Canal wall up versus canal wall down mastoidectomy techniques in the pediatric population with cholesteatoma: A systematic review and meta-analysis of comparative studies.

IMPORTANCE: The optimal surgical management of cholesteatoma remains controversial. Within pediatric otolaryngology, one of the most vital points of contention is the selection of canal wall-up (CWU) versus canal wall-down (CWD) procedures. Pediatric cholesteatoma has high rates of recurrence (16%-54%). In adults, there is evidence that the selection of surgical techniques affects recurrence rates. This has not been shown in children. OBJECTIVES: 1. To systematically review the literature on recurrent and residual cholesteatoma after CWU and CWD in children and perform a meta-analysis of the data. 2. To assess the rates of recurrent and residual cholesteatoma between CWU and CWD techniques in pediatric patients. 3. To assess hearing outcomes by evaluating postoperative differences in the air-bone gap (ABG) between CWU and CWD techniques. DATA SOURCES: A systematic search of PubMed, Embase, Scopus, and Cochrane Collaboration was performed from inception to May 1st, 2020, to identify studies that compared CWU and CWD procedures for acquired cholesteatoma in children. STUDY SELECTION: Search records were screened in duplicate by four reviewers. Inclusion criteria consisted of comparative randomized clinical trials and observational studies assessing outcomes of CWU and CWD techniques in the pediatric population. Studies involving patients with congenital cholesteatoma were excluded. DATA EXTRACTION AND SYNTHESIS: Four reviewers working independently and in duplicate systematically reviewed and extracted study data. Dichotomous variables were analyzed as risk ratios (RR), while continuous variables were compared using weighted mean differences (MD). The risk of bias was assessed using the CLARITY Scale. PRIMARY OUTCOMES AND MEASURES: The outcomes were recurrence, residual disease, air-bone gap (ABG), and air conductive (AC) thresholds. RESULTS: After screening 1036 publications, 17 retrospective cohort studies were selected. 1333 children were included; the overall mean age was ten years (SD 7.9), and the overall mean follow-up time was 5.9 years (SD 6.6). CWU and CWD techniques were performed in 60% (796) and 40% (537) cases. We did not find differences in cholesteatoma recurrence (RR: 1.50, 95% CI 0.94; 2.40; n = 544; I2 0%; Tau [2]: 0.00), or rates of residual cholesteatoma (RR 1.51, 95% CI 0.96; 2.38, n = 506; I2: 0%; Tau [2]: 0.00) in patients who underwent CWU and CWD mastoidectomy. The mean air-bone gap was lower with CWU than CWD (mean difference: 7.60, 95% CI -10.65; -4.54; n = 242; I2: 71%; Tau [2]: 5.98). CONCLUSION: and relevance: We show similar rates of recurrence and residual disease after either CWU or CWD tympanoplasty. Our results challenge the fundamental principle of CWD surgery as a standard technique, as there is no difference in rates of recurrence and residual disease in CWU and CWD. Moreover, audiometric results support CWU with improved hearing outcomes. TRIAL REGISTRATION: PROSPERO identifier: CRD42020184029.

Evaluation of Asymmetries in Speech-in Noise Abilities in Audiologic Screening for Vestibular Schwannoma.

OBJECTIVES: Measures of speech-in-noise, such as the QuickSIN, are increasingly common tests of speech perception in audiologic practice. However, the effect of vestibular schwannoma (VS) on speech-in-noise abilities is unclear. Here, we compare the predictive ability of interaural QuickSIN asymmetry for detecting VS against other measures of audiologic asymmetry. METHODS: A retrospective review of patients in our institution who received QuickSIN testing in addition to a regular audiologic battery between September 2015 and February 2019 was conducted. Records for patients with radiographically confirmed, unilateral, pretreatment VSs were identified. The remaining records excluding conductive pathologies were used as controls. The predictive abilities of various measures of audiologic asymmetry to detect VS were statistically compared. RESULTS: Our search yielded 73 unique VS patients and 2423 controls. Receiver operating characteristic curve analysis showed that QuickSIN asymmetry was more sensitive and specific than pure-tone average asymmetry and word-recognition-in-quiet asymmetry for detecting VS. Multiple logistic regression analysis revealed that QuickSIN asymmetry was more predictive of VS (odds ratio [OR] = 1.23, 95% confidence interval [CI] [1.10, 1.38], p < 0.001) than pure-tone average asymmetry (OR = 1.04, 95% CI [1.00, 1.07], p = 0.025) and word-recognition-in-quiet asymmetry (OR = 1.03, 95% CI [0.99, 1.06], p = 0.064). CONCLUSION: Between-ear asymmetries in the QuickSIN appear to be more efficient than traditional measures of audiologic asymmetry for identifying patients with VS. These results suggest that speech-in noise testing could be integrated into clinical practice without hindering the ability to identify retrocochlear pathology.

Natural History of Cystic Vestibular Schwannomas.

OBJECTIVE: To determine the natural history of cystic vestibular schwannomas (VS). STUDY DESIGN: Retrospective cohort. SETTING: Single tertiary academic hospital. PATIENTS: Adults diagnosed with cystic VS who had at least 2 MRIs performed at least 6 months apart between 2008 and 2016 with no intervening treatment. MAIN OUTCOME MEASURES: Volumetric growth rates of both the entire tumor and individual cystic and solid components were measured. Linear growth rate of the entire tumor was assessed using the largest diameter parallel to the petrous face at the cerebellopontine angle (CPA). RESULTS: Twenty-one patients met inclusion criteria. The average volumetric growth rate of the tumor was 1.1 ± 2 (range: -1.2 to 7.8), while the average growth rate of the cystic component was 0.8 ± 1.6 (range: -0.5 to 5.3) cm3/year. The CPA diameter of the tumor demonstrated an average linear growth rate of was 1.2 ± 4.4 (range: -8to 13.5) mm/year. With regards to tumor diameter, 9/21 (42.9%) remained stable, 3/21 (14.2%) decreased in size, while 9/21 (42.9%) increased in size. CONCLUSIONS: Cystic tumors demonstrate a wide variability in growth rate. Larger, multi-center studies will be required to further compare this relationship to solid tumors.

Matched Cohort Study of Radiographic Superior Semicircular Canal Dehiscence and Tegmen Dehiscence and Obstructive Sleep Apnea.

OBJECTIVE: To report the frequency of radiographic superior semicircular canal dehiscence (SSCD) and tegmen dehiscence in patients with and without obstructive sleep apnea (OSA). STUDY DESIGN: Retrospective matched cohort study. SETTING: Tertiary care center. PATIENTS: Adults with OSA and fine-cut computed tomographic scans including the temporal bone were matched to patients without OSA by age, sex, and type of computed tomography (protocol, scanner type, slice thickness). Ears with otologic surgery or temporal bone tumors were excluded. MAIN OUTCOME MEASURES: Prevalence of SSCD and tegmen dehiscence assessed by two independent reviewers. RESULTS: The average body mass index of the OSA patients was 29.2 kg/m 2 with an average apnea-hypopnea index of 36.8. The control group had an average body mass index of 26.2 kg/m 2 . Of the 352 temporal bones, 34 (9.7%) had SSCD in the OSA cohort versus 37 (10.5%) in the control group ( p > 0.05). Seven OSA patients (25.6% of those with SSCD) had bilateral SSCD versus 8 controls (27.6% of those with SSCD; p > 0.05). The majority (87.3%) of dehiscences involved the temporal lobe, with the remaining involving the superior petrosal sinus or both. Of the 352 OSA ears, 90 (25.6%) had a tegmen dehiscence versus 95 (27.0%) in the control group ( p > 0.05). Neither group had a laterality preference for SSCD or tegmen dehiscence. CONCLUSION: The prevalence of radiographic SSCD and tegmen dehiscences in OSA patients does not significantly differ from age- and sex-matched controls. This is in contrast to a previous case-control study finding patients with symptomatic SSCD to have higher rates of OSA. This may suggest that the effect size of OSA on SSCD prevalence may be limited despite OSA being a risk factor for elevated intracranial pressure.

Postoperative Venous Thromboembolism after Neurotologic Surgery.

Objective This study aimed to determine the incidence of postoperative venous thromboembolism (VTE) in adults undergoing neurotologic surgery at a single center. Methods The records of adults undergoing neurotologic surgery from August 2009 to December 2016 at a tertiary care hospital were reviewed for VTE within 30 postoperative days. Particular attention was focused on postoperative diagnosis codes, imaging, and a keyword search of postoperative notes. Caprini risk scores were calculated. Results Among 387 patients, 5 experienced postoperative VTE including 3 cases of pulmonary embolism (PE) and 2 cases of isolated deep vein thrombosis (DVT). All patients were given sequential compression devices perioperatively, and none received preoperative chemoprophylaxis. Patients with Caprini score > 8 had a significantly higher rate of VTE compared with those < 8 (12.5 vs. 1%, p = 0.004). Receiver operating characteristic analysis revealed the Caprini risk assessment model to be a fair predictor of VTE, with a C-statistic of 0.70 (95% confidence interval [CI]: 0.49-0.92). Conclusion While no specific validated VTE risk stratification scheme has been widely accepted for patients undergoing neurotologic surgery, the Caprini score appears to be a useful predictor of risk. The benefits of chemoprophylaxis should be balanced with the risks of intraoperative bleeding, as well as the potential for postoperative intracranial hemorrhage.

Loxhd1 Mutations Cause Mechanotransduction Defects in Cochlear Hair Cells.

Sound detection happens in the inner ear via the mechanical deflection of the hair bundle of cochlear hair cells. The hair bundle is an apical specialization consisting of actin-filled membrane protrusions (called stereocilia) connected by tip links (TLs) that transfer the deflection force to gate the mechanotransduction channels. Here, we identified the hearing loss-associated Loxhd1/DFNB77 gene as being required for the mechanotransduction process. LOXHD1 consists of 15 polycystin lipoxygenase α-toxin (PLAT) repeats, which in other proteins can bind lipids and proteins. LOXHD1 was distributed along the length of the stereocilia. Two LOXHD1 mouse models with mutations in the 10th PLAT repeat exhibited mechanotransduction defects (in both sexes). While mechanotransduction currents in mutant inner hair cells (IHCs) were similar to wild-type levels in the first postnatal week, they were severely affected by postnatal day 11. The onset of the mechanotransduction phenotype was consistent with the temporal progression of postnatal LOXHD1 expression/localization in the hair bundle. The mechanotransduction defect observed in Loxhd1-mutant IHCs was not accompanied by a morphologic defect of the hair bundle or a reduction in TL number. Using immunolocalization, we found that two proteins of the upper and lower TL protein complexes (Harmonin and LHFPL5) were maintained in the mutants, suggesting that the mechanotransduction machinery was present but not activatable. This work identified a novel LOXHD1-dependent step in hair bundle development that is critical for mechanotransduction in mature hair cells as well as for normal hearing function in mice and humans.SIGNIFICANCE STATEMENT Hair cells detect sound-induced forces via the hair bundle, which consists of membrane protrusions connected by tip links. The mechanotransduction machinery forms protein complexes at the tip-link ends. The current study showed that LOXHD1, a multirepeat protein responsible for hearing loss in humans and mice when mutated, was required for hair-cell mechanotransduction, but only after the first postnatal week. Using immunochemistry, we demonstrated that this defect was not caused by the mislocalization of the tip-link complex proteins Harmonin or LHFPL5, suggesting that the mechanotransduction protein complexes were maintained. This work identified a new step in hair bundle development, which is critical for both hair-cell mechanotransduction and hearing.

The risk of ergonomic injury across surgical specialties.

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

Direct laryngoscopy assisted fiberoptic intubation: A novel technique for the pediatric airway.

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

Sleep surgery in syndromic and neurologically impaired children.

PURPOSE: To examine surgery performed for obstructive sleep apnea (OSA) in children with syndromic or neurologic comorbidities. MATERIAL AND METHODS: Medical records of 375 children with OSA were retrospectively reviewed, including 142 patients with trisomy 21, 105 with cerebral palsy, 53 with muscular dystrophy, 32 with spinal muscular atrophy, 18 with mucopolysaccharidoses, 14 with achondroplasia, and 11 with Prader-Willi. OUTCOME MEASURES: Apnea-hypopnea index (AHI), complications, length of postoperative stay, and endoscopic findings. RESULTS: 228 patients received 297 surgical interventions, with the remainder undergoing observation or positive pressure ventilation. Adenoidectomy was the most common procedure performed (92.1% of patients), followed by tonsillectomy (91.6%). Average AHI decreased following tonsillectomy, from 12.4 to 5.7 (p = 0.002). The most common DISE finding was the tongue base causing epiglottic retroflexion. Lingual tonsillectomy also resulted in an insignificant decrease in the AHI. CONCLUSIONS: Adenotonsillectomy, when there is hypertrophy, remains the mainstay of management of syndromic and neurologically-impaired children with OSA. However, additional interventions are often required, due to incomplete resolution of the OSA. DISE is valuable in identifying remaining sites of obstruction and guiding future management.

Metformin Potential Impact on the Growth of Vestibular Schwannomas.

OBJECTIVE: Previous work has suggested that metformin may possess antineoplastic properties. This study aims to assess the effect of metformin on the growth of sporadic vestibular schwannomas. METHODS: A retrospective cohort study was performed on patients presenting with radiologically confirmed vestibular schwannomas to Stanford medical center between January 1990 and October 2018. Patients who received metformin during the follow-up period were included and were compared with the control group who were not receiving metformin. Tumor progression and hearing loss are primary and secondary outcomes, respectively. RESULTS: A total of 149 patients were analyzed, with 42 patients receiving metformin. The mean age at presentation is 69.6 (±11.7) years. There are 69 (46.3%) females and 80 (53.7%) males and there is no significant age difference between the groups. Tumor size at presentation is similar between both groups, 8 mm (4-13) in control group and 7.5 mm (4-14) in metformin group. The average follow-up period is 34.2 months (18.3-57.8) and 30.3 months (13.6-69.8) in the metformin and control cohorts, respectively, and they are not significantly different. No significant differences between both groups were found in final American Academy of Otolaryngology - Head and Neck Surgery hearing outcome or poor audiogram outcome. Metformin users are significantly less likely to present with tumor growth at final follow-up compared with nonmetformin users (28.6 versus 49.5%, respectively; p = 0.02). CONCLUSIONS: This preliminary result suggests metformin may reduce vestibular schwannoma tumor growth rate and shows potential promise as a novel chemotherapeutic agent. Further studies are needed to validate this finding.

Neonatal retropharyngeal abscess with complications: Apnea and cervical osteomyelitis.

OBJECTIVE: To evaluate the clinical presentation and management strategies for neonatal retropharyngeal abscess (RPA). METHODS: Retrospective chart review was performed, and literature reviewed. RESULTS: We report two cases of neonatal RPA, with one complicated by cervical osteomyelitis, and the other presenting with apparent life-threatening events (ALTEs). A 6-week-old female underwent transoral drainage of an RPA, which grew methicillin sensitive Staphylococcus aureus. She had a prolonged recovery course and was found to have developed osteomyelitis of the dens and atlas. She was treated with 14 weeks of IV antibiotics and rigid collar fixation for spinal cord instability. A 2-month-old female was admitted after multiple ALTEs with episodes of apnea and pallor. Direct laryngoscopy revealed a bulging RPA, which was drained transorally. This grew multiple organisms including methicillin resistant Staphylococcus aureus, Streptococcal oralis and Prevotella species. CONCLUSIONS: Uncommon in neonates, RPA can present in this age group without fever, and are is likely to have airway complications than in older children. In cases with prolonged recovery, additional diagnostic intervention is recommended to rule out rare complications such as osteomyelitis. Emphasis in such complex cases is placed on a multidisciplinary approach to patient care, coordinating neonatologists, infectious disease specialists, neurosurgeons, and otolaryngologists.

Postoperative Venous Thromboembolism after Extracranial Otologic Surgery.

OBJECTIVE: To determine the incidence of postoperative venous thromboembolism (VTE) in adults undergoing otologic surgery. STUDY DESIGN: Cross-sectional retrospective study. SETTING: Single tertiary academic center. SUBJECTS AND METHODS: Adults undergoing nononcologic, extracranial otologic surgery from August 2009 to December 2016. Patients with postoperative diagnosis VTE codes were identified. Imaging and clinical documents were searched for VTE evidence within the first 30 postoperative days. Methods of thromboprophylaxis were documented, and Caprini risk scores were calculated. RESULTS: In total, 1213 otologic surgeries were evaluated. No postoperative VTE events were identified (0/1268). Mean age was 51.0 ± 17.3 years (range, 18.1-93.4 years). Average length of surgery was 136.0 ± 79.0 minutes (range, 5-768 minutes). The average Caprini score in all patients was 4.0 ± 1.7 (range, 1-15). Eighty-five percent of patients had a Caprini score ≥3, the threshold at which chemoprophylaxis has been recommended in general surgery patients by the American College of Chest Physicians 2012 guidelines. Six patients had documented preoperative chemoprophylaxis and a Caprini score of 4.8 ± 1.7. This was not significantly different from that of patients who did not receive preoperative chemoprophylaxis (t test, P = .3). The literature would estimate a rate of 3.7% VTE in adults with similar Caprini scores undergoing general surgery procedures with no VTE prophylaxis. CONCLUSION: The Caprini risk assessment model may overestimate VTE risk in patients undergoing extracranial otologic surgery. Postoperative VTE following otologic surgery is rare, even in patients traditionally considered moderate or high risk. Chemoprophylaxis guidelines in this group should be balanced against the potential risk of increased intraoperative bleeding and its associated effects on surgical visualization and morbidity.