Estimation of Minor Conductive Hearing Loss in Humans Using Distortion Product Otoacoustic Emissions.

Objectives: Conductive hearing loss (CHL) systematically alters distortion product otoacoustic emission (DPOAE) levels through attenuation of both the primary tones and the evoked response by the middle ear, as well as through modification of the effective L1-L2 relationship within the cochlea. It has been postulated that, if optimal primary tone level relationships for an ear without CHL are known or can be estimated accurately and a CHL can be presumed to attenuate both primary tones to a similar extent, the adjustment to L1 required to restore an optimal L1-L2 separation following CHL induction can be utilized to estimate CHL magnitude objectively. The primary aim of this study was to assess the feasibility of objectively estimating experimentally produced CHL in humans by comparing CHL estimates resulting from DPOAE- and pure-tone audiometry-based methods. A secondary aim was to compare the accuracy of DPOAE-based CHL estimates when obtained using generic, as opposed to ear-specific, optimal primary tone level formula parameters. Design: For a single ear of 30 adults with normal hearing, auditory threshold for a 1 kHz tone was obtained using automated Bekesy audiometry at an ear-canal pressure of 0 daPa, as well as at a negative pressure sufficient for increasing threshold by 3 to 10 dB. The difference in threshold for the ear-canal pressure conditions was defined as the pure-tone audiometry-based estimate of CHL (CHLPT). For the same two ear-canal pressures, optimal DPOAE primary tone level relationships were identified for f2 = 1 kHz. Specifically, for 20

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Clinical and pathological characteristics of Fabry disease combined with IgA nephropathy in Chinese patients .

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Clinical and pathological characteristics of Fabry disease combined with IgA nephropathy in Chinese patients
.

Clin Nephrol. 2017 Feb 03;:

Authors: Yang N, Wang X, Xu F, Zeng C, Wang J, Liu Z

Abstract
AIMS: To improve diagnosis and treatment, we characterized Fabry disease combined with IgA nephropathy and its response to treatment clinically and pathologically in Chinese patients.
MATERIALS AND METHODS: Clinical and pathological characteristics of 6 Chinese patients with renal biopsy-proven Fabry disease combined with IgA nephropathy were retrospectively analyzed.
RESULTS: There were 4 males and 2 females in this study. All of the 6 patients presented with proteinuria. Microscopic hematuria was observed in case 4. Extrarenal symptoms included: acroparesthesia in case 1, 2, and 6, hypohidrosis in case 6 and angiokeratomas with hearing loss in case 3. By light microscopy, podocyte distension, with vacuolization, mesangial expansion, and interstitial lesions were found in all 6 cases; and focal segmental glomerulosclerosis was observed in 3 cases. Immunofluorescence microscopy showed deposition of IgA or predominant IgA with C3 in the mesangium. By electron microscopy, myelin figures and/or zebra bodies as well as electron-dense materials, were observed in the mesangium in the 6 cases. After admission and treatment with angiotensin converting enzyme inhibitors (ACEI) or angiotensin receptor blocker (ARB) together with immunosuppressant, glomerular filtration rate (GFR) decreased in 3 cases while it increased in the other 3 without statistical differences.
CONCLUSIONS: The clinical and pathological features of Fabry disease combined with IgA nephropathy are diverse. Therefore, it is important to combine family history, clinical manifestations, α-galactosidase A activity and pathological features, especially ultrastructural changes, to improve the diagnosis and treatment of the disease.
.

PMID: 28157068 [PubMed - as supplied by publisher]

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Deficits in Coordinative Bimanual Timing Precision in Children With Specific Language Impairment

Purpose

Our objective was to delineate components of motor performance in specific language impairment (SLI); specifically, whether deficits in timing precision in one effector (unimanual tapping) and in two effectors (bimanual clapping) are observed in young children with SLI.

Method

Twenty-seven 4- to 5-year-old children with SLI and 21 age-matched peers with typical language development participated. All children engaged in a unimanual tapping and a bimanual clapping timing task. Standard measures of language and motor performance were also obtained.

Results

No group differences in timing variability were observed in the unimanual tapping task. However, compared with typically developing peers, children with SLI were more variable in their timing precision in the bimanual clapping task. Nine of the children with SLI performed greater than 1 SD below the mean on a standardized motor assessment. The children with low motor performance showed the same profile as observed across all children with SLI, with unaffected unimanual and impaired bimanual timing precision.

Conclusions

Although unimanual timing is unaffected, children with SLI show a deficit in timing that requires bimanual coordination. We propose that the timing deficits observed in children with SLI are associated with the increased demands inherent in bimanual performance.

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Deficits in Coordinative Bimanual Timing Precision in Children With Specific Language Impairment

Purpose

Our objective was to delineate components of motor performance in specific language impairment (SLI); specifically, whether deficits in timing precision in one effector (unimanual tapping) and in two effectors (bimanual clapping) are observed in young children with SLI.

Method

Twenty-seven 4- to 5-year-old children with SLI and 21 age-matched peers with typical language development participated. All children engaged in a unimanual tapping and a bimanual clapping timing task. Standard measures of language and motor performance were also obtained.

Results

No group differences in timing variability were observed in the unimanual tapping task. However, compared with typically developing peers, children with SLI were more variable in their timing precision in the bimanual clapping task. Nine of the children with SLI performed greater than 1 SD below the mean on a standardized motor assessment. The children with low motor performance showed the same profile as observed across all children with SLI, with unaffected unimanual and impaired bimanual timing precision.

Conclusions

Although unimanual timing is unaffected, children with SLI show a deficit in timing that requires bimanual coordination. We propose that the timing deficits observed in children with SLI are associated with the increased demands inherent in bimanual performance.

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Deficits in Coordinative Bimanual Timing Precision in Children With Specific Language Impairment

Purpose

Our objective was to delineate components of motor performance in specific language impairment (SLI); specifically, whether deficits in timing precision in one effector (unimanual tapping) and in two effectors (bimanual clapping) are observed in young children with SLI.

Method

Twenty-seven 4- to 5-year-old children with SLI and 21 age-matched peers with typical language development participated. All children engaged in a unimanual tapping and a bimanual clapping timing task. Standard measures of language and motor performance were also obtained.

Results

No group differences in timing variability were observed in the unimanual tapping task. However, compared with typically developing peers, children with SLI were more variable in their timing precision in the bimanual clapping task. Nine of the children with SLI performed greater than 1 SD below the mean on a standardized motor assessment. The children with low motor performance showed the same profile as observed across all children with SLI, with unaffected unimanual and impaired bimanual timing precision.

Conclusions

Although unimanual timing is unaffected, children with SLI show a deficit in timing that requires bimanual coordination. We propose that the timing deficits observed in children with SLI are associated with the increased demands inherent in bimanual performance.

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Groundbreaking gene therapy restores hearing, balance

Improved gene therapy penetrates inner ear and restores hearing and balance in genetically deaf mice, finds Boston's Children's Hospital researchers.

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Sign language users have better reaction times and peripheral vision

People who use British Sign Language (BSL) have better reaction times in their peripheral vision, a new study from the University of Sheffield has found.

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Groundbreaking gene therapy restores hearing, balance

Improved gene therapy penetrates inner ear and restores hearing and balance in genetically deaf mice, finds Boston's Children's Hospital researchers.

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Sign language users have better reaction times and peripheral vision

People who use British Sign Language (BSL) have better reaction times in their peripheral vision, a new study from the University of Sheffield has found.

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Groundbreaking gene therapy restores hearing, balance

Improved gene therapy penetrates inner ear and restores hearing and balance in genetically deaf mice, finds Boston's Children's Hospital researchers.

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Sign language users have better reaction times and peripheral vision

People who use British Sign Language (BSL) have better reaction times in their peripheral vision, a new study from the University of Sheffield has found.

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The Change in Electrical Stimulation Levels During 24 Months Postimplantation for a Large Cohort of Adults Using the Nucleus(R) Cochlear Implant.

Objectives: To examine electrical stimulation data over 24 months postimplantation in adult implant users. The first aim was to calculate mean T and C levels for seven time points, for four cochlear segments, and two array types. The second aim was to (a) analyze the degree of change in each of the T and C levels as a function of dynamic range for six consecutive time point comparisons, for the four segments, and (b) to determine the proportion of participants with an acceptable degree of change. The third aim was to examine relationships between demographic factors and degree of change. Design: T levels, C levels, and dynamic ranges were extracted for 680 adults using Nucleus implants for the following postimplant time points: 2-, 3-, 6-, 9-, 12-, 18-, and 24-month. For each time point, mean levels were calculated for the four segments. The degree of change in each of the levels was analyzed for six consecutive time point comparisons. The criterion for an acceptable degree of change was 20% change in levels after 3 months postimplantation. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Directional Microphone Contralateral Routing of Signals in Cochlear Implant Users: A Within-Subjects Comparison.

Objectives: For medical or financial reasons, bilateral cochlear implantation is not always possible in bilaterally deafened patients. In such cases, a contralateral routing of signals (CROS) device could complement the monaural implant. The goal of our study was to compare the benefit of three different conditions: (1) unilateral cochlear implant (CI) alone, (2) unilateral CI complemented with a directional CROS microphone, and (3) bilateral CIs. Design: Twelve bilateral experienced CI users were tested. Speech reception in noise and sound localization were measured in the three above-mentioned conditions. Patients evaluated which condition they presumed to be activated and the subjective benefit on a hearing scale. Results: Compared with the unilateral CI condition, the additional CROS device provided significantly better speech intelligibility in noise when speech signals came from the front or side of the CROS microphone. Only small subjective improvement was observed. Bilateral-activated CIs further improved the hearing performance. This was the only condition where sound localization was possible. Subjective evaluation showed a clear preference for the bilateral CI treatment. Conclusions: In bilateral deafened patients, bilateral implantation is the most preferable form of treatment. However, patients with one implant only could benefit from an additional directional microphone CROS device. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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The Change in Electrical Stimulation Levels During 24 Months Postimplantation for a Large Cohort of Adults Using the Nucleus(R) Cochlear Implant.

Objectives: To examine electrical stimulation data over 24 months postimplantation in adult implant users. The first aim was to calculate mean T and C levels for seven time points, for four cochlear segments, and two array types. The second aim was to (a) analyze the degree of change in each of the T and C levels as a function of dynamic range for six consecutive time point comparisons, for the four segments, and (b) to determine the proportion of participants with an acceptable degree of change. The third aim was to examine relationships between demographic factors and degree of change. Design: T levels, C levels, and dynamic ranges were extracted for 680 adults using Nucleus implants for the following postimplant time points: 2-, 3-, 6-, 9-, 12-, 18-, and 24-month. For each time point, mean levels were calculated for the four segments. The degree of change in each of the levels was analyzed for six consecutive time point comparisons. The criterion for an acceptable degree of change was 20% change in levels after 3 months postimplantation. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Directional Microphone Contralateral Routing of Signals in Cochlear Implant Users: A Within-Subjects Comparison.

Objectives: For medical or financial reasons, bilateral cochlear implantation is not always possible in bilaterally deafened patients. In such cases, a contralateral routing of signals (CROS) device could complement the monaural implant. The goal of our study was to compare the benefit of three different conditions: (1) unilateral cochlear implant (CI) alone, (2) unilateral CI complemented with a directional CROS microphone, and (3) bilateral CIs. Design: Twelve bilateral experienced CI users were tested. Speech reception in noise and sound localization were measured in the three above-mentioned conditions. Patients evaluated which condition they presumed to be activated and the subjective benefit on a hearing scale. Results: Compared with the unilateral CI condition, the additional CROS device provided significantly better speech intelligibility in noise when speech signals came from the front or side of the CROS microphone. Only small subjective improvement was observed. Bilateral-activated CIs further improved the hearing performance. This was the only condition where sound localization was possible. Subjective evaluation showed a clear preference for the bilateral CI treatment. Conclusions: In bilateral deafened patients, bilateral implantation is the most preferable form of treatment. However, patients with one implant only could benefit from an additional directional microphone CROS device. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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The Change in Electrical Stimulation Levels During 24 Months Postimplantation for a Large Cohort of Adults Using the Nucleus(R) Cochlear Implant.

Objectives: To examine electrical stimulation data over 24 months postimplantation in adult implant users. The first aim was to calculate mean T and C levels for seven time points, for four cochlear segments, and two array types. The second aim was to (a) analyze the degree of change in each of the T and C levels as a function of dynamic range for six consecutive time point comparisons, for the four segments, and (b) to determine the proportion of participants with an acceptable degree of change. The third aim was to examine relationships between demographic factors and degree of change. Design: T levels, C levels, and dynamic ranges were extracted for 680 adults using Nucleus implants for the following postimplant time points: 2-, 3-, 6-, 9-, 12-, 18-, and 24-month. For each time point, mean levels were calculated for the four segments. The degree of change in each of the levels was analyzed for six consecutive time point comparisons. The criterion for an acceptable degree of change was 20% change in levels after 3 months postimplantation. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Directional Microphone Contralateral Routing of Signals in Cochlear Implant Users: A Within-Subjects Comparison.

Objectives: For medical or financial reasons, bilateral cochlear implantation is not always possible in bilaterally deafened patients. In such cases, a contralateral routing of signals (CROS) device could complement the monaural implant. The goal of our study was to compare the benefit of three different conditions: (1) unilateral cochlear implant (CI) alone, (2) unilateral CI complemented with a directional CROS microphone, and (3) bilateral CIs. Design: Twelve bilateral experienced CI users were tested. Speech reception in noise and sound localization were measured in the three above-mentioned conditions. Patients evaluated which condition they presumed to be activated and the subjective benefit on a hearing scale. Results: Compared with the unilateral CI condition, the additional CROS device provided significantly better speech intelligibility in noise when speech signals came from the front or side of the CROS microphone. Only small subjective improvement was observed. Bilateral-activated CIs further improved the hearing performance. This was the only condition where sound localization was possible. Subjective evaluation showed a clear preference for the bilateral CI treatment. Conclusions: In bilateral deafened patients, bilateral implantation is the most preferable form of treatment. However, patients with one implant only could benefit from an additional directional microphone CROS device. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Performance Plateau in Prelingually and Postlingually Deafened Adult Cochlear Implant Recipients.

Objective: To characterize the performance plateau after unilateral cochlear implantation (CI) in prelingually and postlingually deafened adults and to compare their relative progress. Study Design: Retrospective chart review. Setting: Tertiary referral center. Patients: Prelingually and postlingually deaf adults who received a unilateral CI and completed a minimum of 2 years of follow-up at our center. Intervention: Unilateral CI. Main Outcome Measures: : Standard speech perception testing (consonant-nucleus-consonant [CNC] monosyllabic word test and hearing in noise test [HINT] or AzBio sentence test) were performed preoperatively and 3 and 12 months postoperatively, and annually thereafter. Results: In postlingually deaf patients (n = 102), there was a significant improvement in word scores for 3 years postimplantation (p

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Cochlear Implantation in Children With Congenital Single-Sided Deafness.

Objective: To determine audiological and clinical results of cochlear implantation in children with congenital single sided deafness (SSD), with an emphasis on children implanted before and after 6 years of age. Study Design: Retrospective study. Setting: Tertiary referral center. Subjects: Twenty one children with congenital SSD who were implanted aged 10 months to 11;3 years. Intervention: Unilateral cochlear implantation. Main Outcome Measures: Speech recognition in noise via the German Oldenburg Sentence Test for Children (OlKiSa), lateralization ability, and subjective evaluation of hearing results using self- and third-party assessment questionnaires. Results: Significant improvements of all three aspects of true binaural hearing were found. The most striking improvement was the combined head shadow effect by 2.11 dB (squelch effect: 0.95 dB, summation effect 0.98 dB). An improvement of lateralization ability was also demonstrated. Parents had a high overall level of satisfaction with their children's cochlear implantation. Subjective benefit was verified in all three subscales of the Speech, Spatial, and Qualities of Hearing Questionnaire. No significant difference was found between subjects implanted before the age of 6 with those implanted later. Three of the five subjects with a follow-up of greater than 3 years were limited users or nonusers. Conclusions: Cochlear implant (CI) provision provides children with congenital SSD with significant audiological and subjective benefits which can be seen even in children implanted after the age of 3;6. The problem of limited use and nonuser, however, should not be ignored and has to be considered for further studies. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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The Role of Rho/Rho-Kinase Pathway in the Pathogenesis of Cholesteatoma.

Objective: To assess the role of Rho/Rho-kinase pathway in the pathogenesis of cholesteatoma. Materials and Methods: Thirty-eight patients with cholesteatoma, who had gone mastoidectomies were enrolled in this prospective study. Cholesteatomas matrix (CM) and a piece of the external ear canal skin (EECS as control) were taken and transferred to the liquid nitrogen and kept at -86 [degrees]C for Rho A and Rho-kinase (ROCK) analysis with Western blotting and commercial ELISA kits (Cell Biolabs Inc., San Diego, CA). The tissues were homogenized by an appropriate ice-cold lysis buffer. Following centrifugation, the supernatant was taken and total protein amount was detected by the Bradford method. Thereafter, tissue homogenates were subjected to sodium dodecyl sulphate (SDS)-polyacrylamide gel electrophoresis electrophoresis then transferred to nitrocellulose membrane where it was treated with specific monoclonal primary antibody against to ROCK-2 and HRP-conjugated seconder antibody, respectively. The protein blots were visualized with commercial x-ray film and dansitometrically analyzed by the Scion Image Program (Cell Biolabs Inc., San Diego, CA). In another series of experiments, Rho-kinase activities were assessed by ROCK-2 ELISA kits. Results: There were no statistical differences in Rho A translocation between CM and EECS. However, ROCK activity was found to be lower in CM than EECS as detected by ELISA kits. Furthermore, ROCK protein expression was also significantly lower in CM than EECS as demonstrated by Western blotting. Conclusion: Given Rho-kinase could take essential roles in cell differentiation, the results of this study implicate that down-regulated Rho-kinase could be responsible for the keratinocyte undifferentiation seen in cholesteatoma pathogenesis. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Conductive Hearing Loss From a Jugular Bulb Anomaly.

No abstract available

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Benign Temporomandibular Joint Lesions Presenting as Masses in the External Auditory Canal.

Objective: Describe benign lesions arising from the temporomandibular joint (TMJ) that presented as masses in the external auditory canal (EAC). Study Design: Retrospective case series of two academic medical centers. Patients: Six patients with lesions emanating from the TMJ that presented as EAC masses. Lesions included pigmented villonodular synovitis (PVNS), nodular fasciitis, foramen of Huschke herniation with salivary fistula, fibroepithelial polyp, superficial angiomyxoma, and giant cell tumor (GCT). Intervention: Surgical resection. Main Outcome Measures: Tumor control, TMJ function, and hearing. Results: All patients presented with hearing loss, TMJ pain, and otalgia. Three patients with EAC occluding masses developed entrapment cholesteatoma between the mass and tympanic membrane. Following surgical resection, four patients were free of disease. The patient with PVNS has a stable remnant at 3 years follow up and the patient with giant cell tumor is under active surveillance. Hearing improved in three patients, remained stable in one patient, and worsened in two patients. All had resolution of their TMJ and ear pain. Conclusions: EAC masses of uncertain origin should be imaged preoperatively to exclude lesions best not biopsied (e.g., encephalocele, facial nerve schwannoma, paraganglioma). In the differential of EAC masses, lesions emanating from the TMJ need to be considered, especially for those based anteriorly. Understanding the extent of TMJ involvement is crucial for surgical planning which optimizes outcome. Goals for therapy are to control the underlying disease process, restore patency of the EAC, improve hearing, and preserve function of TMJ. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Intraoperative Bleeding and the Risk of Residual Cholesteatoma: A Multivariate Analysis.

Objective: Residual cholesteatoma most frequently occurs where visualization and surgical access are restricted by anatomic constraints. Other factors that compromise surgical field visualization might also increase rates of residual cholesteatoma. We evaluated whether impaired surgical field clarity from bleeding increases rates of residual cholesteatoma. Study Design: Prospective cohort study. Setting: Tertiary care hospital. Patients: Consecutive series of children having intact canal wall surgery for cholesteatoma. Interventions: Impact of bleeding on surgical field clarity was assessed intraoperatively on a six-point scale. Main Outcome measures: Presence of residual cholesteatoma was established at follow up clinical encounters, second stage procedures, and with magnetic resonance imaging. Multiple logistic regression was used to determine the influence of surgical field clarity and other factors on rates of residual cholesteatoma. Results: Surgery was completed on 232 ears and residual cholesteatoma found in 45 (19%) ears. Multivariate regression analysis of cases completed with atticotomy or tympanoplasty demonstrated that surgical field clarity was a significant predictor of residual cholesteatoma (OR [odds ratio] 4, 95%CI 1.05-15; p = 0.04). Cholesteatoma extent was the most significant predictor of residual cholesteatoma when including cases requiring combined approach tympanomastoidectomy (OR 2.2, 95%CI 1.4-3.3; p

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