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.

Myeloid Sarcoma of the Temporal Bone: A Unique Cause of Hearing Loss, Otalgia, and Facial Nerve Weakness.

OBJECTIVE: To characterize a clinical triad of symptoms associated with myeloid sarcomas of the temporal bone via a review of all previously reported cases. METHODS: Case report and Ovid MEDLINE database literature review. RESULTS: A literature search revealed that a clinical triad of hearing loss, otalgia, and facial nerve weakness are commonly associated with this rare presentation of myeloid sarcoma in the temporal bone. 44% (18/41) of patients presented with all three symptoms, while 76% (31/41) presented with at least two. The presence of t(8;21) was reported in nine patients with myeloid sarcomas of the temporal bone. CONCLUSIONS: Although myeloid sarcomas are exceedingly rare, it is necessary to consider them as part of the differential diagnosis for patients who might present with middle ear and mastoid opacification on computed tomography (CT) scan, hearing loss, otalgia, and facial nerve palsy. Physicians should maintain a high degree of suspicion in patients with a history of acute myelogenous leukemia (AML), especially if previous cytogenetic analysis revealed a t(8;21).

Microsurgical Technique for Resection of a Cerebellopontine Angle Epidermoid Tumor.

Epidermoid tumors are slow-growing, benign, congenital lesions.1 They commonly arise in the cerebellopontine angle, fourth ventricle, suprasellar region, or spinal cord.2 Symptoms may include hearing loss, facial pain, and headaches. The management options include observation or surgical resection. If the patient has symptoms, surgical resection is the treatment option of choice with the goal of gross total resection. In Video 1, we discuss the microsurgical technique for the resection of a right cerebellopontine angle epidermoid tumor. A 22-year-old male patient presented with chronic headache, decreased right-sided hearing, right facial pain, and right facial twitching. Magnetic resonance imaging revealed the characteristic finding of an epidermoid tumor, which appeared as isointense on T1 and hyperintense on T2 with diffusion-weighted imaging. The patient was taken to the operating room, and a retrosigmoid craniotomy was performed on the basis of the transverse and sigmoid sinuses. The tumor capsule was opened, and the tumor was decompressed by removing the internal components consisting of epithelial keratin and cholesterol crystals, allowing for a gross total resection to be achieved. The patient's postoperative computed tomography scan showed no residual tumor, and the patient was discharged on postoperative day 1 in stable condition.

Incidence of Concomitant Semicircular Canal Dehiscence With Otosclerosis.

Objective: The concurrence of otosclerosis and superior semicircular canal dehiscence (SSCD) presents a diagnostic challenge and failure to differentiate between these 2 diagnoses results in mischaracterization and unsuccessful surgery. The objective of this study is to identify the incidence of SSCD in patients who have computed tomography (CT) evidence of otosclerosis. Study Design: Retrospective chart review. Setting: Tertiary referral hospital. Patients: Adults with CT scan of the temporal bone diagnosed with radiological unilateral or bilateral fenestral otosclerosis from January 1995 to April 2018. Methods: Retrospective review of patient imaging from a multi-center tertiary-referral health system from January 1995 to April 2018. Imaging was reviewed to quantify the incidence of SSCD among patients with CT-diagnosed bilateral fenestral otosclerosis. Poor quality imaging was excluded from review. Results: One-thousand two-hundred eight patients (1214 CT scans) were identified with otosclerosis, of which 373 were diagnosed with fenestral otosclerosis (663 ears) with imaging of sufficient quality for review. This population was predominantly female (57.2%) with bilateral fenestral otosclerosis (78%). Of these, 23 ears (3.5%) had definitive evidence of SSCD, with an additional 15 ears (2.3%) with possible radiographic evidence of SSCD. There was no significant difference in laterality between the SSCD and otosclerosis. Conclusions: Among 373 patients with fenestral otosclerosis per CT temporal bone imaging at a tertiary referral hospital, as many as 8.3% of patients had radiographic evidence of SSCD. Given this incidence, it continues to be important to consider SSCD when diagnosing and treating otosclerosis.

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.

Microvascular Decompression and Transposition of the 8th Cranial Nerve Using a Fenestrated Clip.

Neurovascular compression is a potential, yet rare cause of vertigo and unilateral tinnitus. Despite the high success rate of microvascular decompression (MVD) in other vascular compression syndromes, the outcome reported after MVD of the vestibulocochlear nerve is variable.1,2 The presence of combined symptoms of tinnitus and vertigo treated by MVD has demonstrated a higher predictive value for success.3 In this video, we present the case of a 68-year-old male who presented with vertigo and tinnitus refractory to medical management and vestibular therapy. Audiologic evaluation was normal apart from mild sensorineural hearing loss. Vestibular testing was suggestive of uncompensated right peripheral vestibulopathy. Magnetic resonance imaging with fast imaging employing steady-state acquisition sequences revealed vascular compression by the right anteroinferior cerebellar artery (AICA) at the cisternal component of vestibulocochlear nerve. After a multidisciplinary discussion, a microvascular decompression was performed through a right retrosigmoid craniotomy. The AICA was mobilized off the vestibulocochlear nerve and was secured to the petrous dura using a fenestrated clip. Indocyanine green angiography with Glow-800 was conducted before and after AICA transposition to confirm adequate flow through the mobilized vessel. Postoperatively, the patient's vestibular symptoms improved significantly. This case demonstrates that microvascular decompression can provide a satisfactory outcome in patients with unilateral tinnitus and vertigo associated with vascular compression in appropriately selected cases (Video 1).

Transmastoid Endoscopic-Assisted Eustachian Tube Packing After Translabyrinthine Tumor Resection: A Cadaveric Feasibility Study.

HYPOTHESIS: Endoscopically assisted packing of the Eustachian tube (ET) will improve visualization of the protympanic space compared with standard techniques with the microscope. BACKGROUND: Postoperative cerebrospinal fluid (CSF) leakage after translabyrinthine tumor resection remains a problem. Current techniques of packing the ET are limited by inadequate visualization. The objective of this study is to demonstrate the feasibility and usefulness of transmastoid endoscopic-assisted ET packing during translabyrinthine tumor resection. METHODS: Eight human cadaveric temporal bone dissections were performed on four heads to test the visualization that could be obtained of the ET orifice with an endoscope via transmastoid-facial recess approach. The incus body and incus buttress were removed, the aditus ad antrum enlarged, and tensor tympani muscle was cut. The scope was placed where the incus buttress had previously resided. The ET orifice was visualized and subsequently packed. RESULTS: In all eight temporal bones, the endoscope was successfully able to visualize the ET orifice, with improved visualization of the orifice compared with standard techniques. Surgical technique and potential pitfalls are discussed. CONCLUSIONS: The transmastoid endoscopic approach for packing the ET improves visualization of the ET orifice. Packing under direct visualization provided greater reassurance that the material entered the true ET lumen as opposed to a false passage. The technique can be performed without any significant changes to standard surgical technique, allowing for a fast and accurate closure of the ET orifice and has application in potentially decreasing postoperative CSF leak rates.

Facial Nerve Meningioma: A Cause of Pediatric Facial Weakness.

OBJECTIVE: To present an unusual case of a temporal bone meningioma with intrafascicular spread throughout the temporal facial nerve from cerebellopontine angle (CPA) to stylomastoid foramen. PATIENT: Four-year-old female with progressive facial weakness and normal hearing. MAIN OUTCOME MEASURE: Clinical, radiological, and histopathological findings of temporal bone meningiomas. RESULTS: A patient presented with progressive facial weakness and normal hearing. Imaging demonstrated a mass within the left internal auditory canal radiologically consistent with a schwannoma. Asymmetric enlargement with enhancement of the left facial nerve from CPA to the stylomastoid foramen suggested facial schwannoma. At surgery, gross tumor was noted in the internal auditory canal, the fallopian canal seemed expanded and the facial nerve was enlarged and had an irregular contour. Resection of the facial nerve from the CPA to just proximal to its exit at the stylomastoid foramen was necessary to achieve negative margins. Cable grafting was performed. The histopathologic diagnosis was transitional meningioma with intraneural growth throughout the length of the resected facial nerve segment. CONCLUSION: Meningiomas involving the temporal bone are exceedingly rare. We report a rare case of a child presenting with progressive facial weakness due to a presumed facial schwannoma spreading along the facial nerve throughout its intratemporal course that at surgery was found to be an intrafascicular CN VII meningioma.

Gamma Knife surgery for the management of glomus tumors: a multicenter study.

OBJECT: Glomus tumors are rare skull base neoplasms that frequently involve critical cerebrovascular structures and lower cranial nerves. Complete resection is often difficult and may increase cranial nerve deficits. Stereotactic radiosurgery has gained an increasing role in the management of glomus tumors. The authors of this study examine the outcomes after radiosurgery in a large, multicenter patient population. METHODS: Under the auspices of the North American Gamma Knife Consortium, 8 Gamma Knife surgery centers that treat glomus tumors combined their outcome data retrospectively. One hundred thirty-four patient procedures were included in the study (134 procedures in 132 patients, with each procedure being analyzed separately). Prior resection was performed in 51 patients, and prior fractionated external beam radiotherapy was performed in 6 patients. The patients' median age at the time of radiosurgery was 59 years. Forty percent had pulsatile tinnitus at the time of radiosurgery. The median dose to the tumor margin was 15 Gy. The median duration of follow-up was 50.5 months (range 5-220 months). RESULTS: Overall tumor control was achieved in 93% of patients at last follow-up; actuarial tumor control was 88% at 5 years postradiosurgery. Absence of trigeminal nerve dysfunction at the time of radiosurgery (p = 0.001) and higher number of isocenters (p = 0.005) were statistically associated with tumor progression-free tumor survival. Patients demonstrating new or progressive cranial nerve deficits were also likely to demonstrate tumor progression (p = 0.002). Pulsatile tinnitus improved in 49% of patients who reported it at presentation. New or progressive cranial nerve deficits were noted in 15% of patients; improvement in preexisting cranial nerve deficits was observed in 11% of patients. No patient died as a result of tumor progression. CONCLUSIONS: Gamma Knife surgery was a well-tolerated management strategy that provided a high rate of long-term glomus tumor control. Symptomatic tinnitus improved in almost one-half of the patients. Overall neurological status and cranial nerve function were preserved or improved in the vast majority of patients after radiosurgery.