Cost-Effectiveness of Diffusion-Weighted Magnetic Resonance Imaging Versus Second-Look Surgery in Treating Cholesteatoma: A Modeling Study.

OBJECTIVE: To evaluate whether canal wall-up (CWU) tympanomastoidectomy with diffusion-weighted magnetic resonance imaging (DW-MRI) is a cost-effective method of treating cholesteatoma compared with CWU with second-look surgery. DESIGN AND SETTING: Cost-effectiveness analysis was conducted using a Markov state transition model. The simulation model adhered to the Panel Recommendations on Cost-Effectiveness in Health and Medicine established by the US Public Health Service. One-way and Monte Carlo probability sensitivity analyses were conducted for validation. INTERVENTIONS: Tympanomastoidectomy with DW-MRI versus tympanomastoidectomy with second-look surgery. MAIN OUTCOME MEASURES: Effectiveness and health utility were measured using quality-adjusted life years (QALYs). Costs were derived from Medicare reimbursement using the perspective of the payer. Probabilities for outcomes and complications were taken from existing literature. Cost-effectiveness was assessed using the incremental cost-effectiveness ratio. RESULTS: With base case analysis, the total cost was $15,069 when treated with CWU and second-look surgery versus $13,126 when treated with CWU and DW-MRI. The second-look treatment pathway yielded 17.05 QALYs, whereas the DW-MRI pathway yielded 16.91 QALYs in terms of health benefit accrued across the lifetime of the patient. The cost-effectiveness incremental cost-effectiveness ratio was $21,800/QALY. Using the conventional $50,000 willingness-to-pay threshold, second-look surgery was the more cost-effective approach 63.7% of the time by simulation. CONCLUSIONS: Both treatment pathways were found to be cost-effective, with second-look surgery incrementally cost-effective 63.7% of the time. Assumptions were validated by one-way and Monte Carlo probability sensitivity analysis. PROFESSIONAL PRACTICE GAP AND EDUCATIONAL NEED: There is ample variation in treatment pathways regarding usage of DW-MRI and second-look surgery for cholesteatoma. LEARNING OBJECTIVE: To evaluate the cost-effectiveness of DW-MRI and second-look surgery approaches, accounting for health-related quality-of-life outcomes and costs for the duration of the patient lifetimes. DESIRED RESULT: To inform the discussion on the treatment for cholesteatoma given emergent noninvasive technologies.Level of Evidence: Level III.Indicate IRB or IACUC: Exempt.

Use of Soft Cervical Collar Improves Surgeon Ergonomics During Simulated Otologic Surgery.

OBJECTIVE: To determine whether surgeon use of a soft cervical collar during endoscopic and microscopic otologic surgery is feasible and impacts surgeon ergonomics as measured by inertial sensors. STUDY DESIGN: Prospective crossover trial. SETTING: US-based otolaryngology training program. PATIENTS: Otolaryngology residents and fellows. INTERVENTIONS: Therapeutic-use of a soft cervical collar during simulated otologic surgery. MAIN OUTCOME MEASURES: Time spent in high-risk angles of neck and back flexion and extension; average angle of neck flexion, extension, rotation, and lateral bending; validated assessment of neck pain; average daily phone use. RESULTS: Fifteen subjects met criteria for inclusion. Ten of 15 (67%) were male. Seven of 15 (47%) were postgraduate year 1-2. Seven of 15 (47%) reported a history of neck pain. None reported prior spinal steroid injections or surgery. Across all subjects, use of the soft cervical collar significantly reduced time spent in high-risk angles of neck flexion/extension during both endoscopic (56% vs. 35%, p < 0.05) and microscopic (60% vs. 32%, p < 0.05) otologic surgery. There was no effect on back flexion or extension. There was no difference in time spent in high-risk neck or back angles between endoscopic and microscopic surgery. Average angles of neck or back flexion, extension, lateral bending, and rotation were not significantly different for subgroups with more operative experience, increased phone use, perception of good posture, or history of neck pain. CONCLUSIONS: Use of a soft cervical collar during simulated otologic surgery significantly reduced time spent in high-risk neck positions. These data support feasibility of soft collar use during otologic surgery and hold promise for reduction in the high rates of neck pain reported by neurotologists. PROFESSIONAL PRACTICE GAP AND EDUCATIONAL NEED: Improving surgeon ergonomics for otologic surgery. LEARNING OBJECTIVE: To identify a therapeutic intervention to mitigate neck pain in surgeons caused by assumption of high-risk cervical neck flexion and extension. DESIRED RESULT: To demonstrate that use of a readily available soft cervical collar reduces risk of neck pain in otologic surgeons. LEVEL OF EVIDENCE: II. INDICATE IRB OR IACUC: Exempt.

Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement.

Adult and paediatric patients with pathogenic variants in the gene encoding succinate dehydrogenase (SDH) subunit B (SDHB) often have locally aggressive, recurrent or metastatic phaeochromocytomas and paragangliomas (PPGLs). Furthermore, SDHB PPGLs have the highest rates of disease-specific morbidity and mortality compared with other hereditary PPGLs. PPGLs with SDHB pathogenic variants are often less differentiated and do not produce substantial amounts of catecholamines (in some patients, they produce only dopamine) compared with other hereditary subtypes, which enables these tumours to grow subclinically for a long time. In addition, SDHB pathogenic variants support tumour growth through high levels of the oncometabolite succinate and other mechanisms related to cancer initiation and progression. As a result, pseudohypoxia and upregulation of genes related to the hypoxia signalling pathway occur, promoting the growth, migration, invasiveness and metastasis of cancer cells. These factors, along with a high rate of metastasis, support early surgical intervention and total resection of PPGLs, regardless of the tumour size. The treatment of metastases is challenging and relies on either local or systemic therapies, or sometimes both. This Consensus statement should help guide clinicians in the diagnosis and management of patients with SDHB PPGLs.

Clinical consensus guideline on the management of phaeochromocytoma and paraganglioma in patients harbouring germline SDHD pathogenic variants.

Patients with germline SDHD pathogenic variants (encoding succinate dehydrogenase subunit D; ie, paraganglioma 1 syndrome) are predominantly affected by head and neck paragangliomas, which, in almost 20% of patients, might coexist with paragangliomas arising from other locations (eg, adrenal medulla, para-aortic, cardiac or thoracic, and pelvic). Given the higher risk of tumour multifocality and bilaterality for phaeochromocytomas and paragangliomas (PPGLs) because of SDHD pathogenic variants than for their sporadic and other genotypic counterparts, the management of patients with SDHD PPGLs is clinically complex in terms of imaging, treatment, and management options. Furthermore, locally aggressive disease can be discovered at a young age or late in the disease course, which presents challenges in balancing surgical intervention with various medical and radiotherapeutic approaches. The axiom-first, do no harm-should always be considered and an initial period of observation (ie, watchful waiting) is often appropriate to characterise tumour behaviour in patients with these pathogenic variants. These patients should be referred to specialised high-volume medical centres. This consensus guideline aims to help physicians with the clinical decision-making process when caring for patients with SDHD PPGLs.

Ergonomic Analysis of Otologic Surgery: Comparison of Endoscope and Microscope.

OBJECTIVE: The comparative postural health of surgeons performing endoscopic and microscopic otologic surgeries has been a topic of active debate, with many nascent or anecdotal reports suggesting the latter encourages suboptimal ergonomics. Using inertial body sensors to measure joint angles, this study sought to objectively evaluate and compare the ergonomics of surgeons during endoscopic and microscopic otologic surgeries. STUDY DESIGN: Prospective pilot trial. SETTING: Large, multicenter, academic hospital system. Performed 21 otologic operations (10 endoscopic and 11 microscopic) in November 2020 and January 2021. All attendings were fellowship trained in otology/neurotology. SUBJECTS: Eight otolaryngologists (four attendings and four residents) performing 21 otologic surgeries (11 microscopic and 10 endoscopic). INTERVENTION: Approach to otologic surgery: endoscope or microscope. MAIN OUTCOME MEASURES: Surgeons' neck and back angles while wearing ergonomic sensors affixed to either side of each major joint, mental and physical burdens and pain after each surgery (via modified NASA Task Load Index). RESULTS: Residents' necks (9.54° microscopic vs. -4.79° endoscopic, p = 0.04) and backs (16.48° microscopic vs. 3.66° endoscopic, p = 0.01) were significantly more flexed when performing microscopic surgery than when performing endoscopic surgery, although attending neck and back flexion were comparable during microscopic and endoscopic surgeries. Attendings reported significantly higher pain levels after operating microscopically than after operating endoscopically (0.13 vs. 2.76, p = 0.01). CONCLUSIONS: Residents were found to operate with significantly higher risk back and neck postures (as defined by the validated ergonomic tool, Rapid Entire Body Assessment) when operating microscopically. Attendings reported significantly higher levels of pain after operating microscopically versus endoscopically, suggesting that the suboptimal microscopic postures adopted earlier in training may pose an indelible risk later in a surgeon's career.

Translabyrinthine Approach for Sporadic Vestibular Schwannoma: Patient Selection, Technical Pearls, and Patient Outcomes.

Herein we briefly describe the translabyrinthine approach to vestibular schwannoma resection as well as a focused literature review as to the best candidates, technical recommendations, and key outcomes with respect to other approaches.

A Case of HPV+ Squamous Cell Carcinoma of the Perigeniculate Area and Middle Fossa.

Herein we present an unusual case of a primary HPV+ perigeniculate, extra-axial middle fossa skull base tumor and our management thereof. Laryngoscope, 133:3158-3160, 2023.

Defining the Learning Curve for Endoscopic Ear Skills Using a Modular Trainer: A Multi-Institutional Study.

OBJECTIVE: Quantify the learning curve for endoscopic ear skills acquisition in otolaryngology residents using a simulator. The secondary objective was to determine if demographic factors or previous endoscopic experience influenced skill development. STUDY DESIGN: Prospective, multicenter study. Resident participants each completed 10 amassed trials using a validated endoscopic ear skill trainer. SETTING: Two academic teaching hospitals. SUBJECTS: Otolaryngology residents. MAIN OUTCOME MEASURES: Trial completion times; rate of improvement over time. RESULTS: Thirty-eight residents completed the study, 26 from program A and 12 from program B. Fifteen participants were women and 23 were men. Mean age was 30 years old (range 26 to 34 years). Previous experience with otoendoscopy (B = -16.7, p = 0.005) and sinus endoscopy (B = -23.4, p = 0.001) independently correlated with lower overall trial times. Age, gender, postgraduate year, handedness, interest in otology, and video gaming were not associated with trial times. On multivariate logistic regression, resident completion times improved with trial number, and residents without previous endoscopy experience improved at a faster rate than those with experience ( p < 0.001). CONCLUSIONS: Novice surgeons may acquire basic endoscopic ear experience with self-directed simulation training. The learning curve for transcanal endoscopic ear surgery is comparable to those demonstrated for other otologic surgeries, and specific task competencies can be achieved within 10 trials, suggesting that previous experiences, or lack thereof, may not dictate the ability to acquire new skills. There may be a translational value to previous endoscopic sinus experience on learning transcanal endoscopic ear surgery.

Institutional Experience With Cochlear Implants Falling Under the 2020 FDA Corrective Action.

OBJECTIVE: To determine the rate of device failure for those cochlear implants falling under the 2020 Food and Drug Administration (FDA) voluntary corrective action. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary otology-neurotology practice. PATIENTS: Those with cochlear implant failure falling under the FDA corrective action. INTERVENTIONS: Cochlear implant explant and reimplantation. OUTCOME MEASURES: Reason for cochlear implant failure, time to failure, symptoms of failure, and benefit from reimplantation. RESULTS: The overall failure rate was 20.0% (18 of 90 ears); of the failures, 15 of 18 (83.3%) were hard device failures, and 3 of 18 (16.7%) were medical or surgical failures. All hard device failures were confirmed with integrity testing as performed by the company. The average time to integrity testing was 38.0 months. Of the hard failures, 14 of 15 had successful initial activation and benefit. Lack of expected progress was seen in 7 of 15 and a sudden decline in function in 8 of 15. Electrodes 9 to 16 were most often defunct. Significant drops in speech perception were often seen in device failure cases. Three medical/surgical failures were explanted; one had migration of the receiver/stimulator causing discomfort, and the other two had electrode migration after partial insertion. Of the reimplanted patients, 11 of 12 are deriving benefit from their new devices. CONCLUSIONS: The rate of device failure for the cochlear implants of interest is significantly higher in our series than reported in the initial FDA voluntary field corrective action publication.

Cochlear Implantation in Adults With Single-Sided Deafness: A Systematic Review and Meta-analysis.

OBJECTIVE: To determine the clinical outcomes of adult patients with single-sided deafness (SSD) undergoing ipsilateral cochlear implantation. DATA SOURCE: An electronic search of Medline and Embase articles. REVIEW METHODS: A systematic review was performed with a search strategy developed by a licensed librarian to identify studies of adult patients with SSD who underwent ipsilateral cochlear implantation. Articles were managed in Covidence and evaluated by 2 independent reviewers. Risk of bias was assessed and data were extracted, including patient demographics, etiology of deafness, duration of deafness, and postoperative change in speech recognition, tinnitus, sound localization, and quality of life (QoL). A meta-analysis was performed, and pooled mean differences were calculated for each outcome of interest via random effects models by each outcome, as well as subgroup analyses by the individual clinical score used. RESULTS: Of 2309 studies identified, 185 full texts were evaluated, and 50 were ultimately included involving 674 patients. Speech perception scores in quiet and noise, tinnitus control, sound localization, and QoL all significantly improved after implantation. Pooled outcomes demonstrated score improvements in speech perception (standardized mean difference [SMD], 2.8 [95% CI, 2.16-3.43]), QoL (SMD, 0.68 [95% CI, 0.45-0.91]), sound localization (SMD, -1.13 [95% CI, -1.68 to -0.57]), and tinnitus score reduction (SMD, -1.32 [95% CI, -1.85 to -0.80]). CONCLUSIONS: Cochlear implantation in adults with SSD results in significant improvements in speech perception, tinnitus control, sound localization, and QoL.

Initial Experience With Two Active Transcutaneous Bone-Anchored Hearing Implants.

OBJECTIVE: To analyze our institutional experience with two active transcutaneous bone-anchored hearing aids. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary academic otology-neurotology practice. PATIENTS: Patients with conductive or mixed hearing losses meeting criteria to receive active transcutaneous bone-anchored hearing aids. INTERVENTIONS: Implantation with one of two active transcutaneous bone-anchored hearing aids. OUTCOME MEASURES: Operative time, dural exposure and decompression, use of lifts, implant position, ease of use, qualitative patient satisfaction, complication rates. RESULTS: Ten patients received Implant 1 and 11 patients received Implant 2. The most common underlying etiologies of hearing loss were chronic suppurative otitis media in 33.3%, atresia/microtia in 23.8%, and cholesteatoma in 23.8%. Average operative times were 99.3 minutes for Implant 1 and 80.9 minutes for Implant 2 ( p = 0.263). Implant 1 required lifts in 60%, dural exposure in 50%, and dural compression in 30%. Overall, placement was difficult in 47.6% of cases. There were no intraoperative complications. There were higher rates of issues with sound quality (27.3% versus 0.0%, p = 0.123) and poor cosmesis (36.4% versus 10.0%, p = 0.360) with Implant 2. Functional gain was not recorded, but all patients derived qualitative benefit from their implant. Three patients had local wound complications that self-resolved or were treated with antibiotics. One patient implanted at an outside institution required explant because of multiple infections. CONCLUSIONS: There were no statistically significant differences in outcomes comparing Implants 1 and 2; however, Implant 2 had much higher rates of issues with audio quality and poor cosmesis. Placing Implant 1 often required special techniques.

Sharing in-office otoendoscopy recordings may improve patient satisfaction: A prospective cohort study.

OBJECTIVE: Evaluate the impact of sharing otoendoscopy exams on patient satisfaction in the outpatient clinical setting. METHODS: Randomized, prospective cohort study. Consecutive adults presenting to otology clinic at one tertiary referral center were randomized into two groups: standard microscopy (SM) and video otoendoscopy (VO). The SM group had ears examined using a standard, otomicroscope; the VO group had ears examined using a 0° rigid endoscope connected to a video tower. All subjects were counseled on their exam findings in a routine manner; the VO group was concurrently shown a recording of their ear exam. All subjects completed the 18-item Patient Satisfaction Questionnaire (PSQ-18) at the conclusion of their clinic visit. RESULTS: The SM group consisted of 27 patients and VO group consisted of 23 patients. VO subjects reported higher PSQ-18 scores compared to SM subjects within the domains of communication (p = 0.04) and technical quality (p = 0.005). On linear regression models, demographic factors and positive exam findings were not predictive of patient satisfaction. CONCLUSIONS: Sharing otoendoscopy recordings may be a valuable tool that can improve patient satisfaction. PRACTICE IMPLICATIONS: Clinicians should consider sharing recordings of otoscopic exams with patients, particularly when faced with the possibility of surgery.

Cost-effectiveness of Canal Wall-Up vs Canal Wall-Down Mastoidectomy: A Modeling Study.

OBJECTIVE: To assess the relative lifetime costs, benefits, and cost-effectiveness between the 2 approaches, canal wall-up (CWU) and canal wall-down (CWD) tympanomastoidectomy, used in the treatment of cholesteatomas. STUDY DESIGN: Markov state transition model. SETTING: Tertiary academic health system. METHODS: A Markov state transition model was used to simulate outcomes across the patient lifetime. Outcome and complication probabilities were obtained from the existing literature. Costs were calculated from the payer perspective, with procedure, hospital, clinic, and physician cost derived from Medicare reimbursement. Quality-adjusted life years (QALYs) were used to represent effectiveness and utility. One-way and probability sensitivity analyses (PSAs) were conducted. RESULTS: The base case analysis, assuming a 40-year-old patient, yielded a lifetime cost of $14,214 for a patient treated with the CWU approach assuming second-look surgery and $22,290 with a CWD approach. CWU and CWD generated a benefit of 17.11 and 17.30 QALYs, respectively. The incremental cost-effectiveness ratio for CWU was $43,237 per QALY. The Monte Carlo PSA validated the base case scenario. Using a standard $50,000 willingness-to-pay threshold, CWD was the more cost-effective approach and was selected 54.8% of the time by the simulation. CONCLUSION: Both CWU and CWD were found to be cost-effective, with CWD being cost-effective 54.8% of the time at a WTP threshold of $50,000. The assumptions used in the analysis were validated by the results of 1-way and PSA.

Outcomes in primary and revision surgery for pediatric onset stapedial pathology.

OBJECTIVES: To analyze audiometric outcomes of surgery for pediatric onset stapedial pathology (POSP). STUDY DESIGN: Retrospective cohort study. SETTING: Single-institution database. METHODS: Retrospective analysis of 809 stapes procedures performed at a single high-volume tertiary referral otology practice, 75 of which were POSP cases. RESULTS: Oval window drillout for thick footplate and aborting the procedure were more common in POSP cases compared to the rest of the cohort (28.0% versus 9.8% [p < .001] and 5.3% versus 1.2% [p = .007], respectively). Postoperative complications were rare. Postoperative Air-Bone Gap (pABG) closure to ≤20 dB was significantly lower in the POSP group (80.0% versus 89.0%, p = .021). Rates of sensorineural hearing loss (SNHL) were not different between the two groups. Poor audiometric outcomes in the POSP group were largely driven by revision cases; pABG≤10 dB was 60.3% in primary cases but only 11.8% in revisions (p < .001), and postoperative SNHL was significantly higher in revisions (29.4% versus 0.0%, p < .001). In multivariate analysis, POSP was not a predictor of successful closure of the pABG at either level, nor did it predict significant postoperative SNHL. CONCLUSIONS: Surgery for pediatric onset stapedial pathology had significantly worse audiometric outcomes, particularly in revision cases, as compared to the rest of the cohort.

Endoscopic Versus Microscopic Stapedotomy: A Single-Blinded Randomized Control Trial.

OBJECTIVE: To demonstrate non-inferiority of endoscopic stapedotomy to microscopic stapedotomy for the treatment of otosclerosis. STUDY DESIGN: Single-blinded randomized control trial. SETTING: Tertiary, academic otology-neurotology practice. PATIENTS: Adult subjects with a diagnosis of otosclerosis and a preoperative air-bone gap (ABG) more than or equal to 20 dB undergoing primary stapedotomy. INTERVENTION: Endoscopic or microscopic stapedotomy. MAIN OUTCOME MEASURES: Primary audiometric outcome was postoperative ABG. Secondary audiometric outcomes included speech reception threshold (SRT), word recognition score (WRS), bone- and air-conduction pure tone averages (PTA), change in ABG, and ABG closure rates to less than or equal to 10 dB and less than or equal to 20 dB. RESULTS: Twenty-two patients were recruited. Eleven patients underwent endoscopic stapedotomy and 11 underwent microscopic stapedotomy. The endoscopic group was non-inferior to the microscopic group in terms of postoperative audiometric outcomes (endoscope versus microscope, p-value): ABG (8.1 dB versus 8.1 dB, <0.001), SRT (27.7 dB versus 25.9 dB, <0.001), WRS (92% at 65 dB versus 98% at 62 dB, <0.001), air-conduction PTA (33.5 dB versus 30.8 dB, <0.01), and change in ABG (23.0 dB versus 20.7 dB, <0.0001). ABG closure rates to less than or equal to 10 dB (72.7% versus 81.2%, p = 1.0) and less than or equal to 20 dB (90.9% versus 100%, p = 1.0) were not significantly different. There was no significant difference in operative time, necessity of scutum curettage, or postoperative dysgeusia. No patients required chorda tympani sacrifice. Preoperative tinnitus resolved in three patients in each group postoperatively. CONCLUSIONS: This study is the first randomized control trial to demonstrate non-inferiority of endoscopic to microscopic stapedotomy.

Opioids Are Infrequently Required following Ambulatory Otologic Surgery.

OBJECTIVE: To determine the frequency with which postoperative opioid prescriptions are required after ambulatory otologic surgery. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary otology-neurotology practice. PATIENTS: Patients (n = 447) given over-the-counter acetaminophen and ibuprofen following ambulatory otologic surgery between July 1, 2018 and June 30, 2020. INTERVENTION: Opioid prescription upon request. MAIN OUTCOME MEASURES: Patient, disease, and surgical variables such as age, sex, past medical history, chronic pain condition, surgical procedure, primary versus (vs.) revision surgery, and endoscopic vs. microscopic approach were examined for relationship to ad hoc opioid prescription rate. RESULTS: Of 370 adult patients (mean age 49.0 yrs, range 18.0-88.5 yrs), 75 (20.3%) were prescribed opioids for postoperative pain, most commonly oxycodone-acetaminophen 5/325 mg. Of 77 pediatric patients (mean age 8.8 yrs, range 0.7-17.9 yrs), 5 (6.5%) were prescribed postoperative opioid analgesia. In the adult population, chronic pain condition, pain medication use at baseline, canal wall up mastoidectomy, tympanoplasty, tympanomeatal flap, bone removal of the mastoid, postauricular incision, and intraoperative microscopy were independent predictors of opioid pain prescription. When controlling for all significant variables, only chronic pain condition remained significant (odds ratio = 3.94; p = 0.0007). In the pediatric population, atresiaplasty, meatoplasty, and conchal cartilage removal were independently associated with opioid prescription, but none remained significant when analyzed in a multivariate linear model. CONCLUSIONS: Pain following ambulatory otologic surgery may be adequately managed with over-the-counter pain medications in the majority of cases. Opioids may be necessary in adults with preexisting pain conditions.

Is There a Role for Intraoperative Navigation During Cochlear Implantation in Patients With Aural Atresia?

OBJECTIVE: Concurrent bilateral congenital aural atresia (CAA) and profound sensorineural hearing loss are rare. While not a contraindication, temporal bone and cochleovestibular abnormalities are an important consideration for cochlear implantation (CI) candidacy. Intraoperative image-guided surgical navigation may play a role during CI surgery in patients with complex anatomy, such as CAA. PATIENT: One patient with bilateral CAA, cochlear dysplasia, speech delay, and profound sensorineural hearing loss underwent candidacy evaluation for cochlear implantation. INTERVENTIONS: Cochlear implantation using intraoperative image-guided navigation. MAIN OUTCOME MEASURES: (1) Registration accuracy, (2) surgical outcomes, (3) audiometry. RESULTS: A four-year-old girl with complete bilateral CAA and profound sensorineural hearing loss successfully underwent a right transmastoid approach for CI using intraoperative image-guided navigation with sticker fiducials. Bony landmarks included the mastoid tip, tympanomastoid suture line, helical root, zygomatic root, and lateral brow. A registration accuracy of 0.9 mm was achieved. There were no intraoperative or immediate postoperative complications. Postoperatively, Neural Response Imaging was confirmed on 9 electrodes and behavioral testing demonstrated Ling-6 access at 30 dB. On most recent follow-up, she has demonstrated gains in language development, vocalizations, and uses total communication in a hearing-impaired educational environment. CONCLUSIONS: Children with CAA and profound sensorineural hearing loss may be candidates for cochlear implantation, with successful outcomes in the setting of complex anatomy. Surgical navigation may play a role corroborating intraoperative landmarks.

Intraoperative navigation during atresiaplasty for congenital aural atresia.

OBJECTIVE: The purpose of this study was to evaluate the feasibility of intraoperative navigation (ION) using adhesive fiducials and high-resolution computed tomography (HRCT) of the temporal bone in pediatric patients undergoing atresiaplasty for congenital aural atresia (CAA). METHODS: From June 2018 to August 2019, a retrospective review was performed on pediatric patients with unilateral or bilateral CAA who underwent atresiaplasty with or without concurrent bone anchored implant (BAI) placement. Single stage atresiaplasty was performed at a tertiary referral center with ION linked to image-guidance compatible HRCT non-contrast temporal bone images. Up to six adhesive fiducials were placed for navigation. Patient demographics were collected including Schuknecht classification type and Jahrsdoerfer score. Main outcome measures included the number of useable adhesive fiducials for navigation, navigation system registration accuracy, operative time, intraoperative complications, and postoperative outcomes including facial nerve function, surgical site infection rate, restenosis rate, and audiometric data. RESULTS: Five patients (3 male, 1 AU atresia, 2 AS atresia, all Schuknecht type C) with an average age of 9.2 years (range 6.8-11.8 years) underwent single sided atresiaplasty with ION. Two patients underwent concurrent BAI placement. Average Jahrsdoerfer score was 8.6 (range 7-10). Preoperative audiogram demonstrated a mean air-bone gap (ABG) of 45 dB (range 35-54 dB). The navigation system registration accuracy ranged from 0.08 to 1.80 mm (mean 1.00 mm). Mean operative time was 268 min (range 217-307 min). There were no intraoperative complications. At an average follow up of 7.2 months (range 2-15 months), postoperative facial nerve function was normal in all patients and no patients developed postoperative stenosis or surgical site infections. Four patients underwent postoperative audiogram at an average of 4 months after surgery (range 2-5 months) and all demonstrated an ABG of less than 30 dB (mean 20 dB, range 15-26 dB) with an average improvement in ABG of 23 dB (range 11-39 dB). The one patient who did not undergo postoperative pure tone audiometry underwent AzBio speech perception testing and demonstrated an improvement from 81% to 89%. CONCLUSIONS: The use of ION in otologic surgery is uncommon. Inherent aberrant temporal bone anatomy in CAA makes this a unique population to study the value of this technology. The use of adhesive fiducials is feasible, with navigation registration accuracy and surgical outcomes comparable to those in the literature. More data is necessary regarding the impact of ION on long-term surgical and audiometric outcomes.