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Objective Multiple short series have evaluated the efficacy of salvage microsurgery (MS) after stereotactic radiosurgery (SRS) for treatment of vestibular schwannomas (VSs); however, there is a lack of a large volume of patient data available for interpretation and clinical adaptation. The goal of this study was to provide a comprehensive review of tumor characteristics, management, and surgical outcomes of salvage of MS after SRS for VS. Methods The Medline/PubMed, Scopus, CINAHL, Cochrane Library, and Google Scholar databases were queried according to PRISMA guidelines. All English-language and translated publications were included. Studies lacking adequate study characteristics and outcomes were excluded. Cases involving neurofibromatosis type 2, previous MS, or malignant transformation were excluded when possible. Results Twenty studies containing 297 cases met inclusion criteria. Three additional cases from Rush University Medical Center were added for 300 total cases. Tumor growth with or without symptoms was the primary indication for salvage surgery (92.3% of cases), followed by worsening of symptoms without growth (4.6%) and cystic enlargement (3.1%). The average time to MS after SRS was 39.4 months. The average size and volume of tumor at surgery were 2.44 cm and 5.92 cm3, respectively. The surgical approach was retrosigmoid (42.8%) and translabyrinthine (57.2%); 59.5% of patients had a House-Brackmann (HB) grade of I or II. The facial nerve was preserved in 91.5% of cases. Facial nerve preservation and HB grades were lower for the translabyrinthine versus retrosigmoid approach (p = 0.31 and p = 0.18, respectively); however, fewer complications were noted in the translabyrinthine approach (p = 0.29). Gross-total resection (GTR) was completed in 55.7% of surgeries. Studies that predominantly used subtotal resection (STR) were associated with a lower rate of facial nerve injury (5.3% vs 11.3%, p = 0.07) and higher rate of HB grade I or II (72.9% vs 48.0%, p = 0.00003) versus those using predominantly GTR. However, majority STR was associated with a recurrence rate of 3.6% as compared to 1.4% for majority GTR (p = 0.29). Conclusions This study showed that the leading cause of MS after SRS was tumor growth at an average of 39.4 months after radiation. There were no significant differences in outcomes of facial nerve preservation, postoperative HB grade, or complication rate based on surgical approach. Patients who underwent STR showed statistically significant better HB outcomes compared with GTR. MS after SRS was considered by most authors to be more difficult than primary MS. These data support the notion that the surgical goals of salvage surgery are debulking of tumor mass, decreasing compression of the brainstem, and not necessarily pursuing GTR. Abbreviations CK = CyberKnife; fSRT = fractionated stereotactic radiotherapy; GKS = Gamma Knife surgery; GTR = gross-total resection; HB = House-Brackmann; LINAC = linear accelerator; MS = microsurgery; NF2 = neurofibromatosis type 2; SRS = stereotactic radiosurgery; STR = subtotal resection; VS = vestibular schwannoma.

OBJECTIVE To characterize the radiologic findings of petrous apex cephalocele (PAC) in a patient cohort, and report the surgical management for three symptomatic PAC patients and cerebrospinal fluid (CSF) leak via the middle cranial fossa approach. STUDY DESIGN Retrospective case series. SETTING Academic center. PATIENTS Thirty-five patients with PAC were identified by review of the imaging archive between 2008 and 2019 (29 females; mean, 55 yrs; range, 4-86 yrs). All patients underwent magnetic resonance imaging of the skull base and/or computed tomography examination. INTERVENTIONS Surgical repair of PAC. MAIN OUTCOME MEASURES Radiologic features of PAC. RESULTS Radiological features of PAC: 25.7% bilateral; partial or expanded empty sella in 82.9%; arachnoid pits in 14.2%; and enlarged CSF space of optic nerve sheath in 20.0%. Coexisting pathology included temporal, sphenoid, and bilateral jugular foramen meningocele; as well as cribriform, middle crania fossa, and right temporal defect. Three case studies describing the surgical course of spontaneous CSF leak secondary to PAC were managed with the middle cranial fossa approach. CONCLUSIONS PAC is an exceedingly unusual cause for CSF leak in the adult and pediatric population. A middle fossa approach may be used to treat CSF leak as a result of PAC. Typically, CSF otorrhea originates from an encephalocele that extends via a bony defect in the tegmen tympani or tegmen mastoideum. However, on occasion the source of the CSF otorrhea is not via the tegmen, instead defects in the middle fossa floor, medial to the ridge for the gasserian ganglion (tubercle of Princeteau), need to be considered.

Objectives: Historically, eventual loss of cochlear nerve function has limited patients with neurofibromatosis type 2 (NF2) to auditory brainstem implants (ABI), which in general are less effective than modern cochlear implants (CI). Our objective is to evaluate hearing outcomes following ipsilateral cochlear implantation in patients with NF2 and irradiated vestibular schwannomas (VS), and sporadic VS that have been irradiated or observed. Methods: Multi-center retrospective analysis of ipsilateral cochlear implantation in the presence of observed and irradiated VS. MESH search in NCBI PubMed database between 1992 and 2019 for reported cases of cochlear implantation with unresected vestibular schwannoma. Results: Seven patients underwent ipsilateral cochlear implantation in the presence of observed or irradiated vestibular schwannomas. Four patients had sporadic tumors with severe-profound contralateral hearing loss caused by presbycusis/hereditary sensorineural hearing loss, and three patients with NF2 lost contralateral hearing after prior surgical resection. Prior to implantation, one VS was observed without growth for a period of 7 years and the others were treated with radiotherapy. Mean post-operative sentence score was 63.9% (range 48-91) at an average of 28 (range 2-84) months follow up. All patients in this cohort obtained open set speech perception. While analysis of the literature is limited by heterogenous data reporting, 85% of implants with observed schwannomas achieved some open set perception, and 67% of patients previously radiated schwannomas. Furthermore, blending literature outcomes for post implantation sentence testing in quiet without lip-reading show 59.0 ± 35% for patients with CI and observed tumors and 55.7 ± 35% for patients with radiated tumors, with both groups ranging 0 to 100%. Conclusion: This retrospective series and literature review highlight that hearing outcomes with CI for VS patients are superior to those achieved with ABI. However, important considerations including imaging, delayed hearing loss, and observation time cannot be ignored in this population.

Objective The objective of this study was to assess the strategic changes implemented in the departmental mission to continue safe delivery of otolaryngology care and to support the broader institutional mission during the COVID-19 pandemic response. Study Design Retrospective assessment was performed to the response and management strategy developed to transform the clinical and academic enterprise. Setting Large urban tertiary care referral center. Results The departmental structure was reorganized along new clinical teams to effectively meet the system directives for provision of otolaryngology care and support for inpatient cases of COVID-19. A surge deployment schedule was developed to assist frontline colleagues with clinical support as needed. Outpatient otolaryngology was consolidated across the system with conversion of the majority of visits to telehealth. Operative procedures were prioritized to ensure throughput for emergent and time-critical urgent procedures. A tracheostomy protocol was developed to guide management of emergent and elective airways. Educational and research efforts were redirected to focus on otolaryngology care in the clinical context of the COVID-19 crisis. Conclusion Emergence of the COVID-19 global health crisis has challenged delivery of otolaryngology care in an unparalleled manner. The concerns for preserving health of the workforce while ethically addressing patient career needs in a timely manner has created significant dilemmas. A proactive, thoughtful approach that reorganizes the overall departmental effort through provider and staff engagement can facilitate the ability to meet the needs of otolaryngology patients and to support the greater institutional mission to combat the pandemic.

Background Deep brain stimulation (DBS) surgery in patients with pre-existing cochlear implants (CIs) poses various challenges. We previously reported successful magnetic resonance imaging (MRI)-based, microelectrode recording (MER)-guided subthalamic DBS surgery in a patient with a pre-existing CI. Other case reports have described various DBS procedures in patients with pre-existing CIs using different techniques, leading to varying issues to address. A standardized operative technique and workflow for DBS surgery in the setting of pre-existing CIs is much needed. Objective To provide a standardized operative technique and workflow for DBS lead placement in the setting of pre-existing CIs. Methods Our operative technique is MRI-based and MER-guided, following a workflow involving coordination with a neurotology team to remove and re-implant the internal magnets of the CIs in order to safely perform DBS lead placement, altogether within a 24-h time frame. Intraoperative nonverbal communication with the patient is easily possible using a computer monitor. Results A 65-yr old woman with a 10-yr history of craniocervical dystonia and pre-existing bilateral CIs underwent successful bilateral pallidal DBS surgery at our institution. No merging errors or difficulties in targeting globus pallidus internus were experienced. Also, inactivated CIs do not interfere with MER nor with stimulation, and intraoperative communication with the patient using a computer monitor proved feasible and satisfactory. Conclusion DBS procedures are safe and feasible in patients with pre-existing CIs if precautions are taken following our workflow.

Background Facial paralysis can drastically diminish satisfaction in one's social interactions and overall quality of life. Bell palsy is the most common cause of facial palsy, however, a diagnosis of "atypical" BP may originate from an entirely different pathological process. This case highlights a rare case of facial nerve paraganglioma, initially misdiagnosed as BP, that resulted in facial paralysis from neoplastic invasion of the facial nerve. Case presentation A 66-year old Hispanic woman with systemic lupus erythematosus presented to the plastic surgery clinic with complaints of drooling and being unable to smile. She experienced several episodes of left facial paralysis and was diagnosed with BP at an outside institution. Each episode was only partially responsive to steroid therapy. Imaging at our institution demonstrated lobulated enhancement along the vertical and extratemporal segments of the facial nerve, which prompted surgical intervention. The patient underwent left transmastoid approach for removal of the lesion involving the facial nerve followed by facial nerve reanimation via gracilis free flap without complication. Conclusions This report outlines an extraordinarily rare case of a patient with facial nerve paraganglioma. This case represents the importance of reconstructive surgeons in considering a thorough diagnostic work-up with imaging and histopathology in the setting of idiopathic facial paralysis. Successful collaboration between otolaryngology and plastic surgery made streamlined diagnosis and surgical treatment of this unique case possible.

To highlight emerging preoperative screening protocols and document workflow challenges and successes during the early weeks of the COVID-19 pandemic.This retrospective cohort study was conducted at a large urban tertiary care medical center. Thirty-two patients undergoing operative procedures during the COVID-19 pandemic were placed into 2 preoperative screening protocols. Early in the pandemic a "high-risk case protocol" was utilized to maximize available resources. As information and technology evolved, a "universal point-of-care protocol" was implemented.Of 32 patients, 25 were screened prior to surgery. Three (12%) tested positive for COVID-19. In all 3 cases, the procedure was delayed, and patients were admitted for treatment or discharged under home quarantine. During this period, 86% of operative procedures were indicated for treatment of oncologic disease. There was no significant delay in arrival to the operating room for patients undergoing point-of-care screening immediately prior to their procedure (Currently, few studies address preoperative screening for COVID-19. A substantial proportion of individuals in this cohort tested positive, and both protocols identified positive cases. The major strengths of the point-of-care protocol are ease of administration, avoiding subsequent exposures after testing, and relieving strain on "COVID-19 clinics" or other community testing facilities.Preoperative screening is a critical aspect of safe surgical practice in the midst of the widespread pandemic. Rapid implementation of universal point-of-care screening is possible without major workflow adjustments or operative delays.

Objectives This study is aimed to report the largest independent case series of spontaneous otogenic pneumocephalus (SOP) and review its pathophysiology, clinical presentation, and treatment. Design Four patients underwent a middle cranial fossa approach for repair of the tegmen tympani and tegmen mastoideum. A comprehensive review of the literature regarding this disease entity was performed. Setting U.S. tertiary academic medical center. Participants: Patients presenting to the lead author's clinic or to the emergency department with radiographic evidence of SOP. Symptoms included headache, otalgia, and neurologic deficits. Main Outcome Measures Patients were assessed for length of stay, postoperative length of stay, and neurologic outcome. Three of four patients returned to their neurologic baseline following repair. Results Four patients were successfully managed via a middle cranial fossa approach to repairing the tegmen mastoideum. Conclusion The middle cranial fossa approach is an effective strategy to repair defects of the tegmen mastoideum. SOP remains a clinically rare disease, with little published information on its diagnosis and treatment.

Background Temporal bone dehiscence (TBD) often results in leakage of cerebrospinal fluid (CSF) and/or encephalocele. TBD can also occur over the superior semicircular canal, causing debilitating vertigo. Both can be repaired surgically, but traditional treatment is focused only on one pathology, not both. Objective To report our experience in the treatment of TBD via the middle cranial fossa (MCF) approach. Methods A retrospective review was conducted for all patients who underwent MCF approach for surgical repair of any temporal fossa dehiscence. Results A total of 34 patients underwent a total of 37 surgeries. Obesity was prevalent; 21 patients (61.8%) were obese (BMI [body mass index] > 30 kg/m2), and 7 (20.6%) were overweight (BMI 25-30 kg/m2). The most common presenting symptom was hearing disturbance (70.3%), followed by otorrhea (51.4%). Empty sella was noted on computed tomography or magnetic resonance imaging in 15 patients (45.5%). Eight of the 34 patients (23.5%) were found to have superior semicircular canal dehiscence (SCD). Hearing improved with surgical intervention in 25 of 26 patients with hearing loss as a presenting symptom (96%). CSF resolved in 18 of 19 cases (95%). Seventy-three percent of patients reported at least minimal improvement in vertigo. Conclusion TBD may present with symptoms of CSF leak/encephalocele, but may also present with superior SCD. We recommend consistent review of the temporal bone imaging to check for superior SCD, and repair of the SCD first to prevent complications involving the labyrinth and cochlea. MCF approach using a multilayer repair without a lumbar drain is highly effective with minimal risk of complications.

Background Very few studies have examined vestibular schwannoma (VS) management trends across centers and between providers. The objective of this study is to examine current practice trends, variance in treatment philosophies, and nuanced or controversial aspects of VS care across North America. Methods This is a cross-sectional survey of North American Skull Base Society (NASBS) members who report regular involvement in VS care. Results A total of 57 completed surveys were returned. Most respondents claimed to have over 20 years of experience and the majority reported working in an academic practice with an affiliated otolaryngology and/or neurosurgery residency program. Sixty-three percent of respondents claimed to evaluate VS patients in clinic with both an otolaryngologist and neurosurgeon involved. Eighty-six percent of respondents claimed to operate on VS with both an otolaryngologist and neurosurgeon involved, while only 18% of neurosurgeons and 9% of otolaryngologists performed surgery alone. There was a wide range in the number of cases evaluated at each center annually. Similarly, there was wide variation in the number of patients treated with microsurgery and radiation at each center. Additional details regarding management preferences for microsurgery, stereotactic radiosurgery, stereotactic radiotherapy, and conservative observation are presented. Conclusion VS management practices vary between providers and centers. Overall, most centers employ a multidisciplinary approach to management with collaboration between otolaryngology and neurosurgery. Overall, survey responses concur with previous studies suggesting a shift toward conservatism in management.

Introduction Perioperative care of vestibular schwannoma (VS) patients is extremely variable across surgeons and institutions making practice patterns difficult to standardize. No data currently exist detailing this practice variability. Methods The North American Skull Base Society membership was electronically surveyed regarding perioperative care of surgically operated VS patients. Results There were 87 respondents to the survey. Surgical positioning, surgical approach utilized, and perioperative medical adjuncts are quite variable. However, of those performing retrosigmoid approaches, 49% perform this in the supine position, while 33% use a park-bench position with only 2% using the sitting position. In those performing translabyrinthine approaches, 86% perform this in supine position. Although the use of neuromonitoring appears to be standard of care (98%), other than the seventh nerve, there is substantial variability between respondents regarding monitoring of additional cranial nerves. Postoperative antibiotics are used by 65%, postoperative steroids 81%, and postoperative chemical deep vein thrombosis prophylaxis in 68% of survey respondents. Conclusion Although the perioperative adjuncts to VS surgery are variable, there does appear to be a trend in common practice. Therefore, making surgeons aware of these trends may lead to standardized practice or alternatively trials of these variances to instruct which truly improve patient outcomes.

Background In this report, we present a unique case of intraneural squamous cell carcinoma of unknown primary found within the facial nerve and the proposed algorithms for diagnosis and management of progressive idiopathic facial paralysis. Case presentation A 66-year-old female with a previous history of basal cell carcinoma presented with right-sided progressive facial paralysis. Repeated magnetic resonance imaging as well as targeted workup failed to reveal a diagnosis. 20 months following symptom onset, after the patient's facial function slowly progressed to a complete paralysis, repeat magnetic resonance imaging revealed enhancement at the stylomastoid foramen. The patient underwent superficial parotidectomy, transmastoid facial nerve decompression and resection of descending and proximal extratemporal facial nerve segments, as well as great auricular nerve interposition grafting. Intraoperatively, frozen sections from the surface of the facial nerve, and the proximal and distal segments of the facial nerve following resection, were negative for malignancy. The final pathology revealed infiltrating poorly differentiated squamous cell carcinoma of the facial nerve with negative margins. Conclusion In cases of slowly progressive facial paralysis the clinician needs to consider malignancy until proven otherwise. Without an identifiable primary malignancy, early algorithmic assessment of presenting characteristics may facilitate expedited clinical decision making and surgical management of malignancy involving the facial nerve. In cases of slowly progressive facial paralysis, when the time comes for surgical exploration and biopsy, head and neck surgeons must be aware that malignancy can exist entirely within the facial nerve, without pathologic changes on the surface of the nerve or in the surrounding tissue.

This article reviews the causes of ossicular impairment, interpretations of chronic ear disease, and provides a structured approach to the surgical correction of conductive hearing loss. It discusses reasons for inadequate air bone gap closure following ossicular chain reconstruction. The article reviews the Austin-Kartush classification of ossicular impairment, which allows for classification of nearly any defect in the ossicular chain. Using the Austin-Kartush classification, our most common ossicular chain reconstruction techniques are presented. Alternatives to ossicular chain reconstruct are discussed. A surgical video of transcanal Vibroplasty is included and available for viewing online.

(2010). The Predictive Value of Transtympanic Promontory EABR in Congenital Temporal Bone Malformations. Cochlear Implants International: Vol. 11, Proceedings of the 9th European Symposium on Paediatric Cochlear Implantation, Warsaw, 2009, pp. 181-186.

Objective: To evaluate the long-term hearing outcomes of neurofibromatosis type 2 (NF2) patients with cochlear implants. Methods: Retrospective analysis of cochlear implant performance in NF2 patients using open- and closed-set speech perception testing. Results: Patients with NF2-associated bilateral vestibular schwannomas frequently become profoundly deaf. The aim of surgical resection should be to preserve serviceable hearing in at least one ear; however, this goal can be difficult to achieve. Frequently, tumor size or poor preoperative hearing status can require a surgical approach that leaves the patient with a profound, bilateral sensorineural hearing loss. If the cochlear nerve is preserved anatomically after vestibular schwannoma surgery, and if promontory stimulation confirms the functionality of the cochlear nerve, then cochlear implantation is an excellent option to restore hearing. We present six cochlear implant patients with NF2 who attained a significant improvement in open- and closed-set speech understanding with a mean follow-up of 7.9 (range: 5–13) years after surgery. In all but one case, the hearing results did not deteriorate over the follow-up period. Conclusion: Early surgical intervention for vestibular schwannomas in NF2 patients when the cochlear nerve can be spared is an important consideration to allow for possible cochlear implantation. A 6- to 8-week recovery period for the anatomically intact cochlear nerve may be necessary to obtain a positive promontory stimulation response following tumor resection and should be performed prior to cochlear implantation.

Background Patients with previously implanted cranial devices pose a special challenge in deep brain stimulation (DBS) surgery. We report the implantation of bilateral DBS leads in a patient with a cochlear implant. Technical nuances and long-term interdevice functionality are presented. Case description A 70-year-old patient with advancing Parkinson's disease and a previously placed cochlear implant for sensorineural hearing loss was referred for placement of bilateral DBS in the subthalamic nucleus (STN). Prior to DBS, the patient underwent surgical removal of the subgaleal cochlear magnet, followed by stereotactic MRI, frame placement, stereotactic computed tomography (CT), and merging of imaging studies. This technique allowed for successful computational merging, MRI-guided targeting, and lead implantation with acceptable accuracy. Formal testing and programming of both the devices were successful without electrical interference. Conclusion Successful DBS implantation with high resolution MRI-guided targeting is technically feasible in patients with previously implanted cochlear implants by following proper precautions.

Objective To evaluate the sound localization capabilities of patients with unilateral, profound sensorineural hearing loss who had been treated with either a bone-anchored hearing device (Baha BP100) or a TransEar 380-HF bone-conduction hearing device. Study Design Nonrandomized, prospective study. Setting Tertiary referral private practice. Patients Patients with unilateral, profound sensorineural hearing loss treated with a BP100 (n = 10) or a TransEar (n = 10) device. Patients wore the hearing device for at least 1 month and had normal hearing in the contralateral ear. Ten patients with normal, bilateral hearing were used for control. Interventions Sound localization of a 3-second recorded sound with and without a TransEar or Baha device was assessed using an array of 7 speakers at head level separated by approximately 45 degrees. The recorded sounds were that of a barking dog or a police siren. Randomized trials of 4 presentations per speaker were given for each hearing condition. Main Outcome Measures Sound localization was assessed by the accuracy in response and the generalized laterality of response. Results The mean accuracy of speaker localization was 24% and 26% for the aided condition using the BP100 and TransEar devices, respectively. The mean accuracy of laterality judgment was 59% and 69% for the aided condition using the BP100 and TransEar devices, respectively. These results were only slightly better than chance. There was no statistical difference in localization accuracy or laterality judgment between the BP100 and TransEar groups. Conclusion Neither the BP100 nor the TransEar device improved sound localization accuracy or laterality judgment ability in patients with unilateral, profound sensorineural hearing loss compared with performance in the unaided condition.

To compare short- and long-term hearing results following stapedectomy using 3 different oval window grafting materials with the same stapes prosthesis.Database review.Tertiary referral private practice.Subjects were ears that underwent stapedectomy for otosclerosis, with placement of fat, fascia, or vein as an oval window seal and reconstruction with a titanium bucket handle prosthesis. A total of 365 procedures met these inclusion criteria: 98 fat grafts, 135 fascia grafts, and 132 vein grafts. Outcome measures included short-term (1 year) and long-term follow-up air-bone gap. We compared the preoperative and postoperative amount of change in air-bone gap and preoperative and postoperative amount of change in the high-frequency bone conduction average.Overall median times to short-term and long-term follow-ups were 2.2 months and 36.1 months, respectively. There were no statistically significant differences between the 3 tissue seal groups in the amount of change in air-bone gap. There was no significant difference in amount of change in high-frequency bone conduction (representing sensorineural hearing level) between the 3 tissue seal groups. Most patients in all 3 groups had an air-bone gap at long-term follow-up of ≤ 10 dB (fat, 79.5%; fascia, 78.8%; and vein, 75.6%), with 90.3% of all patients at ≤ 20 dB.In both the short-term postoperative period and long-term follow-up, there were no significant differences in hearing results among 3 types of tissue seals of the oval window in stapes surgery. Fat, fascia, and vein grafts all provide satisfactory hearing outcomes in stapedectomy.