OBJECTIVE To objectively evaluate adherence to timing and dosing of insulin by using Bluetooth pen caps and examine factors related to adherence. RESEARCH DESIGN AND METHODS Bluetooth-enabled insulin pen caps were used in younger (ages 18–35 years) and older (ages ≥65 years) adults on two or more insulin injections per day. RESULTS We evaluated 75 participants with diabetes, 42 younger (29 ± 4 years) and 33 older (73 ± 7 years). Nonadherence was found in 24% of bolus (Apidra) doses and 36% of basal (Lantus) doses. We divided participants into tertiles on the basis of overall adherence, with the most adherent tertile having 85% dose adherence compared with 49% in the least adherent tertile (P < 0.001). Participants in the most adherent tertile had better glycemic control than those in the least adherent tertile (7.7 ± 1.1% [61 ± 12 mmol/mol] vs. 8.6 ± 1.5% [70 ± 16.4 mmol/mol], P < 0.03). CONCLUSIONS Nonadherence to insulin dosing and timing can be objectively assessed by Bluetooth pen caps and is associated with poor glycemic control.

Objective: To evaluate whether increased body mass index (BMI), age, or frailty influence vestibular schwannoma (VS) short-term surgical morbidity. Methods:The 2005-2017 National Surgical Quality Improvement Program database was queried for patients with VS undergoing surgical resection. Age was stratified according to age <50, 50-64, and ≥65, while BMI was stratified based on a threshold of 30. Frailty score (0-5) was indicated based on functional status, diabetes, chronic obstructive pulmonary disease, congestive heart failure, and hypertension.Results: A total of 1405 patients were included consisting of 56.7% females with a mean age of 50.7±13.8 years and mean BMI of 29.4±6.6. Patients <50 (n=604), 50-64 (n=578), and ≥65 (n=223), had different duration of surgery (428±173 vs. 392±149 vs. 387±154 minutes; p<0.001) and 30-day mortality rates (0.7% vs. 0% vs. 1.8%; p=0.01). However, post-operative length of stay (LOS) (p=0.16), readmission (p=0.08), reoperation (p=0.54), and complication rates were similar. Post-operative myocardial infarction (p=0.03) and wound infection (p=0.02) were more commonly observed in the obese cohort (BMI≥30) but readmission (p=0.18), reoperation (p=0.44), and complication rates were similar to those with BMI<30. Severely obese patients (BMI≥35) also had higher rates of deep vein thrombosis (p=0.004). Frailty score 0 (n=921), 1 (n=375), and 2-4

Objectives: To evaluate the presence of migraine features in patients with persistent postural-perceptual dizziness (PPPD). Methods: In a retrospective survey study, consecutive patients presenting to a tertiary care neurotology clinic during an 18-month period were given questionnaires about headache and dizziness symptoms. The survey responses plus history and examination of the patient were used to diagnose patients with PPPD. The prevalence of migraine headache, vestibular migraine (VM), and migraine characteristics was evaluated. Results: In total, 36 subjects with PPPD were included in the study. The mean age of the subjects was 56 ± 16 years with a female (72%) predominance. A total of 19 (53%) patients met the International Classification of Headache Disorders criteria for migraine headache, and 6 of those (17%) met the criteria for definite VM. Of the patients who did not meet full migraine headache criteria, 6 (17%) patients met 4 of 5 criteria, and 5 (14%) patients met 3 of 5 criteria. There was no significant difference between PPPD patients who fulfilled full migraine headache criteria and those who did not in sensitivity to light, sound, smells, weather changes, feelings of mental fog/confusion, and sinus pain/facial pressure. Conclusions: This study demonstrates that a majority of patients with PPPD fulfill the criteria for migraine headache. A large proportion of PPPD patients who do not meet the full criteria for migraine headache still meet a majority of the migraine headache criteria. This suggests an association between the 2 conditions. PPPD may be a part of the spectrum of otologic migraine, where migraine manifests as otologic symptoms.

Objectives: To identify migraine features present in a cohort of patients with recurrent benign paroxysmal positional vertigo (BPPV). Methods: Patients presenting with recurrent BPPV were surveyed. Recurrent BPPV was defined as three episodes or greater in 6 months before presentation, with resolution of symptoms after Epley maneuver. Current or past migraine headache (MH) diagnosis was made according to the International Headache Society guidelines. Results: Fifty-eight patients with recurrent BPPV with a mean age of 53.8 ± 17.4 years were included. Half (29 patients) fulfilled criteria for MH and half (29 patients) did not meet the criteria for MH (non-MH). No statistically significant difference was found in a majority of migraine-related symptoms between the MH and non-MH cohorts with recurrent BPPV. History of migraine medication usage (p = 0.008), presence of a weekly headache (p = 0.01), and duration of dizziness after positional vertigo (p = 0.01) were the only variables that were different on multivariate analysis between the MH and non-MH cohorts. Conclusions: Half of recurrent BPPV patients suffer from migraine headaches. The other half presented with migraine-related symptoms, but do not meet criteria for MH. The high comorbidity of MH in our recurrent BPPV cohort as well as the absence of a statistically significant difference in a majority of migraine-related features among patients who did and did not fulfill criteria for MH may suggest that recurrent BPPV has a relationship with migraine. Recurrent BPPV may potentially be a manifestation of migraine in the inner ear, which we term otologic migraine including cochlear, vestibular, or cochleovestibular symptoms.

There are an estimated 6000-8000 rare Mendelian diseases that collectively affect 30 million individuals in the United States. The low incidence and prevalence of these diseases present significant challenges to improving diagnostics and treatments. Next-generation sequencing (NGS) technologies have revolutionized research of rare diseases. This article will first comment on the effectiveness of NGS through the lens of long-tailed economics. We then provide an overview of recent developments and challenges of NGS-based research on rare diseases. As the quality of NGS studies improve and the cost of sequencing decreases, NGS will continue to make a significant impact on the study of rare diseases moving forward.