.9% vs 4.9%, P = .0016). Additional postoperative complications included diabetes insipidus (26.7%), hypopituitarism (46.6%), visual deficits (2.6%), meningitis (3.4%), and weight gain (3.4%). CONCLUSION: ESBS for pediatric sellar and suprasellar lesions is overall an effective management approach with an increasingly favorable risk-benefit profile. Younger children may be more susceptible to postoperative CSF leak as compared with older pediatric patients. Tumor type does not appear to be an independent risk factor for postoperative CSF leak in this population.

Transoral Robotic Surgery versus Plasma Ablation for Tongue Base Reduction in Obstructive Sleep Apnea: Meta-analysis.

OBJECTIVES: To compare the effectiveness of transoral robotic surgery (TORS) versus plasma ablation (PA) in tongue base reduction surgery for obstructive sleep apnea (OSA). DATA SOURCES: PubMed, Scopus, Cochrane Library, OVID. REVIEW METHODS: Keywords searched included OSA, tongue base surgery, TORS, and coblation. Outcomes included pre- to postoperative apnea-hypopnea index, Epworth Sleepiness Scale (ESS), and lowest oxygen saturation. Additional outcomes included surgical success rate, postoperative bleeding, operative time, and length of stay. RESULTS: A total of 690 unique articles were identified, of which 60 underwent full-text review. Twenty-six articles were included in final analysis, comprising 18 studies on TORS (834 patients) and 11 studies on PA (294 patients). Mean differences of apnea-hypopnea index, ESS, and lowest oxygen saturation for TORS were -23.92, -7.6, and 5.83% (all P < .01). Corresponding values for PA were -22.07, -4.14, and 5.48% (all P < .00001). TORS had greater ESS reduction than PA (P = .02). Follow-up duration was shorter in TORS than PA (mean ± SD: 4.2 ± 2.6 vs 4.6 ± 1.4 months, P = .0482). Surgical success rates in TORS and PA were similar (57.6% vs 60.3%, P = .4474). Postoperative bleeding occurred less frequently in TORS versus PA (3.3% vs 7.5%, P = .0103). Operative time was longer for TORS than PA (77.9 ± 16.4 minutes vs 44.0 ± 12.9 minutes, P < .0001). Length of stay was similar between TORS and PA (3.9 ± 1.6 days vs 3.9 ± 2.5 days, P = .9047). CONCLUSION: Tongue base reduction with TORS or PA each effectively treats OSA and provides comparable results. The choice between techniques might depend on patient factors, availability of technology, and associated costs.

Posterior Semicircular Canal Dehiscence: Case Series and Systematic Review.

OBJECTIVE: To conduct a systematic review of posterior semicircular canal dehiscence (PSCD) and to present a series of patients with PSCD with and without classic third-window symptoms. DATA SOURCES: PubMed, Scopus, and the Cochrane Library from inception until April 2019. Case series of five patients seen in a multidisciplinary, vestibular-focused, neurotology clinic. STUDY SELECTION: Inclusion criteria: PSCD studies of symptomatology, diagnostic testing, radiology, and histopathology. EXCLUSION CRITERIA: non-English articles, reviews, letters, animal studies. DATA EXTRACTION: Quality evaluated according to Oxford Center for Evidence-Based Medicine criteria and funnel plot via the Stern and Egger method. DATA SYNTHESIS: Two hundred five studies were found, and 58 studies were included. In 47 total patients, sound-induced vertigo, mixed hearing loss, and tinnitus were the most common presenting symptom. A meta-analysis of proportions using eight radiological and histopathological studies revealed an incidence of 0.38% adult ears [95% CI 0.08, 0.89] and 2.16% of adult patients [0.64, 4.54]. The incidence in pediatric patients ranged from 1.3 to 43%. Jugular bulb abnormalities were common. In our case series, four of five patients presented without third-window symptoms, while one had sound- and pressure-induced vertigo. Hearing loss in these patients was not salvageable. CONCLUSIONS: PSCD is a rare phenomenon most commonly presenting with third-window type symptoms. However, PSCD might also present with dizziness and hearing loss inconsistent with third-window symptomatology. One should be conscious of potentially poorer prognosis for hearing recovery in these patients.

Hearing outcomes in children of diabetic pregnancies.

OBJECTIVE: Children of diabetic pregnancies (CDPs) face numerous risk factors for hearing loss (HL). The objective of this study was to investigate the hearing outcomes of CDPs on a population scale. METHODS: Using the Audiological and Genetic Database, the prevalence, severity, and progression of HL in CDPs was compared against children of non-diabetic pregnancies (CNDPs) who served as controls. RESULTS: Among 311 CDPs, 71.1% demonstrated evidence of HL compared to 45.5% in CNDPs (p < 0.001). The mean age at which CDPs received audiograms was 3.6 years compared to 5.4 years for CNDPs (p < 0.001). Compared to CNDPs, CDPs were similarly affected by common otologic conditions such as acute otitis media (25.7%), chronic otitis media (38.3%), and Eustachian tube dysfunction (41.8%) (all p > 0.05). CDPs were more likely to have bilateral HL (81%) and sensorineural hearing loss (SNHL) (8%) relative to CNDPs (p < 0.001 and p = 0.004, respectively). Rates of conductive HL and mixed HL were not significantly different between groups (p = 0.952 and p = 0.058, respectively). CDPs were at significant risk for the development of HL (aOR 1.66 [1.28-2.17], SNHL (aOR 1.63 [1.01-2.52], and high-frequency HL (aOR 1.32 [1.03-1.68]). Of the comorbidities evaluated, CDPs with hyperbilirubinemia (aOR 1.85 [1.18-2.84]), perinatal asphyxia (aOR 1.90 [1.06-3.16]), or congenital heart disease (aOR 1.21 [1.07-1.37]) demonstrated higher risk of SNHL. CONCLUSION: Children of diabetic pregnancies face increased risks of developing HL, particularly bilateral and sensorineural hearing loss. Given these findings, we recommend close audiologic follow-up for these children, especially those with complicated birth histories or additional medical problems.

Endonasal endoscopic surgery for pediatric anterior cranial fossa encephaloceles: A systematic review.

BACKGROUND: Endoscopic endonasal skull base surgery (ESBS) is a well-established management strategy for anterior cranial fossa (ACF) encephaloceles in adults. However, its application to the pediatric population has not been evaluated in large-scale studies. This study systematically reviews the safety of ESBS for pediatric ACF encephaloceles. METHODS: Articles reporting on pediatric patients undergoing purely ESBS for encephaloceles were reviewed from three databases (PubMed, Scopus, and Cochrane Library). Main outcomes were post-operative cerebrospinal fluid (CSF) leak and other surgical complications. RESULTS: Twenty-three articles provided data on 110 eligible patients with a mean age of 4.3 years (range birth - 18 years). Intraoperative CSF leaks occurred in 18.5% of children while post-operative CSF leaks developed in 6.0% of cases. Rates of meningitis and hydrocephalus were each 3.7%. Other complications included alar collapse, nasal stenosis, transient diabetes insipidus, pneumonia, and neurologic injury, collectively occurring in 7.0% of cases. The rate of death was 4.8%. The average duration of follow-up was 25.3 months. Encephaloceles recurred in 5.2% of patients. CONCLUSIONS: ESBS for pediatric ACF encephaloceles provides a relatively favorable risk-benefit profile, with a low rate of post-operative CSF leak, other surgical complications, and disease recurrence.

Cross-Sectional Analysis of Cognitive Dysfunction in Patients With Vestibular Disorders.

OBJECTIVE: The relationship of cognitive dysfunction and vestibular dysfunction has been established by various studies. However, the available Patient-Reported Outcome Measures questionnaires that address the main vestibular complaint fail to highlight this domain of dysfunction in this patient population. The objective of this study was to quantify and compare cognitive impairment using a validated cognitive questionnaire across several vestibular diagnoses. STUDY DESIGN: Cross-sectional study of 186 patients presenting to a tertiary care vestibular clinic with a diagnosis of vestibular migraine, Meniere's disease (MD), benign positional paroxysmal vertigo, or persistent postural-perceptual dizziness (PPPD). Patients completed the Cognitive Failures Questionnaire (CFQ) and the Dizziness Handicap Inventory (DHI). RESULTS: Mean CFQ scores for this cohort were significantly higher than similarly aged published controls (34.9/100 versus 31.3/100; p < 0.01; mean age, 45 years) as well as published controls between 65 and 74 years of age (34.9/100 versus 31.2/100; p < 0.05). Patients with PPPD or combined vestibular migraine and MD scored the highest on the CFQ and significantly higher than controls (45.1/100, p = 0.001; and 44.1/100, p = 0.006, respectively). Patients with benign positional paroxysmal vertigo had lower scores than normal controls. There is a weak but significant correlation between CFQ and DHI (r = 0.31; p < 0.001). Multivariate linear regression shows that CFQ scores were largely driven by the duration of symptoms (p < 0.001), type of diagnosis (notably PPPD; p = 0.026), and DHI (p < 0.001). CONCLUSIONS: Our results indicate that cognitive impairment is prevalent with chronic vestibular disorders, even in peripheral disorders such as MD. The duration of vestibular symptoms before diagnosis (and management), as well as certain etiologies, may play a bigger role in cognitive disability than age. This is not currently well-addressed in Patient-Reported Outcome Measures questionnaires and may be overlooked at the time of the diagnosis. Appropriate identification may help tailor treatment, namely rehabilitation programs, to individual patients.

Programmed Death-1 Pathway in Orbital Invasion of Cutaneous Carcinomas.

PURPOSE: The programmed death-1 pathway negatively regulates the immune system. Previous reports have indicated worse tumor-related outcomes with increased expression of the ligand for this pathway. This study was undertaken to assess the role of the PD pathway in cutaneous malignancies that invade the orbit. METHODS: Immunohistochemical staining for the programmed death-1 receptor and ligand was performed on exenteration specimens of invasive cutaneous orbital malignancies (n = 12) and nodular basal cell carcinoma (n = 10). The numbers of positively-staining cells/40× field were counted across 5 consecutive fields, and statistical analyses were performed to compare the differences between the 2 groups. RESULTS: Programmed death-1 receptor positivity was seen in means of 30.9 cells/40× field and 62.4 cells/40× field for nodular basal cell carcinomas and invasive malignancies, respectively (p = 0.0046). A mean of 4.54 cells/40× field stained positively for the programmed death-1 ligand in nodular basal cell carcinoma, whereas a mean of 46.4 cells/40× field stained positively for programmed cell death ligand-1 in orbital invasive cutaneous carcinomas (p = 0.0015). Both of these differences were statistically significant. CONCLUSIONS: Both the programmed death-1 receptor and its ligand are enriched in invasive cutaneous malignancies. This finding indicates that negative regulation of the immune system likely prohibits tumor surveillance, and facilitates increasing aggressiveness and invasion of cutaneous malignancies.