Monday, December 14, 2020

Precision Medicine in Ossiculoplasty

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Introduction: Long term results of ossiculoplasty surgery are considered poor with displacement and extrusion amongst the common reasons for failure. Application of 3Dimensional (3D) printing may help overcome some of these barriers, however digital methods to attain accurate 3D morphological studies of ossicular anatomy are lacking, exacerbated by the limitation of resolution of clinical imaging. Methods: 20 human cadaveric temporal bones were assessed using micro computed tomography (CT) imaging to demonstrate the lowest resolution required for accurate 3D reconstruction. The bones were then scanned using conebeam CT (125 μm) and helical CT (0.6 mm). 3D reconstruction using clinical imaging techniques with microCT imaging (40 μm resolution) as a reference was assessed. The incus was chosen as the focus of study. Two different methods of 3D printing techniques were assessed. Results: A minimum resolution of 100 μm was needed for adequate 3D reconstruction of the ossicular chain. Conebeam CT gave the most accurate data on 3D analysis, producing the smallest mean variation in surface topography data relative to microCT (mean difference 0.037 mm, p 
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Frequency-specific Electrocochleography and Traveling Wave Time as a Clinical Test for Menière's Disease

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Introduction: Menière's disease is a clinical entity with no definitive objective testing. It has been hypothesized that underlying endolymphatic hydrops stiffens the basilar membrane leading to increased speed of the acoustic stimulus, therefore traveling wave velocity has been proposed as an objective test to aid in the diagnosis. The objective of this study is to compare electrocochleography frequency-specific action potential latency, basilar membrane traveling wave time, and summation to action potential (SP/AP) ratio in Menière's and non-Menière's patients. Methods: Tympanic electrocochleography was performed with frequency-specific action potential latency time and SP/AP ratio recorded. Patient demographics, symptoms, audiogram data, AAO-HNS classification of Menière's disease, management interventions, and follow-up were recorded. Statistical analysis was performed to compare outcome measures across patient groups, demographics, and clinical data. Results: Ninety-one patients (182 ears) were included. There was a significant difference between a "definite" Menière's diagnosis and an "unlikely" or "probable" diagnosis by an average of 13 dB HL for the pure-tone thresholds at 250 Hz on the affected side (p = 0.006). There was no significant difference in pure-tone thresholds at any other frequency, AP latency at any frequency, or AP/SP ratio between the different Menière's classification groups. Conclusions: Our study fails to show significance of the traveling wave velocity as an objective test for Menière's disease. A significant correlation was found with low-frequency hearing loss between AAO-HNS Menière's classification groups. Address correspondence and reprint requests to Robert M. Conway, D.O., 27351 Dequindre Road, Madison Heights, MI 48071; E-mail: conwayr4@msu.edu S.C.B., M.D., Acclarent/Johnson & Johnson Honorarium, Cochlear Corporation research grant, Oticon Medical research grant. The authors disclose no conflicts of interest. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company
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Subcutaneous Air Around Receiver-Stimulator Causing Open-Circuit Failures: An Uncommon But Readily Treatable Condition Related to CPAP Use After Cochlear Implant Surgery

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Objective: To raise awareness of a unique complication associated with continuous positive airway pressure (CPAP) use after cochlear implantation to improve early detection, prevent unnecessary testing, and facilitate treatment. Study Design: Case series. Setting: Tertiary referral center. Subjects: Patients who developed subcutaneous air around the receiver-stimulator device and the associated ground electrode in the setting of CPAP usage, which resulted in open-circuit electrode failures. Main Outcome Measures: Clinical course and intervention. Results: Two patients were identified that fit this criterion. Both patients were noted to have poor implant performance secondary to high or open impedances and concomitant emphysema surrounding the cochlear implant receiver-stimulator. Manual massage offered transient improvement, but programming changes ultimately led to improved sound quality and resolution of impedance anomalies in both cases. Conclusions: Subcutaneous air collection surrounding the cochlear implant receiver-stimulator in the setting of CPAP use is an uncommon but clinically relevant complication that can be recognized by characteristic physical examination findings and impedance changes. Early and accurate recognition of this event can prevent unnecessary testing and facilitate early effective treatment. Address correspondence and reprint requests to Matthew L. Carlson, M.D., Department of Otolaryngology–Head and Neck Surgery, Mayo Clinic, 200 1st St SW, Rochester, MN 55905; E-mail: carlson.matthew@mayo.edu Financial Material & Support: This study was performed without commercial sponsorship or support. Institutional Review Board Approval: Mayo Clinc IRB Protocol 16-006130. Statement of Originality: This material has not been previously published in part or whole and is not currently under consideration for publication elsewhere. Aniket A. Saoji PhD is a consultant for Advanced Bionics and Envoy Medical. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company
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Radiological and Audiological Outcomes of the LISTENT LCI-20PI Cochlear Implant Device

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Objective: To study the surgical results, intracochlear position of the electrode array (EA) and auditory performance of the LISTENT LCI-20PI cochlear implant device, and daily use status at 3 years. Study Design: A retrospective study. Setting: A single-tertiary referral center. Patients: Between January and December 2016, 20 patients underwent cochlear implantation using the LISTENT LCI-20PI (lateral wall EA). Intervention: Cochlear implantation. Main Outcome Measures: Measurement of cochlear size, extent of posterior tympanotomy, and insertion depth. Scalar position of the EA evaluated by 3D reconstruction. Auditory outcomes 1 year after implantation and daily use status at 3 years. Results: EAs were completely inserted in all cases with an insertion depth of 288 ± 36.8 degrees. One year later, the average sentence recognition score (SRS) was 90 ± 21.7%. EA scalar location was analyzed in 18 patients. Thirteen EAs (72.2%) were fully inserted into the scala tympani (ST) and 5 (27.8%) had shifted from the ST to the scala vestibuli (SV). There was no statistically significant difference in cochlear size, extent of posterior tympanotomy, or insertion depth between these two groups. EAs inserted by cochleostomy had a higher chance of scalar shift than those inserted via the round window (60% vs 15.4%, p = 0.099). SRS at 1 year with full ST insertion was significantly better than in those with scalar shift (99 ± 1.3% vs 83 ± 16.5%, p = 0.002). Three years after implantation, 92% of patients were daily users and 46% were telephone users. Conclusions: The LISTENT LCI-20PI provided accredited hearing rehabilitation with a short insertion depth. Full insertion into the ST was associated with better cochlear implantation outcomes. Address correspondence and reprint requests to Huan Jia, M.D., Ph.D., Department of Otolaryngology Head and Neck Surgery, Sino-French Joint Clinic Research Center on Otology and Neuro-Otology, Shanghai Ninth People's Hospital, School of Medicine, Shanghai Jiaotong University, No 639 Zhizaoju Road, 200011 Shanghai, China; E-mail: huan.jia.orl@shsmu.edu.cn; Hao Wu, M.D., Ph.D., Department of Otolaryngology Head and Neck Surgery, Sino-French Joint Clinic Research Center on Otology and Neuro-Otology, Shanghai Ninth People's Hospital, School of Medicine, Shanghai Jiaotong University, No 639 Zhizaoju Road, 200011 Shanghai, China; E-mail: wuhao@shsmu.edu.cn H.T. and J.Y. contributed equally to this work. This study was independent of the clinical trial of LISTENT LCI-20PI. This work was supported by the Shanghai Scientific and Technological Innovation Action Plan (17441903600, 17ZR1416100). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. The authors disclose no conflicts of interest. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company
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Effect of Graft Size on Air-bone Gap Closure in Cartilage Button Tympanoplasty

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Objective: To study the effect of graft size on postoperative air-bone gap in children undergoing butterfly inlay cartilage tympanoplasty using circular punch grafts. Study Design: Retrospective case review. Setting: Tertiary, academic children's hospital. Patients: Children less than 16 years old undergoing circular butterfly inlay tympanoplasty using 4, 5, or 6 mm round grafts. Intervention: Butterfly inlay tympanoplasty using circular punch graft harvest technique. Main Outcome Measures: Postoperative pure-tone average and air-bone gap. Results: Fifty-two children were included in the analysis: 18 in the 4 mm group, 28 in the 5 mm group, and 6 in the 6 mm group. There was no significant difference in either postoperative pure-tone average or air-bone gap among the three groups. Closure rates for the 4, 5, and 6 mm graft groups were 94, 96, and 67%, respectively, for an overall rate of 92%. Conclusions: Cartilage button butterfly inlay tympanoplasty with punch graft is an effective method for tympanic membrane repair with similar hearing results among various graft diameters but may have diminished success with perforations requiring grafts larger than 5 mm. Larger case series are necessary to determine if larger defects are best managed with other repair techniques. Address correspondence and reprint requests to Jeffrey D. Carron, M.D., F.A.A.P., F.A.C.S., Department of Otolaryngology—Head and Neck Surgery, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS 39216; E-mail: jcarron@umc.edu The authors disclose no conflicts of interest. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company
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Projected Cost Simulation in Chronic Ear Surgery: Cost in Chronic Ear Surgery

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Objective: To examine the projected healthcare costs of intact canal wall (ICW) and canal wall down (CWD) tympanomastoidectomy surgery and associated clinic follow-up visits in the management of chronic ear disease. Study Design: A review of literature was performed to analyze the cholesteatoma recidivism rates of ICW and CWD tympanomastoidectomy. Projected Medicare cost simulation applied to representative samples of patients undergoing otologic surgery and follow-up clinic appointments projected over 15 years with an annual 25% cholesteatoma recidivism rate. Setting: Tertiary academic center. Patients: Simulated patient population status postchronic ear surgery and representative follow-up clinic visits with or without in-office mastoid debridement procedures. Interventions: Therapeutic and rehabilitative. Main Outcome Measures: Healthcare cost. Results: Simulation of cost for chronic ear surgery with follow-up visits produced a higher mean cost for the ICW group compared with the CWD group at year 1 ($9112.06 versus $4954.16). However, CWD patients accumulate cost over time due to routine mastoid debridements. The accumulated costs for the CWD group surpassed the ICW group at 12.54 (CI: 12.02–13.11) years. Conclusions: Using a projected cost analysis model, intact canal wall tympanomastoidectomy incurs a larger upfront cost than the canal wall down technique. However, long-term mastoid cavity maintenance costs accumulate to surpass the intact canal wall group at 12.54 years. Address correspondence and reprint requests to Matthew L. Kircher, M.D., 2160 S First Avenue, Maywood, IL 60153; E-mail: mkircher@lumc.edu IRB approved, LU # 211389. Level of Evidence: Level IV. The authors disclose no conflicts of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website (www.jcraniofacialsurgery.com). Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company
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Does Cognitive Dysfunction Correlate With Dizziness Severity in Meniére's Disease Patients

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Objective: Characterize the relationship between cognitive dysfunction and the dizziness severity in Meniére's disease (MD) patients. Study Design: Retrospective review. Setting: University-based tertiary medical center. Patients: Three hundred patients were evaluated for MD from 2015 to 2019. Excluding comorbid or alternative vestibular disorders, 29 patients with definite MD and available pre- and postintervention data were included for analysis. Interventions: A progressive protocol of salt restriction, diuretics, steroid and/or gentamycin injection, and endolymphatic sac decompression for those refractory to medical therapy. Main Outcome Measures: Quality of life measured with the Dizziness Handicap Inventory (DHI) and cognitive function measured with the Cognitive Failures Questionnaire (CFQ). Pre- and posttreatment DHI and DHI subscale scores and change in these scores were correlated with pre- and posttreatment CFQ scores and change in CFQ with therapy. Results: Analysis showed a number of limited associations between improvement in DHI and improvement in CFQ. Total DHI scores failed to correlate scores or variation in scores with change in CFQ (p = 0.091 and p = 0.085, respectively). Improvement in the CFQ false-triggering domain was significantly associated with improvement in the DHI physical subscale (r = 0.491, p = 0.007) and was nonsignificantly associated with improvement in total DHI and DHI emotional subscale scores (r = 0.422, p = 0.016 and r = 0.399, p = 0.032). Conclusions: The DHI correlates with several pre- and posttreatment measures of cognitive dysfunction (CFQ) in MD patients. However, change in DHI and CFQ with therapy correlate poorly. Overall, the commonly used DHI may fail to adequately assess cognitive dysfunction in MD patients possibly due to factors not directly implicated by measures of vestibular dysfunction, such as central nervous system or cognitive dysfunction; however, the specific physical and emotional subscales may offer helpful insight into cognitive dysfunction change/improvement with treatment. Address correspondence and reprint requests to Habib G. Rizk, M.D., 135 Rutledge Avenue, MSC 550, Charleston, SC 29425; E-mail: rizkh@musc.edu The authors disclose no conflicts of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website (). Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company
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Factors That Influence Second-side Cochlear Implant Speech Recognition Outcomes

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Objective: Assess relationships between patient, hearing, and cochlear implant (CI)-related factors and second-side CI speech recognition outcomes in adults who are bilaterally implanted. Study Design: Retrospective review of a prospectively maintained CI database. Setting: Tertiary academic center. Patients: One hundred two adults receiving bilateral sequential or simultaneous CIs. Outcome Measures: Postimplantation consonant-nucleus-consonant (CNC) word and AzBio sentence scores at ≥12 months. Results: Of patient, hearing and CI-specific, factors examined only postimplantation speech recognition scores of the first CI were independently associated with speech recognition performance of the second CI on multivariable regression analysis (CNC: ß = 0.471[0.298, 0.644]; AzBio: ß = 0.602[0.417, 0.769]). First-side postoperative CNC scores explained 24.3% of variation in second CI postoperative CNC scores, while change in first CI AzBio scores explained 40.3% of variation in second CI AzBio scores. Based on established 95% confidence intervals, 75.2% (CNC) and 65.9% (AzBio) of patients score equivalent or better with their second CI compared to first CI performance. Age at implantation, duration of hearing loss, receiving simultaneous versus sequential CIs, and preoperative residual hearing (measured by pure-tone average and aided speech recognition scores) were not associated with 12 month speech recognition scores at 12 months. Conclusions: The degree of improvement in speech recognition from first CI may predict speech recognition with a second CI. This provides preliminary evidence-based expectations for patients considering a second CI. Counseling should be guarded given the remaining unexplained variability in outcomes. Nonetheless, these data may assist decision making when considering a second CI versus continued use of a hearing aid for an unimplanted ear. Level of Evidence: III Address correspondence and reprint requests to Theodore R. McRackan, M.D., MSCR, 135 Rutledge Ave, MSC 550, Charleston, SC 29425; E-mail: mcrackan@musc.edu; James Dornhoffer, M.D., Medical University of South Carolina, Charleston, SC; E-mail: dornhoff@musc.edu J.D. and Y.F.L. contributed equally to this work. This research was partially supported by funding from a K23 award from National Institutes of Health Grant Number K23DC016911, and a grant from the American Cochlear Implant Alliance. The authors disclose no conflicts of interest. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company
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Potential Gene Therapy Trials in Autosomal Dominant Progressive Sensorineural Hearing Loss

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Background: Advances in gene therapeutic approaches to treat sensorineural hearing loss (SNHL) confront us with future challenges of translating these animal studies into clinical trials. Little is known on patient attitudes towards future innovative therapies. Objective: We aimed to better understand the willingness of patients with progressive SNHL and vestibular function loss of autosomal dominant (AD) inheritance to participate in potential gene therapy trials to prevent, stabilize, or slow down hearing loss. Methods: A survey was performed in carriers of the P51S and G88E pathogenic variant in the COCH gene (DFNA9). Various hypothetical scenarios were presented while using a Likert scale. Results: Fifty three participants were included, incl. 49 symptomatic patients, one presymptomatic patient, and three participants at risk. Their attitude towards potential trials studying innovative therapies was overall affirmative, even if the treatment would only slow down the decline of hearing and vestibular function, rather than cure the disease. Among the different potential scenarios, the less invasive and less frequent treatments increased the likelihood to enroll. Daily oral medication and annual intravenous infusion were awarded the highest scores. The more invasive, more frequent, and more at-risk treatments were still likely to be accepted but decreased the willingness to participate. The presence of a placebo arm was met with the lowest scores of willingness to participate. Conclusions: Overall, most symptomatic DFNA9 patients would likely consider participation in future innovative inner ear therapy trials, even if it would only slow down the decline of hearing and vestibular function. Address correspondence and reprint requests to Camille Levie, M.D., Department of Otorhinolaryngology and Head & Neck surgery, Antwerp University Hospital, Wilrijkstraat 10, 2610 Edegem, Belgium; E-mail: camille.levie@uza.be Disclosure of funding: The authors received no specific funding for this work. The authors disclose no conflicts of interest. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company
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Management of Intractable Petrous Bone Cholesteatoma With a Combined Translabyrinthine–Transsphenoidal Approach

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Objective: To evaluate whether a combined translabyrinthine–transsphenoidal approach can be used to achieve adequate surgical resection of an extensive petrous bone cholesteatoma and create a debris drainage route for the residual cholesteatoma that is maintained long-term. Patient: A 71-year-old man with residual petrous temporal bone cholesteatoma that had spread extensively to the internal carotid artery and posterior cranial fossa. Intervention: Surgical resection of the cholesteatoma via a translabyrinthine approach and creation of a debris drainage route into the nasopharynx via a transsphenoidal approach. Main Outcome Measures: Control of unresectable petrous temporal bone cholesteatoma and occurrence of cholesteatoma- or surgery-related complications. Results: Although complete removal of the cholesteatoma was attempted via a translabyrinthine approach, this was not possible because the epithelium of the cholesteatoma was strongly attached to the internal carotid artery and posterior cranial fossa. A debris drainage route leading to the nasopharynx was created by drilling the clivus on the side of the lesion via a transsphenoidal approach. The patient has had no complications since surgery. The drainage route remains open, and the cholesteatoma has been controlled for 57 months. Conclusions: A markedly advanced petrous temporal bone cholesteatoma can be managed safety and reliably by combining a translabyrinthine approach with a transsphenoidal approach. Creation of a debris drainage route into the nasopharynx can prevent isolation of the cholesteatoma and mastoid cavity problems after surgery. This one-stage surgery may be a suitable method for keeping cholesteatoma under control in patients with unresectable petrous bone cholesteatoma. Address correspondence and reprint requests to Tsunetaro Morino, M.D., Ph.D., Department of Otorhinolaryngology, Jikei University School of Medicine, 3-25-8 Nishi-shinbashi, Minato-ku, Tokyo, Japan; E-mail: moritune@jikei.ac.jp The study received no source of funding. The authors disclose no conflicts of interest. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company
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Subjective Visual Vertical Evaluation by a Smartphone-Based Test—Taking the Phone Out of the Bucket

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Objective: To measure the subjective visual vertical (SVV) in patients suffering from peripheral vestibular disorders versus controls, using a smartphone-based test designed to simulate the bucket test, in order to validate it as an available tool for the clinician. Study design: Prospective cohort study. Setting: Academic tertiary medical center. Patients: Forty-five adult patients were recruited to the study, 25 had vestibular disorders, and 20 did not (controls). Intervention: All patients underwent conventional bucket-SVV (b-SVV) and smartphone-based SVV (s-SVV) testing. Main outcome measures: Correlation and agreement of b-SVV and s-SVV scores in patients with peripheral vestibular disorders compared to controls. Results: SVV score in the vestibular disorders group was significantly higher compared to controls in both testing methods (p 
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