bluuebunny/crossref_metadata_2025_split huggingface dataset:
Evaluating the Long-Term Hearing Outcomes of Cochlear Implant Users With Single-Sided Deafness
Authors: Távora-Vieira Dayse , Rajan Gunesh P. , Van de Heyning Paul , Mertens Griet 
Published: May 2019

Objectives:
To investigate the long-term outcomes of cochlear implantation in individuals with single-sided deafness (SSD) in terms of speech perception, subjective hearing performance, and sound localization.

Methods:
Thirty-four subjects with SSD were recruited across two large cochlear implant (CI) centers (Antwerp, Belgium and Perth, Australia). The long-term hearing outcomes (between 4 and 10 years of CI use) were evaluated using speech in noise tests, a subjective hearing performance questionnaire (Speech, Spatial and Qualities Questionnaire [SSQ12]), and sound localization tests.

Results:
Statistically significant improvements were observed in speech perception in noise and sound localization results postoperatively with the use of a CI in comparison to preoperative measurements. Subjective hearing abilities also significantly improved after long-term CI use.

Conclusion:
Access to binaural hearing is important for subjects with SSD. CI users with SSD experience long-term benefits in speech understanding, sound localization, and quality of life.
Comprehensive Analysis of Factors Leading to Poor Performance in Prelingual Cochlear Implant Recipients
Authors: Panda Smriti , Sikka Kapil , Singh Vishwajeet , Agarwal Shivani , Kumar Rakesh , Thakar Alok , Sharma Suresh C. 
Published: May 2019

Objective:
To comprehensively analyze the prognostic factors responsible for affecting outcomes following cochlear implantation in prelinguals.

Study Design:
Retrospective cohort study from June 2004 to November 2015.

Setting:
Tertiary care center.

Patients:
Patients who had undergone cochlear implantation during June 2004 to November 2015 for prelingual sensorineural hearing loss with a minimum follow-up of 2 years.

Intervention:
Patients were evaluated for the presence of 20 risk factors possibly influencing postimplantation outcomes using a questionnaire.

Main Outcome Measures:
Assessment for speech and auditory function was done at the last follow-up with speech intelligibility ratings (SIR) and categories of auditory performance (CAP) scores, respectively.

Results:
One hundred fifty-one patients were included in this study. On univariate analysis for CAP, age at implantation, noncompliance to pre and postimplantation auditory and speech habilitation, poor parental motivation, socioeconomic status and literacy were found to be associated with lower scores (p < 0.05). Whereas, for SIR, additionally, attention deficit hyperactivity disorder and inner ear malformation were statistically significant negative predictors on univariate analysis. Finally, factors responsible for low CAP scores on multivariate analysis were poor parental literacy, poor socioeconomic status, irregular pre/postimplantation rehabilitation, and attention deficit hyperactivity disorder. While for SIR, additionally age at implantation was also a significant negative predictor. Increasing IQ and duration of implant use were associated with higher CAP (univariate) and SIR scores (univariate and multivariate) (p < 0.05).

Conclusion:
We were able to demonstrate negative impact of higher age at implantation, minimal cognitive disorder, adverse parental/socioeconomic profile, and poor compliance to pre/postimplantation auditory verbal habilitation on auditory and speech outcomes.
Episodic Vestibular Symptoms in Children With a Congenital Cytomegalovirus Infection: A Case Series
Authors: Dhondt Cleo , Maes Leen , Oostra Ann , Dhooge Ingeborg 
Published: May 2019

Objective:
Congenital cytomegalovirus (cCMV) infection is the most common non-genetic cause of sensorineural hearing loss in children. Although cCMV-induced vestibular loss is demonstrated in several studies, the occurrence of vertigo has been described in only two cases to date. The aim of this paper is to discuss the underlying pathophysiology of recurrent vestibular symptoms in children with cCMV, based on five cases investigated in our center and an extensive research of the literature.

Study Design:
Retrospective case series.

Setting:
Tertiary referral center.

Patients:
This case series describes five pediatric cCMV-patients (three boys, two girls). Four of them were symptomatic at birth, one was asymptomatic. Three patients underwent cochlear implantation. The age of onset of the vestibular symptoms varied from 2;0 to 7;3 years of age.

Intervention:
None.

Main Outcome Measures:
Details regarding the patient history and results of cranial imaging, audiological, vestibular, and neurological assessments were collected retrospectively.

Results:
The selected cases suffered from recurrent vestibular symptoms. All patients had delayed onset, fluctuating, and/or progressive hearing loss. In all cases, the attacks were accompanied with nausea and vomiting and occurred without clear-cut trigger. Migraine and epilepsy often were proposed as first diagnosis, although they could not be confirmed eventually. Four out of five patients were diagnosed with a peripheral vestibular deficit.

Conclusions:
Diagnosis of vestibular symptoms in children with cCMV is complex, given the multiple morbidities than can occur. Peripheral vestibular causes should be considered in the diagnosis, as important vestibular deficits are demonstrated in this population.
Datalogging Statistics and Speech Recognition During the First Year of Use in Adult Cochlear Implant Recipients
Authors: Schvartz-Leyzac Kara C. , Conrad Chelsea A. , Zwolan Teresa A. 
Published: May 2019

Objective:
To examine device datalogging characteristics and postoperative speech recognition performance in adult cochlear implant (CI) recipients.

Study Design:
Retrospective study examining datalogging characteristics throughout the first year of device use and postoperative speech recognition outcomes measured at 1 year.

Patients:
One hundred seventy-seven adults who received a Cochlear CI.

Main Outcome Measures:
Average values for environmental scene classification (hours of device use per day, and percent of hours per day in quiet, noise, and speech) as reported by Cochlear datalogging over the first year of device use. Speech recognition was assessed at 1 year postactivation.

Results:
During the first year of devices use, CI listeners >80 years of age used their device significantly less (average = 10.97 h/d) than the youngest adult listeners (18–30 yrs), who used their device an average of 13.29 hours/d. There was no consistent effect of age on the number of hours a CI user listened to speech in noise each day. Correlational and regression analyses suggest that the number of average hours of device use is the primary factor that accounts for variance observed in postoperative consonant-nucleus-consonant word scores.

Conclusion:
Results suggest that the average number of hours listening to speech in noise is not related to postoperative performance, but the average number of hours of device use per day is correlated with postoperative performance. Further research is needed to determine if these findings are merely correlational or causal in nature.
Bilateral Posterior Canal Benign Paroxysmal Positional Vertigo Tends to Reoccur
Authors: Pollak Lea , Michael Tal 
Published: May 2019

Objective:
We analyzed the clinical characteristics of quite a large cohort of patients with bilateral posterior canal benign paroxysmal positional vertigo (bil-BPPV) with respect to the rate and types of recurrence.

Study Design:
Retrospective case review.

Setting:
Outpatient dizziness clinic.

Patients:
About 2,050 patients diagnosed with BPPV during the years 2003 to 2018 were reviewed.

Results:
Sixty (2.9%) were diagnosed with bil-BPPV. Fifty three (88%) were idiopathic and seven (12%) posttraumatic. Multiple BPPV episodes were encountered in 28 (47%) patients. Patients with bil-BPPV and multiple BPPV episodes were comparable with patients with a single attack of bil-BPPV except for older age and longer follow up. The most frequent type of recurrence following a bil-BPPV episode was posterior canal canalolithiasis (37.5%) and bil-BPPV (31.3%). Combined (posterior and horizontal) BPPV was encountered in 12.5% while horizontal canal involvement was found in only 7% of recurrences.

Conclusions:
The prevalence of bil-BPPV amongst all BPPV patients was lower than previously reported possibly due to stricter selection criteria. Older age and longer follow up of patients with multiple BPPV episodes than those with a single episode of bil-BPPV is in accordance with the theory of degenerative otoconia loosening. In contrast to the general consensus regarding random recurrences of BPPV side and subtypes, bil-BPPV recurred more frequently than expected. This might be the consequence of individual vulnerability to head movements, increased bone metabolism or an underlying vestibular pathology.
Easy Stapes Subluxation in 22q11.2 Deletion Syndrome: A Clinical Capsule and Literature Review
Authors: Kennel Christopher E. , Cousins Joseph P. , Rivera Arnaldo L. 
Published: May 2019

Objectives:
This report describes iatrogenic stapes subluxation in a 22q11 deletion syndrome patient and reviews the human and murine literature for evidence that these patients have stapes malformations. We aim to alert otologic surgeons regarding the possibility of stapes footplate abnormalities in 22q11 deletion patients.

Patient:
An adult woman with known 22q11 deletion syndrome. Additionally, the literature review focused on other patients with 22q11 deletion syndrome as well as mouse models of this disorder.

Interventions:
A combination of diagnostic and therapeutic interventions were conducted consisting of middle ear exploration, removal of ossicular chain adhesions, and ultimately ossicular reconstruction.

Results:
The stapes footplate was poorly attached to the oval window in our patient. During removal of ossicular adhesions, the entire stapes subluxed requiring placement of a stapes prosthesis. The postoperative audiogram was similar to the preoperative audiogram. Literature review identified one other case of stapes subluxation in a patient with 22q11 deletion syndrome, and mouse models suggest that the stapes footplate has an abnormal connection to the oval window in those affected by 22q11 deletion syndrome.

Conclusions:
Patients with 22q11 deletion syndrome have chronic middle ear pathology, and if middle ear exploration is undertaken, the surgeon should be aware that the stapes may have a weak attachment to the oval window. This could put the stapes at risk of injury and contribute to conductive hearing loss.
Angular Electrode Insertion Depth and Speech Perception in Adults With a Cochlear Implant: A Systematic Review
Authors: Heutink Floris , de Rijk Simone R. , Verbist Berit M. , Huinck Wendy J. , Mylanus Emmanuel A. M. 
Published: May 2019

Objective:
By discussing the design, findings, strengths, and weaknesses of available studies investigating the influence of angular insertion depth on speech perception, we intend to summarize the current status of evidence; and using evidence based conclusions, possibly contribute to the determination of the optimal cochlear implant (CI) electrode position.

Data Sources:
Our search strategy yielded 10,877 papers. PubMed, Ovid EMBASE, Web of Science, and the Cochrane Library were searched up to June 1, 2018. Both keywords and free-text terms, related to patient population, predictive factor, and outcome measurements were used. There were no restrictions in languages or year of publication.

Study Selection:
Seven articles were included in this systematic review. Articles eligible for inclusion: (a) investigated cochlear implantation of any CI system in adults with post-lingual onset of deafness and normal cochlear anatomy; (b) investigated the relationship between angular insertion depth and speech perception; (c) measured angular insertion depth on imaging; and (d) measured speech perception at, or beyond 1-year post-activation.

Data Extraction and Synthesis:
In included studies; quality was judged low-to-moderate and risk of bias, evaluated using a Quality-in-Prognostic-Studies-tool (QUIPS), was high. Included studies were too heterogeneous to perform meta-analyses, therefore, effect estimates of the individual studies are presented. Six out of seven included studies found no effect of angular insertion depth on speech perception.

Conclusion:
All included studies are characterized by methodological flaws, and therefore, evidence-based conclusions regarding the influence of angular insertion depth cannot be drawn to date.
Epidemiology and Diagnosis of Gastroparesis in the United States
Authors: Syed Aslam R. , Wolfe Michael M. , Calles-Escandon Jorge 
Published: May 2019

Goals:
We aimed to establish the epidemiological characteristics and documentation of diagnostic workup for gastroparesis (GP).

Background:
No study has used a national database to evaluate the prevalence, demographics, and associated comorbid conditions of GP, and document rates of proper diagnosis.

Materials and Methods:
This was a cross-sectional population-based study using the Explorys Platform to determine the prevalence of GP in a large and diverse population highly representative of the US population and to examine the diagnostic approach of GP. Data collected were individual characteristics from electronic medical records (EMRs) included age, ethnicity/race, sex, diagnostic report for esophagogastroduodenoscopy (EGD) and gastric emptying study (GES).

Results:
A total of 43,827,910 medical records were surveyed (1999 to 2014), of which 69,950 had a diagnosis of GP, yielding an overall prevalence of 0.16%. We identified 249,930 EMRs with type 1 diabetes mellitus (T1DM), and 2,940,280 EMR’s with type 2 diabetes mellitus (T2DM), of which 11,470 (4.59%) and 38,670 (1.31%) EMR’s had concurrent GP, respectively. The remainder 19,810 EMRs with a diagnosis of GP were classified as having idiopathic GP. In all three subgroups, women and Caucasians had the highest prevalence of GP. The diagnosis of GP was confirmed by both GES and EGD in 9,950 of patients (14.22%). For patients with T1DM, T2DM, or idiopathic GP, GP was confirmed by both diagnostic tests in 16.8%, 14.0%, and 13.2%, respectively.

Conclusions:
Our estimated rates of prevalence of GP in T1DM and T2DM indicate that GP is not a common clinical complication in these populations. Majority of EMRs that indicated a diagnosis of GP did not include any documentation of definitive diagnostic testing (EGD and/or GES).
Fibroscan and low-density lipoprotein as determinants of severe liver fibrosis in diabetic patients with nonalcoholic fatty liver disease
Authors: Jaafar Rola F. , Hajj Ali Adel M. , Zaghal Ahmad M. , Kanso Mariam , Habib Salim G. , Halaoui Adham F. , Daniel Fady , Mokaddem Farah , Khalife Mohamad J. , Mukherji Deborah M. , Faraj Walid G. 
Published: May 2019

Background
Fibroscan is an effective and noninvasive tool to quantify fibrosis and steatosis in liver diseases including nonalcoholic fatty liver disease (NAFLD). Type-2-diabetes is a known risk factor for worse prognosis in NAFLD. In this study, we compare liver status in NAFDL diabetic and nondiabetic patients, identify potential risk factors, and determine the usefulness of Fibroscan in this population.

Patients and methods
The charts of all patients with NAFLD who underwent Fibroscan at our institution were reviewed. Fibroscan results, demographics, and clinical data were collected and analyzed using SPSS software.

Results
Of the 248 NAFLD patients, 73 (29.4%) were diabetic and 175 (70.6%) were nondiabetic. As detected by the NAFLD’ liver stiffness measure, 35 (47.94%) diabetic patients had severe liver fibrosis (F4) in contrast to only 46 (26.3%) nondiabetics. Diabetic patients also presented more with hypertension, dyslipidemia, coronary artery disease, and chronic kidney disease. Liver steatosis, liver function tests, and noninvasive scores did not vary significantly between the two groups, except for γ-glutamyltransferase, prothrombin time-international normalized ratio, and BMI-alanine aminotransferase ratio-diabetes score. Diabetic patients had significantly lower high-density lipoproteins and low-density lipoproteins.

Conclusion
Fibroscan results and low-density lipoprotein are potential diagnostic factors of liver fibrosis in diabetic patients with NAFLD. Further studies are necessary to verify liver fibrosis diagnostic tools and prognostic and genetic markers in diabetic patients.
Update on low-density lipoprotein cholesterol quantification
Authors: Chung Soie 
Published: May 2019

Purpose of review
β-Quantification is considered the reference measurement procedure for low-density lipoprotein cholesterol (LDL-C). However, this technique is time-consuming and thus is inappropriate for routine clinical practice. Therefore, the Friedewald equation or homogeneous assays have been widely utilized. As several pitfalls exist with these two methods, a novel method for estimating LDL-C was developed by Martin et al.

Recent findings
Martin's method uses a strata-specific median for the triglycerides/very low-density lipoprotein cholesterol (VLDL-C) ratio on the basis of triglycerides and non-HDL-C concentrations. Recent studies show that Martin's method better correlates with β-quantification or homogeneous assays compared with the Friedewald equation, especially with values of triglycerides at least 150 mg/dl and/or LDL-CD less than 70 mg/dl. Such findings have also been demonstrated in other ethnic groups (Japanese and Korean) and disease populations, including diabetes and cardiovascular disease, in which the triglycerides/VLDL-C ratio can be affected.

Summary
For the current therapeutic goal of LDL-C values below 70 mg/dl in high-risk patients, accurate assessment of LDL-C levels at very low levels is required. Martin's method could overcome pitfalls such as underestimation of the Friedewald equation at this level. Further evaluation of the triglycerides/VLDL-C ratio in participants with diverse ethnic backgrounds or clinical conditions would expand the implementation of this novel method.
Epitope matching in kidney transplantation: recent advances and current limitations
Authors: Larkins Nicholas G. , Wong Germaine , Taverniti Anne , Lim Wai H. 
Published: May 2019

Purpose of review
Evolution of human leukocyte antigen (HLA) molecular typing techniques has progressively enabled more accurate determination of the three-dimensional building blocks that form the antibody accessibility and binding sites of each HLA allele. These immunogenic HLA regions known as epitopes are composed of polymorphic sequences of amino acid residues termed eplets. This review provides a critical appraisal of the current understanding of epitope compatibility in kidney transplantation.

Recent findings
There is a tendency to suggest that epitope matching is likely to be superior to broad antigen HLA matching such that the allocation of donor kidneys to patients with a more favorable epitope compatibility profile may lead to better allograft outcomes. A growing body of work has highlighted the association between a greater number of eplet mismatches and adverse allograft outcomes, and approaches using eplet matching have been successfully implemented in organ allocation programs. However, our understanding of epitope compatibility remains in its infancy, requiring further and more in-depth evaluation. Critically, it remains unclear how best to translate findings derived at the population level to the care of individual patients. Questions that need to be answered include a lack of consensus in the definition and interpretation of epitope compatibility, are class I and II compatibility of similar clinical importance, how best to define predetermined mismatch thresholds for utilization in organ allocation, and whether other properties such as differences in electrostatic potential between donor and recipient HLA alleles are also important in determining immunological compatibility.

Summary
Epitope matching likely represents a valid progression in understanding donor–recipient HLA compatibility. However, more clinical data and a better understanding about differences in methods to determine epitope compatibility are required before the approach can be widely applied in clinical practice.
A diagnostic ‘C’ saw: the ups and downs of C1q testing
Authors: Gebel Howard M. , Bray Robert A. 
Published: May 2019

Purpose of review
The present review will focus on recently published data of solid organ allograft recipients reporting that patients with de-novo donor-specific HLA antibodies (DSA) that fix complement in vitro have a significantly higher risk for antibody-mediated rejection (AMR) and/or graft loss compared to patients whose de-novo DSA do not fix complement or patients who present with preexisting complement fixing DSA.

Recent findings
HLA DSAs that fix complement in vitro appear to be a key indicator for rejection and failure of kidney, heart, and lung allografts from studies performed around the world. The majority of these studies are population based and retrospective in nature. Although these studies seemingly indicate that in-vitro complement activating DSAs represent a higher clinical risk than noncomplement fixing DSAs, the majority have not accounted for false-negative reactions attributable to the so-called prozone/interference phenomenon. In the limited number of published studies addressing that concern, high mean fluorescence intensity (MFI) value noncomplement fixing DSAs correlate as well as complement fixing DSAs with AMR and graft loss. Combined with the cost of additional testing, these observations bring into question whether there is sufficient clinical applicability to warrant routine testing for complement fixing antibodies.

Summary
Complement fixing DSAs are clearly associated with AMR and/or loss of transplanted allografts. However, under appropriate testing conditions, complement fixing capability typically correlates with MFI values of the DSAs. As such, the routine implementation of in-vitro assays to determine whether DSAs fix complement is of questionable value especially when considering additional issues such as cost of testing, logistics, and whether the test results factor into individualized patient care.
Elevated International Normalized Ratio (INR) is Associated With an Increased Risk of Intraventricular Hemorrhage in Extremely Preterm Infants
Authors: Glover Williams Alessandra , Odd David , Bates Sarah , Russell Geoff , Heep Axel 
Published: May 2019

Introduction:
The international normalized ratio (INR), a standardized method of reporting the prothrombin time, can be a surrogate marker of the vitamin K-dependent coagulation pathways.

Objective:
To evaluate the relationship between INR measurements in the first 48 hours of life and subsequent development of intraventricular hemorrhage (IVH) in extremely preterm infants.

Materials and Methods:
A single-center retrospective, observational cohort study of infants born at <28 weeks gestation. The main outcome measure was defined as the degree of IVH seen on cranial ultrasound examinations at day 7 postnatal age.

Results:
Of 200 infants, 109 (mean gestational age, 25.2 wk [SD, 1.27]) had coagulation results available. Of 109, 26 developed IVH. Elevated INR was associated with increased risk of a severe IVH (odds ratio [OR] 6.50; 95% confidence interval [CI], 1.65-25.62; P=0.008) adjusted for gestation, birth weight, and sex. INR was significantly associated with severe IVH in infants who did not receive blood products (OR, 64.60; 95% CI, 1.35-3081.25; P=0.035), but not in those who did (OR, 2.93; 95% CI, 0.67-12.71; P=0.151) (P
interaction=0.086).

Conclusion:
An elevated INR in the first 48 hours of life may be useful to identify preterm infants at risk of severe IVH and may guide strategies to prevent the development, or limit the extension, of IVH.