Deep, Nicholas L.

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.

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. Intervention(s) Diagnostic only. Main Outcome Measure(s) 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.

Abstract Background Vestibular migraine (VM) is one of the common causes of episodic dizziness, but it is underdiagnosed and poorly understood. Previous research suggests that otolith reflex pathway performance is often impaired in this patient group, leading to altered perception of roll plane stimuli. Clinically, this perception can be measured with subjective visual vertical (SVV) testing. Purpose The aim of this study is to compare static SVV performance (absolute mean SVV tilt, variance) in a cohort of patients diagnosed with VM to results obtained from clinically derived normative data. Study Design Retrospective case review. Study Sample Ninety-four consecutive patients between 18 and 65 years of age diagnosed with VM were included in this comparison to clinically derived normative data. Data Collection and Analysis Retrospective chart review was completed. Demographic data, symptom report, and vestibular laboratory results were documented. SVV performance was documented in terms of absolute mean SVV tilt and response variance. Results Abnormal mean SVV tilt was described in 54% (n = 51) of patients with VM. Including abnormal response variance increased those identified with abnormal presentation to 67% (n = 63). Laboratory findings were insignificant for semicircular canal function, but of those with abnormal ocular vestibular myogenic potential results (n = 30), 77% (n = 23) demonstrated both abnormal SVV and utriculo-ocular reflex performance. There were no associations noted for SVV performance and demographic or other self-report variables. Conclusion Absolute mean SVV tilt and response variance are often abnormal in patients diagnosed with VM. These findings support theories suggesting atypical intralabyrinthine integration within the vestibular nuclei and cerebellar nodular pathways.

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.

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.