PURPOSE: To compare the results of visual acuity testing in a population of deaf children using the Handy Eye Chart versus the Lea Symbols Chart and to compare testability and preference between charts. METHODS: A total of 24 participants were recruited at the Atlanta Area School for the Deaf. Visual Acuity was evaluated using the Handy Eye Chart and the Lea Symbols Chart. Patient preference and duration of testing were measured. RESULTS: The mean difference between the visual acuity as measured by each chart was -0.02 logMAR (95% CI, -0.06 to 0.03). Testing with the Handy Eye Chart was an average of 13.79 seconds faster than testing with the Lea Symbols Chart (95% CI, 1.1-26.47; P = 0.03). Of the 24 participants, 17 (71%) preferred the Handy Eye Chart (95% CI: 49%-87%; P = 0.07). CONCLUSIONS: The Handy Eye Chart is a fast, valid, and useful tool for measuring visual acuity in deaf children age 7-18 years. Additional research is needed to evaluate the utility of the Handy Eye Chart in younger children and deaf adults.

Importance: Diagnostic errors can lead to the initial misdiagnosis of optic nerve sheath meningiomas (ONSM), which can lead to vision loss. Objective: To identify factors contributing to the initial misdiagnosis of ONSM. Design, Setting, and Participants: We retrospectively reviewed 35 of 39 patients with unilateral ONSM (89.7%) who were seen in the tertiary neuro-ophthalmology practice at Emory University School of Medicine between January 2002 and M arch 2017. The Diagnosis Error Evaluation and Research taxonomy tool was applied to cases with missed/delayed diagnoses. Exposures: Evaluation in a neuro-ophthalmology clinic. Main Outcomes and Measures: Identifying the cause of diagnostic errors for patients who initially received a misdiagnosis who were found to have ONSM. Results: Of 35 patients with unilateral ONSM (30 women [85.7%]; mean [SD] age, 45.26 [15.73] years), 25 (71%) had a diagnosis delayed for a mean (SD) of 62.60 (89.26) months. The most common diagnostic error (19 of 25 [76%]) was clinician assessment failure (errors in hypothesis generation and weighing), followed by errors in diagnostic testing (15 of 25 [60%]). The most common initial misdiagnosis was optic neuritis (12 of 25 [48%]), followed by the failure to recognize optic neuropathy in patients with ocular disorders. Five patients who received a misdiagnosis (20%) underwent unnecessary lumbar puncture, 12 patients (48%) unnecessary laboratory tests, and 6 patients (24%) unnecessary steroid treatment. Among the 16 patients who initially received a misdiagnosis that was later correctly diagnosed at our institution, 11 (68.8%) had prior magnetic resonance imaging (MRI) results that were read as healthy; 5 (45.5%) showed ONSM but were misread by a non-neuroradiologist and 6 (54.5%) were performed incorrectly (no orbital sequence or contrast). Sixteen of the 25 patients (64%) had a poor visual outcome. Conclusions and Relevance: Biased preestablished diagnoses, inaccurate funduscopic examinations, a failure to order the correct test (MRI brain/orbits with contrast), and a failure to correctly interpret MRI results were the most common sources of diagnostic errors and delayed diagnosis with worse visual outcomes and increased cost (more visits and tests). Easier access to neuro-ophthalmologists, improved diagnostic strategies, and education regarding neuroimaging should help prevent diagnostic errors..

Purpose: To estimate the proportion of patients presenting with isolated third, fourth, or sixth cranial nerve palsy of presumed microvascular origin versus other causes. Design: Prospective, multicenter, observational case series. Participants: A total of 109 patients aged 50 years or older with acute isolated ocular motor nerve palsy. Testing: Magnetic resonance imaging (MRI) of the brain. Main Outcome Measures: Causes of acute isolated ocular motor nerve palsy (presumed microvascular or other) as determined with early MRI and clinical assessment. Results: Among 109 patients enrolled in the study, 22 had cranial nerve III palsy, 25 had cranial nerve IV palsy, and 62 had cranial nerve VI palsy. A cause other than presumed microvascular ischemia was identified in 18 patients (16.5%; 95% confidence interval, 10.7-24.6). The presence of 1 or more vasculopathic risk factors (diabetes, hypertension, hypercholesterolemia, coronary artery disease, myocardial infarction, stroke, and smoking) was significantly associated with a presumed microvascular cause (P = 0.003, Fisher exact test). Vasculopathic risk factors were also present in 61% of patients (11/18) with other causes. In the group of patients who had vasculopathic risk factors only, with no other significant medical condition, 10% of patients (8/80) were found to have other causes, including midbrain infarction, neoplasms, inflammation, pituitary apoplexy, and giant cell arteritis (GCA). By excluding patients with third cranial nerve palsies and those with GCA, the incidence of other causes for isolated fourth and sixth cranial nerve palsies was 4.7% (3/64). Conclusions: In our series of patients with acute isolated ocular motor nerve palsies, a substantial proportion of patients had other causes, including neoplasm, GCA, and brain stem infarction. Brain MRI and laboratory workup have a role in the initial evaluation of older patients with isolated acute ocular motor nerve palsies regardless of whether vascular risk factors are present.

Idiopathic intracranial hypertension (IIH) is increasingly recognized as a cause of spontaneous cerebrospinal fluid (CSF) leak in the otolarnygological and neurosurgical literature. The diagnosis of IIH in patients with spontaneous CSF leaks typically is made a few weeks after surgical repair of the leak when symptoms and signs of elevated intracranial pressure (ICP) appear. Case reports and literature review. Two young obese women developed spontaneous CSF rhinorrhea related to an empty sella in one and a cribriform plate encephalocele in the other. Both patients underwent surgical repair of the CSF leak. A few weeks later, they developed chronic headaches and bilateral papilledema. Lumbar punctures showed elevated CSF opening pressures with normal CSF contents, with temporary improvement of headaches. A man with a 3-year history of untreated IIH developed spontaneous CSF rhinorrhea. He experienced improvement of his headaches and papilledema after a CSF shunting procedure, and the rhinorrhea resolved after endoscopic repair of the leak. These cases and the literature review confirm a definite association between IIH and spontaneous CSF leak based on: 1) similar demographics; 2) increased ICP in some patients with spontaneous CSF leak after leak repair; 3) higher rate of leak recurrence in patients with raised ICP; 4) patients with intracranial hypertension secondary to tumors may develop CSF leak, confirming that raised ICP from other causes than IIH can cause CSF leak. CSF leak occasionally may keep IIH patients symptom-free; however, classic symptoms and signs of intracranial hypertension may develop after a CSF leak is repaired, exposing these patients to a high risk of recurrence of the leak unless an ICP-lowering intervention is performed.

BACKGROUND: The prevalence of optic nerve and retinal vascular changes within the obstructive sleep apnea (OSA) population are not well-known, although it has been postulated that optic nerve ischemic changes and findings related to an elevated intracranial pressure may be more common in OSA patients. We prospectively evaluated the ocular fundus in unselected patients undergoing overnight diagnostic polysomnography (PSG). METHODS: Demographic data, medical/ocular history, and nonmydriatic fundus photographs were prospectively collected in patients undergoing PSG at our institution and reviewed for the presence of optic disc edema for which our study was appropriately powered a priori. Retinal vascular changes were also evaluated. OSA was defined using the measures of both sleep-disordered breathing and hypoxia. RESULTS: Of 250 patients evaluated in the sleep center, fundus photographs were performed on 215 patients, among whom 127 patients (59%) had an apnea/hypopnea index (AHI) ≥15 events per hour, including 36 with severe OSA. Those with AHI <15 served as the comparison group. None of the patients had optic disc edema (95% confidence interval [CI]: 0%-3%). There was no difference in rates of glaucomatous appearance or pallor of the optic disc among the groups. Retinal arteriolar changes were more common in severe OSA patients (odds ratio: 1.09 per 5 unit increase in AHI; 95% CI, 1.02-1.16; P = 0.01), even after controlling for mean arterial blood pressure. CONCLUSIONS: We did not find an increased prevalence of optic disc edema or other optic neuropathies in our OSA population. However, retinal vascular changes were more common in patients with severe OSA, independent of blood pressure.

Backgrounds: Recent studies have identified the role of serologic markers in characterizing disease phenotype, location, complications, and severity among Northern Europeans (NE) with Crohn's disease (CD). However, very little is known about the role of serology in CD among African Americans (AA). Our study explored the relationship between serology and disease phenotype in AA with CD, while controlling for genetic ancestry. Methods: AAs with CD were enrolled as participants through multicenter collaborative efforts. Serological levels of IgA anti-Saccharomyces cervisiae antibody (ASCA), IgG ASCA, E. coli outermembrane porin C, anti-CBir1, and ANCA were measured using enzyme-linked immunosorbent assays. Genotyping was performed using Illumina immunochip technology; an admixture rate was calculated for each subject. Multiple imputation by chained equations was performed to account for data missing at random. Logistic regression was used to calculate adjusted odds ratio (OR) for associations between serological markers and both complicated disease and disease requiring surgery. Results: A total of 358 patients were included in the analysis. The majority of our patients had inflammatory, noncomplicated disease (58.4%), perianal disease (55.7%), and documented colonic inflammation (86.8%). On multivariable analysis, both IgG ASCA and OmpC were associated with complicated disease (OR, 2.67; 95% CI, 1.67-4.28; OR, 2.23; 95% CI, 1.41-3.53, respectively) and disease requiring surgery (OR, 2.51; 95% CI, 1.49-4.22; OR, 3.57; 95% CI, 2.12-6.00). NE admixture to the African genome did not have any associations or interactions in relation to clinical outcome. Conclusions: Our study comprises the largest cohort of AAs with CD. The utility of serological markers for the prognosis of CD in NE applies equally to AA populations.

Ocular fundus examination is a fundamental component of the neurologic examination. Finding papilledema in headache patients or retinal arterial emboli in stroke patients can be extremely useful. Although examination of the ocular fundus with a direct ophthalmoscope is an important skill for all neurologists, it is rarely and unreliably performed. Nonmydriatic ocular fundus photography, which allows direct visualization of high-quality photographs of the ocular fundus, has been recently proposed for screening neurologic patients in urgent care settings such as emergency departments. This new technology has many potential applications in neurology, including e-transmission of images for remote interpretation.

Ocular fundus examination is a critical part of the physical examination in patients with severely elevated blood pressure (BP), which is defined by the Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure (JNC7) as a BP exceeding 180/120 mm Hg. Indeed, the presence or absence of severe, grade III/IV hypertensive retinopathy helps differentiate hypertensive emergencies requiring intensive care from less severe hypertensive urgencies. As a secondary analysis in the Fundus photography versus Ophthalmoscopy Trial Outcomes in the Emergency Department (FOTO-ED) study, we sought to explore potential risk factors, in particular BP, for the presence of ocular fundus abnormalities relevant to the care of emergency department (ED) patients. We found evidence of acute end-organ ocular damage at lower blood pressures than the JNC7 criteria.

Idiopathic Intracranial Hypertension (IIH) is a disease of unknown etiology typically affecting young, obese women, producing a syndrome of increased intracranial pressure without identifiable cause. Despite a large number of hypotheses and publications over the past decade, the etiology is still unknown. Vitamin A metabolism, adipose tissue as an actively secreting endocrine tissue, and cerebral venous abnormalities are areas of active study regarding IIH’s pathophysiology. There continues to be no evidence-based consensus or formal guidelines regarding management and treatment of the disease. Treatment studies show that the diagnostic lumbar puncture is a valuable intervention beyond its diagnostic importance, and that weight management is critical. However, open questions remain regarding the efficacy of acetazolamide, cerebrospinal fluid shunting procedures, and cerebral transverse venous sinus stenting.

Objectives Examination of the ocular fundus is imperative in many acute medical and neurologic conditions, but direct ophthalmoscopy by non-ophthalmologists is underutilized, poorly performed, and difficult without pharmacologic pupillary dilation. The objective was to examine the feasibility of non-mydriatic fundus photography as a clinical alternative to direct ophthalmoscopy by emergency physicians (EPs). Methods Adult patients presenting to the emergency department (ED) with headache, acute focal neurologic deficit, diastolic blood pressure ≥ 120 mmHg, or acute visual change had ocular fundus photographs taken by nurse practitioners using a non-mydriatic fundus camera. Photographs were reviewed by a neuro-ophthalmologist within 24 hours for findings relevant to acute ED patient care. Nurse practitioners and patients rated ease, comfort, and speed of non-mydriatic fundus photography on a 10-point Likert scale (10 best). Timing of visit and photography were recorded by automated electronic systems. Results Three hundred fifty patients were enrolled. There were 1,734 photographs taken during 230 nurse practitioner shifts. Eighty-three percent of the 350 patients had at least one eye with a high quality photograph, while only 3% of patients had no photographs of diagnostic value. Mean ratings were ≥ 8.7 (standard deviation [SD] ≤ 1.9) for all measures. The median photography session lasted 1.9 minutes (interquartile range [IQR] 1.3 to 2.9 minutes), typically accounting for less that 0.5% of the patient’s total ED visit. Conclusions Non-mydriatic fundus photography taken by nurse practitioners is a feasible alternative to direct ophthalmoscopy in the ED. It is performed well by non-physician staff, is well-received by staff and patients, and requires a trivial amount of time to perform.