Optic Nerve T2 Signal Intensity and Caliber Reflect Clinical Severity in Genetic Optic Atrophy.

BACKGROUND: Genetic optic atrophies comprise phenotypically heterogenous disorders of mitochondrial function. We aimed to correlate quantitative neuroimaging findings of the optic nerves in these disorders with clinical measures. METHODS: From a retrospective database of 111 patients with bilateral optic atrophy referred for genetic testing, 15 patients diagnosed with nonglaucomatous optic atrophy of genetic origin (7 patients with pathogenic variants in OPA1, 3 patients with Wolfram syndrome, and 5 patients with Leber hereditary optic neuropathy) who had accessible magnetic resonance (MR) images of the orbits and/or brain were analyzed. The primary outcome measures of T2 short Tau inversion recovery (STIR) signal and optic nerve caliber were quantified according to a standardized protocol, normalized to internal standards, and compared between cases and controls. Inter-rater reliability was assessed and clinical features were analyzed according to MRI features. RESULTS: Compared with control patients, the 15 genetic optic atrophy patients demonstrated significantly increased T2 STIR signal (fold-change 1.6, P = 0.0016) and decreased optic nerve caliber (fold-change 0.72, P = 0.00012) after internal normalization. These metrics were reliable (inter-reader reliability correlation coefficients of 0.98 [P = 0.00036] and 0.74 [P = 0.0025] for normalized STIR and nerve caliber, respectively) and significantly correlated with visual acuity, cup-to-disc ratio, and visual field testing. CONCLUSION: Normalized optic nerve STIR signal and optic nerve caliber significantly correlate with visual acuity, cup-to-disc ratio, and perimetric performance in patients with genetic optic atrophy. A formalized protocol to characterize these differences on MRI may help to guide accurate and expedient diagnostic evaluation.

Adult Auditory Brainstem Implant Outcomes and Three-Dimensional Electrode Array Position on Computed Tomography.

OBJECTIVES: Factors contributing to auditory brainstem implant (ABI) outcomes are poorly understood. The aims of this study are to (1) characterize ABI electrode array position on postoperative imaging and (2) determine if variability in position is related to perceptual outcomes. DESIGN: Retrospective cohort study. Subjects were selected from the adult ABI recipient population at Massachusetts Eye and Ear. Postoperative three-dimensional (3D) computed tomography (CT) reconstruction of the head was used to measure ABI array position in 20 adult ABI recipients (17 with Neurofibromatosis Type 2 (NF2) and three non-NF2 recipients). Three-dimensional electrode array position was determined based on angles from the horizontal using posterior and lateral views and on distances between the proximal array tip superiorly from the basion (D1), laterally (D2P) and posteriorly (D2L) from the midline. Array position was correlated with perceptual data (in 15 of the 20 recipients who used their ABI). Perceptual data included the number of electrodes that provided auditory sensation, location and type of side effects, level of speech perception (from no sound to open-set word recognition of monosyllables) and the amount of charge required for auditory perception. RESULTS: Although the 3D orientation of the ABI array exhibited a variety of angles, all arrays were posteriorly tilted from the lateral view and most were medially tilted from the posterior view. ABI position relative to the basion from posterior showed mean distances of 1.71 ± 0.42 and 1.1 ± 0.29 cm for D1 and D2, respectively, and a mean D2 of 1.30 ± 0.45 cm from the lateral view. A strong linear negative correlation was found between the number of active electrodes and the distance of the proximal array tip laterally from the basion (D2P; rs = -0.73, p = 0.006) when measured in the posterior view. Although side effects were experienced in all recipients and varied in type and location across the array, electrodes in the middle part of the array tended to elicit auditory sensations while the proximal and distal tips of the array tended to elicit nonauditory side effects. Arrays with and without low charge thresholds appeared to generally overlap in position. However, the two recipients with the best (open-set) speech perception had low charge thresholds and had arrays that were tilted superiorly in the posterior view. CONCLUSION: ABI recipients with better speech perception appear to share a profile of arrays that are tilted superiorly as compared to recipients with lower speech perception levels. These ABI recipients have a high number of active electrodes (10 or more) and require less electrical charge on individual electrodes to achieve optimal stimulation.

Site of Origin of the Ophthalmic Artery Influences the Risk for Retinal Versus Cerebral Embolic Events.

BACKGROUND: Embolic events leading to retinal ischemia or cerebral ischemia share common risk factors; however, it has been well documented that the rate of concurrent cerebral infarction is higher in patients with a history of transient ischemic attack (TIA) than in those with monocular vision loss (MVL) due to retinal ischemia. Despite the fact that emboli to the ophthalmic artery (OA) and middle cerebral artery share the internal carotid artery (ICA) as a common origin or transit for emboli, the asymmetry in their final destination has not been fully explained. We hypothesize that the anatomic location of the OA takeoff from the ICA may contribute to the differential flow of small emboli to the retinal circulation vs the cerebral circulation. METHODS: We report a retrospective, comparative, case-control study on 28 patients with retinal ischemia and 26 patients with TIA or cerebral infarction caused by embolic events. All subjects underwent either computed tomography angiography or MRA. The location of the ipsilateral OA origin off the ICA was then graded in a blinded fashion and compared between cohorts. Vascular risk factors were collected for all patients, including age, sex, hypertension, hyperlipidemia, arrhythmia, diabetes, coronary artery disease, and smoking. RESULTS: We find that in patients with retinal ischemia of embolic etiology, the ipsilateral OA takeoff from the ICA is more proximal than in patients with cerebral infarcts or TIA (P = 0.0002). We found no statistically significant differences in demographic, vascular, or systemic risk factors. CONCLUSIONS: We find that the mean anatomical location of the OA takeoff from the ICA is significantly more proximal in patients with MVL due to retinal ischemia compared with patients with TIA or cerebral ischemia. This finding contributes significantly to our understanding of a long observed but poorly understood phenomenon that patients with MVL are less likely to have concurrent cerebral ischemia than are patients with TIA.

Comparison between computed tomography and ultrasound for presurgical evaluation of oral tongue squamous cell carcinoma tumor thickness.

PURPOSE: To compare the accuracy of oral tongue squamous cell carcinoma (OTSCC) tumor thickness (TT) measured on CT to intraoperative ultrasound (US) and histopathology. METHODS AND MATERIALS: Twenty-six patients with OTSCC who underwent tumor resection by a single surgeon with simultaneous intraoperative US between 3/2016 and 4/2019 were prospectively identified, and their data reviewed. TT was independently measured in 19 patients who underwent preoperative CT (cTT) by two neuroradiologists blinded to US and histological results. The confidence level of interpretation of cTT was recorded by each reader using a 5-point Likert scale. The degree of dental artifact on CT was also scored. cTT was compared to TT measured on intraoperative US (uTT) and histopathologic assessment of TT (hTT). RESULTS: OTSCC was visualized on CT in 52% (10/19) and 63% (12/19) of cases for readers 1 and 2, respectively. Mean Likert score was 0.42 for reader 1 and 0.73 for reader 2. Mean cTT of OTSCCs was 5.8 mm +/- 1.7 mm (n = 11). In comparison, mean uTT and hTT were 7.6 mm±3.5 mm and 7.1 +/- 4.2 mm, respectively. The Pearson coefficient (95% confidence interval) was 0.10 (-0.53-0.66) between cTT and hTT (n = 11) and 0.93 (0.74-0.98) between uTT and hTT. CONCLUSIONS: Preoperative CT is not reliable for assessment of TT in OTSCC compared to US and histopathology, particularly for OTSCC under 10 mm. US offers a practical complementary imaging tool with a unique role for primary tumor assessment that can aid in pre-operative planning, especially for small tumors.

Subperiosteal Masqueraders as Compared to Subperiosteal Abscess: Contrasting Clinical Presentation and Radiographic Densities.

PURPOSE: Subperiosteal orbital lesions are most commonly abscesses secondary to sinusitis but, in rare cases, may represent other processes. Here, the authors compare the clinical and radiographic presentation of subperiosteal abscesses and alternate subperiosteal processes ("masqueraders") in an effort to establish distinguishing preoperative diagnostic criteria. METHODS: A retrospective chart review of cases of subperiosteal orbital lesions that underwent surgical intervention over a 3-year period was performed. The medical records of 6 cases of subperiosteal masqueraders and 6 cases of abscesses were reviewed for the clinical course, imaging (including radiographic density of lesions), and pathology. Clinical and radiographic features of the 2 groups were compared. RESULTS: All cases presented with orbital signs on exam. Fever and leukocytosis were absent in the masquerader group and present in 3 patients from the abscess group. Common radiographic findings in both groups included a rim-enhancing convex mass along the orbital wall and adjacent sinus opacification, often with bony dehiscence. Of the masqueraders, the final diagnosis was hematoma in 3 cases, mucocele in 1, and malignancy in 2. The difference between the mean radiodensity of the subperiosteal abscesses, 38 ± 5 Hounsfield units (95% CI, 34-42), as compared with the average radiodensity of the masqueraders, 71 ± 5 Hounsfield units (95% CI, 67-75), was significant (p = 0.042). Comparing radiodensity of the orbital lesion to adjacent sinus lesions and metastatic lesions elsewhere was also informative in establishing the diagnosis. CONCLUSIONS: Radiographic features, particularly radiodensity, may help distinguish subperiosteal abscesses from other lesions and aid in preoperative diagnosis and management.

Auditory Brainstem Implant Array Position Varies Widely Among Adult and Pediatric Patients and Is Associated With Perception.

OBJECTIVES: The auditory brainstem implant (ABI) provides sound awareness to patients who are ineligible for cochlear implantation. Auditory performance varies widely among similar ABI cohorts. We hypothesize that differences in electrode array position contribute to this variance. Herein, we classify ABI array position based on postoperative imaging and investigate the relationship between position and perception. DESIGN: Retrospective review of pediatric and adult ABI users with postoperative computed tomography. To standardize views across subjects, true axial reformatted series of scans were created using the McRae line. Using multiplanar reconstructions, basion and electrode array tip coordinates and array angles from vertical were measured. From a lateral view, array angles (V) were classified into types I to IV, and from posterior view, array angles (T) were classified into types A to D. Array position was further categorized by measuring distance vertical from basion (D1) and lateral from midline (D2). Differences between array classifications were compared with audiometric thresholds, number of active electrodes, and pitch ranking. RESULTS: Pediatric (n = 4, 2 with revisions) and adult (n = 7) ABI subjects were included in this study. Subjects had a wide variety of ABI array angles, but most were aimed superiorly and posteriorly (type II, n = 7) from lateral view and upright or medially tilted from posterior view (type A, n = 6). Mean pediatric distances were 8 to 42% smaller than adults for D1 and D2. In subjects with perceptual data, electrical thresholds and the number of active electrodes differed among classification types. CONCLUSIONS: In this first study to classify ABI electrode array orientation, array position varied widely. This variability may explain differences in auditory performance.

Location of Small Intracanalicular Vestibular Schwannomas Based on Magnetic Resonance Imaging.

Vestibular schwannomas (VSs) were proposed to arise from the glial-Schwann cell junction within the internal auditory canal (IAC). However, otopathology studies indicate that VS may arise anywhere along the course of the vestibular nerve. Recent studies suggested that the majority of tumors are located centrally within the IAC with an equal distribution near the porus acusticus and the fundus. However, these studies analyzed tumors of all sizes, obscuring their precise origin. Herein, we aim to quantify the position of small intracanalicular tumors (<5 mm), assessing hearing outcomes and growth patterns in relation to tumor position. Of the 38 small intracanalicular tumors analyzed, 61% originated closest to the fundus, 34% at the midpoint, and only 5% closest to the porus acusticus. Tumors were observed with serial magnetic resonance imaging for 3.37 ± 2.65 years (mean ± SD) without intervention. Our findings indicate a lateral predominance of small VS within the IAC, an independence between tumor location and hearing outcomes, and further support the slow natural progression of VS.

Utility of intraoral ultrasound in managing oral tongue squamous cell carcinoma: Systematic review.

OBJECTIVES/HYPOTHESIS: Adequate surgical resection of early stage oral tongue cancer provides the best chance at preventing locoregional disease recurrence. Determination of tumor dimensions and margin location is challenging and can lead to inadequate resections with close/positive margins. Ultrasonography has proven its utility in determining the thickness and extent of tongue tumors. Preoperative tumor dimension measurements carry increased significance with the addition of depth of invasion (DOI) to the eighth edition of the American Joint Committee on Cancer (AJCC) TNM staging system. We report the results of a systematic review of the literature pertaining to the use of ultrasound in the diagnosis and management of oral tongue carcinoma. METHODS: The Preferred Reporting Items for Systematic Reviews and Meta-analysis statement checklist was used to inform the design of this systematic review. All studies that utilized ultrasound in the diagnosis/management of primary carcinoma of the oral tongue were included. PubMed, Embase, and Cochrane were reviewed to identify eligible studies. RESULTS: Nineteen articles were included in our analysis. Six hundred seventy-eight patients were studied in the articles included. Ultrasound tumor thickness measurements correlate well with those on histopathology and show promise as a predictor of cervical lymph node metastasis. Ultrasound can be safely used intraoperatively for deep margin assessment. CONCLUSIONS: Ultrasound is useful in the evaluation of oral tongue malignancies. More experience is needed to determine if it is reliable in determining preoperative DOI in light of the role this tumor parameter plays in the eighth edition of the AJCC staging manual. Laryngoscope, 129:662-670, 2019.

Intraoperative Ultrasound in Oral Tongue Cancer Resection: Feasibility Study and Early Outcomes.

The current standard of care in oral tongue cancer surgery is complete resection with a target of 5-mm microscopic clearance at all margins on final pathologic review. While current methods of resection are often successful at determining the mucosal margins of the lesion, they may be limited when attempting to achieve an adequate deep margin. A number of previous studies suggested that ultrasound is superior to manual palpation and other imaging modalities (computed tomography, magnetic resonance imaging) at demarcating the margins of tongue lesions. Recent clinical reports of the intraoperative use of this modality have used an invasive method to mark the proposed deep resection margin. In this communication, we report our initial experience with the use of intraoperative ultrasound as an adjunct to oral tongue cancer surgery without the use of an invasive method to mark the deep resection margin.

Chronic orbital and calvarial fungal infection with Apophysomyces variabilis in an immunocompetent patient.

Apophysomyces is a rare fungal organism causing rhino-orbito-cerebral mycotic infections with high morbidity and mortality, typically in immunocompetent individuals. Several cases of Apophysomyces elegans orbital disease have been reported. Herein, we report a case of Apophysomyces variabilis infection involving the orbit, sinuses, and calvarium in an immunocompetent 74-year-old woman, with a review of the literature. Unlike prior cases of A. elegans classic rhino-orbito-cerebral infection, our case included diffuse calvarial lytic lesions and overlying soft tissue nodules, but without parenchymal intracranial involvement. There was radiographic and clinical evidence of infarction of the orbital contents and cavernous sinus thrombosis. Anastomoses between the superior orbital (ophthalmic) vein and diploic veins of the calvarium are believed to be primarily responsible for the unusual mode of spread on the extradural surface of the brain. Although the patient stabilized without definitive surgical intervention, her disease slowly and intermittently progressed for over a year after presentation, requiring multiple courses of antifungal treatment.

Correlation Between Aspirin Intake and Reduced Growth of Human Vestibular Schwannoma: Volumetric Analysis.

OBJECTIVE: To determine whether people with sporadic vestibular schwannoma (VS) who take aspirin for unrelated medical reasons exhibit less tumor growth than nonaspirin users. We previously demonstrated the efficacy of salicylates in inhibiting VS growth in vitro, corroborating the results of our retrospective clinical study, which found halted VS growth (based on linear tumor measurements) in aspirin users. The current study evaluates this association using more accurate tumor volumetric measurements, and quantifies the degree of frequency-specific, VS-induced hearing loss. STUDY DESIGN: Retrospective analysis. SETTING: Tertiary care hospital. PATIENTS: Diagnosed with VS between 1980 and 2012, followed by serial magnetic resonance imaging for at least 1 year. MAIN OUTCOME MEASURES: Patient history of aspirin intake; change in VS volume over time of observation; frequency-specific, VS-induced audiometric threshold shifts. RESULTS: Of the 347 patients followed by serial magnetic resonance imaging scans, 86 had sequential scans available for 3D-segmented volumetric analysis for up to 11 years of follow-up (median 53 mo). Twenty-five (29%) had documented history of aspirin intake; 8 (32%) of these demonstrated VS growth. Of the 61 (71%) nonusers, 36 (59%) demonstrated growth. A significant inverse association was found among aspirin users and VS growth: odds ratio 0.32, 95% confidence interval 0.11 to 0.91. VS-induced audiometric thresholds shifts were larger above than below 2000 Hz. CONCLUSION: Our volumetric analysis of VS growth reaffirms the results of our linear analysis and suggests that aspirin may inhibit VS growth. The audiometric findings are consistent with the previously reported VS-induced predominantly high-frequency sensorineural hearing loss.

Cochlear Implantation in a Patient With Pfeiffer Syndrome and Temporal Bone Vascular Anomalies.

OBJECTIVE: Pfeiffer syndrome is a rare craniosynostotic disorder resulting in premature bony fusion of the skull, which can result in abnormal temporal bone and vascular anatomy and hearing loss. Cochlear implantation in these patients requires thoughtful surgical planning given the potential for limited access to the cochlea because of subcutaneous or intratemporal vasculature. Herein, we present a patient with Pfeiffer syndrome who underwent successful cochlear implantation using preoperative Doppler ultrasound to identify large extracranial venous anatomy followed by a modified transcanal surgical approach. PATIENTS: An adult female patient with Pfeiffer syndrome. INTERVENTION(S): Preoperative axial computed tomography, catheter angiography, Doppler ultrasound, and right side cochlear implant. MAIN OUTCOME MEASURE(S): Surgical feasibility and audiometric outcomes including aided thresholds and word recognition score. RESULTS: Successful cochlear implantation was performed via a modified transcanal approach with blind sac closure of the external auditory meatus. Full electrode insertion was obtained through a round window approach. No extracranial or intratemporal vessels were encountered during surgery. CONCLUSION: Patients with significant craniosynostoses and vascular malformations of the temporal bone can undergo successful cochlear implantation. Careful preoperative planning with high-resolution CT, MRA, and MRV, and/or traditional catheter angiography can assist in determining surgical feasibility and minimizing risk. We recommend preoperative Doppler ultrasound of abnormal extracranial vessels to assist in safe placement of incisions and hardware.

Radiographic evaluation of nasal septal deviation from computed tomography correlates poorly with physical exam findings.

BACKGROUND: Performance of septoplasty is dependent on objective evidence of nasal septal deviation. Although physical examination including anterior rhinoscopy and endoscopic examination is the gold standard for evaluation of septal deviation, third-party payors' reviews of septoplasty claims are often made on computed tomography (CT) findings. However, the correlation between radiographic evaluation of septal deviation with physical examination findings is unknown. METHODS: Retrospective, blinded, independent evaluation of septal deviation in 39 consecutive patients from physical examination, including anterior rhinoscopy and endoscopic examination, by an otolaryngologist and radiographic evaluation of sinus CT scan by a neuroradiologist. Four distinct septal locations (nasal valve, cartilaginous, inferior/maxillary crest and osseous septum) were evaluated on a 4-point scale representing (1) 0% to 25%, (2) >25% to 50%, (3) >50% to 75%, and (4) >75% obstruction. Correlation between physical examination and radiographic evaluations was made by Pearson's correlation and quantitative agreement assessed by Krippendorf's alpha. RESULTS: Statistically significant correlation was detected between physical examination including nasal endoscopy and radiographic assessment of septal deviation only at the osseous septum (p = 0.007, r = 0.425) with low quantitative agreement (α = 0.290). No significant correlation was detected at the cartilaginous septum (p = 0.286, r = 0.175), inferior septum (p = 0.117, r = 0.255), or nasal valve (p = 0.174, r = 0.222). Quantitative agreement at the nasal valve suggested a bias in CT to underestimate physical exam findings (α = -0.490). CONCLUSION: CT is a poor substitute for physical examination, the gold standard, in assessment of septal deviation. Clinical decisions about pursuit of septoplasty or third-party payors' decisions to approve septoplasty should not be made on radiographic evidence.

Objective radiographic density measurements of sinus opacities are not strong predictors of noninvasive fungal disease.

BACKGROUND: High-density paranasal sinus opacities are often deemed consistent with fungal elements. No studies of objective quantitative radiographic density measures have been performed to support this assertion. METHODS: A consecutive series of 120 patients with chronic rhinosinusitis who underwent maxillary antrostomy with microbiological evaluation of contents within 60 days of sinus computed tomography scanning was investigated. Radiographic density characteristics of opacities in cultured maxillary sinuses (minimum, maximum, average, and standard deviation of Hounsfield units [HUstd]) were recorded. Receiver operator characteristic (ROC) curves were used to analyze the accuracy of radiographic characteristics in predicting fungal opacities. RESULTS: Of 133 maxillary sinus opacities, 22 were ultimately consistent with noninvasive fungal disease: 11 allergic fungal rhinosinusitis and 11 fungal balls. Fungal balls had higher-density components and were more heterogeneous and allergic fungal mucin was generally more radiodense. These findings were reflected by statistically significant ROC curves for maximum HU (p = 0.019) and HUstd (p = 0.023) for fungal balls and for average HU (p = 0.002) for allergic fungal mucin. A maximum HU cutoff of 334.0 detected fungal balls with 90.9% sensitivity and 72.7% specificity. An average HU cutoff of 42.9 HU detected allergic fungal mucin with 100% sensitivity and 46.3% specificity, although specificity improved to 73.2% with inclusion of nasal polyposis as a second requirement. CONCLUSION: Higher average HU more accurately predicts allergic fungal mucin whereas heterogeneity/high-density components more accurately predict fungal balls. No objective radiographic density measure, in isolation, is both sensitive and specific in predicting noninvasive fungal sinusitis.