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.

Classifying the Large Vestibular Aqueduct: Morphometry to Audiometry.

OBJECTIVE: Large vestibular aqueduct (LVA) is the most common inner ear dysplasia identified in patients with hearing loss. Our objective was to systematically quantify LVA morphologies and correlate imaging findings with established audiometric outcomes. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: Patients with large vestibular aqueduct identified radiographically, with or without hearing loss. INTERVENTIONS: Diagnostic only. MAIN OUTCOME MEASURES: Vestibular aqueduct (VA) width at midpoint, width at external aperture, and length were measured on cross-sectional imaging. Morphology was classified as type I (borderline), type II (tubular), or type III (funneled). Audiometric endpoints included air/bone conduction, pure tone averages, and air-bone gaps at 250 and 500 Hz. Statistical associations were evaluated using linear regression models, adjusted for age at first audiogram and sex. RESULTS: One hundred seventeen patients (197 ears) were included, with mean age at first audiogram of 22.2 years (standard deviation, 21.7 yr). Imaging features associated with poor audiometric outcomes were increasing VA width at midpoint and external aperture, decreasing VA length, dilated extraosseous endolymphatic sac, cochleovestibular malformations, and increasing VA type (III > II > I). CONCLUSIONS: Quantitative LVA measurements and a standardized morphologic classification system aid in prediction of early audiometric endpoints.

Modifications to Implanting the OSIA® 2 Bone Conduction Hearing Implant: How I Do it.

This "How I Do It" report describes modifications made to the OSIA bone conduction hearing implant surgery in order to reduce wound complications. Laryngoscope, 132:1850-1854, 2022.

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.

Endoscopic Transcanal and Transmastoid Laser-Assisted Resection of Middle Ear Capillary Hemangioma.

OBJECTIVE: To present a rare case of a middle ear capillary hemangioma in an adult. PATIENT: A 31-year-old woman with a 6-month history of left ear fullness, pressure, tinnitus, and progressive hearing loss. INTERVENTION: Endoscopic laser-assisted resection. MAIN OUTCOME MEASURES: Clinical, radiographic, and histopathological findings of a capillary hemangioma. RESULTS: Otoscopy revealed an erythematous and slightly pulsating multilobulated middle ear retrotympanic mass. Her audiogram demonstrated a left-sided mixed hearing loss with air-conduction thresholds in the severe-to-profound range. Computed tomography (CT) imaging was significant for total opacification of the left middle ear and mastoid air cells. She underwent a combined endoscopic transcanal and transmastoid excision of the mass with ossicular chain reconstruction. A KTP laser was used to ablate and shrink down the periphery of the lesion. Pathology of the specimen was consistent with a capillary hemangioma. The patient's pulsatile tinnitus and spontaneous vertigo resolved postoperatively. CONCLUSIONS: Capillary hemangiomas are an uncommon cause of vascular middle ear lesions in adults and typically present with symptoms of aural fullness, pulsatile tinnitus, conductive hearing loss, otalgia, and vertigo. Surgery resection provides definitive treatment and the use of laser ablation techniques can allow for hemostasis and excellent visualization.

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.

Iatrogenic Third Window After Retrosigmoid Approach to a Vestibular Schwannoma Managed with Cochlear Implantation.

OBJECTIVE: To present a case of an iatrogenic inner ear third window after vestibular schwannoma microsurgery. PATIENTS: A 42-year-old male presented 9-months after left-sided retrosigmoid approach for an intracanalicular vestibular schwannoma with hearing-preservation attempt performed elsewhere. Immediately postoperatively, he developed the following disabling and persistent symptoms on the ipsilateral side: autophony, pulsatile tinnitus, high-pitched ringing tinnitus, and hearing his footsteps. He denied vertigo. Otoscopy was normal. Tuning fork (512-Hz) lateralized to the left and Rinne was negative on the left. Audiogram demonstrated a severe mixed hearing loss and 10% aided word-recognition score. High-resolution CT demonstrated violation of the common crus and dehiscence of bone along the medial vestibule suggestive of an iatrogenic inner ear third window. INTERVENTIONS: Labyrinthectomy and concurrent cochlear implantation. MAIN OUTCOME MEASURES: Resolution of third window symptoms, open-set speech recognition, tinnitus suppression. RESULTS: Patient reported immediate resolution of third window symptoms after labyrinthectomy and cochlear implantation. He demonstrates open-set word recognition of 64% at 1-year postoperatively and tinnitus suppression with his cochlear implant on. CONCLUSIONS: Iatrogenic third window symptoms can occur after hearing-preservation vestibular schwannoma microsurgery. Patients with sufficient hearing preservation who are disabled by third window symptoms yet lack sound clarity and useful hearing may be considered for labyrinthectomy and concurrent cochlear implantation. This intervention effectively extinguishes third window symptoms by destroying residual auditory function and simultaneously provides an opportunity to restore useful hearing and suppress tinnitus, thereby enhancing overall quality of life.

Cochlear Implantation in Infants: Why and How.

In children with congenital deafness, cochlear implantation (CI) prior to 12 months of age offers the opportunity to foster more typical auditory development during late infancy and early childhood. Recent studies have found a positive association between early implantation and expressive and receptive language outcomes, with some children able to achieve normal language skills by the time of school entry. Universal newborn hearing screening improved early detection and diagnosis of congenital hearing loss, allowing for earlier intervention, including decision-making regarding cochlear implant (CI) candidacy. It can be more challenging to confirm CI candidacy in infants; therefore, a multidisciplinary approach, including objective audiometric testing, is recommended to not only confirm the diagnosis but also to counsel families regarding expectations and long-term management. Surgeons performing CI surgery in young children should consider both the anesthetic risks of surgery in infancy and the ways in which mastoid anatomy may differ between infants and older children or adults. Multiple studies have found CI surgery in infants can be performed safely and effectively. This article reviews current evidence regarding indications for implantation in children younger than 12 months of age and discusses perioperative considerations and surgical technique.

Cochlear Implantation in Infants: Evidence of Safety.

The aim of this study was to investigate surgical, anesthetic, and device-related complications associated with cochlear implantation (CI) in children younger than 1 year of age. This was a multicenter, retrospective chart review of all children with severe-to-profound sensorineural hearing loss who underwent cochlear implantation with a Cochlear Nucleus Implant System before 1 year of age. Endpoints included perioperative course, major and minor surgical, anesthetic and device-related complications, and 30-day readmission rates. One hundred thirty-six infants (242 ears) met criteria. The mean age at implantation was 9.4 months (standard deviation 1.8). Six-month follow-up was reported in all patients. There were no major anesthetic or device-related complications. Adverse events were reported in 34 of implanted ears (14%; 7 major, 27 minor). Sixteen adverse events occurred ≤30 days of surgery, and 18 occurred >30 days of surgery. The 30-day readmission rate was 1.5%. The rate of adverse events did not correlate with preexisting medical comorbidities or duration under anesthesia. There was no significant difference detected in complication rate for patients younger than 9 months of age versus those 9 to 11 months of age. This study demonstrates the safety of CI surgery in infants and supports reducing the indication for cochlear implantation to younger than 1 year of age for children with bilateral, profound sensorineural hearing loss obtaining a Cochlear Nucleus Implant System.

Cochlear Implantation in Adults With Single-sided Deafness: Outcomes and Device Use.

OBJECTIVE: To describe our experience with adults undergoing cochlear implantation (CI) for treatment of single-sided deafness (SSD). STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Fifty-three adults with SSD. INTERVENTIONS: Unilateral CI. MAIN OUTCOME MEASURES: Speech perception testing in quiet and noise, tinnitus suppression, and device usage from datalogs. RESULTS: The mean age at CI was 53.2 years (SD 11.9). The mean duration of deafness was 4.0 years (SD 7.8). The most common etiology was idiopathic sudden SNHL (50%). Word recognition improved from 8.7% (SD 15) preoperatively to 61.8% (SD 20) at a mean follow-up of 3.3 years (SD 1.8) (p < 0.0001). Adaptive speech recognition testing in the "binaural with CI" condition (speech directed toward the front and noise toward the normal hearing ear) revealed a significant improvement by 2.6-dB SNR compared to the preoperative unaided condition (p = 0.0002) and by 3.6-dB SNR compared to when a device to route sound to the contralateral side was used (p < 0.0001). Tinnitus suppression was reported to be complete in 23 patients (43%) and improved in 20 patients (38%) while the device was on. The addition of the CI did not lead to a decrement in hearing performance in any spatial configuration. Device usage averaged 8.7 (SD 3.7) hours/day. CONCLUSIONS: Cochlear implantation in adult SSD patients can suppress tinnitus and achieve speech perception outcomes comparable with CI in conventional candidates. Modest improvements in spatial hearing were also observed and primarily attributable to the head shadow effect. Careful patient selection and counseling regarding potential benefits are important to optimize outcomes.

Cochlear Implantation in Children with Single-Sided Deafness.

OBJECTIVE: To describe our experience with children undergoing unilateral cochlear implantation (CI) for treatment of single-sided deafness (SSD). STUDY DESIGN: Retrospective case series. METHODS: A retrospective case review from a tertiary referral center involving 14 pediatric patients (<18 years) with SSD who underwent unilateral CI. Speech perception testing in quiet and noise in the CI-only and bimodal conditions with at least 1 year of device use and device usage from data logs represent the main outcome measures. RESULTS: The mean age at CI was 5.0 years (median 4.4, range 1.0-11.8 years). The mean duration of deafness was 3.0 years (median 2.4, range 0.6-7.0 years). Mean follow-up was 3.4 years. Speech perception testing with a minimum of 1 year post-CI was available in eight patients. The mean word recognition scores (WRS) in the CI-only condition was 56%; a significant improvement from baseline. Testing in background noise with spatially separated speech and noise revealed that patients scored as well or better with the CI-on versus CI-off in all conditions and in no cases was interference from the CI noted. Data logs were reviewed for device usage which revealed an average use of 6.5 hr/d. CONCLUSION: Cochlear implantation is a viable treatment option for pediatric SSD in this self-selected cohort. Open-set speech and improvement in background noise can be achieved. Careful patient selection and thorough counseling on expectations is paramount to achieving successful outcomes. LEVEL OF EVIDENCE: IV Laryngoscope, 131:E271-E277, 2021.

Tracking Spontaneous Vestibular Schwannoma Regression with Volumetric Measurements.

OBJECTIVE: To characterize a series of patients with MRI evidence of spontaneous vestibular schwannoma (VS) regression. STUDY DESIGN: Retrospective case series. METHODS: Retrospective review between 2012 and 2020 from a single, tertiary-care center of all patients with an untreated, sporadic VS and spontaneous regression in volumetric tumor size over the course of observation. The main outcome measures included VS size and location, presenting symptoms, medication use, changes in pure-tone averages and word recognition scores. RESULTS: The 13 treatment-naïve patients (62% female, mean age 67.1 years) with spontaneous VS regression represented 3.9% of all patients undergoing observation with serial imaging during the study period. Median tumor size from initial MRI was 529.0 mm3 (range: 108 mm3 -13,180 mm3 ). The mean interval between MRI measurements was 5.5 years (SD 4.4 years). The average percent decrease in tumor size was 36.1% (SD 21.9%) and the average rate of volume decrease was 15.8 mm3 /yr (SD 25.4 mm3 /yr). Five patients were classified as having major regression, defined by a relative decrease in volume of >40%, while eight patients had minor regression (<40% relative volume reduction). No significant differences in initial tumor size, rate of regression, or audiometric changes were observed between the major and minor regression cohorts. CONCLUSIONS: Patients with evidence of a spontaneously shrinking VS have a heterogeneous presentation. Due to the scarcity of this phenomenon, predicting which tumors will eventually undergo regression remains unclear. Employing volumetric measurements to compare serial MRI scans may improve the accuracy of detecting shrinking tumors. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E1647-E1652, 2021.

Cochlear Implantation Under Local Anesthesia With Conscious Sedation in the Elderly: First 100 Cases.

OBJECTIVE: To report the outcomes on a large series of elderly patients who underwent cochlear implantation (CI) surgery under local anesthesia with conscious sedation (LA-CS). METHODS: Retrospective chart review on 100 consecutive elderly patients (> 65 years) who underwent CI with LA-CS at a tertiary care center between August 2013 and January 2020. An age-matched control group of 50 patients who underwent CI with general anesthesia (GA) are used for comparison. Outcomes measured included time in the operating room, time in the postanesthesia care unit (PACU), and rate of adverse events. RESULTS: Cochlear implant surgery under LA-CS was successfully performed in 99 (99%) patients. One patient requiring conversion to GA intraoperatively. No patients in the LA-CS group experienced cardiopulmonary adverse events; however, three patients (6%) in the GA group experienced minor events including atrial fibrillation and/or demand ischemia. Overnight observation in the hospital due to postoperative medical concerns or prolonged wake-up from anesthesia was required in one patient (1%) from the LA-CS cohort and 12 patients (24%) from the GA cohort. Perioperative adverse events exclusive to the LA-CS group included severe intraoperative vertigo (8%), temporary facial nerve paresis (3%), and wound infection (1%). The average amount of time spent in the operating room was 37 minutes less for procedures performed under LA-CS compared to GA (P < .05). The average amount of time in recovery was similar for both groups (P > .05). CONCLUSION: Cochlear implant surgery under LA-CS offers many benefits and is a safe, feasible, and cost-effective alternative to GA when performed by experienced CI surgeons. LEVEL OF EVIDENCE: 3 Laryngoscope, 131:E946-E951, 2021.

Cochlear Implant Outcomes in Neurofibromatosis Type 2: Implications for Management.

OBJECTIVE: To describe our institutional experience with cochlear implantation (CI) for rehabilitation of hearing loss in Neurofibromatosis type 2 (NF2) patients. STUDY DESIGN: Retrospective review between 1989 and 2019. SETTING: Tertiary-care center. PATIENTS: Twenty-four patients (67% female, mean age 45.6years) with NF2. Management of their ipsilateral vestibular schwannoma included microsurgery (n=12), stereotactic radiation (n=5), and observation (n=7). INTERVENTIONS: Cochlear implantation. MAIN OUTCOME MEASURES: Ability to obtain open-set speech, daily device usage and long-term device benefit. RESULTS: All patients achieved some degree of sound awareness with CI. Nineteen patients (79%) achieved open-set speech understanding with a mean word-recognition score of 43% (range 0-88%). Patients with tumors 1.5 cm or less demonstrated the better speech understanding, without significant differences among treatment modalities. For tumors greater than 1.5 cm, patients who underwent microsurgery had a lower rate of open-set speech understanding compared to those treated with radiation or observation. Regular daily device use in 83% of patients was found. Long-term use (>10years) was observed in several patients, though some ultimately required reimplantation with an auditory brainstem implant due to progressive tumor growth. Mean follow-up duration was 4.1 years (range 0.4-15). CONCLUSIONS: Cochlear implantation can be an effective treatment for hearing loss in NF2 patients provided the cochlear nerve is intact, regardless of prior management for the ipsilateral tumor. The degree of benefit varies and is influenced by tumor size. Management strategies that preserve the cochlear nerve maximize the interval during which a CI could be of benefit to NF2 patients.

Cochlear Implantation in Sporadic Vestibular Schwannoma and Other Retrocochlear Pathology: A Case Series.

OBJECTIVE: To describe outcomes with cochlear implantation (CI) for rehabilitation of hearing loss in patients with sporadic vestibular schwannomas (VS) and other retrocochlear pathologies. STUDY DESIGN: Retrospective review. SETTING: Tertiary-care center. PATIENTS: Twenty three cases in 19 patients (53% men, mean age 55.8 yr) with non-neurofibromatosis type 2 related retrocochlear pathology. INTERVENTIONS: Unilateral or bilateral CI. MAIN OUTCOME MEASURES: Word recognition score, device usage. RESULTS: Etiology of deafness included sporadic VS (n = 9, 39%), radiation after head and neck or central nervous system (CNS) malignancy (n = 8, 35%), superficial siderosis (n = 3, 13%), neurosarcoidosis (n = 2, 9%), and pontine stroke (n = 1, 4%). Mean follow-up duration was 2.3 years (standard deviation [SD] 3.0; range, 0.2-9.4). Auditory perception was achieved in 20 out of 22 patients (91%) who have been activated. Mean WRS in patients with sporadic VS was 18% (SD 20; range, 0-44). Mean WRS in patients with non-VS retrocochlear pathology was 55% (SD 30; range, 0-94). Data logs showed 7.0 h/d of average use (SD 4.3; range, 0-13). CONCLUSIONS: Appropriately selected patients with retrocochlear pathology may benefit from CI so long as the patient has a cochlear fluid signal and an intact cochlear nerve. Patients with sporadic VS patients and normal contralateral hearing exhibited guarded outcomes with CI, whereas most patients with non-VS retrocochlear pathologies demonstrated open-set speech understanding scores comparable to or slightly worse than conventional CI candidates. Since variable performance benefit is observed with CI in patients with retrocochlear pathology, counseling is imperative to align patient expectations with realistic outcomes.

From Bimodal Hearing to Sequential Bilateral Cochlear Implantation in Children-A Within-Subject Comparison.

OBJECTIVE: To evaluate the performance changes after sequential bilateral cochlear implantation in a pediatric population of bimodal cochlear implant (CI) users. To evaluate the factors which influence the parental and recipient decision to discontinue hearing aid use and seek a second implant. STUDY DESIGN: Retrospective case review, within-subject comparison. SETTING: Tertiary referral center. PATIENTS: Thirty-one pediatric (<18 yr) bimodal CI users who underwent sequential bilateral CI. INTERVENTIONS: Sequential bilateral CI. MAIN OUTCOME MEASURES: Parental and/or recipient's reasons for discontinuing their hearing aid and pursuing a second implant, device usage from datalogs, speech understanding in the bimodal and bilateral CI condition. RESULTS: Parents/patients were motivated to pursue sequential bilateral CI based on their positive performance with CI1, the expectation of further improvement with a second CI, and the prospect of having a second independently functional ear. In the bimodal condition, mean word recognition score (WRS), sentence recognition in quiet (SIQ), and sentence recognition in noise (SIN) scores were 87.4, 97.3, and 92.9% respectively. At 1-year post-sequential bilateral CI, the mean WRS, SIQ, and SIN score were 92.7, 98.7, and 97.7%, respectively. The improvement in bilateral CI speech scores compared with bimodal scores was statistically significant for WRS (p = 0.015). A ceiling effect limited the ability to detect further meaningful differences on speech perception testing. CONCLUSIONS: The bilateral CI condition demonstrates equivalent or slightly superior performance compared with the bimodal condition. Several non-speech benefits were elicited from parents as reasons for pursuing a second implant. Close monitoring of the residual acoustic hearing, inquiring about the perceived benefits provided by the HA, and early counseling regarding the potential for sequential bilateral CI are important aspects in determining if and when a second implant is indicated.