Management of Cholesteatoma: Hearing Rehabilitation.

Surgical treatment of cholesteatoma requires a highly individualized approach, balancing disease eradication with hearing preservation, and necessitates thorough patient counseling on expected auditory outcomes and options for rehabilitation. The choice between canal wall up and canal wall down mastoidectomy can influence hearing outcomes. Hearing rehabilitation options following cholesteatoma surgery include air- and bone-conduction devices and are individualized for the patient. Assistive listening devices such as frequency modulation systems, hearing loops, and Bluetooth technology are critical for enhancing speech understanding in noisy environments for patients who employ various hearing rehabilitation methods and require further advanced solutions.

Temporal Bone Trauma.

Temporal bone trauma represents a potentially underrecognized condition during head injuries and remains an important consideration during the evaluation of these patients. The temporal bone contains many critical neurovascular structures in addition to the primary organs of the auditory and vestibular systems that may be violated during these injuries. Despite the lack of consensus guidelines on the management of these injuries, this review highlights the current literature regarding the diagnosis and management of temporal bone trauma and its potential complications.

Sensory Impairment and the Odds of Adverse Consequences of Unmet Needs for Care Among Older Adults.

OBJECTIVES: Vision and hearing impairments are highly prevalent conditions among older adults, and well-established links exist between sensory impairment and household, mobility, and self-care activity limitations. However, studies examining the impact of unmet long-term services and supports (LTSS) needs have not considered the role of vision and hearing impairment on the risk of experiencing adverse consequences, including wetting or soiling oneself. METHOD: Using Rounds 1 and 5 of the National Health and Aging Trends Study, a nationally representative survey of Medicare beneficiaries aged 65 and older, we examine the association of vision and hearing impairment on the odds of experiencing an adverse consequence while accounting for other sociodemographic and health status factors. RESULTS: Among a weighted population of 49,770,947 community-living older adults with limitations in household, mobility, or self-care activities, 20.1% (95% CI: 19.2-21.0) experienced an adverse consequence as a result of unmet LTSS needs. In the fully adjusted regression, individuals with vision or hearing impairment had 96% (odds ratio [OR]: 1.96; 95% CI: 1.64-2.34) and 43% increased odds (OR: 1.43; 95% CI: 1.24-1.65), respectively, of experiencing any adverse consequence. Hearing impairment was associated with higher odds of household or self-care adverse consequences, while vision impairment was associated with higher odds of mobility or self-care adverse consequences. DISCUSSION: Sensory impairment may increase the risk for adverse consequences for older adults with unmet LTSS needs. Activities that support older adults living safely in the community should consider the role of sensory impairment and how to address the unique needs of those with hearing or vision impairment.

Early Myeloid Derived Suppressor Cells (eMDSCs) Are Associated With High Donor Myeloid Chimerism Following Haploidentical HSCT for Sickle Cell Disease.

Haploidentical hematopoietic stem cell transplantation (haplo-HSCT) is a widely available curative option for patients with sickle cell disease (SCD). Our original non-myeloablative haplo-HSCT trial employing post-transplant (PT) cyclophosphamide had a low incidence of GVHD but had high rejection rates. Here, we aimed to evaluate immune reconstitution following haplo-HSCT and identify cytokines and cells associated with graft rejection/engraftment. 50 cytokines and 10 immune cell subsets were screened using multiplex-ELISA and flow cytometry, respectively, at baseline and PT-Days 30, 60, 100, and 180. We observed the most significant differences in cytokine levels between the engrafted and rejected groups at PT-Day 60, corresponding with clinical findings of secondary graft rejection. Of the 44 cytokines evaluated, plasma concentrations of 19 cytokines were different between the two groups at PT-Day 60. Factor analysis suggested two independent factors. The first factor (IL-17A, IL-10, IL-7, G-CSF, IL-2, MIP-1a, VEGF, and TGFb1 contributed significantly) was strongly associated with engraftment with OR = 2.7 (95%CI of 1.4 to 5.4), whereas the second factor (GROa and IL-18 contributed significantly) was not significantly associated with engraftment. Sufficient donor myeloid chimerism (DMC) is critical for the success of HSCT; here, we evaluated immune cells among high (H) DMC (DMC≥20%) and low (L) DMC (DMC<20%) groups along with engrafted and rejected groups. We found that early myeloid-derived suppressor cell (eMDSC) frequencies were elevated in engrafted patients and patients with HDMC at PT-Day 30 (P< 0.04 & P< 0.003, respectively). 9 of 20 patients were evaluated for the source of eMDSCs. The HDMC group had high mixed chimeric eMDSCs as compared to the LDMC group (P< 0.00001). We found a positive correlation between the frequencies of eMDSCs and Tregs at PT-Day 100 (r=0.72, P <0.0007); eMDSCs at BSL and Tregs at PT-Day 100 (r=0.63, P <0.004). Of 10 immune regulatory cells and 50 cytokines, we observed mixed chimeric eMDSCs and IL-17A, IL-10, IL-7, G-CSF, IL-2, MIP-1a, VEGF, TGFb1 as potential hits which could serve as prognostic markers in predicting allograft outcome towards engraftment following haploidentical HSCT employing post-transplant cyclophosphamide. The current findings need to be replicated and further explored in a larger cohort.

Otolaryngology residency selection: are we doing it right?

PURPOSE OF REVIEW: This article will provide an overview of recent disruptions to the otolaryngology residency match process and conclude with questions and resources that can guide future selection system design. RECENT FINDINGS: During the implementation of the single accreditation system, the loss of osteopathic programs, reduction of osteopathic leadership positions, and lack of interest in Osteopathic Recognition represent serious threats to the profession; this has implications for the distribution of the otolaryngology workforce, plausibly decreasing healthcare access in less-populated communities. Next, the impacts of COVID-19 reverberated throughout the application process, including the reduction/elimination of away rotations, modification of application requirements, conversion to virtual interviews, and initiation of preference signaling. Soon, the transition to pass/fail scoring for the United States Medical Licensing Exam Step 1 could stimulate a paradigm shift, with a heightened emphasis on holistic review. SUMMARY: The last two match cycles have been the most dynamic and unpredictable in decades. Out of the commotion, the otolaryngology community has an opportunity for a fresh start, combining insights from past literature with recent articles compiled for this review. Moving forward, it will be advantageous to approach residency selection as a well-executed quality improvement project, requiring continuous assessment and adjustment.

A Review of Adult-Onset Hearing Loss: A Primer for Neurologists.

PURPOSE OF THE REVIEW: The goal of this review is to highlight current approaches to diagnosis and treatment for adult-onset hearing loss in patients likely to present to a neurologist's office. The review will discuss primary and secondary causes of acute and chronic hearing loss, and will discuss common situations that can be managed by a neurologist as well as situations that require immediate care and referral for further management by an otolaryngologist-head and neck surgeon. RECENT FINDINGS: Hearing screening assessments using mobile applications and tablet devices are now available and can be integrated into many clinical practice settings, including in the evaluation of hearing concerns related to various neurological pathologies. For patients presenting with a sudden worsening in hearing, bedside evaluation, including with objective measures of hearing, can inform neurologists about diagnosis and subsequent management. For patients who present with gradual worsening in hearing, particularly those related to neurologic disorders, hearing care can be an important adjunct to ongoing neurologic care. More commonly encountered, age-related hearing loss is highly prevalent among older adults and may affect overall neurological assessment, including neurocognitive testing, as well as patient-provider communication, patient satisfaction, and care outcomes. Hearing loss is increasingly recognized as a potentially modifiable risk factor for dementia. Neurologists can support the hearing health of their patients through the routine use of communication strategies and by integrating simple, low-cost technology with their current clinical practices. SUMMARY: Both acute and chronic hearing loss can be a symptom of many conditions managed by neurologists. Few conditions are emergent, requiring immediate referral to and treatment by an otolaryngologist-head and neck surgeon. Despite the range of hearing interventions available, including hearing aids, over-the-counter devices, and aural rehabilitation, hearing loss is a common and under-treated chronic health condition. By promptly addressing a patient's hearing concerns, neurologists can improve patients' awareness of the deficit and support the overall importance of maintaining sensory health across the life course.

Racial/Ethnic and Sex Representation in US-Based Clinical Trials of Hearing Loss Management in Adults: A Systematic Review.

Importance: Although the National Institutes of Health (NIH) mandated the inclusion and reporting of women and racial or ethnic minority groups in NIH-funded research in 1993, little is known regarding the representation of women and racial or ethnic minority groups in trials that investigate hearing loss management. Objective: To assess sex and racial/ethnic representation in US-based clinical trials of hearing loss management in an adult population. Data Sources: Pertinent studies were identified using search strategies in PubMed, Embase, and ClinicalTrials.gov. Study Selection: Our search strategy yielded 6196 studies. We included prospective studies that were written in English, performed in the US, and evaluated hearing loss management in adults, including amplification devices, such as hearing aids or assistive listening devices, cochlear implants, aural rehabilitation, and therapeutics. Given its prevalence, only studies that addressed bilateral sensorineural hearing loss were included. Data Extraction and Synthesis: Data from 125 studies were extracted. The Preferred Reporting Items for Systematic Reviews and Meta-analyses diagram for systematic reviews was used for abstracting data. The guidelines were applied using independent extraction by multiple observers. Results: Among 125 clinical studies performed from January 1990 to July 2020 regarding hearing loss management, only 16 (12.8%) reported race/ethnicity, and 88 (70.4%) reported sex. Of the 16 studies that reported race/ethnicity, only 5 included more than 30% non-White representation. Among the 88 articles that reported sex, 44 (35.2%) reported more than 45% female representation. While the mean number of participants included in the observed trials was 80 (range, 7-644), the median number of participants from racial or ethnic minority groups in studies that reported race/ethnicity was 9 (range, 1-77), and a median of 12 female participants were included in studies with a numerical breakdown by sex. A mean of 41% (range, 1.55%-77.5%) of participants were female among studies that reported sex, and a mean of 30% (range, 1.96%-100%) of participants were from racial or ethnic minority groups among the 16 studies that reported race/ethnicity. Reporting of race/ethnicity varied substantially by funding source and journal type, while reporting by sex differed only by journal type. Conclusions and Relevance: Studies investigating hearing loss management do not adequately reflect the US population. A closer examination of the inclusion of diverse adults in clinical research associated with hearing health may work to ameliorate disparities and contribute to the development of tailored interventions that address the needs of an increasingly diverse US population.

Telemedicine and Dementia Care: A Systematic Review of Barriers and Facilitators.

OBJECTIVES: An increasing reliance on telemedicine for older adults with cognitive impairment requires a better understanding of the barriers and facilitators for this unique patient population. DESIGN: The study team queried PubMed, Embase, the Cochrane Library, PsycINFO, CINAHL, Scopus, and ClinicalTrials.gov on May 1, 2020, for studies in English published from January 2010 to May 2020. SETTING AND PARTICIPANTS: We conducted a systematic review of articles investigating the use of telemedicine among older adults with Alzheimer's disease and related dementia (ADRD) or mild cognitive impairment (MCI) that focused on the patient and care partner perspectives. METHODS: Telemedicine encounter purpose, technological requirements, and findings regarding sensory needs were extracted. The Cochrane Collaboration's Risk of Bias Tool was applied for quality assessment. RESULTS: The search yielded 3551 abstracts, from which 90 articles were reviewed and 17 were included. The purpose of telemedicine encounters included routine care, cognitive assessment, and telerehabilitation. All studies reported successful implementation of telemedicine, supported by patient and care partner satisfaction, similar results on cognitive assessment and diagnosis compared to in-person visits, and improvement in outcome measures following rehabilitation. Sixteen studies relied on staff and care partners to navigate technologies. Six studies reported participants reporting difficulty hearing the provider during the telemedicine visits. Five studies excluded participants with visual or hearing impairment because of the potential difficulty of using telemedicine technology. No studies reported technological adaptations to account for sensory impairment. CONCLUSIONS AND IMPLICATIONS: Telemedicine is well received among patients and care partners, but successful delivery incorporates support staff and the care partners to navigate technologies. The exclusion of older adults with sensory impairment, especially given that it is highly prevalent, in developing telemedicine systems may further exacerbate access to care in this population. Adapting technologies for sensory needs is critical to the advancement of accessible dementia care through telemedicine.

Hearing Loss and Home Health: An Unmet Need and an Opportunity for Action.

The purpose of this study was to identify unmet hearing care needs among older adults receiving home healthcare as a potential method to reach a population unserved by clinic-based care. Cross-sectional analyses were used to identify hearing loss and hearing aid use among beneficiaries enrolled in home care, using data from the nationally representative 2017 Medicare Current Beneficiary Survey (MCBS). Survey participants who reported enrollment in home healthcare services in 2017 (n = 3,183,693) were included for the purposes of analyses. Home health and hearing status were assessed through MCBS questionnaires, reported by Medicare beneficiaries or an appointed proxy, along with hearing status evaluated at initial home care intake. Among older Medicare beneficiaries receiving home healthcare, 51.8% self-reported hearing concerns. Of this population, only 16.1% reported hearing aid use. Recipients with hearing difficulty received an average of 30 visits per year, totaling to $5,208.25 in expenditure. By self-report, 44% of older home care recipients with hearing difficulty were misclassified as having "adequate" hearing on initial assessment and 31% of initial assessments identified hearing loss among those who self-reported no difficulty hearing. Effective management of chronic health conditions is core to healthy aging, including sensory health. Hearing loss represents a largely unaddressed need among home care recipients and frequently goes unidentified. Home-based hearing services that integrate into existing assessments and practices may be an avenue in identifying hearing loss and extending care to older adults who have traditionally gone without access to hearing intervention.

Computer Algorithm-Based Hydroxyurea Dosing Facilitates Titration to Maximum Tolerated Dose in Sickle Cell Anemia.

Adults with sickle cell disease (SCD) experience acute and chronic complications and die prematurely. When taken at maximum tolerated dose (MTD), hydroxyurea prolongs survival; however, it has not consistently reversed organ dysfunction. Patients also frequently do not take hydroxyurea, at least in part because of physician discomfort with prescribing hydroxyurea. We sought to develop a computer program that could easily titrate hydroxyurea to MTD. This was a single-arm, open-label pilot study. Fifteen patients with homozygous SCD were enrolled in the protocol, and 10 patients were followed at baseline and then for 1 year after hydroxyurea initiation or dose titration. Fetal hemoglobin significantly increased in all 10 patients from 8.3% to 25.1% (P < .001). Nine patients were titrated to MTD in an average of 7.9 months, and the tenth patient's hydroxyurea dose was increased to 33 mg/kg/day. Computer program dosing recommendations were the same as manual dosing decisions made using the same algorithm for all patients and at all times. We also evaluated markers of cardiopulmonary, liver and renal damage. Although cardiopulmonary function did not significantly improve, direct bilirubin and alanine aminotransferase levels significantly decreased (P < .001 and P < .01, respectively). Last, although kidney function did not improve, degree of proteinuria was significantly reduced (P < .05). We have developed a computer program that reliably titrates hydroxyurea to MTD. A larger study is indicated to test the program either as a computer program or a downloadable application.

Measuring Deformability and Red Cell Heterogeneity in Blood by Ektacytometry.

Decreased red cell deformability is characteristic of several disorders. In some cases, the extent of defective deformability can predict severity of disease or occurrence of serious complications. Ektacytometry uses laser diffraction viscometry to measure the deformability of red blood cells subject to either increasing shear stress or an osmotic gradient at a constant value of applied shear stress. However, direct deformability measurements are difficult to interpret when measuring heterogenous blood that is characterized by the presence of both rigid and deformable red cells. This is due to the inability of rigid cells to properly align in response to shear stress and results in a distorted diffraction pattern marked by an exaggerated decrease in apparent deformability. Measurement of the degree of distortion provides an indicator of the heterogeneity of the erythrocytes in blood. In sickle cell anemia, this is correlated with the percentage of rigid cells, which reflects the hemoglobin concentration and hemoglobin composition of the erythrocytes. In addition to measuring deformability, osmotic gradient ektacytometry provides information about the osmotic fragility and hydration status of erythrocytes. These parameters also reflect the hemoglobin composition of red blood cells from sickle cell patients. Ektacytometry measures deformability in populations of red cells and does not, therefore, provide information on the deformability or mechanical properties of individual erythrocytes. Regardless, the goal of the techniques described herein is to provide a convenient and reliable method for measuring the deformability and cellular heterogeneity of blood. These techniques may be useful for monitoring temporal changes, as well as disease progression and response to therapeutic intervention in several disorders. Sickle cell anemia is one well-characterized example. Other potential disorders where measurements of red cell deformability and/or heterogeneity are of interest include blood storage, diabetes, Plasmodium infection, iron deficiency, and the hemolytic anemias due to membrane defects.

Measurements of red cell deformability and hydration reflect HbF and HbA2 in blood from patients with sickle cell anemia.

Decreased erythrocyte deformability, as measured by ektacytometry, may be associated with disease severity in sickle cell anemia (SCA). Heterogeneous populations of rigid and deformable cells in SCA blood result in distortions of diffraction pattern measurements that correlate with the concentration of hemoglobin S (HbS) and the percentage of irreversibly sickled cells. We hypothesize that red cell heterogeneity, as well as deformability, will also be influenced by the concentration of alternative hemoglobins such as fetal hemoglobin (HbF) and the adult variant, HbA2. To test this hypothesis, we investigate the relationship between diffraction pattern distortion, osmotic gradient ektacytometry parameters, and the hemoglobin composition of SCA blood. We observe a correlation between the extent of diffraction pattern distortions and percentage of HbF and HbA2. Osmotic gradient ektacytometry data indicate that minimum elongation in the hypotonic region is positively correlated with HbF, as is the osmolality at which it occurs. The osmolality at both minimum and maximum elongation is inversely correlated with HbS and HbA2. These data suggest that HbF may effectively improve surface-to-volume ratio and osmotic fragility in SCA erythrocytes. HbA2 may be relatively ineffective in improving these characteristics or cellular hydration at the levels found in this patient cohort.