Τετάρτη, 18 Μαρτίου 2020

1.
 2020 Mar 16;16(3):e1008447. doi: 10.1371/journal.ppat.1008447. [Epub ahead of print]

STUB1 is targeted by the SUMO-interacting motif of EBNA1 to maintain Epstein-Barr Virus latency.

Wang Y1Du S1Zhu C1Wang C1Yu N1Lin Z1Gan J1,2Guo Y3Huang X4He Y4Robertson E5Qu D1Wei F2Cai Q1,6.

Abstract

Latent Epstein-Barr virus (EBV) infection is strongly associated with several malignancies, including B-cell lymphomas and epithelial tumors. EBNA1 is a key antigen expressed in all EBV-associated tumors during latency that is required for maintenance of the EBV episome DNA and the regulation of viral gene transcription. However, the mechanism utilized by EBV to maintain latent infection at the levels of posttranslational regulation remains largely unclear. Here, we report that EBNA1 contains two SUMO-interacting motifs (SIM2 and SIM3), and mutation of SIM2, but not SIM3, dramatically disrupts the EBNA1 dimerization, while SIM3 contributes to the polySUMO2 modification of EBNA1 at lysine 477 in vitro. Proteomic and immunoprecipitation analyses further reveal that the SIM3 motif is required for the EBNA1-mediated inhibitory effects on SUMO2-modified STUB1, SUMO2-mediated degradation of USP7, and SUMO1-modified KAP1. Deletion of the EBNASIM motif leads to functional loss of both EBNA1-mediated viral episome maintenance and lytic gene silencing. Importantly, hypoxic stress induces the SUMO2 modification of EBNA1, and in turn the dissociation of EBNA1 with STUB1, KAP1 and USP7 to increase the SUMO1 modification of both STUB1 and KAP1 for reactivation of lytic replication. Therefore, the EBNA1SIM motif plays an essential role in EBV latency and is a potential therapeutic target against EBV-associated cancers.
2.
 2020 Mar 2. doi: 10.3233/PRM-190601. [Epub ahead of print]

Training, detraining, and retraining: Two 12-week respiratory muscle training regimens in a child with infantile-onset Pompe disease.

Abstract

BACKGROUND:

Respiratory muscle weakness is a primary cause of morbidity and mortality in patients with Pompe disease. We previously described the effects of our 12-week respiratory muscle training (RMT) regimen in 8 adults with late-onset Pompe disease [1] and 2 children with infantile-onset Pompe disease [2].

CASE REPORT:

Here we describe repeat enrollment by one of the pediatric participants who completed a second 12-week RMT regimen after 7 months of detraining. We investigated the effects of two 12-week RMT regimens (RMT #1, RMT #2) using a single-participant A-B-A experimental design. Primary outcome measures were maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP). Effect sizes for changes in MIP and MEP were determined using Cohen's d statistic. Exploratory outcomes targeted motor function.

RELEVANCE:

From pretest to posttest, RMT #2 was associated with a 25% increase in MIP and a 22% increase in MEP, corresponding with very large effect sizes (d= 2.92 and d=A 2.65, respectively). Following two 12-week RMT regimens over 16 months, MIP increased by 69% and MEP increased by 97%, corresponding with very large effect sizes (d= 3.57 and d= 5.10, respectively). MIP and MEP were largely stable over 7 months of detraining between regimens. Magnitude of change was greater for RMT #1 relative to RMT #2.

KEYWORDS:

Pompe disease; glycogen storage disease type II; neuromuscular disease; rehabilitation; respiratory muscle training; skeletal muscle
PMID:
 
32176666
 
DOI:
 
10.3233/PRM-190601
3.
 2020 Feb 29. doi: 10.3233/PRM-180576. [Epub ahead of print]

Incidence and risk factors of communication, swallowing, and orofacial myofunctional disorders in children and adolescents with cancer and benign neoplasms.

Abstract

PURPOSE:

To describe the incidence and risk factors of communication, swallowing, and orofacial myofunctional disorders in a cohort of children and adolescents with cancer and benign neoplasms.

METHODS:

A prospective cohort study conducted with children aged ⩾ 2 years and adolescents of both genders admitted at the Pediatric Oncology Department of the Instituto Nacional de Câncer (INCA) between March 2014 and April 2015. Study participants were submitted to a Speech-Language Pathology (SLP) assessment at three different times: (T1) at hospital admission; (T2) six months after admission; (T3) one year after admission.

RESULTS:

One hundred and sixty individuals were evaluated. At the time of hospital admission, 68 individuals (42.5%) presented with some type of SLP disorder. After one year of follow-up, 22.8% of the patients had developed new impairments. The occurrence of new speech-language disorders had a statistically significant association with the tumor site. In the risk analysis for the development of speech-language disorders with respect to the primary tumor site, compared to other sites, the central nervous system (CNS) tumor group was 8.29 times more likely to present some new alterations, while the head and neck (HN) tumor group had a 10.36-fold higher risk.

CONCLUSION:

An incidence of 22.8% for communication, swallowing, and orofacial myofunctional disorders was observed. The development of these disorders was greater in individuals with tumors in the CNS and in the HN region.

KEYWORDS:

Speech; communication disorders; deglutition disorders; language and hearing sciences; oncology; pediatrics
PMID:
 
32176665
 
DOI:
 
10.3233/PRM-180576
4.
 2020 Mar 16:20190400. doi: 10.1259/dmfr.20190400. [Epub ahead of print]

Peripheral nerve blocks in the cervical region: from anatomy to ultrasound-guided techniques.

Abstract

Cervical plexus nerve blocks have been employed in various head and neck operations. Both adequate anaesthesia and analgesia are attained in clinical practice. Nowadays, ultrasound imaging in regional anaesthesia is driven towards a certain objective that dictates high accuracy and safety during the implementation of peripheral nerve blocks. In the cervical region, ultrasound-guided nerve blocks have routinely been conducted only for the past few years and thus only a small number of publications pervade the current literature. Moreover, the sonoanatomy of the neck, the foundation stone of interventional techniques, is very challenging; multiple muscles and fascial layers compose a complex of compartments in a narrow anatomic region, in which local anaesthetics are injected. Therefore, this review intends to deliver new insights into ultrasound-guided peripheral nerve block techniques in the neck. The sonoanatomy of the cervical region, in addition to the cervical plexus, cervical ganglia, superior and recurrent laryngeal nerve blocks are comprehensively discussed.

KEYWORDS:

cervical plexus; interventional ultrasonography; nerve block
PMID:
 
32176537
 
DOI:
 
10.1259/dmfr.20190400
5.
 2020 Mar 16. doi: 10.1021/jacs.0c00706. [Epub ahead of print]

A Micropeptide MIAC Inhibits HNSCC Progression by Interacting with Aquaporin 2.

Abstract

Several important micropeptides encoded by noncoding RNAs have been identified in recent years, however, there has never been any reports of micropeptide in head and neck squamous cell carcinoma (HNSCC). Here we report the discovery and characterization of a human endogenous peptide named micropeptide inhibiting actin cytoskeleton (MIAC). Comprehensive analysis of TCGA (The Cancer Genome Atlas) database (n = 500), clinical fresh samples (n = 94) and tissue microarrays (n = 60) revealed that lower MIAC expression is correlated with poor overall survival of HNSCC patients. Meanwhile, RNA-sequencing analysis of 9657 human tissues across 32 cancer types from TCGA cohorts found that MIAC significantly associates with the progression of 5 other different tumors. Mechanistically, MIAC directly interacts with AQP2 (Aquaporin 2) to inhibit actin cytoskeleton by regulating the SEPT2 (Septin 2)/ITGB4 (Integrin Beta 4), and ultimately suppress tumor growth and metastasis of HNSCC. Collectively, the mechanism investigation and evaluation of MIAC activity in vivo and in vitro highlight that MIAC plays an important role in HNSCC tumorigenesis.
PMID:
 
32176498
 
DOI:
 
10.1021/jacs.0c00706
6.
 2020 Mar 16. doi: 10.1002/lary.28606. [Epub ahead of print]

Hospitalized Patients With New-Onset Vocal Fold Immobility Warrant Inpatient Injection Laryngoplasty.

Abstract

OBJECTIVES:

To evaluate the impact of early inpatient bedside injection laryngoplasty (IL) in hospitalized patients with iatrogenic unilateral vocal fold immobility (UVFI).

STUDY DESIGN:

Retrospective cohort study.

METHODS:

A retrospective review of hospitalized patients with iatrogenic UVFI undergoing IL between September 2013 and June 2017 was performed. Patients had a swallow evaluation by a speech-language pathologist and bedside IL. Evaluated outcomes included swallow scores, return to diet, secondary events/procedures, and hospital length of stay. Outcomes related to etiology of UVFI were also examined.

RESULTS:

The cohort consisted of 90 patients (61% male, 52% after cardiac/cardiothoracic surgery). Seventy-seven percent of all patients who could improve had increased swallow scores after IL. The lowest number (40%) from the subgroup of patients with high vagal injuries as the cause of UVFI obtained improvement in swallow scores, whereas 87% of those in the cardiac surgery group improved. There were more bronchoscopies and reintubations in patients before IL than after IL.

CONCLUSION:

Hospitalized patients with UVFI are at increased risk of morbidity and mortality due to dysphagia. We advocate for early swallow evaluation and intervention with IL if there is dysphagia and risk of aspiration. Coordination of care between interdisciplinary teams is paramount to a successful inpatient IL program.

LEVEL OF EVIDENCE:

2b Laryngoscope, 2020.

KEYWORDS:

Injection laryngoplasty; aspiration; dysphagia; injection augmentation; vocal fold paralysis
PMID:
 
32176334
 
DOI:
 
10.1002/lary.28606
7.
 2020 Mar 16. doi: 10.1002/lary.28613. [Epub ahead of print]

Neuromonitored Thyroid Surgery: Optimal Stimulation Based on Intraoperative EMG Response Features.

Abstract

OBJECTIVES:

To evaluate/compare normative electrophysiologic electromyography (EMG) response characteristics of recurrent laryngeal, vagus, and external branch of superior laryngeal nerve evoked with different stimulators used in neuromonitored thyroid surgery.

STUDY DESIGN:

Prospective crossover study METHODS: EMG responses obtained via endotracheal tube surface electrodes in 11 patients undergoing thyroid surgery were recorded when stimulated with four stimulators: two monopolar (Prass standard and ball tip), one bipolar, and one dissecting instrument. Normative mean EMG results including latency, amplitude, threshold, saturation currents, and distance-sensitivity were compared.

RESULTS:

The Prass standard stimulator had shorter latency time when nerve was not covered with fascia (P = .04). The bipolar, dissecting instrument, and ball tip demonstrated similar latency times with and without nerve fascia. Pooled mean latency increased significantly from 1.86 ms to 2.16 ms when comparing nerves without fascia and nerves with fascia (P < .05). The Prass standard monopolar stimulator had the lowest mean threshold at 0.40 mA, with the dissecting instrument having the highest threshold at 0.89 mA for dissected nerve. Pooled mean threshold and saturation increased from 0.6 mA to 1.7 mA (P < .0001) and 1.57 mA to 4.15 mA (P < .001) with fascia covering nerve, respectively. The mean depolarization rate was 100% for monopolar and bipolar electrodes and 81% for dissecting instrument at 1 mA. Only 9% of monopolar electrodes generated an EMG response when stimulated from 2 mm away.

CONCLUSION:

Monopolar stimulators are more sensitive for neural mapping, whereas bipolar instruments are more specific, thus reducing false positive stimulation. Dissecting instruments share many features of monopolar stimulators while being more specific, and thus are a viable alternative.

LEVEL OF EVIDENCE:

2b Laryngoscope, 2020.

KEYWORDS:

EMG; Thyroid surgery; electrophysiology; intraoperative neuromonitoring; stimulation; stimulator
PMID:
 
32176321
 
DOI:
 
10.1002/lary.28613
8.
 2020 Mar 16. doi: 10.1002/lary.28605. [Epub ahead of print]

A Novel Laryngoscope With an Adjustable Distal Tip.

Abstract

OBJECTIVES:

Various laryngoscopes are currently available for supraglottic, glottic, and cervical esophageal exposure, yet none allow for adjustable articulation of the laryngoscope distal tip. We sought to create a new laryngoscope to improve anatomic field of view exposure.

STUDY DESIGN:

Novel laryngoscope device validation study.

MATERIALS AND METHODS:

Three-dimensional printed plastic and titanium prototype designs were created using Solidworks. Validation testing was performed in a cadaveric model. Optimal exposure of the cadaveric larynx and supraglottis was determined by ensuring the endoscope tip was exactly 2.5 cm from the level of the vocal cords. The prototype exposure (22-cm adjustable tip laryngoscope) was compared to the Weerda (18-cm distending laryngoscope) and Dedo (18-cm operating laryngoscope) laryngoscope exposures. Anteroposterior (AP) and lateral (L) exposure measurements were obtained from analysis of endoscopic images. Objective millimeter quantification was performed by pixel calibration to the known width of the vocal cord.

RESULTS:

The prototype provided 77.3-mm AP and 40.6-mm L exposure of the cadaveric larynx and supraglottis. These measurements were then compared to the exposure provided by the Weerda (49.9-mm AP, 40.4-mm L) and Dedo (15.7-mm AP, 18.6-mm L) laryngoscopes. The investigators found the prototype had similar handling characteristics to the Weerda laryngoscope and laryngeal instrumentation was enhanced due to a wider field of view.

CONCLUSION:

This novel laryngoscope with an adjustable distal tip provides improved exposure of the supraglottis and glottis in a cadaveric study and has the potential to be used for both supraglottic/glottic and proximal esophageal procedures.

LEVEL OF EVIDENCE:

5 Laryngoscope, 2020.

KEYWORDS:

Laryngoscope; innovation; medical device; novel
PMID:
 
32176319
 
DOI:
 
10.1002/lary.28605
9.
 2019 Dec 30;3(1):22-50. doi: 10.5414/ALX02120E. eCollection 2019.

ARIA guideline 2019: treatment of allergic rhinitis in the German health system.

Abstract

BACKGROUND:

The number of patients affected by allergies is increasing worldwide. The resulting allergic diseases are leading to significant costs for health care and social systems. Integrated care pathways are needed to enable comprehensive care within the national health systems. The ARIA (Allergic Rhinitis and its Impact on Asthma) initiative develops internationally applicable guidelines for allergic respiratory diseases.

METHODS:

ARIA serves to improve the care of patients with allergies and chronic respiratory diseases. In collaboration with other international initiatives, national associations and patient organizations in the field of allergies and respiratory diseases, real-life integrated care pathways have been developed for a digitally assisted, integrative, individualized treatment of allergic rhinitis (AR) with comorbid asthma. In the present work, these integrated care pathways have been adapted to the German situation and health system.

RESULTS:

The present ICP (integrated care pathway) guideline covers key areas of the care of AR patients with and without asthma. It includes the views of patients and other healthcare providers.

DISCUSSION:

A comprehensive ICP guideline can reflect real-life care better than traditional guideline models.

KEYWORDS:

allergen-specific immunotherapy; allergic asthma; allergic diseases; health care system; integrated care pathway
PMID:
 
32176226
 
PMCID:
 
PMC7066682
 
DOI:
 
10.5414/ALX02120E
10.
 2020 Feb 29;(156). doi: 10.3791/60795.

Intrathecal Application of a Fluorescent Dye for the Identification of Cerebrospinal Fluid Leaks in Cochlear Malformation.

Abstract

In cases of cerebrospinal fluid (CSF) leaks, reliable detection of their origins is needed to seal the leak sufficiently and prevent complications, such as meningitis. A method is presented here using intrathecal administered fluorescein in a clinical case of bilateral congenital ear malformation. A fluorescent dye is administered intrathecally to achieve intraoperative visualization of CSF leaks. The dye is applied 20 min before surgery, and concentration of 5% is used. Per every 10 kg of body weight, 0.1 mL of the fluid is applied intrathecally. The fluorescein is visualized using a fully digital microscope. The origin of the fluid leak is identified in the stapes footplate. During primary surgery, it is sealed, and cochlea implantation is performed for hearing restoration. In this specific case, 6 weeks later, the implant was explanted due to acute meningitis, and the electrode array was left as a spacer. Postoperatively, in the aural smear, β-transferrin was detected. During a revision mastoidectomy, dislocated coverage of the leak was found. The stapes was removed and oval window sealed. Five days after revision surgery, no β-transferrin was detected in the aural smear. During the revision of cochlea implantation 6 months later, intact coverage of the oval niche was observed. Thus, intrathecal fluorescein application proves to be a reliable tool for the detection of CSF leaks. It facilitates the orientation in malformations and complicated or unknown surgical situs. In the literature, its use is described for CSF fistulas in endonasal surgery but is rarely described in skull base and mastoid surgeries. The method has been used successfully in several cases with CSF leaks, and the results confirm the feasibility of safely accessing the origin of the leak.
PMID:
 
32176204
 
DOI:
 
10.3791/60795
11.
 2020 Apr;41(4):561. doi: 10.1097/MAO.0000000000002433.

Repair of a Temporal Bone Encephalocele With the Surgical Exoscope.

Abstract

OBJECTIVE:

We describe our experience using the extracorporeal video microscope, the "exoscope" for repair of a temporal bone encephalocele.

METHOD:

The patient is a 69-year-old male with a right temporal lobe encephalocele herniating through a tegmen defect. He underwent definitive tegmen defect repair and bipolar cauterization of the encephalocele. The authors elected for a combined transmastoid and transtemporal approach in order to isolate the tegmen defect and provide watertight repair. The Synaptive robotic BrightMatter (Toronto, ON) drive video exoscope monitor system was used for the entirety of the case including both the transmastoid approach and transtemporal craniotomy.

RESULTS:

No intraoperative complications were encountered during either the transmastoid (mastoidectomy) or transtemporal craniotomy. The authors were able to complete the entire case without abandonment of the exoscope in favor of the traditional binocular microscope. Advantages of this technology in clinical practice includes high-resolution three-dimensional visualization, increased degrees of freedom for exoscope adjustment, and reduced surgeon fatigue in a fixed, unnatural posture. Limitations include decreased depth perception and increased operative time.

CONCLUSION:

The exoscope system is a safe and effective alternative or adjunct to the existing binocular operating microscope for lateral skull based procedures. The exoscope provides the surgeon with a comfortable, high-resolution visualization without compromising surgical exposure and patient safety.SDC video link: http://links.lww.com/MAO/A837.
12.
 2020 Apr;41(4):560. doi: 10.1097/MAO.0000000000002277.

Underwater Endoscopic Repair of Superior Canal Dehiscence.

Abstract

: Superior canal dehiscence (SCD) is a bony defect of the superior semicircular canal (SCC). Patients with SCD Syndrome (SCDS) may experience symptoms such as aural fullness, pulsatile tinnitus, hyperacusis, autophony, or pressure or noise-induced vertigo . The defect can be repaired in various ways, but there is potential for loss of perilymphatic fluid during transmastoid approaches that could result in postoperative sensorineural hearing loss . We hypothesize that if the procedure were performed "underwater" in balanced salt solution (BSS), loss of perilymphatic fluid would be minimized. CASE REPORT:: A 55-year-old male presented with right-sided autophony, pulsatile tinnitus, and hyperacusis. Audiometric testing demonstrated a low-frequency airbone gap and a supranormal bone-conduction threshold at 4 kHz. Ocular VEMP responses were increased amplitude. Temporal bone imaging revealed a SCC dehiscence.The patient was taken to the operating room for an underwater, endoscopic repair of the SCC using a transmastoid approach. A cortical mastoidectomy was performed using Landmarx image navigation. BSS filled the mastoid and a 0-degree endoscope with endoscrub was used to see the SCC underwater. The SCC was entered near the ampullated end with a bur. A stepwise plugging process included applying strips of wet and dried fascia and bone dust. The non-ampullated end was similarly plugged. BSS was suctioned, and under microscopic visualization, labyrinthotomies were capped with bone chips. The patient tolerated the procedure well and was discharged the next day. There was no sensorineural hearing loss postoperatively. CONCLUSION:: SCDS may be addressed surgically using multiple approaches. An underwater endoscopic repair of the SCC may be safe and effective surgical treatment.SDC video link: http://links.lww.com/MAO/A808.
13.
 2020 Apr;41(4):e516-e525. doi: 10.1097/MAO.0000000000002573.

A Mouse Model of Tinnitus Using Gap Prepulse Inhibition of the Acoustic Startle in an Accelerated Hearing Loss Strain.

Abstract

HYPOTHESIS:

To develop a mouse model for temporary and persistent tinnitus using the gap startle paradigm.

BACKGROUND:

Behavioral animal models for tinnitus are classified into conditioning- and reflex-based types. Gap prepulse inhibition of the acoustic startle (GPIAS) is based on the acoustic startle modification by a silent gap and gap detection deficit caused by tinnitus gap filling.

METHODS:

We used C57BL/6J mice inherently susceptible to hearing loss and potentially predisposed to tinnitus. They were divided into the control, salicylate-induced tinnitus, and noise-induced tinnitus groups. Mice were tested with the auditory brainstem response at four frequencies (8, 16, 24, and 32 kHz) and GPIAS in three carrier conditions, 16 and 24 kHz narrow band noises (NBNs) and broadband noise, at multiple time points before and after treatment. The ratio between the gap startle and no-gap startle amplitudes was analyzed by a repeated measures design. In addition, the number of tinnitus-positive mice meeting a specified criterion was counted.

RESULTS:

Salicylate/unilateral noise trauma resulted in temporary/permanent tinnitus evidenced by GPIAS reduction. GPIAS reduction was the most significant at 16 kHz NBN among the three carriers in both tinnitus groups. Control mice also showed good gap detection performance at 16 kHz NBN, which is in the most sensitive hearing range in mice.

CONCLUSION:

The GPIAS test in C57BL/6J mice was very reliable at 16 kHz NBN. This tinnitus model developed in the mouse strain of accelerated hearing loss can be used with two options of temporary and persistent tinnitus.
14.
 2020 Apr;41(4):e507-e515. doi: 10.1097/MAO.0000000000002535.

Histology of the Cochlear Outer Sulcus Cells in Normal Human Ears, Presbycusis, and Menière's Disease.

Abstract

HYPOTHESIS:

Outer sulcus cell features and distribution are hypothesized to differ throughout regions of the human cochlea and between diseased and normal specimens.

BACKGROUND:

Outer sulcus cells play a role in inner ear fluid homeostasis. However, their anatomy and distribution in the human are not well described.

METHODS:

Temporal bone specimens with normal hearing (n = 10), Menière's disease (n = 10), presbycusis with flat audiograms (n = 4), and presbycusis with sloping audiograms (n = 5) were examined by light microscopy. Outer sulcus cells were assessed quantitatively and qualitatively in each cochlear turn. One specimen was stained for tubulin immunofluorescence and imaged using confocal microscopy.

RESULTS:

Outer sulcus cells interface with endolymph throughout the cochlea, with greatest contact in the apical turn. Mean outer sulcus cell counts in the upper apical turn (8.82) were generally smaller (all p < 0.05) than those of the upper basal (17.71), lower middle (18.99) upper middle (18.23), and lower apical (16.42) turns. Mean outer sulcus cell counts were higher (p < 0.05) in normal controls (20.1) than in diseased specimens (15.29). There was a significant correlation between mean cell counts and tonotopically expected hearing thresholds in the upper basal (r = -0.662, p = 0.0001), lower middle (r = -0.565, p = 0.0017), and upper middle (r = -0.507, p = 0.0136) regions. Other differences in cell morphology, distribution, or relationship with Claudius cells were not appreciated between normal and diseased specimens. Menière's specimens had no apparent unique features in the cochlear apex. Immunofluorescence staining demonstrated outer sulcus cells extending into the spiral ligament in bundles forming tapering processes which differed between the cochlear turns in morphology.

CONCLUSION:

Outer sulcus cells vary throughout the cochlear turns and correlate with hearing status, but not in a manner specific to the underlying diagnoses of Menière's disease or presbycusis.
15.
 2020 Apr;41(4):e501-e506. doi: 10.1097/MAO.0000000000002557.

Contextualizing the Modern Epidemiology of Neurofibromatosis Type 2 in an Era of Heightened Detection of Sporadic Vestibular Schwannoma.

Abstract

OBJECTIVE:

Closely paralleling previous radiologic studies, recent population-based prevalence data suggest sporadic vestibular schwannoma (VS) affects over one in 2,000 adults and up to one in 500 in those aged 70 years or older. Attributable to increased utilization of magnetic resonance imaging and screening protocols for asymmetrical sensorineural hearing loss, the increasing detection rate of sporadic VS fundamentally changes the perception of VS as a whole. The primary objective of the current study was to contextualize modern epidemiological trends in neurofibromatosis type 2 (NF2) in light of these recent advancements in the understanding of sporadic VS.

STUDY DESIGN:

Population-based study.

SETTING:

Olmsted County, Minnesota. Population size on January 1, 2017: 159,689 people.

PATIENTS:

All patients with NF2 diagnosed between Jan 1, 1966 and Dec 31, 2016, identified using the Rochester Epidemiology Project.

MAIN OUTCOME MEASURES:

Incidence, prevalence.

RESULTS:

Six incident cases were identified over the past 50 years. From 1966 to 2016, the age- and sex-adjusted incidence rate was 0.10 per 100,000 person-years. The incidence rate remained at 0.12 over the most recent decade. Five cases met disease prevalence criteria, and the age- and sex-adjusted prevalence of NF2 on Jan 1, 2017 was 3.1 per 100,000 persons. All prevalent cases were women, and the resultant prevalence among women only was 6.0 per 100,000 persons.

CONCLUSIONS:

The modern age- and sex-adjusted incidence rate and prevalence of NF2 is 0.10 per 100,000 person-years and 3.1 per 100,000 persons, respectively. In contrast to trends in incidence rates of sporadic VS over the last half-century, this study demonstrates that the incidence of NF2 has remained relatively stable since 1966. This divergence in epidemiological trends is likely attributed to the nature of NF2, with early clinical manifestations resulting in diagnosis regardless of modern advances, whereas the increased incidence of sporadic VS is heavily influenced by improved detection in a greater population of patients with minimally symptomatic or asymptomatic tumors.
16.
 2020 Apr;41(4):522-529. doi: 10.1097/MAO.0000000000002580.

What Genes Can Tell: A Closer Look at Vestibular Schwannoma.

Abstract

OBJECTIVE:

Comprehensive molecular profiling of radioresistant and cystic vestibular schwannoma (VS) subtypes.

STUDY DESIGN:

Our study utilized whole-exome sequencing (WES), RNA-sequencing (RNAseq), and correlated clinical data from 12 samples (2 samples of solid sporadic subtype, 8 with cystic changes, and 2 previously irradiated).

SETTING:

Academic medical center.

PATIENTS:

Patients diagnosed with VS who required surgical treatment. Inclusion: Cystic and radioresistant tumors matched to age and tumor volume, with solid sporadic VS samples as control; Exclusion: NF-2 patients.

INTERVENTION(S):

WES using custom probes for copy number analysis. A modified version of the Agilent Human Whole Exome sequencing hybrid capture system was used to process samples. Recurrent variants were identified and compared between groups. Leukocyte-derived DNA was utilized as internal control to reduce false-positives.

MAIN OUTCOME MEASURE(S):

Analysis of genetic landscape of VS subtypes (naive solid VS, cystic VS, and previously irradiated VS) by performing deep next-generation sequencing.

RESULTS:

WES data achieved a mean coverage of 202X and RNAseq generated an average of 74 million total reads. As a group, 25% of samples had 22q loss. Somatic analysis identified previously reported genes and multiple novel mutations across samples. Differential expression analysis of RNAseq data found significantly mutated genes such as COL6A3, CLMP, ART4, Lumican that were shared by both cystic VS and irradiated VS, but not seen in sporadic VS.

CONCLUSIONS:

Using WES we were able to demonstrate that cystic and irradiated samples are subtypes of VS with an increased mutation burden and a unique genetic fingerprint. We identified differences between the genomic and molecular profile of cystic VS and radioresistant VS. Our results help advance the understanding of the pathophysiology of these tumor subtypes and suggest possible molecular targets for novel treatment strategies.
17.
 2020 Apr;41(4):e494-e500. doi: 10.1097/MAO.0000000000002561.

Development and Validation of VM-PATHI: Vestibular Migraine Patient Assessment Tool and Handicap Inventory.

Abstract

OBJECTIVE:

The aim of this article is to develop and validate a disease-specific, patient-reported outcome measure for vestibular migraine.

SETTING:

Tertiary care vestibular center.

PATIENTS:

Adult patients with definite or probable vestibular migraine per Barany Society Criteria.

STUDY DESIGN:

This was a prospective cohort study. VM-PATHI (Vestibular Migraine Patient Assessment Tool and Handicap Inventory) was developed with expert input, literature review, and patient feedback. VM-PATHI scores were compared between those with vestibular migraine and controls, across several time points, and to other dizziness and quality of life (QoL) measures.

RESULTS:

A 25-item questionnaire was developed. Cronbach's α was high at 0.92. Test-retest reliability was excellent (r = 0.90, p < 0.001). Scores were much higher in patients with vestibular migraine (mean 42.5, SD = 16.1) than control patients (mean = 9.6, SD = 8.5). VM-PATHI scores were responsive to treatment (p = 0.01). Scores were well correlated with general QoL, depression, and anxiety scores. Scores were also correlated with the Dizziness Handicap Inventory (r = 0.69). An exploratory factor analysis was performed, which revealed 6 distinct factors that corresponded well to different aspects of disease-related symptomatology.

CONCLUSION:

VM-PATHI is a valid, reliable, and responsive measure of disease severity in vestibular migraine.
18.
 2020 Apr;41(4):511-521. doi: 10.1097/MAO.0000000000002576.

Posterior Semicircular Canal Dehiscence: Case Series and Systematic Review.

Abstract

OBJECTIVE:

To conduct a systematic review of posterior semicircular canal dehiscence (PSCD) and to present a series of patients with PSCD with and without classic third-window symptoms.

DATA SOURCES:

PubMed, Scopus, and the Cochrane Library from inception until April 2019. Case series of five patients seen in a multidisciplinary, vestibular-focused, neurotology clinic.

STUDY SELECTION:

Inclusion criteria: PSCD studies of symptomatology, diagnostic testing, radiology, and histopathology.

EXCLUSION CRITERIA:

non-English articles, reviews, letters, animal studies.

DATA EXTRACTION:

Quality evaluated according to Oxford Center for Evidence-Based Medicine criteria and funnel plot via the Stern and Egger method.

DATA SYNTHESIS:

Two hundred five studies were found, and 58 studies were included. In 47 total patients, sound-induced vertigo, mixed hearing loss, and tinnitus were the most common presenting symptom. A meta-analysis of proportions using eight radiological and histopathological studies revealed an incidence of 0.38% adult ears [95% CI 0.08, 0.89] and 2.16% of adult patients [0.64, 4.54]. The incidence in pediatric patients ranged from 1.3 to 43%. Jugular bulb abnormalities were common. In our case series, four of five patients presented without third-window symptoms, while one had sound- and pressure-induced vertigo. Hearing loss in these patients was not salvageable.

CONCLUSIONS:

PSCD is a rare phenomenon most commonly presenting with third-window type symptoms. However, PSCD might also present with dizziness and hearing loss inconsistent with third-window symptomatology. One should be conscious of potentially poorer prognosis for hearing recovery in these patients.
19.
 2020 Apr;41(4):e484-e493. doi: 10.1097/MAO.0000000000002565.

Human Cochlear Nucleus on 7 Tesla Diffusion Tensor Imaging: Insights Into Micro-anatomy and Function for Auditory Brainstem Implant Surgery.

Abstract

OBJECTIVE:

The cochlear nucleus (CN) is the target of the auditory brainstem implant (ABI). Most ABI candidates have Neurofibromatosis Type 2 (NF2) and distorted brainstem anatomy from bilateral vestibular schwannomas. The CN is difficult to characterize as routine structural MRI does not resolve detailed anatomy. We hypothesize that diffusion tensor imaging (DTI) enables both in vivo localization and quantitative measurements of CN morphology.

STUDY DESIGN:

We analyzed 7 Tesla (T) DTI images of 100 subjects (200 CN) and relevant anatomic structures using an MRI brainstem atlas with submillimetric (50 μm) resolution.

SETTING:

Tertiary referral center.

PATIENTS:

Young healthy normal hearing adults.

INTERVENTION:

Diagnostic.

MAIN OUTCOME MEASURES:

Diffusion scalar measures such as fractional anisotropy (FA), mean diffusivity (MD), mode of anisotropy (Mode), principal eigenvectors of the CN, and the adjacent inferior cerebellar peduncle (ICP).

RESULTS:

The CN had a lamellar structure and ventral-dorsal fiber orientation and could be localized lateral to the inferior cerebellar peduncle (ICP). This fiber orientation was orthogonal to tracts of the adjacent ICP where the fibers run mainly caudal-rostrally. The CN had lower FA compared to the medial aspect of the ICP (0.44 ± 0.09 vs. 0.64 ± 0.08, p < 0.001).

CONCLUSIONS:

7T DTI enables characterization of human CN morphology and neuronal substructure. An ABI array insertion vector directed more caudally would better correspond to the main fiber axis of CN. State-of-the-art DTI has implications for ABI preoperative planning and future image guidance-assisted placement of the electrode array.
20.
 2020 Apr;41(4):494-503. doi: 10.1097/MAO.0000000000002572.

Direct Drive Simulation-Preoperative Sound Simulation of "Vibroplasty-Hearing" in Patients With Mixed Hearing Loss.

Abstract

OBJECTIVE:

Validation of the preoperative sound simulation test for vibroplasty-hearing so-called "Direct Drive Simulation" (DDS) in case of mixed hearing loss.

STUDY DESIGN:

Retrospective data analysis.

SETTING:

Tertiary referral center with a large hearing implant program.

MAIN OUTCOME MEASURE:

Comparison of sound impression during preoperative Direct Drive Simulation, and postoperative testing with the activated active middle ear implant (AMEI) under free-field condition and in daily routine.

PATIENTS:

Fifty-four data sets from 18 patients with mixed hearing loss with a mean age of 60.78 ± 3.18 were included.

RESULTS:

Comparing the sound impression during DDS preoperatively versus free-field testing with the implanted AMEI, no significant differences were found. DDS offers a slightly better sound quality than the AMEI in daily routine, fitting well to the ideal listening situation in DDS versus some background noise in daily routine.

CONCLUSION:

The DDS offers the possibility of a realistic preoperative sound simulation of the "vibroplasty-hearing" in cases of mixed hearing loss. This probably facilitates patient's decision towards a vibroplasty. The audiologist as well as the surgeon get additional information regarding the indication especially when audiologic inclusion criteria are critical. Thus, the DDS is a useful extension of preoperative diagnostics before vibroplasty.
21.
 2020 Apr;41(4):489-493. doi: 10.1097/MAO.0000000000002554.

Incidence of Congenital Stapes Footplate Fixation Since 1970: A Population-based Study.

Abstract

OBJECTIVE:

Several reports have suggested that the incidence of otosclerosis is declining. However, due to the limitations of epidemiologic databases within the United States, little is known about the incidence of congenital stapes footplate fixation (CSFF), much less epidemiological trends over the past 50 years. The current study was conceived to characterize the epidemiology of CSFF over the past five decades using the unique resources of the Rochester Epidemiology Project (REP).

STUDY DESIGN:

Population-based study.

PATIENTS:

The medical records of every resident of Olmsted County, Minnesota who was diagnosed with CSFF was reviewed from January 1, 1970 to December 12, 2017 using the REP. The population of Olmsted County on January 1, 2017 was 159,689 persons.

MAIN OUTCOME MEASURE:

Incidence of CSFF.

RESULTS:

Sixteen cases of CSFF were identified at a median age at diagnosis of 8 years (IQR, 6-17), eight of whom confirmed by exploratory tympanotomy. Three of the 16 (19%) cases were bilateral, and the median pure-tone average (500, 1k, 2k, 3k Hz) at time of diagnosis was 37 dB (IQR, 31-48). Two (13%) cases were identified in patients bearing a syndromic condition and one (6%) case demonstrated a malformed external ear on physical examination. The incidence rate of surgically confirmed cases among residents of Olmsted County was 0.31 per 100,000 person-years from 1970 to 1979 and 0.28 from 2000 to 2009. No incident cases among residents of Olmsted County were identified since 2010.

CONCLUSIONS:

CSFF constitutes a rare disease. Unlike epidemiological trends in otosclerosis, the incidence of surgically confirmed cases of CSFF remained stable over the past 50 years.
22.
 2020 Apr;41(4):e464-e467. doi: 10.1097/MAO.0000000000002567.

Improving Microscopic Imaging in Otology and Neurotology.

Abstract

OBJECTIVE:

Due to the microscopic nature of otologic surgery, photographic image quality suffers from an inherent inability to maintain all the planes of surgery in focus under binocular microscopy. Our goal was to perform simple editing techniques to create improved imaging for educational and research purposes in the field of Otology and Neurotology.

PATIENTS/DESIGN:

The study was a proof of concept performed with series of cases including patients undergoing transmastoid and transcanal otologic surgery over time period of December 1, 2018 to March 1, 2019 at an academic medical center. The Zeiss OPMI Pentero 800 operating microscope was used with a camera capturing 2.1 megapixel, 1098 × 1080 resolution images.

INTERVENTION:

We created a systematic protocol for capturing images of multiple focal lengths during each surgery. With the image-editing technique of focus-stacking, multiple images of varying focal length, were spliced together to produce high-quality and high-fidelity composite images rendered using the Adobe Photoshop (San Jose, CA).

OUTCOMES:

Subjective comparisons of pre and post photo-edited photographs.

RESULTS:

Composite, focus-stacked images with comparison to unedited microscopic pictures are reviewed in the manuscript.

CONCLUSION:

We describe a simple and objectively practical method for improving the quality of medical imaging in the field of Otology/Neurotology. To achieve this enhanced image quality, a relatively expeditious and reliable photographic protocol can be used for image capturing and editing, requiring little to no additional training for a physician in the field.
23.
 2020 Apr;41(4):e449-e457. doi: 10.1097/MAO.0000000000002570.

Comparison of Hearing Preservation Outcomes Using Extended Versus Single-Dose Steroid Therapy in Cochlear Implantation.

Gotamco GL1,2Chou YF1,3Sun CH1,3Hsu CJ1,3Chen PR3,4Wu HP1,3.

Abstract

OBJECTIVES:

The purpose of this study was to compare the hearing preservation outcomes of patients who received extended versus single-dose steroid therapy in cochlear implant surgery.

DESIGN:

Case-control.

SETTING:

Tertiary referral centers in Taiwan from April 2017 to 2019.

PARTICIPANTS:

A total of 70 patients aged 1 to 78 years old (mean = 18.04, standard deviation [SD] = 21.51) who received cochlear implantation via the round window approach were included in the study. Prospectively, 35 cases were enrolled for cochlear implantation with single-dose therapy. Thirty-five controls who underwent cochlear implantation with extended therapy were retrospectively enrolled after frequency matching.

OUTCOME MEASURES:

The main outcome measure was the rate of hearing preservation. This was calculated based on the HEARRING Network formula and results were categorized as complete, partial, and minimal. Impedances served as secondary outcomes.

RESULTS:

There was no significant difference in the complete hearing preservation rates between the extended and single-dose groups at 6 months postoperatively. Impedances were significantly lower in the extended group after 1 month and 6 months of follow up. When the complete and partial hearing preservation groups were compared, the size of round window opening and speed of insertion were found to be statistically significant.

CONCLUSIONS:

Both extended and single-dose therapies result in good hearing preservation in patients who undergo cochlear implantation. However, better impedances can be expected from patients who received extended therapy. A slower speed of insertion and a widely opened round window play a role in hearing preservation.
24.
 2020 Apr;41(4):e441-e448. doi: 10.1097/MAO.0000000000002577.

Minimally Invasive Cochlear Implantation Assisted by Intraoperative CT Scan Combined to Neuronavigation.

Abstract

OBJECTIVE:

The objective of this work was to study the feasibility of minimally invasive cochlear implantation under intraoperative computerized tomography-scan coupled to navigation.

MATERIALS AND METHODS:

Five human resin temporal bones (two adults and three children) were used. Initially, a temporal bone imaging was obtained by the intraoperative CT-scan coupled to the navigation (O-ARM). The navigation-assisted drilling began at the mastoid surface creating a conical tunnel (4-2 mm in diameter) through the facial recess and down to the round window. A cochleostomy was performed based on the navigation. A sham electrode array was inserted in the drilled tunnel and into the cochlea.Postoperative CT-scan and dissection were performed to evaluate the trajectory, and possible injury to the external auditory canal, ossicles, or facial nerve.

RESULTS:

The mean duration of the procedure was 24.4 ± 3.79 minutes (range, 15-35). Cochleostomy was possible in all cases without injury to other structures. The sham array was inside the cochlea in all cases. The mean distance between the drilled canal and the mastoid portion of the facial nerve was 1.2 ± 0.07 mm (range, 1.08-1.38). The mean tracking error was 0.6 ± 0.26 mm (range, 0.20-0.72) at the entry point, 0.6 ± 0.33 mm (range, 0.2-1.02) at the facial nerve and 0.4 ± 0.07 mm (range, 0.36-0.51) at the cochleostomy.

CONCLUSION:

Cochlear implantation through a minimally invasive approach assisted by intraoperative imaging combined with navigation was feasible in operating room environment and experimental conditions.
25.
 2020 Apr;41(4):e432-e440. doi: 10.1097/MAO.0000000000002578.

Impact of Underlying Diagnosis on Speech and Quality of Life Outcomes After Cochlear Implantation for Single-Sided Deafness.

Abstract

OBJECTIVE:

Our objective was to compare outcomes in speech and quality of life in those undergoing cochlear implantation for single-sided deafness (SSD), with the aim to characterize the clinical impact of underlying diagnosis in the affected ear and pre-operative hearing status.

STUDY DESIGN:

Prospective case series.

SETTING:

Academic Cochlear Implant Center.

PATIENTS:

42 adult patients implanted with the diagnosis of SSD.

INTERVENTIONS:

Patients were evaluated at 3-, 6-, and 12-months post-operatively using AZBio sentence and speech, and consonant-nucleus-consonant (CNC) depending on appropriate testing level. Our previously validated Comprehensive Cochlear Implant Quality of Life (CCIQ) questionnaire was administered.

MAIN OUTCOME MEASURES:

Speech perception, quality of life.

RESULTS:

Subjects were stratified by the underlying diagnosis: Meniere's Disease (MD; n = 10), sudden sensorineural hearing loss (SSNHL; n = 13), and Other (eg TBI, acoustic neuroma, progressive, noise-induced; n = 19). Mean preoperative PTA of the implanted ear was 82dB ± 17; that of the nonimplanted ear was 32dB ± 17. SSNHL and MD demonstrated the highest speech perception score at 3 months (93 and 95%), and "Other" demonstrated the lowest scores at 88%. All 3 groups demonstrated nadir in speech scores at 6 months before improving at 12 months, but the "Other" diagnoses maintained the lowest speech testing across all time points. All 3 groups reported improved quality of life on CCIQ.

CONCLUSIONS:

Subjects with SSNHL and MD demonstrate excellent speech perception and quality of life outcomes after cochlear implantation for SSD. Subjects with "Other" diagnoses underlying their SSD demonstrated lower scores on speech testing but nonetheless reported improved quality of life.
26.
 2020 Apr;41(4):e422-e431. doi: 10.1097/MAO.0000000000002525.

Impact of Auditory-Motor Musical Training on Melodic Pattern Recognition in Cochlear Implant Users.

Abstract

OBJECTIVE:

Cochlear implant (CI) users struggle with tasks of pitch-based prosody perception. Pitch pattern recognition is vital for both music comprehension and understanding the prosody of speech, which signals emotion and intent. Research in normal-hearing individuals shows that auditory-motor training, in which participants produce the auditory pattern they are learning, is more effective than passive auditory training. We investigated whether auditory-motor training of CI users improves complex sound perception, such as vocal emotion recognition and pitch pattern recognition, compared with purely auditory training.

STUDY DESIGN:

Prospective cohort study.

SETTING:

Tertiary academic center.

PATIENTS:

Fifteen postlingually deafened adults with CIs.

INTERVENTION(S):

Participants were divided into 3 one-month training groups: auditory-motor (intervention), auditory-only (active control), and no training (control). Auditory-motor training was conducted with the "Contours" software program and auditory-only training was completed with the "AngelSound" software program.

MAIN OUTCOME MEASURE:

Pre and posttest examinations included tests of speech perception (consonant-nucleus-consonant, hearing-in-noise test sentence recognition), speech prosody perception, pitch discrimination, and melodic contour identification.

RESULTS:

Participants in the auditory-motor training group performed better than those in the auditory-only and no-training (p < 0.05) for the melodic contour identification task. No significant training effect was noted on tasks of speech perception, speech prosody perception, or pitch discrimination.

CONCLUSIONS:

These data suggest that short-term auditory-motor music training of CI users impacts pitch pattern recognition. This study offers approaches for enriching the world of complex sound in the CI user.
27.
 2020 Apr;41(4):458-466. doi: 10.1097/MAO.0000000000002564.

Prospective Multicentric Follow-up Study of Cochlear Implantation in Adults With Single-Sided Deafness: Tinnitus and Audiological Outcomes.

Abstract

OBJECTIVE:

This study investigated the audiological and tinnitus outcomes of cochlear implantation (CI) in adults with single-sided deafness (SSD) and tinnitus.

STUDY DESIGN:

Multicentered prospective, non-randomized intervention study.

SETTING:

Six French CI centers.

PATIENTS:

Twenty-six patients with SSD and incapacitating tinnitus (Tinnitus Handicap Inventory [THI] >58) underwent cochlear implantation.

INTERVENTIONS:

First, CIs delivered only masking white noise stimulation for 1 month and then standard CI stimulation.

MAIN OUTCOME MEASURES:

Before and after CI surgery, patients completed the THI, Tinnitus Reaction Questionnaire (TRQ), Subjective Tinnitus Severity Scale (STSS), and two visual analogue scales quantifying tinnitus loudness and annoyance. Speech perception in spatialized noise was tested at 13 months.

RESULTS:

The first month of white noise stimulation triggered a significant improvement in THI scores (72 ± 9 to 55 ± 20, p < 0.05). No change was observed for the other measures. After 1 year of standard CI stimulation, 23 patients (92%) reported a significant improvement in tinnitus. This improvement started 1 to 2 months after CI and exceeded 40% improvement for 14 patients (54%). Average speech-in-noise perception after 1 year significantly improved for the 23 patients who completed these measures.

CONCLUSIONS:

CI is efficacious to reduce the handicap of patient with SSD and incapacitating tinnitus, leading to a decrease in reported tinnitus and partial restoration of binaural hearing abilities.
28.
 2020 Apr;41(4):452-457. doi: 10.1097/MAO.0000000000002566.

The Use of Artificial Intelligence to Program Cochlear Implants.

Abstract

OBJECTIVE:

Cochlear implant (CI) technology and techniques have advanced over the years. There has not been the same degree of change in programming and there remains a lack of standardization techniques. The purpose of this study is to compare performance in cochlear implant subjects using experienced clinician (EC) standard programming methods versus an Artificial Intelligence, FOX based algorithm for programming.

STUDY DESIGN:

Prospective, nonrandomized, multicenter study using within-subject experimental design SETTING:: Tertiary referral centers.

PATIENTS:

Fifty-five adult patients with ≥ 3 months experience with a Nucleus 5, 6, Kanso, or 7 series sound processor.

INTERVENTION:

Therapeutic Main Outcome Measures: CNC words and AzBio sentences in noise (+10 dB SNR) tests were administered in a soundproof booth followed by a direct connect psychoacoustic battery using the EC program. Tests were repeated 1 month later using the optimized FOX program. Subjective measures of patient satisfaction were also measured.

RESULTS:

Performance for the EC program was compared to the FOX program for both measures. Group mean results revealed equivalent performance (Kruskal-Wallis ANOVA p = 0.934) with both programming methods. While some patients had better performance with the FOX method and some performed more poorly, the majority had equivalent performance and preferred the FOX system.

CONCLUSION:

The study demonstrated that on average, FOX outcomes are equivalent to those using traditional programming techniques. In addition, the FOX programming method can effect standardization across centers and increase access for many individuals who could benefit.
29.
 2020 Apr;41(4):444-451. doi: 10.1097/MAO.0000000000002555.

A Systematic Review of Cochlear Implant Outcomes in Prelingually-deafened, Late-implanted Patients.

Abstract

OBJECTIVE:

To analyze outcomes of cochlear implantation (CI) in prelingually-deafened, late-implanted patients.

DATA SOURCES:

A search of MEDLINE and EMBASE was performed in February 2018 using SCOPUS for the intersection of "cochlear implant," "prelingual," "deaf," and "delay."

REVIEW METHODS:

Two independent reviewers screened all abstracts and titles for relevance, with conflicts resolved by either the primary or senior author. All articles passing this screen were subjected to a full-text review, during which the primary and senior author each examined manuscripts for inclusion and exclusion criteria. The Cochrane Risk of Bias Assessment Tool was used to assess potential sources of systematic error, and postoperative clinical outcomes were collected at the latest clinical follow-up.

RESULTS:

Twenty-eight articles were yielded in the final systematic review, accounting for 542 patients. For open-set sentence scores, 10 studies representing 240 patients showed an overall estimated improvement of 44.6% (95% CI: 38.0-51.2%). In terms of quality of life, studies generally showed improvement when looking at specific emotional, social, or hearing-specific domains, but not in global measures. Nonuser rates ranged from 0 to 9.5%.

CONCLUSION:

Despite performance that is generally poorer than what is generally seen in "traditional" candidates, prelingually-deafened, late-implanted (PL-LI) CI users can experience benefit in terms of both QOL and audiometric scores. The wide range of performance that is reported in the literature highlights the importance of careful patient counseling.
30.
 2020 Apr;41(4):e414-e421. doi: 10.1097/MAO.0000000000002568.

Audiometric Predictors of Bothersome Tinnitus in a Large Clinical Cohort of Adults With Sensorineural Hearing Loss.

Abstract

OBJECTIVE:

To identify demographic and audiometric predictors of bothersome tinnitus within a large clinical cohort.

STUDY DESIGN:

Retrospective chart review.

SETTING:

Tertiary care hospital.

PATIENTS:

51,989 English-speaking patients between 18 and 80 years of age that received initial audiometric evaluations at the Massachusetts Eye and Ear Infirmary between the years 2000 and 2016.

MAIN OUTCOME MEASURES:

Patients were categorized according to whether or not tinnitus was the primary reason for their visit. The likelihood of tinnitus as a primary complaint (TPC) was evaluated as a function of age, sex, and audiometric configuration. Patient-reported tinnitus percepts were qualitatively assessed in relation to audiometric configuration.

RESULTS:

Approximately 20% of adults who presented for an initial hearing evaluation reported TPC. The prevalence of TPC increased with advancing age until approximately 50 to 54 years, and then declined thereafter. In general, men were significantly more likely to report TPC than women. TPC was statistically associated with specific audiogram configurations. In particular, TPC was most prevalent for notched and steeply sloping hearing losses, but was relatively uncommon in adults with flat losses. Patients with frequency-restricted threshold shifts often reported tonal tinnitus percepts, while patients with asymmetric configurations tended to report broadband percepts.

CONCLUSIONS:

The probability of seeking audiological evaluation for bothersome tinnitus is highest for males, middle-aged patients, and those with notched or high-frequency hearing losses. These findings support the theory that tinnitus arises from sharp discontinuities in peripheral afferent innervation and cochlear amplification, which may induce topographically restricted changes in the central auditory pathway.
31.
 2020 Apr;41(4):429-430. doi: 10.1097/MAO.0000000000002560.

Inaugural Otology and Neurotology "Video Report".

32.
 2020 Mar;99(11):e19497. doi: 10.1097/MD.0000000000019497.

Protocol for a prospective multicenter cross-sectional observational study to investigate the role of air pollution on allergic rhinitis prevalence.

Liu J1,2Wang Y2Liu S2Cao S2Xu C3Zhang M2Liu S1.

Abstract

INTRODUCTION:

Allergic rhinitis (AR) is a major chronic inflammatory disease of the respiratory tract. A large number of epidemiological investigations have shown that the prevalence of AR is increasing, resulting in a large social burden. Importantly, the impact of air pollution on health is a widespread concern. We aim to evaluate association of air pollution and AR risk.

METHODS AND ANALYSIS:

This prospective study includes patients undergoing AR. The exclusion criteria will be as follows: Patients with nasal infection, nasal polyps, nasal tumors, mental disorders, and immunodeficiency will be excluded. Air pollution levels of ambient air pollutants including PM2.5, PM10, sulfur dioxide (SO2), nitrogen dioxide (NO2), carbon dioxide (CO), and O3, and patient data will be collected. The correlation analysis will be performed in air pollutants and AR risk.

DISCUSSION:

This study will provide correlation of NO2, SO2, PM10, and PM2.5 for AR in several aspects, including symptom score, drug score, quality of life score, asthma control score, side effects, and laboratory examination such as nasal function test, serum total immunoglobulin E, and nasal secretion smear.
PMID:
 
32176091
 
DOI:
 
10.1097/MD.0000000000019497
33.
 2020 Mar;99(11):e19491. doi: 10.1097/MD.0000000000019491.

Promoter hypermethylation of CD133/PROM1 is an independent poor prognosis factor for head and neck squamous cell carcinoma.

Hu Z1Liu H1Zhang X1Hong B1Wu Z2Li Q3,4,5Zhou C3,4,5.

Abstract

PROM1 has played a pivotal role in the identification and isolation of tumor stem cells. This study aimed to assess the association between PROM1 promoter methylation and head and neck squamous cell carcinoma (HNSCC), and its diagnostic and prognostic value.Bioinformatic analysis was performed using data from the Cancer Genome Atlas-HNSC and Gene Expression Omnibus datasets.The results showed that PROM1 promoter was hypermethylated in HNSCCs compared with normal head and neck tissues (P = 4.58E-37). The area under the receiver-operating characteristic curve based on methylated PROM1 data was 0.799. In addition, PROM1 hypermethylation independently predicted poor overall survival (hazard ratio [HR]: 1.459, 95% confidence interval [CI]: 1.071-1.987, P = .016) and recurrence-free survival (HR: 1.729, 95% CI: 1.088-2.749, P = .021) in HNSCC patients. Moreover, PROM1 methylation was weakly negatively correlated with its mRNA expression (Pearson r = -0.148, P < .001).In summary, our study reveals that methylated PROM1 might serve as a valuable diagnostic biomarker and predictor of poor survival for HNSCC patients. PROM1 hypermethylation might partially contribute to its downregulation in HNSCC.
PMID:
 
32176088
 
DOI:
 
10.1097/MD.0000000000019491
34.
 2020 Mar;99(11):e19284. doi: 10.1097/MD.0000000000019284.

Low-grade albuminuria is associated with hearing loss in non-diabetic US males: A cross-sectional analysis of 1999-2004 national health and nutrition examination survey.

Abstract

High levels of albuminuria have been demonstrated to associate with hearing loss in non-diabetic people, while the clinical impact of low-grade albuminuria has attracted less attention. This cross-sectional population-based study aimed to examine whether hearing loss in non-diabetic United States (US) adults is independently associated with low-grade albuminuria or reduced estimated glomeruli filtration rate (eGFR).A total of 2518 participants aged 20 to 69 years were selected from the US National Health and Nutritional Examination Survey database. Participants with diabetes or high-grade albuminuria were excluded. Hearing loss was assessed using low-frequency pure-tone average (LFPTA) thresholds (0.5, 1.0, 2.0 kHz) and high-frequency pure-tone average (HFPTA) thresholds (3.0, 4.0, 6.0, 8.0 kHz). Logistic and linear regression analyses were used to evaluate associations between renal function indicators and hearing loss.The median age of included participants was 37.4 years, and 55% of them were female. Multivariate analysis revealed that participants with urinary albumin-to-creatinine ratio (UACR) in the highest tertile had a significantly higher risk of hearing loss (OR, 1.79; 95% CI, 1.01-3.19) and higher HFPTA thresholds (β: 2.23; SE: 0.77). Participants with eGFR <60 mL/min/1.73 m had higher LFPTA thresholds (β: 4.31; SE: 1.79). After stratification by sex, a significant risk remained only for males in the highest UACR tertile, with 2.18 times the risk of hearing loss (95% CI, 1.06-4.48).Non-diabetic US males with low-grade albuminuria are at increased risk of hearing loss, independent of eGFR.
PMID:
 
32176052
 
DOI:
 
10.1097/MD.0000000000019284
35.
 2020 Mar 13. doi: 10.1097/SCS.0000000000006388. [Epub ahead of print]

Incidence and Prevalence of Head and Neck Pathological Spectrum in a Pediatric Population.

Abstract

AIM:

The aim of this study is to determine the frequency of pediatric pathologies and their distribution according to age, gender, site and types of lesions reported over a period of 2004 - 2019 in two prominent Dental Colleges at Western UP, India.

MATERIAL AND METHODS:

All the cases of pediatric pathology were retrospectively analyzed and reviewed that reported in the Department of Oral And Maxillofacial Surgery of 2 prominent dental colleges of Western UP, India. All consecutive patients (< 18 years), between 2004 to 2019, histologically diagnosed as having an intraosseous tumor or tumor-like lesions and cystic lesion, vascular lesions, patients with space infections and hardware infection formed the study population. Patients fulfilling the inclusion criteria were only considered for further study. The study was granted an exemption by the institutes. The entire study material was analyzed and grouped into prominent categories for logical conclusions: The accumulated data was grouped, entered, and analyzed.

RESULTS:

One hundred two cases fulfilled the criteria. Gender distribution was equal, with mandible predominance and a predominance of non-odontogenic lesions. There were 4 malignant and 57 benign conditions. There were 23 lesions in the anterior jaw and 38 lesions were present in the posterior jaw.

CONCLUSION:

The pattern of pediatric pathology presentation from this part of India has been documented. In this study, the pediatric jaw tumors are less common compared to those in adult jaw tumors with non-odontogenic tumors being more common.

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