Cochlear Implantation in Patients With Osteolytic Labyrinthitis: A Case Series.

OBJECTIVE: To describe the rare process of osteolytic labyrinthitis, previously referred to as labyrinthine sequestrum, which involves progressive obliteration of the bony and membranous labyrinth with eventual supplantation with soft tissue and, in some cases, bony sequestrum. PATIENTS: Three patients with diverse presentations of osteolytic labyrinthitis from two tertiary care academic medical centers. INTERVENTIONS: Case series report analyzing the relevant clinical, radiologic, pathologic, and surgical data on our patients with osteolytic labyrinthitis and comparing these index cases to the existing literature. MAIN OUTCOME MEASURES: We describe the varying image findings seen in osteolytic labyrinthitis on computed tomography and magnetic resonance imaging. Also, we report successful surgical intervention and hearing rehabilitation with cochlear implantation in patients with osteolytic labyrinthitis. RESULTS: Our three patients presented with profound sudden sensorineural hearing loss and vertigo consistent with labyrinthitis. None of the three patients had a history of chronic otitis media. Imaging workup revealed varying degrees of erosion to the otic capsule bone demonstrating the spectrum of disease seen in osteolytic labyrinthitis. Although two cases showed osteolytic changes to the semicircular canals and vestibule, the first case revealed frank bony sequestrum within the obliterated labyrinth. The three cases were taken for surgical debridement and cochlear implantation. CONCLUSIONS: We propose the new term, osteolytic labyrinthitis-previously referred to as labyrinthine sequestrum-to describe the rare spectrum of disease characterized by destruction of the osseous and membranous labyrinth and potential supplantation with bony sequestrum. Cochlear implantation is a viable option in selected patients with osteolytic labyrinthitis.

Rationale for the Development of a Novel Clinical Grading Scale for Postoperative Facial Nerve Function: Results of a Multidisciplinary International Working Group.

OBJECTIVE: The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications. STUDY DESIGN: Survey. SETTING: A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery. RESULTS: House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting. CONCLUSIONS: Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland.

Treatment of tegmen dehiscence using a middle fossa approach and autologous temporalis fascia graft: Outcomes from a single center.

INTRODUCTION: Although bony defects of the tegmen surface are relatively common, the majority of dehiscences are asymptomatic. For those who experience symptoms, there is a wide spectrum of relatively benign manifestations such as hearing loss and otorrhea to potentially more serious but rare sequelae such as epilepsy and meningitis. Surgical management of tegmen dehiscences (TDs) can help prevent these symptoms. In this manuscript, we present one of the largest reported single team experiences of using a temporal craniotomy with middle cranial fossa approach and temporalis fascia graft in the treatment of tegmen defects. METHODS: We retrospectively reviewed every case of a TD surgically repaired by the same neurosurgeon/otolaryngologist team at Loyola University Medical Center from May 2015 to January 2022. In our chart review, we identified 44 patients with 48 cases of tegmen defect repair. We analyzed patient characteristics, operative details, and postoperative outcomes. RESULTS: 44 patients met inclusion criteria for the presence of TD (mean age 55 years, 55% male, and average body mass index 35.6). 89% of these patients had no clear etiology for the dehiscence. Commonly reported symptoms were hearing loss (89%) and CSF otorrhea (82%). The least reported presenting signs and symptoms were seizures (5%) and meningitis (2%). Most defects were repaired with both temporalis fascial and calvarial bone grafts (63%), while a minority were treated with temporalis fascia only (33%), temporalis fascia with muscle (2%), or fascia lata (2%). Every patient in our sample experienced resolution of CSF otorrhea after tegmen repair and 81% of the sample reported subjective hearing improvements after surgery. 6% of our sample had post-operative infections and 8% of patients underwent repeat unilateral surgery for a surgical complication. CONCLUSION: Craniotomy for middle fossa approach using autologous temporalis fascial grafts is a safe and effective method for the treatment of TD. These procedures should be performed by experienced and multidisciplinary teams.

The Natural History of Small Vestibular Schwannomas.

Objective The incidence of vestibular schwannomas is increasing, and the average tumor size at diagnosis is decreasing. Therefore, understanding the specific growth pattern of small vestibular schwannomas is becoming increasingly important to guide clinical management. The objectives of this study were to evaluate the growth patterns of very small intracanalicular vestibular schwannomas measuring ≤ 4 mm in linear diameter and to assess the likelihood of these lesions ever requiring treatment.  Methods A retrospective review was performed. A search of all MRI brain and internal auditory canal studies suggestive of a vestibular schwannoma from 1995 to 2019 was performed at our institution. This resulted in 372 cases, which were then evaluated for the presence of a vestibular schwannoma measuring ≤ 4 mm. All patients had to have at least one follow-up MRI to be included. Images were reviewed by a neuroradiologist. Results Eight ≤ 4 mm vestibular schwannomas were found that met all search criteria. The distribution of tumor sizes was as follows: three 2 mm, one 3 mm and four 4 mm. None of the ≤ 4 mm vestibular schwannomas identified demonstrated any significant growth in the linear dimension defined as greater than 2 mm of growth over observation times of 1-13 years (mean 6.3 years). None of the lesions ever required a treatment intervention per available medical records. Conclusion None of the ≤ 4 mm intracanalicular vestibular schwannomas identified in this study grew significantly or required treatment. Overall, the findings in this study suggest that vestibular schwannomas measuring ≤ 4 mm are unlikely to grow and ever require treatment.

Cochlear Implantation in Vestibular Schwannoma: A Systematic Literature Review.

Objective Ipsilateral cochlear implantation (CI) in vestibular schwannoma (VS) has been an emerging trend over the last two decades. We conducted the first systematic review of hearing outcomes comparing neurofibromatosis 2 (NF2) and sporadic VS undergoing CI. A comparison of the two populations and predictor of outcome was assessed. This is an update to a previously presented study. Data Sources Systemic data searches were performed in PubMed NCBI and Scopus by an academic librarian. No restrictions based on the year of publication were used. Study Selection Studies were selected if patients had a diagnosis of NF2 and a CI placed in the affected side with reports of hearing outcome. Two independent reviewers screened each abstract and full-text article. Data Extraction Studies were extracted at the patient level, and the assessment of quality and bias was evaluated according to the National Institutes of Health Quality Assessment Tool. Main Outcome Measures Outcome predictors were determined by using the chi-square test and Student's t -test. Results Overall, most CI recipients functioned in the high-to-intermediate performer category for both sporadic and NF2-related VS. Median AzBio (Arizona Biomedical Institute Sentence Test) was 72% (interquartile range [IQR]: 50) in NF2 patients and 70% (IQR: 7.25) in sporadic patients. Larger tumor size predicted a poorer final audiometric outcome. Conclusions Categorization of hearing outcome into superior performance and inferior performance based on sentence recognition revealed a generally good hearing outcome regardless of treatment or patient population. Select patients with sporadic and NF2 VS may benefit from CI.

Superior Semicircular Canal Dehiscence Syndrome Following Head Trauma: A Multi-institutional Review.

OBJECTIVE: To evaluate patients who become symptomatic from superior semicircular canal dehiscence (SSCD) following head trauma. STUDY DESIGN: Case series assessing patients presenting with SSCD after a trauma. METHODS: A case series was completed assessing patients presenting with SSCD after trauma. Data from three academic medical centers were evaluated, including the following: imaging, videonystagmography (VNG)/vestibular evoked myogenic potential (VEMP) testing, audiometric assessment, and surgical repair. Outcome measures included the following: 1) Description of audio-vestibular symptoms, 2) mean pre- and post-operative pure tone average (PTA), word recognition score (WRS), and air bone gap (ABG). RESULTS: A total of 14 patients were included; 86% were male. Approximately 43% were found to have bilateral SSCD on imaging, with 57% of patients pursuing surgical management. The most common presenting symptoms included pulsatile tinnitus (93%), autophony (79%), and hearing loss (64%). Approximately 36% of patients underwent VNG/VEMP testing, with 83.3% of those demonstrating abnormal results. The mean audiometric findings on the symptomatic side included an air-conduction PTA of 38.0 dB, bone-conduction PTA of 24.3 dB, WRS of 81%, and ABG of 17.9 dB. Among patients who underwent surgery (57%), there was no significant change in the air-conduction PTA, bone-conduction PTA, or WRS (P > .05). However, there was an improvement in the ABG (preoperative = 22.8 dB versus postoperative = 9.7 dB; P = .005). CONCLUSION: Head trauma may be a potentiating event for SSCD syndrome. This study advances the hypothesis that these patients likely have underlying radiographic SSCD prior to their trauma, and a traumatic event increases in intra-vestibular or intracranial pressures, unmasking SSCD syndrome. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2810-E2818, 2021.

Projected Cost Simulation in Chronic Ear Surgery: Cost in Chronic Ear Surgery.

OBJECTIVE: To examine the projected healthcare costs of intact canal wall (ICW) and canal wall down (CWD) tympanomastoidectomy surgery and associated clinic follow-up visits in the management of chronic ear disease. STUDY DESIGN: A review of literature was performed to analyze the cholesteatoma recidivism rates of ICW and CWD tympanomastoidectomy. Projected Medicare cost simulation applied to representative samples of patients undergoing otologic surgery and follow-up clinic appointments projected over 15 years with an annual 25% cholesteatoma recidivism rate. SETTING: Tertiary academic center. PATIENTS: Simulated patient population status postchronic ear surgery and representative follow-up clinic visits with or without in-office mastoid debridement procedures. INTERVENTIONS: Therapeutic and rehabilitative. MAIN OUTCOME MEASURES: Healthcare cost. RESULTS: Simulation of cost for chronic ear surgery with follow-up visits produced a higher mean cost for the ICW group compared with the CWD group at year 1 ($9112.06 versus $4954.16). However, CWD patients accumulate cost over time due to routine mastoid debridements. The accumulated costs for the CWD group surpassed the ICW group at 12.54 (CI: 12.02-13.11) years. CONCLUSIONS: Using a projected cost analysis model, intact canal wall tympanomastoidectomy incurs a larger upfront cost than the canal wall down technique. However, long-term mastoid cavity maintenance costs accumulate to surpass the intact canal wall group at 12.54 years.

Fetal Bovine Collagen Grafts for Repair of Tegmen Defects and Encephaloceles Via Middle Cranial Fossa Approach.

To evaluate the use of commercially available allogenic dural graft materials made of fetal bovine collagen, we present an analysis of our case series with use of autologous and allogenic graft materials. Patients who underwent surgical repair of a tegmen tympani defect associated with ipsilateral conductive hearing loss and cerebrospinal fluid (CSF) otorrhea using a middle cranial fossa (MCF) approach from 2004 to 2018 at Loyola University Medical Center were included. Resolution of CSF otorrhea, audiologic outcomes, facial nerve preservation, and surgical complications was analyzed. Thirty-three patients with an average age of 55.3 years (range: 21-78, standard deviation [SD]: 12.9) and body mass index of 34.4 (range: 22-51, SD: 7.4) underwent an MCF repair of a tegmen and dural defect. All patients presented with CSF otorrhea and conductive hearing loss ipsilateral to the defect. Repairs were made with combinations of allograft and autograft in 17 cases, allograft only in 15 cases, and autograft only in 5 cases. Improvement in hearing was noted in 33 cases, and resolution of CSF otorrhea was noted in 36 cases; one patient required repeat surgery which resolved CSF otorrhea. Three patients had minor complications; all these were in the autograft group. The MCF approach coupled with the use of fetal bovine collagen grafts is a safe and viable method to repair tegmen tympani and associated dural defects with salutary outcomes and low morbidity.

A Novel Technique for the Diagnosis and Management of Middle Ear Myoclonus.

A novel technique for the diagnosis and management of middle ear myoclonus is described. A patient with middle ear myoclonus underwent a trans-canal microscopic middle ear exploration with injection of botulinum toxin into the stapedius and tensor tympani muscles. Postoperatively, the patient noted complete resolution of symptoms. This is the first report of the use of botulinum toxin directly applied to the middle ear musculature via a trans-canal approach for the management of middle ear myoclonus. This approach is both a useful diagnostic and therapeutic tool that allows for temporary muscle paralysis prior to offering definitive surgical management. Laryngoscope, 131:E248-E249, 2021.

Prevalence of and Associations With Distress and Professional Burnout Among Otolaryngologists: Part II, Attending Physicians.

OBJECTIVE: To ascertain the prevalence of and associations with distress and professional burnout among academic otolaryngology attending physicians. STUDY DESIGN: Cross-sectional survey. SETTING: Twelve US academic otolaryngology programs. METHODS: A questionnaire was administered that encompassed sociodemographic and professional features, the Expanded Physician Well-being Index for distress, the 2-item Maslach Burnout Inventory for professional burnout, the Patient Health Questionnaire-2 screen for major depressive disorder, and the Generalized Anxiety Disorder-2 screen for generalized anxiety disorder. RESULTS: The survey response rate was 56% and included 186 attending physicians. The average respondent age was 47 years; 72% were men; 93% were married or partnered; and 86% had children. Distress was present in 40%, professional burnout in 26%, positive depression screening in 8%, and positive anxiety screening in 11%. In a univariable setting, age, hours worked in a typical week, nights on call in a typical week, and years of practice were significantly associated with distress, although in a multivariable setting, only hours worked in a typical week remained significantly associated with a positive Expanded Physician Well-being Index screen (odds ratio for each 10-hour increase, 2.61; 95% CI, 1.73-3.93; P < .001). In a univariable setting, hours worked in a typical week was significantly associated with a positive Maslach Burnout Inventory screen. CONCLUSION: Distress or professional burnout occurs in more than a quarter of academic otolaryngology attending physicians, whereas the prevalence of depression or anxiety is approximately 10%. The number of hours worked per week had the strongest association with distress and burnout. These findings may be used to develop and implement programs to promote physician well-being and mitigate professional burnout.

Prevalence of and Associations With Distress and Professional Burnout Among Otolaryngologists: Part I, Trainees.

OBJECTIVE: To assess the prevalence of distress and burnout in otolaryngology trainees, including associations with relevant sociodemographic and professional factors, and to compare these results with those of attending otolaryngologists. STUDY DESIGN: A cross-sectional survey of trainees and attending physicians. SETTING: Twelve academic otolaryngology programs. METHODS: Distress and burnout were measured with the Expanded Physician Well-being Index and the 2-item Maslach Burnout Inventory. The Patient Health Questionnaire-2 and Generalized Anxiety Disorder-2 were used to screen for depressive disorders and anxiety disorders, respectively. Associations with sociodemographic and professional characteristics were assessed. RESULTS: Of the 613 surveys administered to trainees and attending physicians, 340 were completed (56%). Among 154 trainees, distress was present in 49%, professional burnout in 35%, positive depressive disorder screening in 5%, and positive anxiety disorder screening in 16%. In univariable analysis, female gender, hours worked in a typical week (HW), and nights on call in a typical week (NOC) were significantly associated with distress. In multivariable analysis, female gender (odds ratio, 3.91; P = .001) and HW (odds ratio for each 10 HW, 1.89; P = .003) remained significantly associated with distress. Female gender, HW, and NOC were significantly associated with burnout univariably, although only HW (odds ratio for each 10 HW, 1.92; P = .003) remained significantly associated with burnout in a multivariable setting. Attending physicians had less distress than trainees (P = .02) and felt less callous and less emotionally hardened than trainees (P < .001). CONCLUSION: Otolaryngology trainees experience significant work-place distress (49%) and burnout (35%). Gender, HW, and NOC had the strongest associations with distress and burnout.

Neurotologic Complications Following Microvascular Decompression: A Retrospective Study.

Objective The main purpose of this article is to determine the frequency of neurotologic complications after posterior fossa microvascular decompression (MVD) surgery. Design Retrospective case review. Setting Tertiary care referral center. Participants A total of 215 consecutive MVD operations by a single surgeon between March 1996 and May 2016 were reviewed with 192 surgeries on 183 patients meeting inclusion criteria. Main Outcome Measures Neurotologic complications secondary to MVD. Results The 52 males and 131 females had a mean age of 58.52 years (range, 28-92 years). Indications for MVD were trigeminal neuralgia ( n = 162), hemifacial spasm ( n = 23), glossopharyngeal neuralgia ( n = 13), vagal palsy ( n = 1), and tinnitus ( n = 1). The outcomes examined were postoperative hearing loss, tinnitus, vertigo, and hemifacial paresis. At least one of these complications was present in 17.7% of patients. There were 4.17% with permanent hearing loss, 6.77% with transient hearing loss, 5.21% with tinnitus, 5.73% with vertigo, and 0.52% with hemifacial paresis. There was no significant difference in complication rates based on surgical indication. Conclusions Neurotologic complications are a significant risk when performing MVD. It is important for otolaryngologists as well as neurosurgeons to be aware of such complications. We recommend perioperative audiometry in all patients undergoing MVD and believe there is utility in routine otolaryngologist involvement.

Cochlear Implantation in Sporadic Vestibular Schwannoma: A Systematic Literature Review.

Objective This study was aimed to perform a systematic literature review by examining outcomes in patients with sporadic vestibular schwannoma (VS) undergoing ipsilateral cochlear implant (CI). Data Sources PubMed-NCBI (National Center for Biotechnology Information) and Scopus databases were searched through October 2017. Study Selection Studies reporting auditory outcomes for each patient when a CI was placed with an ipsilateral sporadic VS were included. Main Outcome Measures Demographic variables, VS characteristics, preoperative hearing metrics, duration of deafness, CI type, approach to tumor resection, postoperative auditory outcomes, and postoperative tinnitus outcomes were reported for each eligible patient within studies. Each study was evaluated for quality and bias. Results Fifteen studies and 45 patients met inclusion criteria. Mean speech discrimination score (SDS) improved from 30.0 to 56.4% after CI placement. The majority when reported had an improvement in tinnitus. Preoperative ipsilateral SDS was a negative predictor of postoperative SDS, while neither tumor resection status, tumor location, duration of deafness, ipsilateral pure tone average, nor timing of CI placement had a significant effect on patient outcome. Conclusions Notwithstanding the challenges inherent with surveillance magnetic resonance imaging (MRI) in the setting of a cochlear implant magnet, select sporadic vestibular schwannoma patients can be considered for cochlear implantation.

Hearing Loss following Posterior Fossa Microvascular Decompression: A Systematic Review.

Objectives (1) Determine the prevalence of hearing loss following microvascular decompression (MVD) for trigeminal neuralgia (TN) and hemifacial spasm (HFS). (2) Demonstrate factors that affect postoperative hearing outcomes after MVD. Data Sources PubMed-NCBI, Scopus, CINAHL, and PsycINFO databases from 1981 to 2016. Review Methods Systematic review of prospective cohort studies and retrospective reviews in which any type of hearing loss was recorded after MVD for TN or HFS. Three researchers extracted data regarding operative indications, procedures performed, and diagnostic tests employed. Discrepancies were resolved by mutual consensus. Results Sixty-nine references with 18,233 operations met inclusion criteria. There were 7093 patients treated for TN and 11,140 for HFS. The overall reported prevalence of hearing loss after MVD for TN and HFS was 5.58% and 8.25%, respectively. However, many of these studies relied on subjective measures of reporting hearing loss. In 23 studies with consistent perioperative audiograms, prevalence of hearing loss was 13.47% for TN and 13.39% for HFS, with no significant difference between indications ( P = .95). Studies using intraoperative brainstem auditory evoked potential monitoring were more likely to report hearing loss for TN (relative risk [RR], 2.28; P < .001) but not with HFS (RR, 0.88; P = .056). Conclusion Conductive and sensorineural hearing loss are important complications following posterior fossa MVD. Many studies have reported on hearing loss using either subjective measures and/or inconsistent audiometric testing. Routine perioperative audiogram protocols improve the detection of hearing loss and may more accurately represent the true risk of hearing loss after MVD for TN and HFS.

Facial Nerve Schwannomas Mimicking as Vestibular Schwannomas.

Objective The objective of this study was to identify preoperative and intraoperative findings that may aid in distinguishing facial nerve schwannomas (FNS) from vestibular schwannomas (VSs), particularly in cases limited to the internal auditory canal (IAC) and cerebellopontine angle (CPA). Study Design This was a retrospective study. Setting This study was set at a Tertiary Referral Center. Patients Seventeen cases from October 2002 to July 2015 with an IAC/CPA mass presumed to be a VS who were found to have a FNS intraoperatively. Main Outcome Measures The main outcome measures included preoperative presentation, intraoperative findings, and subsequent intervention. Results Preoperative hearing loss and imbalance were seen in 70.5 and 64.7%, respectively. Suspicious intraoperative findings included: facial nerve incorporated intimately with the tumor capsule in 12 cases; spontaneous action potentials noted while drilling the bony IAC in 3 cases; and action potentials noted on stimulation of the entire tumor capsule in 10 cases. The mean long-term facial function was House-Brackmann grade II and the mean length of follow-up was 4.86 years. Conclusion FNSs are rare and may be difficult to distinguish from VS preoperatively. Surgical findings that should raise concern include spontaneous action potentials during drilling the bony IAC, absence of a plane of dissection between the facial nerve and tumor, or stimulation of the tumor capsule.

Plummer-Vinson syndrome following gastric bypass surgery.

Plummer-Vinson syndrome (PVS) is the combination of dysphagia, angular cheilitis, atrophic glossitis, and esophageal webbing in the setting of iron deficiency anemia. Although it is relatively uncommon, this condition is important to recognize because it is a source of dysphagia and it confers an increased risk for hypopharyngeal cancer. Cases of PVS associated with gastrointestinal conditions such as celiac disease and gastric cancer have been previously reported in the literature, but as far as we know, no case of PVS associated with bariatric surgery has been previously reported. We describe the case of a 39-year-old woman who developed PVS following gastric bypass surgery, and we briefly discuss the current knowledge of this syndrome.

Utility and cost analysis of cholesteatoma histopathologic evaluation.

OBJECTIVES/HYPOTHESIS: To evaluate the correlation between the surgeon's intraoperative findings and histopathologic diagnosis of cholesteatoma specimens and the associated health care cost in requesting pathologic evaluation. STUDY DESIGN: Retrospective chart analysis. METHODS: Chart data were collected at a tertiary neurotology referral center from patients undergoing tympanomastoidectomy for chronic otitis media, with specimens submitted for pathologic review between 2010 and 2011. Correlation between the surgeon's intraoperative findings and the pathologic diagnosis was evaluated using a kappa statistic. Cost analysis for pathologic consultation was also reviewed. RESULTS: A Cohen's kappa value of 0.93 (P < .01) was found between the surgeon's intraoperative findings and pathologic diagnosis. Using accepted kappa magnitude guidelines, there is perfect agreement between the surgeon's intraoperative findings and pathologic diagnosis of cholesteatoma after tympanomastoidectomy. The average cost for microscopic evaluation of cholesteatoma (current procedural terminology code 88304) as estimated per 2012 Medicare reimbursement rates is $61.95. CONCLUSIONS: In the absence of concern for other pathology, intraoperative findings of cholesteatoma are adequate to confirm diagnosis in patients undergoing tympanomastoidectomy for chronic otitis media without the use of histopathology. The increased cost of routine cholesteatoma histopathologic evaluation should be considered in future health care cost-containing measures, as clinical utility appears to be low. LEVEL OF EVIDENCE: 4.