bluuebunny/crossref_metadata_2025 huggingface dataset:
Characterization of Ivoclar Vivadent Dental Restoration Material for 137Cs Retrospective Radiation Dosimetry
Authors: Ratliff Steven T. , Barry Kawsu
Published: June 2018

Retrospective dosimetry is the method of using materials on or near a person who is exposed to ionizing radiation to determine the amount of radiation received by the person. A possible candidate material for retrospective dosimetry is Ivoclar Vivadent IPS e.max® CAD ceramic dental restoration material, which exhibits radiation-induced thermoluminescence when exposed to gamma- and x-ray radiation from a 137Cs source. The purpose of this paper is to characterize the material and study the behavior of the thermoluminescence signal with radiation dose and with delay time between radiation exposure and thermoluminescence measurement. The first glow peak is well-modeled by a first-order glow curve deconvolution formula. The height of the first glow peak is approximately linear with dose. The fading of the signal with time is approximately described by a power law curve with cutoff. The material appears to be suitable for retrospective radiation dosimetry.
Review of Cases of Occult Radiation Exposure and an Analysis of Time to Diagnosis
Authors: Kunkler Jillian , Adams Grace G. , Manger Morgan , Casagrande Rocco
Published: June 2018

Radiation sickness is challenging to diagnose, and the diagnosis is often based on nonclinical data. In accidents where patients are not aware of their exposure to radiation (so-called “occult exposures”), a correct diagnosis can take weeks or months. The purpose of this study is to review the time to diagnosis of radiation sickness for occult exposures where neither the physician nor the patients are aware of their exposure. We reviewed eight case reports involving occult exposures and examined the factors that contributed to a diagnosis. We found that an average of 23 d after exposure were required for diagnosis, regardless of route of intake. In only one case was radiation injury diagnosed within the first week. Although all but one of these cases occurred in the developing world, the factors confounding diagnosis are common to the U.S. medical system. Therefore, an urgent need exists for properly training physicians on radiation sickness so they can more quickly diagnose and treat radiation exposures when the patients are unaware of the exposure event.
Case Study of the Minimum Provable Risk Considering the Variation in Background Risk: Effect of Residual Risk on Epidemiological Studies and a Comparative Assessment of Fatal Disease Risk Due to Radiation Exposure
Authors: Sasaki Michiya , Ogino Haruyuki , Hattori Takatoshi
Published: June 2018

In order to prove a small increment in a risk of concern in an epidemiological study, a large sample of a population is generally required. Since the background risk of an end point of interest, such as cancer mortality, is affected by various factors, such as lifestyle (diet, smoking, etc.), adjustment for such factors is necessary. However, it is impossible to inclusively and completely adjust for such factors; therefore, uncertainty in the background risk remains for control and exposed populations, indicating that there is a minimum limit to the lower bound for the provable risk regardless of the sample size. In this case study, we developed and discussed the minimum provable risk considering the uncertainty in background risk for hypothetical populations by referring to recent Japanese statistical information to grasp the extent of the minimum provable risk. Risk of fatal diseases due to radiation exposure, which has recently been the focus of radiological protection, was also examined by comparative assessment of the minimum provable risk for cancer and circulatory diseases. It was estimated that the minimum provable risk for circulatory disease mortality was much greater than that for cancer mortality, approximately five to seven times larger; circulatory disease mortality is more difficult to prove as a radiation risk than cancer mortality under the conditions used in this case study.
Characteristics of Children With Invasive Pneumococcal Disease After the Introduction of the 13-valent Pneumococcal Conjugate Vaccine in England and Wales, 2010–2016
Authors: Makwana Ashley , Sheppard Carmen , Borrow Ray , Fry Norman , Andrews Nick J. , Ladhani Shamez N.
Published: June 2018

Background:

In England and Wales, replacement of childhood 7-valent pneumococcal conjugate vaccine (PCV7) with a 13-valent vaccine (PCV13) in 2010 was associated with a significant reduction in PCV13-serotype invasive pneumococcal disease (IPD), with a small increase in IPD due to non-vaccine serotypes. Here, we describe the clinical presentation, comorbidity prevalence, serotype distribution and outcomes of childhood IPD during the first 6 years after PCV13 introduction.

Methods:

Public Health England conducts enhanced IPD surveillance in England and Wales, with detailed information requested from general practitioners for all cases in children <5 years of age. Invasive isolates are routinely serotyped at the Public Health England reference laboratory.

Results:

From April 2010 to March 2016, 1280 IPD episodes were confirmed in 1255 children 3–59 months of age; 84.3% (1059/1255) isolates were serotyped. Clinical presentation with meningitis was most prevalent in 3- to 11-month olds (45.8%, 209/456) and lower respiratory tract infection in 24- to 59-month olds (46.7%, 133/285). Overall, 259 (20.6%) children had 292 comorbidities, particularly immunosuppression (31.6%, 92/292). Twenty-one children (1.8%) had recurrent IPD. The case fatality rate was 5.1% (64/1255; 95% confidence interval [CI]: 3.9%–6.5%) and independently associated with meningitis (aOR 3.53; 95% CI: 1.62–7.70) and presence of comorbidity (aOR, 2.41; 95% CI: 1.25–4.64). In 2015/2016, PCV13 serotypes were responsible for 10.8% (25/232) of serotyped cases; the most prevalent non-PCV13 serotypes were 12F (18%), 10A (12%), 23B (10%), 33F (10%), 15B/C (10%) and 8 (8%).

Conclusions:

Most childhood IPD cases are now due to non-PCV13 serotypes. A higher proportion of children with IPD have underlying comorbidity, but, reassuringly, the risk of recurrent IPD or death remains low.
Nurse Practitioner Triage of the Neurosurgical Patient: A Patient-Centered Care Delivery Model
Authors: Miniard Jody L. , Ballman Kathleen
Published: June 2018

Background: Nurse practitioners (NPs) are helping to bridge the gap not only in primary care but also in specialty practice. In an outpatient, neurosurgical spinal clinic in the midwest, patient volume exceeded provider availability, and to rectify this problem, a proposal for an NP triage system and an NP-led spinal clinic was presented. Purpose: The aim of this project was to use NPs to facilitate patient triage in an outpatient neurosurgical practice to expedite patients’ access to care and ultimately improve patient satisfaction, outcomes, and healthcare expenditure. Methods: A prospective comparison study was conducted in which NP patient triaging involved surgeons and NPs simultaneously reviewing patient cases over a 1-month period, with a total number of reviews equaling 100 reviews per NP, for a grand total of 200 test reviews. Data were analyzed for a comparison of NP and surgeon input, in reference to patient diagnosis and appropriate NP referral for surgical consultation. Institutional review board approval was sought and granted. Results: Nurse practitioner and surgeon diagnoses were congruent 100% of the time. Patients triaged were correctly referred to the clinic for surgical consultation, with a sensitivity of 95.7% (95% confidence interval, 90.8%–98.4%) and a specificity of 73.8% (95% confidence interval, 60.9%–84.2%). Discussion: Utilization of NPs to facilitate triage and treatment of the outpatient neurosurgical spine patient is a sustainable patient-centered care delivery model that leads to improvements in access and ensures exceptional quality outcomes.
The Current Limitations and Future Direction of Instrument Design for Totally Endoscopic Ear Surgery: a Needs Analysis Survey
Authors: Swarup Arushri , le Nobel Gavin J. , Andrysek Jan , James Adrian L.
Published: June 2018

Objective:

This study aimed to identify limitations and challenges associated with existing instruments and techniques used in totally endoscopic ear surgery (TEES).

Background:

Otologic instruments, traditionally developed for two-handed surgery with operating microscopes, are not necessarily optimized for the TEES environment. Better understanding of technical challenges and the limitations of current instrumentation may allow advances in instrument design for TEES surgery.

Methods:

This cross-sectional study employed a mixed-methods nine-question survey that was distributed internationally to surgeons with an interest in TEES. Respondents were asked to classify their TEES experience and instrumentation used, rate their need for better instrumentation to address six TEES-related challenges using visual analog scales, and comment on how to modify or develop new instrumentation.

Results:

With 51 respondents, we quantified a need for better instruments to address the following 6 potential TEES challenges ordered from greatest to least need: 1) reaching structures visualized by the endoscope, 2) dissection and removal of cholesteatoma, 3) cutting and/or removing bone, 4) bleeding control, 5) keeping the endoscope lens clean, 6) moving and positioning a graft into the intended place. The majority of surgeons perceive a need for improved instrumentation to address each challenge. Challenges 1) and 2) were associated with significantly greater need than the others (p < 0.05, Wilcoxon method for nonparametric pairwise comparisons).

Conclusion:

In addition to highlighting and quantifying some of the common TEES challenges, these findings provide valuable insight into the design requirements for developing improved surgical instrumentation and techniques.
Prevalence of Obstructive Sleep Apnea (OSA) in Spontaneous Cerebrospinal Fluid (CSF) Leaks: A Prospective Cohort Study
Authors: Rabbani Cyrus C. , Saltagi Mohamad Z. , Manchanda Shalini K. , Yates Charles W. , Nelson Rick F.
Published: June 2018

Objective:

To determine the prevalence of obstructive sleep apnea (OSA) in a prospective cohort of patients with spontaneous CSF (sCSF) leaks of the temporal bone.

Study Design:

Prospective cohort study.

Setting:

Tertiary referral center.

Patients:

Consecutive sCSF leak patients (21) over a 3-year period. Four patients presented with a history of OSA and 17 patients were prospectively offered polysomnogram (PSG) testing during the initial clinic encounter.

Intervention:

Level I PSG. Main Outcome Measures: Patient characteristics (age, sex, body mass index), apnea hypopnea index (AHI), presence of snoring, and presence of hypoxia (oxygen saturation <88% for >5 min). OSA was defined as mild (AHI ≥5 and <15/h), moderate (AHI ≥15 and <30/h), and severe (AHI ≥30/h).

Results:

The prevalence of OSA in sCSF leak patients is 83.3%. PSG studies were performed on 18 of the 21 patients. There were 15 women and 6 men with an average age (standard deviation) of 56.3 (11.2) years and an average body mass index of 35.3 (7.7) kg/m2. Objectively, the AHI ranged from mild to severe (range = 5.7–92, median = 19.8). Snoring was present in 61% of patients and hypoxia was present in 39% of patients. sCSF leak patients with OSA were significantly older than sCSF leak patients without OSA (56.7 [8.3] versus 42.7 [14.5] yr, p = 0.03).

Conclusions:

OSA is highly prevalent among patients with sCSF leaks. All patients with sCSF leaks should undergo formal PSG testing. Future studies are needed to determine the role of OSA in the development of sCSF leaks.
Lateral Semicircular Canal Pressures During Cochlear Implant Electrode Insertion: a Possible Mechanism for Postoperative Vestibular Loss
Authors: Banakis Hartl Renee M. , Greene Nathaniel T. , Jenkins Herman A. , Cass Stephen P. , Tollin Daniel J.
Published: June 2018

Hypothesis:

Insertion of cochlear implant electrodes generates transient pressure spikes within the vestibular labyrinth equivalent to high-intensity acoustic stimuli.

Background:

Though cochlear implant (CI) surgery is regarded as having low risk of impacting the vestibular system, several studies have documented changes in vestibular function after implantation. The mechanism of these changes is not understood. We have previously established that large, potentially damaging pressure transients can be generated in the cochlea during electrode insertion, but whether pressure transients occur within the vestibular labyrinth has yet to be determined. Here, we quantify the exposure of the vestibular system to potentially damaging pressure transients during CI surgery.

Methods:

Five human cadaveric heads were prepared with an extended facial recess and implanted sequentially with eight different CI electrode styles via a round window approach. Fiber-optic sensors measured intralabyrinthine pressures in scala vestibuli, scala tympani, and the lateral semicircular canal during insertions.

Results:

Electrode insertion produced a range of high-intensity pressure spikes simultaneously in the cochlea and lateral semicircular canal with all electrodes tested. Pressure transients recorded were found to be significantly higher in the vestibular labyrinth than the cochlea and occurred at peak levels known to cause acoustic trauma.

Conclusion:

Insertion of CI electrodes can produce transients in intralabyrinthine fluid pressure levels equivalent to high-intensity, impulsive acoustic stimuli. Results from this investigation affirm the importance of atraumatic surgical techniques and suggest that in addition to the cochlea, the vestibular system is potentially exposed to damaging fluid pressure waves during cochlear implantation.
Does a “Fundal Fluid Cap” Predict Successful Hearing Preservation in Vestibular Schwannoma Resections Via the Middle Cranial Fossa Approach?
Authors: Sun Daniel Q. , Kung Raymond W. , Hansen Marlan R. , Gantz Bruce J.
Published: June 2018

Objective:

To determine the association between radiographic cerebrospinal fluid (CSF) cap in the lateral internal auditory canal (IAC) and likelihood of successful hearing preservation in middle cranial fossa (MCF) vestibular schwannoma (VS) surgery.

Study Design:

Retrospective chart review.

Setting:

Academic tertiary referral center.

Patients:

One hundred thirty-eight consecutive patients (mean age/standard deviation, SD, 50/11 yr) who underwent MCF VS resection.

Interventions:

MCF VS excision.

Main Outcome Measures:

Size of fundal fluid cap on preoperative magnetic resonance imaging (MRI), pre- and postoperative pure-tone average (PTA), and word recognition score (WRS).

Results:

Mean tumor and fundal fluid sizes were 9.7 mm (SD, 3.9 mm) and 2.8 mm (SD, 1.7 mm), respectively. On bivariate analysis, fundal fluid size was associated with larger tumor size (p = 0.005) but not changes in postoperative PTA (p = 0.45) or WRS (p = 0.17). When fundal fluid size was stratified as none (<1 mm), small (≥1 mm and <4 mm), and large (≥4 mm), no significant differences were seen in rates of hearing preservation. Using multivariate linear regression models adjusting for patient age, sex, tumor nerve of origin, neurofibromatosis type II status, and preoperative PTA and WRS, superior vestibular nerve tumor origin but not increasing fundal fluid size was associated with preserved postoperative PTA or WRS.

Conclusions:

Presence or size of CSF fluid cap may not be a reliable prognostic indicator for hearing preservation in MCF VS resection, with important implications for patient counseling.
Quality of Life After Translabyrinthine Vestibular Schwannoma Resection—Reliability of the German PANQOL Questionnaire
Authors: Glaas Marcel Fabian , Schäfer Ralf , Jansen Philipp , Franz Matthias , Stenin Igor , Klenzner Thomas , Schipper Jörg , Eysel-Gosepath Katrin , Kristin Julia
Published: June 2018

Objective:

To quantify the postoperative quality of life (QOL) of patients after translabyrinthine surgery for vestibular schwannoma (VS) using the German version of the Penn acoustic neuroma quality-of-life questionnaire (PANQOL) in a university hospital.

Methods:

The PANQOL questionnaire was administered to 72 patients who were treated in our department with translabyrinthine surgery for VS between January 2007 and January 2017. Descriptive evaluations of results were performed in addition to analyses of the reliability and convergent validity of the results and a subgroup analysis.

Results:

For the first time, QOL was measured in German-speaking VS patients after translabyrinthine surgery. Cronbach's alpha for internal consistency and Guttman's split half, used as measures of reliability, showed values between 0.39 and 0.92 (raw alpha) and 0.58 and 0.98 (Lambda 4), respectively.

Descriptive Statistics:

The mean total PANQOL score of patients after translabyrinthine VS resection was 61.96. The domain “hearing” had the lowest score (50.87), while “facial dysfunction” had the highest score (74.88). Subgroup analysis showed that neither preoperative tumor size nor elapsed time postoperatively significantly influenced QOL outcomes.

Conclusions:

Depending on the different domains the German PANQOL questionnaire showed poor to good internal consistency, reliability, and convergent validity. Moreover, some VS patients suffer from reduced QOL for a long time after the translabyrinthine procedure, thus psycho-oncological care should be recommended in these cases.
The Sound of a Cochlear Implant Investigated in Patients With Single-Sided Deafness and a Cochlear Implant
Authors: Peters Jeroen P. M. , Wendrich Anne W. , van Eijl Ruben H. M. , Rhebergen Koenraad S. , Versnel Huib , Grolman Wilko
Published: June 2018

Hypothesis:

A cochlear implant (CI) restores hearing in patients with profound sensorineural hearing loss by electrical stimulation of the auditory nerve. It is unknown how this electrical stimulation sounds.

Background:

Patients with single-sided deafness (SSD) and a CI form a unique population, since they can compare the sound of their CI with simulations of the CI sound played to their nonimplanted ear.

Methods:

We tested six stimuli (speech and music) in 10 SSD patients implanted with a CI (Cochlear Ltd). Patients listened to the original stimulus with their CI ear while their nonimplanted ear was masked. Subsequently, patients listened to two CI simulations, created with a vocoder, with their nonimplanted ear alone. They selected the CI simulation with greatest similarity to the sound as perceived by their CI ear and they graded similarity on a 1 to 10 scale. We tested three vocoders: two known from the literature, and one supplied by Cochlear Ltd. Two carriers (noise, sine) were tested for each vocoder.

Results:

Carrier noise and the vocoders from the literature were most often selected as best match to the sound as perceived by the CI ear. However, variability in selections was substantial both between patients and within patients between sound samples. The average grade for similarity was 6.8 for speech stimuli and 6.3 for music stimuli.

Conclusion:

We obtained a fairly good impression of what a CI can sound like for SSD patients. This may help to better inform and educate patients and family members about the sound of a CI.
First Results With a New, Pressure-free, Adhesive Bone Conduction Hearing Aid
Authors: Dahm Valerie , Baumgartner Wolf-Dieter , Liepins Rudolfs , Arnoldner Christoph , Riss Dominik
Published: June 2018

Objectives:

The aim of this study was to evaluate the hearing benefit, advantages, and disadvantages in a series of patients using a new, nonimplantable, pressure-free, adhesive bone conduction hearing aid.

Methods:

Twelve patients were included in the study at the ear, nose, and throat department of the Medical University of Vienna. All patients suffered from conductive hearing loss for at least 3 months. A sound field audiometry, Freiburg monosyllables word test and Oldenburg sentence test were carried out. Additionally, sound quality (SSQ12) and quality of life (AQoL-8D) were assessed using questionnaires.

Results:

Analysis revealed an average aided threshold of 30.8 dB HL (±7.1 SD) and an unaided threshold of 45.1 dB HL (±7.0 SD), resulting in a statistically significant (p < 0.001) average functional gain. Additionally, participants experienced about 30% gain in word recognition scores at 65 dB sound pressure level, speech reception threshold in quiet was 56.8 dB (±6.1) and improved to 44.5 dB (±6.4) in the aided condition. Both, the SSQ12 and the AQoL-8D showed a statistically significant improvement when comparing the scores at the beginning of the study to the answers after 2 weeks of device usage (SSQ12 (p < 0.002) and AQoL-8D (p = 0.002)). Neither skin irritations nor pain were reported during the study period.

Conclusion:

In conclusion, this new, adhesive bone conduction hearing aid has a high patient satisfaction rate while causing no skin irritation or pain.
Prognostic Factors of Pediatric Revision Ossicular Chain Reconstruction
Authors: Kaffenberger Thomas M. , Govil Nandini , Shaffer Amber D. , Chi David H.
Published: June 2018

Objective:

Ossicular chain reconstruction (OCR) is a surgical method used to repair conductive hearing loss. In children, up to 40% of OCRs will require revisions. In this study, our aims were to validate improvement in hearing outcomes following revision OCR and identify prognostic factors for successful revision OCRs.

Design:

Retrospective review at the Children's Hospital of Pittsburgh from 2003 to 2014.

Setting:

Tertiary referral center.

Patients:

Pediatric patients undergoing multiple OCRs who had demographic, operative, and pre- and postoperative audiogram data. Patients included in our study had ≥ 2 OCR procedures to the same ear. Forty-three of 123 patients met inclusion criteria.

Main Outcome Measures:

Pre- and postoperative audiograms were used to compare the changes in pure-tone averages (PTA), speech recognition thresholds, and air-bone gaps following OCR surgeries.

Results:

Revision OCRs significantly improved PTA, speech recognition thresholds, and air-bone gaps (p values= 0.003, 0.004, 0.005, respectively) and seven patients (14%) had return of normal hearing (PTA < 25 dB). Cholesteatoma at the time of first revision was associated with additional OCR revisions (hazard ratio [HR]: 10.3; p=0.013), while patients who had canal wall down or no mastoidectomy during first OCR revision were less likely to require additional revision compared with patients who had canal wall up mastoidectomy (HR: 0.170, p = 0.041 versus canal wall down; HR: 0.242, p = 0.041 versus no mastoidectomy).

Conclusions:

Revision OCR(s) are an acceptable method to improve residual conductive hearing loss following primary OCR surgery in pediatric patients. Patients with cholesteatoma and canal wall up at the time of revision OCR have increased chances of requiring further revision.
Vestibular Function and Hippocampal Volume in the Baltimore Longitudinal Study of Aging (BLSA)
Authors: Kamil Rebecca J. , Jacob Athira , Ratnanather John Tilak , Resnick Susan M. , Agrawal Yuri
Published: June 2018

Objective:

This study evaluated whether reduced vestibular function in aging adults is associated with lower hippocampal volume.

Study Design:

Cross-sectional study.

Setting:

Baltimore Longitudinal Study of Aging, a long-running longitudinal cohort study of healthy aging.

Patients:

Eligible participants were aged ≥ 60 years and had both vestibular physiological testing and brain magnetic resonance imaging at the same visit.

Intervention:

Vestibular function testing consisted of the cervical vestibular-evoked myogenic potential (cVEMP) to assess saccular function, ocular VEMP to assess utricular function, and video head-impulse testing to assess the horizontal semicircular canal vestibulo-ocular reflex.

Main Outcome Measure:

Hippocampal volume calculated using diffeomorphometry.

Results:

The study sample included 103 participants (range of 35–90 participants in subanalyses) with mean (±SD) age 77.2 years (±8.71). Multivariate linear models including age, intracranial volume, sex, and race showed that 1 μV amplitude increase of cVEMP was associated with an increase of 319.1 mm3(p = 0.003) in mean hippocampal volume. We did not observe a significant relationship between ocular VEMP amplitude or vestibulo-ocular reflex gain and mean hippocampal volume.

Conclusions:

Lower cVEMP amplitude (i.e., reduced saccular function) was significantly associated with lower mean hippocampal volume. This is in line with previous work demonstrating a link between saccular function and spatial cognition. Hippocampal atrophy may be a mechanism by which vestibular loss contributes to impaired spatial cognition in older adults. Future work using longitudinal data will be needed to evaluate the causal nature of the association between vestibular loss and hippocampal atrophy.
Evaluation of an Infant Temporal-Bone Model as Training Tool
Authors: Probst Rudolf , Stump Reto , Mokosch Markus , Röösli Christof
Published: June 2018

Objective:

Evaluation of the face validity of a new artificial model of an infant temporal bone (TB) suitable for surgical training, including cochlear implantation.

Subject:

Micro-computer-tomography images were obtained from a TB specimen of a 1-year-old normal infant available in an anatomical collection. The TB model was designed and constructed using these images and techniques known from similar models of adult TB.

Intervention:

Fifteen otology departments in Austria, Germany, and Switzerland rated the infant TB model and compared it with the established adult TB model manufactured commercially by the same company.

Main Outcome Measure:

The otologists responded to a semi-quantitative questionnaire with a rating scale ranging from 1 (strongly disagree) to 5 (strongly agree). Macroscopic and microscopic anatomic details, drilling experience, and surgical landmarks were rated. The surgical procedures included mastoidectomy, posterior tympanotomy, cochleostomy, and insertion of a cochlear electrode.

Results:

Overall ratings were similar (3.9) for both the infant and the adult TB models, with ranges of 3.47 to 4.47 (infant model) and 3.5 to 4.33 (adult model). Ratings of specific anatomical details differed as a function of type of model, but without preference of one model over the other.

Conclusions:

Infant TB models can be used similarly as adult TB models for surgical training, including cochlear implantation. They may deserve a more important role in surgical training because cadaveric human temporal bones of infants are not available.
Vestibular Dysfunction in Patients With Superficial Siderosis of the Central Nervous System
Authors: Takeda Takamori , Kawashima Yoshiyuki , Hirai Chiaki , Makabe Ayane , Ito Taku , Fujikawa Taro , Yamamoto Katsura , Maruyama Ayako , Tsutsumi Takeshi
Published: June 2018

Objective:

To describe the vestibular function in patients with superficial siderosis of the central nervous system (SSCN).

Study Design:

Retrospective analysis.

Setting:

Tertiary referral center.

Patients:

Ten consecutive patients with SSCN. This study is the largest case series of SSCN in which detailed neuro-otological findings, including electronystagmography recording, video head impulse test (vHIT), and posturography, were described.

Interventions:

Audiological and neuro-otological examinations, including pure-tone audiometry, distortion product otoacoustic emissions, speech audiometry, auditory brainstem responses, electronystagmography recording, vHIT, and posturography.

Main Outcome Measures:

Pure-tone average, DP level, maximum speech discrimination score, interpeak latency between auditory brainstem responses waves I and V, eye tracking test, examination of optokinetic nystagmus, caloric response, visual suppression, vestibulo-ocular reflex gains, total center of pressure path length, and Romberg's ratio.

Results:

Audiological examinations suggested that the sensorineural hearing loss was of a cochlear etiology in 3 ears, a retrocochlear etiology in 11 ears, and a combined cochlear and retrocochlear etiology in 6 ears. Neuro-otological examinations revealed that eight out of nine patients had cerebellar disorders, while all patients also had peripheral vestibular dysfunction.

Conclusion:

In addition to cerebellar disorders, SSCN patients suffer from severe peripheral vestibular dysfunction, which can exacerbate the patient's imbalance. When otolaryngologists encounter patients with distinctly progressive sensorineural hearing loss and imbalance, they should include SSCN in the differential diagnosis and perform neuro-otological examinations, including an electronystagmography recording and vHIT and brain magnetic resonance imaging.
The Bony Obliteration Tympanoplasty in Pediatric Cholesteatoma: Long-term Hearing Results
Authors: van Dinther Joost Josephus Stephanus , Coopman Renaat , Vercruysse Jean-Philippe , Somers Thomas , Zarowski Andrzej , Vanspauwen Robby , Maryn Youri , Cremers Cornelis Wilhelmus Radboud Jozef , Offeciers Frans Erwin
Published: June 2018

Objective:

To present the hearing results of a 5-year longitudinal study in a pediatric population undergoing surgery for extensive cholesteatoma using a canal wall up (CWU) approach with bony obliteration of the mastoid and epitympanic space, with a standard residual rate of 5.8%, a recurrence rate of 2.9%, and all ears waterproof, free of otorrhea and all external ear canals patent and self-cleaning.

Study Design:

Retrospective consecutive study.

Patients:

Thirty-three children (≤18 yr) undergoing surgery for cholesteatoma (34 ears) between 1997 and 2009.

Interventions:

Therapeutic.

Setting:

Tertiary referral center.

Main Outcome Measures:

Hearing and gain in hearing at 1- and 5-year postsurgery: (1) pure-tone average (PTA), (2) pure-tone average high frequency, (3) pure-tone average including 3 kHz, (4) bone conduction at corresponding frequency averages, (5) gain at corresponding air conduction and bone conduction (gain at corresponding air conduction) frequency averages. (6) The Amsterdam Hearing Evaluation Plots were used to study the individual cases.

Results:

The Amsterdam Hearing Evaluation Plots at 5-year showed in 58.8% of patients a positive gain air conduction. In 23.5% a successful functional result was achieved, defined as an air-bone gap closure to 20 dBHL or less. In 6 patients (17.6%) a limited bone conduction deterioration was shown all limited to maximum 20 dBHL.

Conclusion:

The CWU bony obliteration tympanoplasty in a consecutive series of pediatric extensive cholesteatoma shows a similar to slightly improved hearing outcome as compared with CWU surgery without BOT. Although the series shows a clearly reduced reoperation rate and a significantly improved safety and hygienic outcome as compared with CWU without BOT, better hearing outcomes remain desirable in this group of children.
Role of Targeted Next Generation Sequencing in the Etiological Work-Up of Congenitally Deaf Children
Authors: Boudewyns An , van den Ende Jenneke , Sommen Manou , Wuyts Wim , Peeters Nils , Van de Heyning Paul , Van Camp Guy
Published: June 2018

Objectives:

The purpose of this study is to report the results of a comprehensive etiological work-up for congenitally deaf children including targeted next generation sequencing.

Study Design:

Retrospective case review.

Setting:

Tertiary referral center.

Patients:

Fifty children with congenital, bilateral profound hearing loss (HL) (>90 dBnHL).

Interventions:

Etiological work-up included testing for pathogenic variants in GJB2, a phenotype driven genetic analysis, screening for congenital infections and imaging. When no etiology could be found, comprehensive genetic testing was performed using a HL gene panel including 45 syndromic and 96 non-syndromic HL genes.

Results:

Eleven patients carried bi-allelic pathogenic variants in GJB2. Phenotype driven genetic analysis identified two homozygous KCNQ1 patients (Jervell and Lange Nielsen syndrome) and one heterozygous CHD7 patient (CHARGE syndrome). One patient was diagnosed with achondroplasia and one had a clinical diagnosis of Waardenburg syndrome. A deafness gene panel evaluated 16 patients. In 12 out of 16, we identified a pathogenic (n = 12) or likely pathogenic (n = 2) variant and one variant of unknown significance (VUS). A definite diagnosis of non-syndromic or syndromic HL was made in 18 and seven patients, respectively. Non-genetic causes were congenital cytomegalovirus infection (n = 11), anatomic abnormalities (n = 2), neurological/metabolic/polymalformative conditions (n = 3), meningitis (n = 1), and auditory neuropathy (n = 1).

Conclusions:

A definite genetic cause was found in 25 (50%) of congenital, bilaterally deaf children. Our data show that implementation of a gene panel improves the diagnostic yield for etiological work-up of congenital profound HL to 86%. Identification of the etiology of congenital HL may contribute to predicting outcomes of cochlear implantation.
Audiometric Profiles in Patients With Normal Hearing and Bilateral or Unilateral Tinnitus
Authors: Song Kudamo , Shin Sun Ae , Chang Dong Sik , Lee Ho Yun
Published: June 2018

Hypothesis:

Patients with subjective nonpulsatile tinnitus and a normal conventional audiogram have more objective audiologic evidence of hidden hearing loss and deafferentation-related pathology than patients without tinnitus.

Background:

The aim of this study was to assess the epidemiologic characteristics and audiologic profiles, including auditory brainstem response (ABR), distortion product otoacoustic emission, and threshold-equalizing noise test results, in patients with tinnitus and a normal audiogram.

Methods:

The test results for 20 patients complaining of nonpulsatile chronic tinnitus were compared with those of 91 subjects with normal hearing and no tinnitus.

Results:

Patients with unilateral tinnitus had higher tinnitus handicap inventory scores than those with bilateral tinnitus (p < 0.05). Threshold-equalizing noise tests were normal in all study participants. In patients with unilateral tinnitus, the ABR and distortion product otoacoustic emission test results were similar to those of controls. In contrast, patients with bilateral tinnitus showed a shortening of latency in wave III of the ABR on the right (p = 0.047) and in wave V on the left (p = 0.024). Logistic regression analysis revealed that enhanced wave III/I (p = 0.018) and V/I (p = 0.012) ratios on the left and poorer pure-tone average on the right were significant risk factors for bilateral tinnitus.

Conclusion:

The mechanism involved in the development of tinnitus may depend on its laterality. Bilateral tinnitus may be associated with hyperactivity at the level of the cochlear nucleus whereas a higher-order cortical area may be involved in unilateral tinnitus.
Occupational Noise Exposure and Risk for Noise-Induced Hearing Loss Due to Temporal Bone Drilling
Authors: Vaisbuch Yona , Alyono Jennifer C. , Kandathil Cherian , Wu Stanley H. , Fitzgerald Matthew B. , Jackler Robert K.
Published: June 2018

Background:

Noise-induced hearing loss is one of the most common occupational hazards in the United States. Several studies have described noise-induced hearing loss in patients following mastoidectomy. Although otolaryngologists care for patients with noise-induced hearing loss, few studies in the English literature have examined surgeons’ occupational risk.

Methods:

Noise dosimeters and sound level meters with octave band analyzers were used to assess noise exposure during drilling of temporal bones intraoperatively and in a lab setting. Frequency specific sound intensities were recorded. Sound produced using burrs of varying size and type were compared. Differences while drilling varying anatomic structures were assessed using drills from two manufacturers. Pure tone audiometry was performed on 7 to 10 otolaryngology residents before and after a temporal bone practicum to assess for threshold shifts.

Results:

Noise exposure during otologic drilling can exceed over 100 dB for short periods of time, and is especially loud using large diameter burrs > 4 mm, with cutting as compared with diamond burrs, and while drilling denser bone such as the cortex. Intensity peaks were found at 2.5, 5, and 6.3 kHz. Drilling on the tegmen and sigmoid sinus revealed peaks at 10 and 12.5 kHz. No temporary threshold shifts were found at 3 to 6 kHz, but were found at 8 to 16 kHz, though this did not reach statistical significance.

Conclusion:

This article examines noise exposure and threshold shifts during temporal bone drilling. We were unable to find previous descriptions in the literature of measurements done while multiple people drilling simultaneously, during tranlabyrinthine surgery and a specific frequency characterization of the change in peach that appears while drilling on the tegmen. Hearing protection should be considered, which would still allow the surgeon to appreciate pitch changes associated with drilling on sensitive structures and communication with surgical team members. As professionals who specialize in promoting the restoration and preservation of hearing for others, otologic surgeons should not neglect hearing protection for themselves.
Prevalence of hepatitis B and hepatitis C infection from a population-based study in Southern India
Authors: Shanmugam Rengasamy P. , Balakrishnan Selvakumar , Varadhan Hemalatha , Shanmugam Vivekanandan
Published: June 2018

Objectives

This study aims to estimate the prevalence of hepatitis B (HBV) and C (HCV) in the population through field-screening camps conducted by Chennai Liver Foundation, in the southern state of Tamil Nadu, India. This is the largest population-based study from Tamil Nadu.

Patients and methods

A total of 75 camps were conducted across 14 districts of Tamil Nadu (2014–2017). Screening was done by rapid point-of-care assays (SD-bioline tests) and confirmed by enzyme-linked immunosorbent assay (Monolisa tests). Those tested negative were offered first dose of HBV vaccine. Positive patients with HBV count of more than 2000 IU/ml or HCV-RNA positive on quantitative analysis were treated.

Results

A total of 18 589 people were screened, with HBV infection detected in 303 (prevalence 1.63%) and HCV infection in 56 (prevalence 0.3%), with significant variation among districts. Males contributed to about three-fourths of detected HBV [233/303 (77%)] or HCV [41/56 (73%)] infection. Screening detected a higher overall HBV/HCV infection rate in rural [203 (2.52%) infections in 8047 people] than in urban [156 (1.47%) infections in 10 542 people] areas (P<0.0001). Slum areas had a HBV prevalence of 5%. In a dialysis unit, all patients were found to have either HBV/HCV infection. A total of 162/303 (54%) people with HBV and 27/56 (48%) with HCV infection were treated, and 7704 people received the first dose of HBV vaccine.

Conclusion

The prevalence of HBV was 1.63% and HCV was 0.30% in Tamil Nadu. Three-fourths of HBV/HCV infected people were males. Prevalence of HBV/HCV was higher in rural areas. Slum area and dialysis unit had high HBV and HCV prevalence.
Influence factors and a predictive scoring model for measuring the biochemical response of primary biliary cholangitis to ursodeoxycholic acid treatment
Authors: Chen Jialiang , Xue Dongying , Gao Fangyuan , Tao Le , Li Yuxin , Zhang Qun , Wang Rui , Sun Le , Yang Xue , Liu Yao , Zhu Bingbing , Niu Shuaishuai , Wang Xianbo
Published: June 2018

Aims

The biochemical response after ursodeoxycholic acid (UDCA) treatment contributes toward predicting the prognosis for primary biliary cholangitis (PBC) patients. This study aimed to establish a score model that can be used for predicting the biochemical response.

Patients and methods

A total of 218 patients in the derivation group and 66 patients in the verification group were enrolled. Response endpoints were based on the Barcelona criteria combined with the Paris I criteria. We determined independent factors of the biochemical response by univariate and multivariate analyses. Then, we established a predictive score model on the basis of regression coefficients after adjusted multivariate analyses.

Results

The median follow-up duration in the derivation and the verification group was 12.9 and 12.2 months, respectively. Multivariate logistic regression analysis after adjusting for sex and age indicated that First-UDCA treatment [odds ratio (OR)=2.543, 95% confidence interval (CI): 1.234–5.240,P=0.011], baseline alanine aminotransferase level (OR=1.265, 95% CI: 1.089–1.471,P=0.002), and baseline total bilirubin level (OR=0.571, 95% CI: 0.420–0.776,P<0.001) were independent factors that influenced the biochemical response in PBC patients after 1 year of UDCA treatment. Therefore, the resulting biochemical response prediction score model represented the sum of the points corresponding to these three variables. The area under the receiver operating characteristic curve of the score model in the derivation group and the verification group was 0.763 (95% CI: 0.701–0.817,P<0.001) and 0.798 (95% CI: 0.681–0.887,P<0.001), respectively.

Conclusion

We developed and verified an easy-to-use scoring model for the first time, which showed excellent predictive value for the biochemical response in PBC patients.
Sirolimus for Recurrent Giant Cell Myocarditis After Heart Transplantation: A Unique Therapeutic Strategy
Authors: Patel Apurva D. , Lowes Brian , Chamsi-Pasha Mohammed A. , Radio Stanley J. , Hyden Marshall , Zolty Ronald
Published: June 2018

Clinical Features:

Giant cell myocarditis (GCM) is a rare and a rapidly progressive disorder with fatal outcomes such that patients often require heart transplantation. We present a case of recurrent GCM in a transplanted patient with a history of Crohn disease requiring a novel therapeutic approach.

Therapeutic Challenge:

After the orthotopic heart transplantation, GCM recurred on aggressive immunosuppression over the months, which included corticosteroids, basiliximab, tacrolimus, antithymocyte globulin, and rituximab. Although combination immunosuppressive therapy containing cyclosporine and 2–4 additional drugs including corticosteroids, azathioprine, mycophenolate mofetil, muromonab, gammaglobulin, or methotrexate have shown to prolong the transplant-free survival by keeping the disease under control, its role in preventing and treating recurrence posttransplantation is unclear.

Solution:

We added sirolimus, a macrolide antibiotic, with properties of T- and B-lymphocyte proliferation inhibition on the above immunosuppressive treatment postrecurrence of GCM. After sirolimus initiation and continuation, the patient has remained disease free.
MicroRNA-17, MicroRNA-19b, MicroRNA-146a, MicroRNA-302d Expressions in Hepatoblastoma and Clinical Importance
Authors: Ecevit Çiğdem Ö. , Aktaş Safiye , Tosun Yildirim Hülya , Demirağ Bengü , Erbay Ayşe , Karaca İrfan , Çelik Ahmet , Demir Ayşe Banu , Erçetin Ayşe Pinar , Olgun Nur
Published: June 2018

Hepatoblastoma (HB) is the most common liver malignancy in children. The prognosis changes according to the histologic subtypes of HB. In the present study, we aimed to characterize the expression level of selected microRNAs (miRNAs) in HB as well as in histologic subtypes, and to consider the association with the prognosis. A total of 22 HB tumor samples, subtyped as fetal (n=16) and embryonal (n=6), and 10 nontumorous surrounding liver samples were evaluated in this study. Expressions of miR-17, miR-146a, miR-302d, and miR-19b were analyzed in 22 HB tumor samples and 10 nontumorous surrounding liver samples by quantitative real-time polymerase chain reaction. Lower miRNA-17 expression levels were obtained in tumor samples in comparison with nontumorous surrounding liver samples (P=0.028). Lower miRNA-17 expression was significant for predicting prognosis in HB patients (area under receiver-operator characteristic curve=0.875, P=0.044). A higher-level of miR-19b was found in embryonal samples (P=0.008). Overall and event-free survival was not found to correlate with miRNA expression levels (P>0.05). This research finds miRNA-17 and miRNA-19b expression levels can provide important data on diagnosis and prognosis in HB showing different clinical behaviors.
The Efficiency and Toxicity of Mifamurtide in Childhood Osteosarcoma
Authors: Tacyildiz Nurdan , Incesoy Ozdemir Sonay , Unal Emel , Berber Melda , Dincaslan Handan , Yavuz Gulsan
Published: June 2018

The aim of the present study was to evaluate the efficiency and side effects of mifamurtide in childhood osteosarcoma (OS). In total, 477 doses of 2 mg/m2 intravenous (IV) mifamurtide, along with paracetamol as a premedication, were given to 15 patients with primary nonmetastatic OS after complete surgical resection and to 3 patients with progressive OS. The most common side effects encountered in the patients were chills and fever (17/18). These reactions were observed in 4 patients during the administration of each dose, in a single patient during the last administration, and in the remaining 12 patients during the first or initial 2 administrations. Headache, myalgia, and arthralgia were observed in 2 patients during each infusion. Headache was observed in 1 patient with additional hearing loss during the first 2 infusions. One patient had back pain occuring within the first infusion. Of the 15 patients with primary nonmetastatic OS and treated with the addition of mifamurtide to chemotherapy, 13 showed a complete remission, and 2 patients were still under treatment with a complete remission. Of 3 patients with progressive disease, 2 died while the disease progressed further in the third case over a 51-month period. The 3-year overall survival and event-free survival distributions were 87.5% (mean follow-up time, 46.12; 95% confidence interval, 37.79-52.45 mo) and 75.6% (mean follow-up time, 31.30; 95% confidence interval, 26.54-36.06 mo), respectively. We consider that mifamurtide therapy is a safe and well-tolerated agent in childhood OS.
Successful Treatment of Pediatric Epstein-Barr Virus–positive Aggressive Natural Killer-Cell Leukemia
Authors: Kim Bo Kyung , Hong Kyung Taek , Kang Hyoung Jin , An Hong Yul , Choi Jung Yoon , Hong Che Ry , Park Kyung Duk , Lee Dong Soon , Shin Hee Young
Published: June 2018

Epstein-Barr virus (EBV)–positive aggressive natural killer-cell leukemia (ANKL) is a rare malignancy of mature natural killer cells, with a very poor survival rate. Patients have a rapidly declining clinical course and a poor prognosis, with a median survival of only a few months. Herein, we describe a 16-year-old boy who was diagnosed with EBV-positive ANKL and successfully treated using combination chemotherapy and a subsequent allogeneic hematopoietic stem cell transplantation (alloHSCT). The patient is disease free 4 years and 9 months after alloHSCT. Thus, combination chemotherapy followed by alloHSCT seems to be a promising therapeutic option for EBV-positive ANKL.