Faculty Bibliography

OBJECTIVE: Is conscious sedation an effective, safe, and efficient anesthetic choice in patients undergoing select neurotologic and otologic procedures? STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Six patients underwent eight neurotologic procedures including cerebrospinal fluid leak and encephalocele repair, and primary and revision cochlear implant surgery. Patients were deemed poor candidates for general anesthesia secondary to medical comorbidities. These were compared to 11 control patients who underwent same procedures under general anesthesia with intubation. INTERVENTION: Dexmedetomidine infusion was utilized as the primary agent for conscious sedation in this high-risk patient population because, unlike other commonly used sedatives, it preserves normal respirations while providing adequate analgesia. MAIN OUTCOME MEASURES: Preoperative Physiological and Operative Severity Score for the enUmeration of Mortality and Morbidity (P-POSSUM) scores were calculated for the study group. We measured total anesthesia, and perioperative and recovery times. Cost of anesthetic agents was considered and any adverse effects were noted. Patient satisfaction with the operative experience was assessed with telephone surveys. RESULTS: P-POSSUM scores were high for the study group. Postoperative anesthesia was shorter for the study patients undergoing conscious sedation. Difference in cost of anesthetic agents was negligible. The adverse effects were few and as expected for the type of procedure. Patients reported satisfaction and comfort with their operative experience. CONCLUSION: Select neurotologic and otologic procedures can be safely, effectively, and efficiently performed under conscious sedation with dexmedetomidine infusion as the primary anesthetic choice for patients who are deemed poor medical candidates for general endotracheal anesthesia.

OBJECTIVE: The preferential delayed enhancement of the perilymphatic space enables detection of the non-enhancing endolymphatic hydrops present in patients with Meniere's disease. The aim of this study was to evaluate the diagnostic utility of delayed postcontrast 3D FLAIR images and a color map of fused postcontrast FLAIR and constructive interference steady state (CISS) images in the identification of endolymphatic hydrops in patients with clinically diagnosed Meniere's disease. STUDY DESIGN: Case control, blinded study. SETTING: Tertiary referral center. PATIENTS: Ten patients with Meniere's disease and five volunteer controls. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURE: Two neuroradiologists blinded to the clinical history independently evaluated for the presence of endolymphatic hydrops on the images of both inner ears for test and control subjects. Both the standard gray-scale FLAIR images and the fused color map images were independently reviewed. RESULTS: The gray-scale 3D FLAIR images demonstrated 68.2% sensitivity and 97.4% specificity, and the fused color map images demonstrated 85.0% sensitivity and 88.9% specificity in the identification of endolymphatic hydrops in Meniere's disease. There was significant correlation between the gray-scale 3D FLAIR images and fused color map images with the categorization of involvement (p = 0.002). Inter-evaluator reliability was excellent (kappa = 0.83 for gray-scale images, kappa = 0.81 for fused color map). CONCLUSION: Delayed 3D FLAIR and fused 3D FLAIR-CISS color map images of the inner ears after intravenous contrast administration are potentially useful diagnostic tools in the evaluation of patients with suspected Meniere's disease.

PURPOSE OF REVIEW: To summarize the recent medical literature related to the feasibility of hearing preservation after cochlear implantation and to identify variables that may influence hearing preservation in the implanted ear as well as the hearing perception outcomes. RECENT FINDINGS: The published data provide evidence that hearing preservation in the implanted ear yields significantly higher levels of speech recognition in complex listening environments than having just monaural acoustic hearing. There is ample evidence that hearing preservation is feasible and the reported rate is 50-89% in the low frequencies. This rate tends to be stable over time, except for a small percentage that diminishes around 3 months of follow-up. However, factors or variables which influence hearing preservation are yet unknown. Variables such as patient age, degree of residual hearing, insertion depth, insertion speed, duration of profound high frequency deafness and electrode design have been proposed. SUMMARY: Much attention has been focused on hearing preservation in the implanted ear. Thus, investigations into electrode array design, surgical technique and pharmacological management have been undertaken. Minimizing damage in the inner ear not only enhances the possibility for hearing preservation and better performance in complex listening scenarios but also conserves inner ear structures for future treatments.

Background Activation of the mammalian target of rapamycin (mTOR) signaling pathway is thought to be a key driver of tumor growth in Merlin (NF2)-deficient tumors. Everolimus is an oral inhibitor of mTOR complex 1 (mTORC1) with antitumor activity in a variety of cancers. Methods We conducted a single-institution, prospective, 2-stage, open-label phase II study to estimate the response rate to everolimus in neurofibromatosis type 2 (NF2) patients with progressive vestibular schwannoma (VS). Ten eligible patients were enrolled, including 2 pediatric patients. Everolimus was administered at a daily dose of 10 mg (adults) or 5 mg/m(2)/day (children <18 y) orally in continuous 28-day courses, for up to 12 courses. Response was assessed every 3 months with MRI, using 3-dimensional volumetric tumor analysis, and audiograms. Nine patients were evaluable for the primary response, defined as >/=15% decrease in VS volume. Hearing response was evaluable as a secondary endpoint in 8 patients. Results None of the 9 patients with evaluable disease experienced a clinical or MRI response. No objective imaging or hearing responses were observed in stage 1 of the trial, and the study was closed according to predefined stopping rules. Conclusion Everolimus is ineffective for the treatment of progressive VS in NF2 patients. We are currently conducting a pharmacokinetic/pharmacodynamic ("phase 0") study of everolimus in presurgical VS patients to elucidate the biological basis for apparent treatment resistance to mTORC1 inhibition in these tumors.