Performance-Intensity Function and Aided Improvement in Individuals With Late-Onset Auditory Neuropathy Spectrum Disorder.

Objectives: The purpose of the study was to explore the effect of intensity on speech perception in individuals with late-onset auditory neuropathy spectrum disorder (ANSD) by obtaining their performance-intensity (PI) function. Additionally, the study investigated the effect of PI function on aided speech perception. It was hypothesized that speech perception abilities of individuals with ANSD vary with intensity and may provide information regarding their aided performance. Design: A factorial research design was used to obtain the PI function and aided performance. Thirty individuals with late-onset ANSD in the age range of 14 to 42 years were evaluated. All the participants had bilateral mild to moderately-severe symmetrical hearing loss and were naive hearing aid users. A PI function was obtained for each participant for phonemically-balanced words. Further, aided performance was evaluated for binaural digital hearing aids fitted using the National Acoustic Laboratories-Non-linear 1 method. Results: It was found that the sensation level at which maximum word recognition scores were obtained varied across the participants. However, the majority of the participants had maximum scores below 30 dB SL. A large number of participants had a rollover PI function where the word recognition scores deteriorated with increase in presentation level. However, a few individuals demonstrated improvement in word recognition scores with increase in presentation level. Rollover index calculated from the PI function clearly demarcated those who had improved speech perception with hearing aids from those who did not. It was found that those with a rising pattern had aided speech identification scores that were significantly higher than their unaided scores. No such difference was found in those with a rollover. Further, aided benefit calculated by obtaining the difference between the aided and unaided scores was significantly higher in those with a rising PI function than those with a rollover function. Conclusions: The word recognition scores of individuals with ANSD varied with sensation level. This implies that obtaining the word recognition scores at a single presentation level may not accurately represent the speech perception abilities of those with ANSD. Further, a PI function helps differentiate those individuals with ANSD who benefit from hearing aids from those who do not. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Performance-Intensity Function and Aided Improvement in Individuals With Late-Onset Auditory Neuropathy Spectrum Disorder.

Objectives: The purpose of the study was to explore the effect of intensity on speech perception in individuals with late-onset auditory neuropathy spectrum disorder (ANSD) by obtaining their performance-intensity (PI) function. Additionally, the study investigated the effect of PI function on aided speech perception. It was hypothesized that speech perception abilities of individuals with ANSD vary with intensity and may provide information regarding their aided performance. Design: A factorial research design was used to obtain the PI function and aided performance. Thirty individuals with late-onset ANSD in the age range of 14 to 42 years were evaluated. All the participants had bilateral mild to moderately-severe symmetrical hearing loss and were naive hearing aid users. A PI function was obtained for each participant for phonemically-balanced words. Further, aided performance was evaluated for binaural digital hearing aids fitted using the National Acoustic Laboratories-Non-linear 1 method. Results: It was found that the sensation level at which maximum word recognition scores were obtained varied across the participants. However, the majority of the participants had maximum scores below 30 dB SL. A large number of participants had a rollover PI function where the word recognition scores deteriorated with increase in presentation level. However, a few individuals demonstrated improvement in word recognition scores with increase in presentation level. Rollover index calculated from the PI function clearly demarcated those who had improved speech perception with hearing aids from those who did not. It was found that those with a rising pattern had aided speech identification scores that were significantly higher than their unaided scores. No such difference was found in those with a rollover. Further, aided benefit calculated by obtaining the difference between the aided and unaided scores was significantly higher in those with a rising PI function than those with a rollover function. Conclusions: The word recognition scores of individuals with ANSD varied with sensation level. This implies that obtaining the word recognition scores at a single presentation level may not accurately represent the speech perception abilities of those with ANSD. Further, a PI function helps differentiate those individuals with ANSD who benefit from hearing aids from those who do not. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Cochleariform Process Abutment on TBCT in Early Congenital Cholesteatoma.

Objectives: To study the influence of the cochleariform process abutment (CPA) of early congenital cholesteatomas (CC) (ECCs) on surgical outcomes. Study Design: Retrospective case review. Setting: University hospital otology referral clinic. Patients: Two hundred consecutive pediatric ECC patients. Interventions: The patients were classified into three groups based on the temporal bone computed tomography (TBCT) findings of ECC: A) the absence of CPA, B) the presence of CPA, and C) the presence of CPA and extension posterior to the malleus handle. Main Outcome Measures: Residual CC according to each of the TBCT and surgical stages during 34.9 +/- 20.1 months of follow-up. Results: Group A was significantly associated with effortless delivery without matrix tearing (77%) even with simple myringotomy. Only 17% of group B experienced this outcome, and troublesome matrix tearing occurred in significantly more patients (p

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Reduction of the Harmonic Series Influences Musical Enjoyment With Cochlear Implants.

Objective: Cochlear implantation is associated with poor music perception and enjoyment. Reducing music complexity has been shown to enhance music enjoyment in cochlear implant (CI) recipients. In this study, we assess the impact of harmonic series reduction on music enjoyment. Study Design: Prospective analysis of music enjoyment in normal-hearing (NH) individuals and CI recipients. Setting: Single tertiary academic medical center. Patients: NH adults (N = 20) and CI users (N = 8) rated the Happy Birthday song on three validated enjoyment modalities-musicality, pleasantness, and naturalness. Intervention: Subjective rating of music excerpts. Main Outcome Measures: Participants listened to seven different instruments play the melody, each with five levels of harmonic reduction (Full, F3+F2+F1+F0, F2+F1+F0, F1+F0, F0). NH participants listened to the segments both with and without CI simulation. Linear mixed effect models (LME) and likelihood ratio tests were used to assess the impact of harmonic reduction on enjoyment. Results: NH listeners without simulation rated segments with the first four harmonics (F3+F2+F1+F0) most pleasant and natural (p

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Initial Operative Experience and Short-term Hearing Preservation Results With a Mid-scala Cochlear Implant Electrode Array.

Objective: To describe our initial operative experience and hearing preservation results with the Advanced Bionics (AB) Mid Scala Electrode (MSE). Study Design: Retrospective review. Setting: Tertiary referral center. Patients: Sixty-three MSE implants in pediatric and adult patients were compared with age- and sex-matched 1j electrode implants from the same manufacturer. All patients were severe to profoundly deaf. Intervention: Cochlear implantation with either the AB 1j electrode or the AB MSE. Main Outcome Measures: The MSE and 1j electrodes were compared in their angular depth of insertion and pre to postoperative change in hearing thresholds. Hearing preservation was analyzed as a function of angular depth of insertion. Secondary outcome measures included operative time, incidence of abnormal intraoperative impedance and telemetry values, and incidence of postsurgical complications. Results: Depth of insertion was similar for both electrodes, but was more consistent for the MSE array and more variable for the 1j array. Patients with MSE electrodes had better hearing preservation. Thresholds shifts at four audiometric frequencies ranging from 250 to 2000 Hz were 10, 7, 2, and 6 dB smaller for the MSE electrode than for the 1j (p

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Inhibiting p21-Activated Kinase Induces Cell Death in Vestibular Schwannoma and Meningioma via Mitotic Catastrophe.

Hypothesis: p21-activated kinase (PAK) regulates signaling pathways that promote cell survival and proliferation; therefore, pharmacological inhibition of PAK will induce cell death in vestibular schwannomas (VS) and meningiomas. Background: All VS and many meningiomas result from loss of the neurofibromatosis type 2 (NF2) gene product merlin, with ensuing PAK hyperactivation and increased cell proliferation/survival. Methods: The novel small molecule PAK inhibitors PI-8 and PI-15-tested in schwannoma and meningioma cells-perturb molecular signaling and induce cell death. 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide, flow cytometry, and terminal deoxynucleotidyl transferase dUTP nick end labeling assay analyzed PAK inhibitors' effect on cell viability, cell cycle, and cell death, respectively. Western blots evaluated activation and expression of cell proliferation, apoptotic, and mitotic catastrophe markers. Light microscopy evaluated cell morphology, and immunocytochemistry analyzed cellular localization of phospho-Merlin and autophagy-related protein. Results: Treatment with PI-8 and PI-15 decreased cell viability at 0.65 to 3.7 [mu]M 50% inhibitory concentration (IC50) in schwannoma and meningioma cells. Terminal deoxynucleotidyl transferase dUTP nick end labeling and immunocytochemistry studies show that PI-8 and PI-15 induce mitotic catastrophe but not apoptosis in HEI193 cells while in BenMen1 cells, PI-8 induces autophagy and mitotic catastrophe. PI-15 induces apoptosis in BenMen1 cells. PAK inhibitor treated cells show phospho-Merlin localized to over-duplicated centrosomes of dividing cells, multiple enlarged nuclei, and misaligned/missegregated chromosomes-markers for mitotic catastrophe. Increased autophagy-related protein levels in the nucleus confirmed this cell death type. PI-8 and PI-15 inhibits PAK in both cell lines. However, only PI-15 inhibits v-akt murine thymoma viral oncogene homolog in BenMen1 cells. Conclusion: PAK inhibitors induce cell death in schwannoma and meningioma cells, at least in part, by mitotic catastrophe. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Hemifacial Spasm Secondary to Parotid Pleomorphic Adenoma With Stylomastoid Foramen Extension.

Objective: To report hemifacial spasm (HFS) as a rare presenting symptom of a benign parotid neoplasm involving the stylomastoid foramen. Methods: Case report with literature review. Results: An 18-year-old woman presented with a 1-year history of a progressively enlarging right infra-auricular mass with new onset ipsilateral severe HFS. Clinical vetting unveiled a palpable 2 cm, firm, minimally mobile mass medial to the angle of the mandible. Imaging revealed a heterogeneous intraparotid mass with tumor extension into the stylomastoid foramen. Diagnostic consideration was given to neurogenic tumors and neoplasms of parotid origin. The patient ultimately underwent superficial parotidectomy and the tumor was found superficial and extrinsic to the proximal facial nerve trunk but extended into the stylomastoid foramen. The tumor was removed after performing mastoidectomy with mastoid segment facial nerve decompression. Frozen section pathology was consistent with pleomorphic adenoma. Postoperatively the patient had normal facial nerve function with immediate and complete resolution of HFS. To date there are only three other published reports of benign parotid tumors presenting with HFS and this is the first illustrating intratemporal tumor extension. Conclusions: HFS is a rare presenting symptom associated with benign parotid neoplasms with scarce documentation in the literature. In this index case, tumor extirpation with mastoid segment facial nerve decompression provided immediate and durable resolution of symptoms. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Tinnitus Suppression After Auditory Brainstem Implantation in Patients With Neurofibromatosis Type-2.

Objective: To evaluate whether an auditory brainstem implant (ABI) can impact levels of tinnitus in neurofibromatosis type-2 (NF2) patients who have undergone translabyrinthine craniotomy for vestibular schwannoma (VS) removal and to evaluate the burden of tinnitus in these patients. Study Design: A retrospective case series and patient survey. Setting: Tertiary neurotologic referral center. Patients: NF2 patients who underwent translabyrinthine removal of VS and ABI placement between 1994 and 2015. Interventions: A survey, retrospective review and two validated tinnitus handicap questionnaires (tinnitus handicap inventory [THI] and tinnitus visual analogue scale [VAS]) were used to characterize the degree of tinnitus in NF2 patients and whether an ABI can alter tinnitus levels. Main Outcome Measures(s): Survey results, THI and VAS scores. Results: One hundred twelve ABI users were contacted and 43 patients (38.3)% responded to our survey. Tinnitus was reported in 83.7% of patients. The THI score for responders was 17.8 +/- 20.5 standard deviation (SD). For survey participants, the ABI reduced tinnitus levels (mean VAS: Off = 3.5; On 1-h = 2.1; p = 0.048). For patients who subjectively reported that the ABI reduced tinnitus loudness, tinnitus levels were immediately reduced on ABI activation and after 1 hour of use (mean VAS: Off = 4.8; On = 2.4; On 1-h = 1.8; p

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Long-term Incidence and Degree of Sensorineural Hearing Loss in Otosclerosis.

Objectives: 1) To evaluate the long-term incidence and degree of the sensorineural component of hearing loss (SNHL) in patients with otosclerosis after accounting for expected age-related hearing loss. 2) To identify variables that might predict development of sensorineural hearing loss due to otosclerosis. Study Design: Retrospective audiometric database and chart review. Setting: Tertiary referral center. Patients: Consecutive patients with otosclerosis observed between 1994 and 2004, with >=10 years follow-up, excluding patients with postoperative hearing loss or surgery before the initial audiogram. Intervention: Bone conduction (BC) thresholds at 0.5, 1, 2, and 4 kHz and Word Recognition. Main Outcome Measure: BC threshold change (BCTC) over >=10 years minus estimated age-related threshold change (ARTC) specific to age and sex for each patient (based on ISO 7029 reference population). Results: Three-hundred fifty-seven ears (290 patients) met study criteria, including 217 ears that had undergone stapedectomy during the study period. Mean follow-up was 14.0 years. The average BCTC after subtracting estimated ARTC was 4.6, 2.6, 3, and 2.7 dB for 0.5, 1, 2, and 4 kHz frequencies, respectively. However, 34% of ears (122 ears) had clinically significant progression of SNHL during the study period (>10 dB BCTC beyond expected ARTC at >=2 frequencies). Multivariate analysis demonstrated that the probability of developing clinically significant SNHL was higher for women (odds ratio 1.86, p = 0.018) and lower for operated patients (odds ratio 0.46, p = 0.002). Conclusion: The average long-term sensorineural hearing loss due to otosclerosis was statistically significantly more than for age alone at each frequency, but these average values (from 2.6 to 4.6 dB for tested frequencies) were clinically insignificant. Approximately one-third of patients with otosclerosis demonstrated a clinically significant progression of the sensorineural component of hearing loss, with the average BCTC above expected age-related changes ranging from 10.2 to 14.6 dB for tested frequencies among this subgroup. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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The Role of Telemedicine in Auditory Rehabilitation: A Systematic Review.

Objective: The purpose of this study was to assess the feasibility and effectiveness of live telemedicine applications in hearing amplification and cochlear implantation. Data Sources and Study Selection: A systematic search was performed in PubMed, MEDLINE, PsychINFO, CINALH, and Web of Science to identify peer-reviewed research. Inclusion criteria were titles containing words from the search terms 1) audiology, otolaryngology, and hearing impairment, 2) rehabilitative methods, and 3) telemedicine. Exclusion criteria were: 1) non-English articles, and 2) non-original research. Data Extraction and Synthesis: Twelve eligible studies were identified. The studies employed a prospective design in nine of the articles and retrospective case series in three. The use of telemedicine for the provision of cochlear implant services was examined in eight of the articles and with hearing aids in four of the articles. The types of services include intraoperative cochlear implant telemetry; implant programming and assessment of electrode-specific measures and speech recognition after implantation. Hearing aid programming and remote gain assessments were also reported. Many studies assess patient and provider satisfaction along with encounter time comparison. The studies occurred from 2009 to 2014 and took place in seven countries. Conclusions: This review examined the feasibility of remote telemedicine connection to provide in auditory rehabilitation services through hearing aids and cochlear implants. There are significant concerns regarding Internet bandwidth limitations for remote clinics. There is a paucity of research examining reimbursement and cost-effectiveness for services. Further prospective research investigating cost-effectiveness and bandwidth limitations is warranted to assess long-term sustainability of remote audiological rehabilitative service delivery. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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A Retrospective Review of Temporal Bone Imaging With Respect to Bone-Anchored Hearing Aid Placement.

Objective: Current bone-anchored hearing aid (BAHA) guidelines recommend placement of the titanium implant 5 to 7 cm posterior to the ear canal. Previous studies show that bone conducted hearing is maximized the closer the transducer is to the cochlea. We aim to investigate the position of the sigmoid sinus with respect to BAHA implants to determine whether they may be safely placed closer to the ear canal in patients with chronic ear disease, enhancing the amplification available to the patient. Study Design: We performed a retrospective review of high-resolution temporal bone computed tomographies (CTs), comparing multiple measurements between ears with chronic ear disease and normal controls. Setting: Images were obtained at a single academic medical center. Patients: Eighty patients (160 ears) with temporal bone CTs performed between 2006 and 2009 were measured. Patients with chronic ear disease were identified by international statistical classification of diseases and related health problems, revision 9 code and confirmation by review of the imaging. Main Outcome Measures: Measurements were made on axial CT slices from a point 1 cm posterior to the sigmoid sinus to the posterior margin of the external canal. The squamous temporal bone thickness was also measured at this point. Results: Forty-seven patients (55 ears) had chronic ear disease. Distance from the posterior canal was significantly different between normal and diseased ears (36.3 mm versus 33.5 mm, p

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Factors Affecting Outcomes in Cochlear Implant Recipients Implanted With a Perimodiolar Electrode Array Located in Scala Tympani.

Objective: To identify primary biographic and audiologic factors contributing to cochlear implant (CI) performance variability in quiet and noise by controlling electrode array type and electrode position within the cochlea. Background: Although CI outcomes have improved over time, considerable outcome variability still exists. Biographic, audiologic, and device-related factors have been shown to influence performance. Examining CI recipients with consistent array type and electrode position may allow focused investigation into outcome variability resulting from biographic and audiologic factors. Methods: Thirty-nine adults (40 ears) implanted for at least 6 months with a perimodiolar electrode array known (via computed tomography [CT] imaging) to be in scala tympani participated. Test materials, administered CI only, included monosyllabic words, sentences in quiet and noise, and spectral ripple discrimination. Results: In quiet, scores were high with mean word and sentence scores of 76 and 87%, respectively; however, sentence scores decreased by an average of 35 percentage points when noise was added. A principal components (PC) analysis of biographic and audiologic factors found three distinct factors, PC1 Age, PC2 Duration, and PC3 Pre-op Hearing. PC1 Age was the only factor that correlated, albeit modestly, with speech recognition in quiet and noise. Spectral ripple discrimination strongly correlated with speech measures. Conclusion: For these recipients with consistent electrode position, PC1 Age was related to speech recognition performance. Consistent electrode position may have contributed to high speech understanding in quiet. Inter-subject variability in noise may have been influenced by auditory/cognitive processing, known to decline with age, and mechanisms that underlie spectral resolution ability. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Prevalence of Headache in Cochlear Implant Patients: A Cross-sectional Study.

Introduction: In Internet forums and other social media many reports regarding chronic headaches after cochlear implantation can be found. Although quite rare, there are also some reports in the literature. However, little is known regarding the true prevalence of headaches in persons who have undergone cochlear implant surgery. Objectives: The primary aim of this study was to investigate the 1-year prevalence of headache in patients having received a cochlear implantation ("cochlear implant group") in comparison with patients having undergone middle ear surgery ("surgery group") and persons with no history of head and neck surgery ("non-ear-nose-throat [ENT] group"). Methods: Cross-sectional, monocentric study using a validated headache questionnaire. Results: Three hundred persons were asked to participate. Two hundred thirty four valid questionnaires were returned. The participants' median age was 62 years, of whom 45% were women. The prevalence of headache was 31% (95%-confidence interval [CI]: [21; 42]) in the cochlear implant group and 46% (95%-CI: [35; 57]) in the surgery group with no significant difference between these two subgroups (p = 0.071). In the non-ENT group the prevalence of headache was significantly higher than in the other two subgroups (64%, 95%-CI: [52; 74]). Discussion: The prevalence of headache is not higher in cochlear implant patients in comparison to middle ear surgery patients, other, non-ENT patients and the general German or European population. Conclusion: Cochlear implantation does not seem to be associated with an increased risk for developing headache. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Ultra-High-Field Magnetic Resonance Imaging of the Human Inner Ear at 11.7 Tesla.

Objective: To evaluate the ability of ultra-high-field magnetic resonance imaging (UHF-MRI) at 11.7 T to visualize membranous structures of the human inner ear. Specimens: Three temporal bones were extracted from cadaveric human heads for use with small-bore UHF-MRI. Intervention: Ex vivo cadaveric temporal bone specimens were imaged using an 11.7 T magnetic resonance imaging (MRI) scanner via T1- and T2-weighted-imaging with and without contrast. Main Outcome Measure: Qualitative visualization of membranous components of the inner ear compared with reports of UHF-MRI at lower field strengths. Results: The membranous anatomy of the inner ear was superbly visualized at 11.7 T. In the cochlea, Reissner's membrane, the scala media, and the basilar membrane were clearly shown on the scan. In the vestibular labyrinth, the wedge-shaped crista ampullaris and the maculae of both the saccule and utricle were visible. Details of the endolymphatic sac and duct were also demonstrated. Conclusion: To our knowledge, this report presents the first images of the ex vivo human inner ear using 11.7 T UHF-MRI, offering near-histologic resolution. Increased field strength may be particularly useful when imaging the delicate membranous anatomy of the inner ear. Further research on the use of UHF-MRI in clinical and research settings could illuminate structural changes associated with inner ear disorders. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Usefulness of the Saccharin Test for Assessment of Eustachian Tube Function in Patients With Chronic Otitis Media With Perforation.

Objective: The eustachian tube (ET) has two important functions, ventilation and clearance of the middle ear. We evaluated the ET ventilator function by the sonotubometry and the inflation-deflation test that are widely used today, and the ET clearance function by the saccharin test. Results of both tests were compared with surgical outcomes, and assessed which test was more closely related to the surgical outcomes. Study Design: Prospective case series. Setting: A single university hospital. Patients and Intervention: One hundred four ears of 95 patients with chronic otitis media with perforation underwent type I tympanoplasty. Main Outcomes and Measures: Based on sonotubometry and the inflation-deflation test, the patients were divided into normal, stenosis, and patulous types. Based on the saccharin test, they were divided into a normal function group and a partial/gross dysfunction group. Surgical outcomes about the hearing results and the condition of the eardrum were assessed 1 year postoperatively. Preoperative evaluation of ET function was compared with the surgical outcome. Results: Sonotubometry and inflation-deflation tests showed there was no significant difference among the groups about the hearing improvement and the surgical success rate. The saccharin test showed that the rate of the hearing improvement was significantly lower in the gross dysfunction group and that the success rate of Type I tympanoplasty was significantly higher in the normal group than in the dysfunction group. Conclusion: There is a relationship between the saccharin test results and surgical outcomes. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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The Effect of Scala Tympani Morphology on Basilar Membrane Contact With a Straight Electrode Array: A Human Temporal Bone Study.

Hypothesis: Scala tympani morphology influences the insertion dynamics and intra-scalar position of straight electrode arrays. Background: Hearing preservation is the goal of cochlear implantation with current thin straight electrode arrays. These hug the lateral wall, facilitating full, atraumatic insertions. However, most studies still report some postoperative hearing loss. This study explores the influence of scala tympani morphology on array position relative to the basilar membrane and its possible contribution to postoperative hearing loss. Materials and Methods: Twenty-six fresh-frozen human temporal bones implanted with a straight electrode array were three-dimensionally reconstructed from micro-photographic histological sections. Insertion depth and the proximity between the array and basilar membrane were recorded. Lateral wall shape was quantified as a curvature ratio. Results: Insertion depths ranged from 233 to 470 degrees. The mean first point of contact between the array and basilar membrane was 185 degrees; arrays tended to remain in contact with the membrane after first contacting it. Eighty-nine and 93% of arrays that reached the upper basal (>240-360 degrees) and second (>360-720 degrees) turns respectively contacted the basilar membrane in these regions. Scalar wall curvature ratio decreased significantly (the wall became steeper) from the basal to second turns. This shift correlated with a reduced distance between the array and basilar membrane. Conclusion: Scala tympani morphology influences the insertion dynamics and intra-scalar position of a straight electrode array. In addition to gross trauma of cochlear structures, contact between the array and basilar membrane and how this impacts membrane function should be considered in hearing preservation cases. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Book Review.

No abstract available

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Outcomes of Drill Canalplasty in Exostoses and Osteoma: Analysis of 256 Cases and Literature Review.

Objective: 1) To describe the surgical technique of drill canaplasty for exostosis and osteoma and to evaluate our results. 2) To propose a new grading system for external auditory canal stenosis (EACS). 3) To review the recent literature. Study Design: A retrospective review. Setting: Quarternary referral center for Otology & Skull Base surgery. Subjects and Methods: Two hundred seventeen patients (256 ears) with exostosis or osteoma were included in the study. Surgical and audiological parameters were evaluated. Results: Mean age was 51.5 (+/-13.41) years. One hundred sixty nine cases were men and 48 women. Two hundred forty three (95%) cases were exostosis and 13 (5%) were osteomas. According to the proposed grading system, 81% ears had severe or complete stenosis. Seventy eight (30.5%) ears had a concurrent diagnosis of otosclerosis. Retroauricular approach was used in 245 (95.7%). Intraoperative complications included tympanic membrane (TM) perforation seen in four (2%) and mastoid cell exposure in two (1%). Postoperative stenosis was seen in 11 (4%) patients and required revision in seven cases. Audiologic analysis available for 153 ears-showed the mean change in air-bone gaps (ABG) of 2.18 dB, pure tone averages (PTA) bone conduction (BC) (0.5-4 kHz) of 0.3 dB. Mean healing rate was available for 246 (96.1%) patients and was found to be 6.35 (4-16) weeks. Conclusions: A systematically performed drill canalplasty via retroauricular approach, as described in this article, yields excellent postoperative outcomes as seen in our series. Notably, one-third of exostoses patients in this series, also suffered from otosclerosis. The proposed grading system for EACS enables the surgeon to objectively stage the disease. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Aging and Spectro-Temporal Integration of Speech

The purpose of this study was to determine the effects of age on the spectro-temporal integration of speech. The hypothesis was that the integration of speech fragments distributed over frequency, time, and ear of presentation is reduced in older listeners—even for those with good audiometric hearing. Younger, middle-aged, and older listeners (10 per group) with good audiometric hearing participated. They were each tested under seven conditions that encompassed combinations of spectral, temporal, and binaural integration. Sentences were filtered into two bands centered at 500 Hz and 2500 Hz, with criterion bandwidth tailored for each participant. In some conditions, the speech bands were individually square wave interrupted at a rate of 10 Hz. Configurations of uninterrupted, synchronously interrupted, and asynchronously interrupted frequency bands were constructed that constituted speech fragments distributed across frequency, time, and ear of presentation. The over-arching finding was that, for most configurations, performance was not differentially affected by listener age. Although speech intelligibility varied across condition, there was no evidence of performance deficits in older listeners in any condition. This study indicates that age, per se, does not necessarily undermine the ability to integrate fragments of speech dispersed across frequency and time.

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Aging and Spectro-Temporal Integration of Speech

The purpose of this study was to determine the effects of age on the spectro-temporal integration of speech. The hypothesis was that the integration of speech fragments distributed over frequency, time, and ear of presentation is reduced in older listeners—even for those with good audiometric hearing. Younger, middle-aged, and older listeners (10 per group) with good audiometric hearing participated. They were each tested under seven conditions that encompassed combinations of spectral, temporal, and binaural integration. Sentences were filtered into two bands centered at 500 Hz and 2500 Hz, with criterion bandwidth tailored for each participant. In some conditions, the speech bands were individually square wave interrupted at a rate of 10 Hz. Configurations of uninterrupted, synchronously interrupted, and asynchronously interrupted frequency bands were constructed that constituted speech fragments distributed across frequency, time, and ear of presentation. The over-arching finding was that, for most configurations, performance was not differentially affected by listener age. Although speech intelligibility varied across condition, there was no evidence of performance deficits in older listeners in any condition. This study indicates that age, per se, does not necessarily undermine the ability to integrate fragments of speech dispersed across frequency and time.

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Aging and Spectro-Temporal Integration of Speech

The purpose of this study was to determine the effects of age on the spectro-temporal integration of speech. The hypothesis was that the integration of speech fragments distributed over frequency, time, and ear of presentation is reduced in older listeners—even for those with good audiometric hearing. Younger, middle-aged, and older listeners (10 per group) with good audiometric hearing participated. They were each tested under seven conditions that encompassed combinations of spectral, temporal, and binaural integration. Sentences were filtered into two bands centered at 500 Hz and 2500 Hz, with criterion bandwidth tailored for each participant. In some conditions, the speech bands were individually square wave interrupted at a rate of 10 Hz. Configurations of uninterrupted, synchronously interrupted, and asynchronously interrupted frequency bands were constructed that constituted speech fragments distributed across frequency, time, and ear of presentation. The over-arching finding was that, for most configurations, performance was not differentially affected by listener age. Although speech intelligibility varied across condition, there was no evidence of performance deficits in older listeners in any condition. This study indicates that age, per se, does not necessarily undermine the ability to integrate fragments of speech dispersed across frequency and time.

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Early age noise exposure increases loudness perception - a novel animal model of hyperacusis

Publication date: Available online 13 October 2016
Source:Hearing Research
Author(s): Ana’am Alkharabsheh, Fen Xiong, Senthilvelan Manohar, Guangdi Chen, Richard Salvi, Wei Sun
The neural mechanisms that give rise to hyperacusis, a reduction in loudness tolerance, are largely unknown. Some reports suggest that hyperacusis is linked to childhood hearing loss. However, the evidence for this is largely circumstantial. In order to rigorously test this hypothesis, we studied loudness changes in rats caused by intense noise exposure (12 kHz narrow band noise, 115 dB SPL, 4 hours) at postnatal 16 days. Rats without noise exposure were used as controls. The exposed noise group (n = 7) showed a mean 40-50 dB hearing loss compared to the control group (n = 8) at high frequencies (>= 8 kHz) and less hearing loss at lower frequencies. Loudness was evaluated using sound reaction time and loudness response functions in an operant conditioning-based behavioral task using narrow-band noise (40-110 dB SPL, centered at 2, 4 and 12 kHz). Interestingly, the sound reaction time of the noise group was significantly shorter than the control group at supra-threshold levels. The average reaction time was less than 100 ms in the noise group at 100 dB SPL, which was three times shorter than the control group. Our results indicate that early noise-induced hearing loss leads to a significant increase of loudness, a behavior indicative of hyperacusis. Our results are consistent with clinical reports suggesting that hearing loss at an early age is a significant risk factor for hyperacusis.



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Developmental hearing loss impedes auditory task learning and performance in gerbils

Publication date: Available online 13 October 2016
Source:Hearing Research
Author(s): Gardiner von Trapp, Ishita Aloni, Stephen Young, Malcolm N. Semple, Dan H. Sanes
The consequences of developmental hearing loss have been reported to include both sensory and cognitive deficits. To investigate these issues in a non-human model, auditory learning and asymptotic psychometric performance were compared between normal hearing (NH) adult gerbils and those reared with conductive hearing loss (CHL). At postnatal day 10, before ear canal opening, gerbil pups underwent bilateral malleus removal to induce a permanent CHL. Both CHL and control animals were trained to approach a water spout upon presentation of a target (Go stimuli), and withhold for foils (Nogo stimuli). To assess the rate of task acquisition and asymptotic performance, animals were tested on an amplitude modulation (AM) rate discrimination task. Behavioral performance was calculated using a signal detection theory framework. Animals reared with developmental CHL displayed a slower rate of task acquisition for AM discrimination task. Slower acquisition was explained by an impaired ability to generalize to newly introduced stimuli, as compared to controls. Measurement of discrimination thresholds across consecutive testing blocks revealed that CHL animals required a greater number of testing sessions to reach asymptotic threshold values, as compared to controls. However, with sufficient training, CHL animals approached control performance. These results indicate that a sensory impediment can delay auditory learning, and increase the risk of poor performance on a temporal task.



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Early age noise exposure increases loudness perception - a novel animal model of hyperacusis

Publication date: Available online 13 October 2016
Source:Hearing Research
Author(s): Ana’am Alkharabsheh, Fen Xiong, Senthilvelan Manohar, Guangdi Chen, Richard Salvi, Wei Sun
The neural mechanisms that give rise to hyperacusis, a reduction in loudness tolerance, are largely unknown. Some reports suggest that hyperacusis is linked to childhood hearing loss. However, the evidence for this is largely circumstantial. In order to rigorously test this hypothesis, we studied loudness changes in rats caused by intense noise exposure (12 kHz narrow band noise, 115 dB SPL, 4 hours) at postnatal 16 days. Rats without noise exposure were used as controls. The exposed noise group (n = 7) showed a mean 40-50 dB hearing loss compared to the control group (n = 8) at high frequencies (>= 8 kHz) and less hearing loss at lower frequencies. Loudness was evaluated using sound reaction time and loudness response functions in an operant conditioning-based behavioral task using narrow-band noise (40-110 dB SPL, centered at 2, 4 and 12 kHz). Interestingly, the sound reaction time of the noise group was significantly shorter than the control group at supra-threshold levels. The average reaction time was less than 100 ms in the noise group at 100 dB SPL, which was three times shorter than the control group. Our results indicate that early noise-induced hearing loss leads to a significant increase of loudness, a behavior indicative of hyperacusis. Our results are consistent with clinical reports suggesting that hearing loss at an early age is a significant risk factor for hyperacusis.



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Developmental hearing loss impedes auditory task learning and performance in gerbils

Publication date: Available online 13 October 2016
Source:Hearing Research
Author(s): Gardiner von Trapp, Ishita Aloni, Stephen Young, Malcolm N. Semple, Dan H. Sanes
The consequences of developmental hearing loss have been reported to include both sensory and cognitive deficits. To investigate these issues in a non-human model, auditory learning and asymptotic psychometric performance were compared between normal hearing (NH) adult gerbils and those reared with conductive hearing loss (CHL). At postnatal day 10, before ear canal opening, gerbil pups underwent bilateral malleus removal to induce a permanent CHL. Both CHL and control animals were trained to approach a water spout upon presentation of a target (Go stimuli), and withhold for foils (Nogo stimuli). To assess the rate of task acquisition and asymptotic performance, animals were tested on an amplitude modulation (AM) rate discrimination task. Behavioral performance was calculated using a signal detection theory framework. Animals reared with developmental CHL displayed a slower rate of task acquisition for AM discrimination task. Slower acquisition was explained by an impaired ability to generalize to newly introduced stimuli, as compared to controls. Measurement of discrimination thresholds across consecutive testing blocks revealed that CHL animals required a greater number of testing sessions to reach asymptotic threshold values, as compared to controls. However, with sufficient training, CHL animals approached control performance. These results indicate that a sensory impediment can delay auditory learning, and increase the risk of poor performance on a temporal task.



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Early age noise exposure increases loudness perception - a novel animal model of hyperacusis

Publication date: Available online 13 October 2016
Source:Hearing Research
Author(s): Ana’am Alkharabsheh, Fen Xiong, Senthilvelan Manohar, Guangdi Chen, Richard Salvi, Wei Sun
The neural mechanisms that give rise to hyperacusis, a reduction in loudness tolerance, are largely unknown. Some reports suggest that hyperacusis is linked to childhood hearing loss. However, the evidence for this is largely circumstantial. In order to rigorously test this hypothesis, we studied loudness changes in rats caused by intense noise exposure (12 kHz narrow band noise, 115 dB SPL, 4 hours) at postnatal 16 days. Rats without noise exposure were used as controls. The exposed noise group (n = 7) showed a mean 40-50 dB hearing loss compared to the control group (n = 8) at high frequencies (>= 8 kHz) and less hearing loss at lower frequencies. Loudness was evaluated using sound reaction time and loudness response functions in an operant conditioning-based behavioral task using narrow-band noise (40-110 dB SPL, centered at 2, 4 and 12 kHz). Interestingly, the sound reaction time of the noise group was significantly shorter than the control group at supra-threshold levels. The average reaction time was less than 100 ms in the noise group at 100 dB SPL, which was three times shorter than the control group. Our results indicate that early noise-induced hearing loss leads to a significant increase of loudness, a behavior indicative of hyperacusis. Our results are consistent with clinical reports suggesting that hearing loss at an early age is a significant risk factor for hyperacusis.



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Developmental hearing loss impedes auditory task learning and performance in gerbils

Publication date: Available online 13 October 2016
Source:Hearing Research
Author(s): Gardiner von Trapp, Ishita Aloni, Stephen Young, Malcolm N. Semple, Dan H. Sanes
The consequences of developmental hearing loss have been reported to include both sensory and cognitive deficits. To investigate these issues in a non-human model, auditory learning and asymptotic psychometric performance were compared between normal hearing (NH) adult gerbils and those reared with conductive hearing loss (CHL). At postnatal day 10, before ear canal opening, gerbil pups underwent bilateral malleus removal to induce a permanent CHL. Both CHL and control animals were trained to approach a water spout upon presentation of a target (Go stimuli), and withhold for foils (Nogo stimuli). To assess the rate of task acquisition and asymptotic performance, animals were tested on an amplitude modulation (AM) rate discrimination task. Behavioral performance was calculated using a signal detection theory framework. Animals reared with developmental CHL displayed a slower rate of task acquisition for AM discrimination task. Slower acquisition was explained by an impaired ability to generalize to newly introduced stimuli, as compared to controls. Measurement of discrimination thresholds across consecutive testing blocks revealed that CHL animals required a greater number of testing sessions to reach asymptotic threshold values, as compared to controls. However, with sufficient training, CHL animals approached control performance. These results indicate that a sensory impediment can delay auditory learning, and increase the risk of poor performance on a temporal task.



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Early age noise exposure increases loudness perception - a novel animal model of hyperacusis

Publication date: Available online 13 October 2016
Source:Hearing Research
Author(s): Ana’am Alkharabsheh, Fen Xiong, Senthilvelan Manohar, Guangdi Chen, Richard Salvi, Wei Sun
The neural mechanisms that give rise to hyperacusis, a reduction in loudness tolerance, are largely unknown. Some reports suggest that hyperacusis is linked to childhood hearing loss. However, the evidence for this is largely circumstantial. In order to rigorously test this hypothesis, we studied loudness changes in rats caused by intense noise exposure (12 kHz narrow band noise, 115 dB SPL, 4 hours) at postnatal 16 days. Rats without noise exposure were used as controls. The exposed noise group (n = 7) showed a mean 40-50 dB hearing loss compared to the control group (n = 8) at high frequencies (>= 8 kHz) and less hearing loss at lower frequencies. Loudness was evaluated using sound reaction time and loudness response functions in an operant conditioning-based behavioral task using narrow-band noise (40-110 dB SPL, centered at 2, 4 and 12 kHz). Interestingly, the sound reaction time of the noise group was significantly shorter than the control group at supra-threshold levels. The average reaction time was less than 100 ms in the noise group at 100 dB SPL, which was three times shorter than the control group. Our results indicate that early noise-induced hearing loss leads to a significant increase of loudness, a behavior indicative of hyperacusis. Our results are consistent with clinical reports suggesting that hearing loss at an early age is a significant risk factor for hyperacusis.



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Developmental hearing loss impedes auditory task learning and performance in gerbils

Publication date: Available online 13 October 2016
Source:Hearing Research
Author(s): Gardiner von Trapp, Ishita Aloni, Stephen Young, Malcolm N. Semple, Dan H. Sanes
The consequences of developmental hearing loss have been reported to include both sensory and cognitive deficits. To investigate these issues in a non-human model, auditory learning and asymptotic psychometric performance were compared between normal hearing (NH) adult gerbils and those reared with conductive hearing loss (CHL). At postnatal day 10, before ear canal opening, gerbil pups underwent bilateral malleus removal to induce a permanent CHL. Both CHL and control animals were trained to approach a water spout upon presentation of a target (Go stimuli), and withhold for foils (Nogo stimuli). To assess the rate of task acquisition and asymptotic performance, animals were tested on an amplitude modulation (AM) rate discrimination task. Behavioral performance was calculated using a signal detection theory framework. Animals reared with developmental CHL displayed a slower rate of task acquisition for AM discrimination task. Slower acquisition was explained by an impaired ability to generalize to newly introduced stimuli, as compared to controls. Measurement of discrimination thresholds across consecutive testing blocks revealed that CHL animals required a greater number of testing sessions to reach asymptotic threshold values, as compared to controls. However, with sufficient training, CHL animals approached control performance. These results indicate that a sensory impediment can delay auditory learning, and increase the risk of poor performance on a temporal task.



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Early age noise exposure increases loudness perception - a novel animal model of hyperacusis

Publication date: Available online 13 October 2016
Source:Hearing Research
Author(s): Ana’am Alkharabsheh, Fen Xiong, Senthilvelan Manohar, Guangdi Chen, Richard Salvi, Wei Sun
The neural mechanisms that give rise to hyperacusis, a reduction in loudness tolerance, are largely unknown. Some reports suggest that hyperacusis is linked to childhood hearing loss. However, the evidence for this is largely circumstantial. In order to rigorously test this hypothesis, we studied loudness changes in rats caused by intense noise exposure (12 kHz narrow band noise, 115 dB SPL, 4 hours) at postnatal 16 days. Rats without noise exposure were used as controls. The exposed noise group (n = 7) showed a mean 40-50 dB hearing loss compared to the control group (n = 8) at high frequencies (>= 8 kHz) and less hearing loss at lower frequencies. Loudness was evaluated using sound reaction time and loudness response functions in an operant conditioning-based behavioral task using narrow-band noise (40-110 dB SPL, centered at 2, 4 and 12 kHz). Interestingly, the sound reaction time of the noise group was significantly shorter than the control group at supra-threshold levels. The average reaction time was less than 100 ms in the noise group at 100 dB SPL, which was three times shorter than the control group. Our results indicate that early noise-induced hearing loss leads to a significant increase of loudness, a behavior indicative of hyperacusis. Our results are consistent with clinical reports suggesting that hearing loss at an early age is a significant risk factor for hyperacusis.



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Developmental hearing loss impedes auditory task learning and performance in gerbils

Publication date: Available online 13 October 2016
Source:Hearing Research
Author(s): Gardiner von Trapp, Ishita Aloni, Stephen Young, Malcolm N. Semple, Dan H. Sanes
The consequences of developmental hearing loss have been reported to include both sensory and cognitive deficits. To investigate these issues in a non-human model, auditory learning and asymptotic psychometric performance were compared between normal hearing (NH) adult gerbils and those reared with conductive hearing loss (CHL). At postnatal day 10, before ear canal opening, gerbil pups underwent bilateral malleus removal to induce a permanent CHL. Both CHL and control animals were trained to approach a water spout upon presentation of a target (Go stimuli), and withhold for foils (Nogo stimuli). To assess the rate of task acquisition and asymptotic performance, animals were tested on an amplitude modulation (AM) rate discrimination task. Behavioral performance was calculated using a signal detection theory framework. Animals reared with developmental CHL displayed a slower rate of task acquisition for AM discrimination task. Slower acquisition was explained by an impaired ability to generalize to newly introduced stimuli, as compared to controls. Measurement of discrimination thresholds across consecutive testing blocks revealed that CHL animals required a greater number of testing sessions to reach asymptotic threshold values, as compared to controls. However, with sufficient training, CHL animals approached control performance. These results indicate that a sensory impediment can delay auditory learning, and increase the risk of poor performance on a temporal task.



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The importance of parkinsonian signs for gait and balance in patients with Alzheimer’s disease of mild degree

Publication date: Available online 14 October 2016
Source:Gait & Posture
Author(s): Gro Gujord Tangen, Astrid Bergland, Knut Engedal, Anne Marit Mengshoel
Parkinsonian signs are common in patients with Alzheimer’s disease (AD) of mild degree and predict functional decline, but their relationship with gait speed and balance is unclear. The aims of this study were to describe characteristics of patients with parkinsonian signs among 98 patients with AD of mild degree (with no comorbid Parkinson’s disease), and to examine associations between parkinsonian signs with gait speed and balance. A cross sectional study at a memory clinic was conducted. Presence of each parkinsonian sign (bradykinesia, rigidity and tremor) was derived from the UPDRS, regular gait speed was recorded over 10meters and balance was assessed using the Mini-Balance Evaluation Systems Test (Mini-BESTest). Bradykinesia was present in 30.6% of the sample, rigidity in 13.3% and tremor only in one patient. Patients with bradykinesia were older, had worse cognitive impairment and worse gait and balance performance than those without bradykinesia. More men than women had rigidity. Bradykinesia was significantly associated with mini-BESTest after adjusting for demographic factors (p<0.001, explaining 13.3% of the variance), but was not significantly associated with gait speed. Rigidity was not associated with either gait speed or balance. We conclude that assessment of bradykinesia should be included in examination of balance control in patients with AD of mild degree.



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Correcting waveform bias using principal component analysis: applications in multicentre motion analysis studies

Publication date: Available online 13 October 2016
Source:Gait & Posture
Author(s): Allison L. Clouthier, Eric R. Bohm, John F. Rudan, Barbara L. Shay, Michael J. Rainbow, Kevin J. Deluzio
Multicentre studies are rare in three dimensional motion analyses due to challenges associated with combining waveform data from different centres. Principal component analysis (PCA) is a statistical technique that can be used to quantify variability in waveform data and identify group differences. A correction technique based on PCA is proposed that can be used in post processing to remove nuisance variation introduced by the differences between centres. Using this technique, the waveform bias that exists between the two datasets is corrected such that the means agree. No information is lost in the individual datasets, but the overall variability in the combined data is reduced. The correction is demonstrated on gait kinematics with synthesized cross-talk and on gait data from knee arthroplasty patients collected in two centres. The induced cross-talk was successfully removed from the knee joint angle data. In the second example, the removal of the nuisance variation due to the multicentre data collection allowed significant differences in implant type to be identified. This PCA-based technique can be used to correct for differences between waveform datasets in post processing and has the potential to enable multicentre motion analysis studies.



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The importance of parkinsonian signs for gait and balance in patients with Alzheimer’s disease of mild degree

Publication date: Available online 14 October 2016
Source:Gait & Posture
Author(s): Gro Gujord Tangen, Astrid Bergland, Knut Engedal, Anne Marit Mengshoel
Parkinsonian signs are common in patients with Alzheimer’s disease (AD) of mild degree and predict functional decline, but their relationship with gait speed and balance is unclear. The aims of this study were to describe characteristics of patients with parkinsonian signs among 98 patients with AD of mild degree (with no comorbid Parkinson’s disease), and to examine associations between parkinsonian signs with gait speed and balance. A cross sectional study at a memory clinic was conducted. Presence of each parkinsonian sign (bradykinesia, rigidity and tremor) was derived from the UPDRS, regular gait speed was recorded over 10meters and balance was assessed using the Mini-Balance Evaluation Systems Test (Mini-BESTest). Bradykinesia was present in 30.6% of the sample, rigidity in 13.3% and tremor only in one patient. Patients with bradykinesia were older, had worse cognitive impairment and worse gait and balance performance than those without bradykinesia. More men than women had rigidity. Bradykinesia was significantly associated with mini-BESTest after adjusting for demographic factors (p<0.001, explaining 13.3% of the variance), but was not significantly associated with gait speed. Rigidity was not associated with either gait speed or balance. We conclude that assessment of bradykinesia should be included in examination of balance control in patients with AD of mild degree.



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Correcting waveform bias using principal component analysis: applications in multicentre motion analysis studies

Publication date: Available online 13 October 2016
Source:Gait & Posture
Author(s): Allison L. Clouthier, Eric R. Bohm, John F. Rudan, Barbara L. Shay, Michael J. Rainbow, Kevin J. Deluzio
Multicentre studies are rare in three dimensional motion analyses due to challenges associated with combining waveform data from different centres. Principal component analysis (PCA) is a statistical technique that can be used to quantify variability in waveform data and identify group differences. A correction technique based on PCA is proposed that can be used in post processing to remove nuisance variation introduced by the differences between centres. Using this technique, the waveform bias that exists between the two datasets is corrected such that the means agree. No information is lost in the individual datasets, but the overall variability in the combined data is reduced. The correction is demonstrated on gait kinematics with synthesized cross-talk and on gait data from knee arthroplasty patients collected in two centres. The induced cross-talk was successfully removed from the knee joint angle data. In the second example, the removal of the nuisance variation due to the multicentre data collection allowed significant differences in implant type to be identified. This PCA-based technique can be used to correct for differences between waveform datasets in post processing and has the potential to enable multicentre motion analysis studies.



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The importance of parkinsonian signs for gait and balance in patients with Alzheimer’s disease of mild degree

Publication date: Available online 14 October 2016
Source:Gait & Posture
Author(s): Gro Gujord Tangen, Astrid Bergland, Knut Engedal, Anne Marit Mengshoel
Parkinsonian signs are common in patients with Alzheimer’s disease (AD) of mild degree and predict functional decline, but their relationship with gait speed and balance is unclear. The aims of this study were to describe characteristics of patients with parkinsonian signs among 98 patients with AD of mild degree (with no comorbid Parkinson’s disease), and to examine associations between parkinsonian signs with gait speed and balance. A cross sectional study at a memory clinic was conducted. Presence of each parkinsonian sign (bradykinesia, rigidity and tremor) was derived from the UPDRS, regular gait speed was recorded over 10meters and balance was assessed using the Mini-Balance Evaluation Systems Test (Mini-BESTest). Bradykinesia was present in 30.6% of the sample, rigidity in 13.3% and tremor only in one patient. Patients with bradykinesia were older, had worse cognitive impairment and worse gait and balance performance than those without bradykinesia. More men than women had rigidity. Bradykinesia was significantly associated with mini-BESTest after adjusting for demographic factors (p<0.001, explaining 13.3% of the variance), but was not significantly associated with gait speed. Rigidity was not associated with either gait speed or balance. We conclude that assessment of bradykinesia should be included in examination of balance control in patients with AD of mild degree.



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Correcting waveform bias using principal component analysis: applications in multicentre motion analysis studies

Publication date: Available online 13 October 2016
Source:Gait & Posture
Author(s): Allison L. Clouthier, Eric R. Bohm, John F. Rudan, Barbara L. Shay, Michael J. Rainbow, Kevin J. Deluzio
Multicentre studies are rare in three dimensional motion analyses due to challenges associated with combining waveform data from different centres. Principal component analysis (PCA) is a statistical technique that can be used to quantify variability in waveform data and identify group differences. A correction technique based on PCA is proposed that can be used in post processing to remove nuisance variation introduced by the differences between centres. Using this technique, the waveform bias that exists between the two datasets is corrected such that the means agree. No information is lost in the individual datasets, but the overall variability in the combined data is reduced. The correction is demonstrated on gait kinematics with synthesized cross-talk and on gait data from knee arthroplasty patients collected in two centres. The induced cross-talk was successfully removed from the knee joint angle data. In the second example, the removal of the nuisance variation due to the multicentre data collection allowed significant differences in implant type to be identified. This PCA-based technique can be used to correct for differences between waveform datasets in post processing and has the potential to enable multicentre motion analysis studies.



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Measuring Disability in Population Based Surveys: The Interrelationship between Clinical Impairments and Reported Functional Limitations in Cameroon and India

by Islay Mactaggart, Hannah Kuper, G. V. S. Murthy, Joseph Oye, Sarah Polack

Purpose

To investigate the relationship between two distinct measures of disability: self-reported functional limitations and objectively-screened clinical impairments.

Methods

We undertook an all age population-based survey of disability in two areas: North-West Cameroon (August/October 2013) and Telangana State, India (Feb/April 2014). Participants were selected for inclusion via two-stage cluster randomised sampling (probability proportionate to size cluster selection and compact segment sampling within clusters). Disability was defined as the presence of self-reported functional limitations across eight domains, or presence of moderate or greater clinical impairments. Clinical impairment screening comprised of visual acuity testing for vision impairment, pure tone audiometry for hearing impairment, musculoskeletal functioning assessment for musculoskeletal impairment, reported seizure history for epilepsy and reported symptoms of clinical depression (depression adults only). Information was collected using structured questionnaires, observations and examinations.

Results

Self-reported disability prevalence was 5.9% (95% CI 4.7–7.4) and 7.5% (5.9–9.4) in Cameroon and India respectively. The prevalence of moderate or greater clinical impairments in the same populations were 8.4% (7.5–9.4) in Cameroon and 10.5% (9.4–11.7) in India. Overall disability prevalence (self-report and/or screened positive to a moderate or greater clinical impairment) was 10.5% in Cameroon and 12.2% in India, with limited overlap between the sub-populations identified using the two types of tools. 33% of participants in Cameroon identified to have a disability, and 45% in India, both reported functional limitations and screened positive to objectively-screened impairments, whilst the remainder were identified via one or other tool only. A large proportion of people with moderate or severe clinical impairments did not self-report functional difficulties despite reporting participation restrictions.

Conclusion

Tools to assess reported functional limitation alone are insufficient to identify all persons with participation restrictions and moderate or severe clinical impairments. A self-reported functional limitation tool followed by clinical screening of all those who report any level of difficulty would identify 94% of people with disabilities in Cameroon and 95% in India, meeting the study criteria.

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Medal Music

Athletes from the United States won 117 medals. Those from Ukraine, Great Britain, and China won even more. We are talking about medals at the 2016 Paralympics that followed the summer Olympics in Rio da Janeiro. While the summer Olympics get all their deserved attention, the Paralympics that follow hold a place of particular importance in the Olympic movement. This year’s Paralympics in Rio had a unique twist for us sound geeks.

Tweet

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Assessment of traffic noise levels in urban areas using different soft computing techniques

Available traffic noise prediction models are usually based on regression analysis of experimental data, and this paper presents the application of soft computing techniques in traffic noise prediction. Two mathematical models are proposed and their predictions are compared to data collected by traffic noise monitoring in urban areas, as well as to predictions of commonly used traffic noise models. The results show that application of evolutionary algorithms and neural networks may improve process of development, as well as accuracy of traffic noise prediction.

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The bursts of stops can convey dialectal information

This study investigates the effects of the dialect of the speaker on the spectral properties of stop bursts. Forty-five female speakers—20 Standard Modern Greek and 25 Cypriot Greek speakers—participated in this study. The spectral properties of stop bursts were calculated from the burst spectra and analyzed using spectral moments. The findings show that besides linguistic information, i.e., the place of articulation and the stress, the speech signals of bursts can encode social information, i.e., the dialects. A classification model using decision trees showed that skewness and standard deviation have a major contribution for the classification of bursts across dialects.

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Maturation of Spontaneous Firing Properties after Hearing Onset in Rat Auditory Nerve Fibers: Spontaneous Rates, Refractoriness, and Interfiber Correlations.

Related Articles

Maturation of Spontaneous Firing Properties after Hearing Onset in Rat Auditory Nerve Fibers: Spontaneous Rates, Refractoriness, and Interfiber Correlations.

J Neurosci. 2016 Oct 12;36(41):10584-10597

Authors: Wu JS, Young ED, Glowatzki E

Abstract
Auditory nerve fibers (ANFs) exhibit a range of spontaneous firing rates (SRs) that are inversely correlated with threshold for sounds. To probe the underlying mechanisms and time course of SR differentiation during cochlear maturation, loose-patch extracellular recordings were made from ANF dendrites using acutely excised rat cochlear preparations of different ages after hearing onset. Diversification of SRs occurred mostly between the second and the third postnatal week. Statistical properties of ANF spike trains showed developmental changes that approach adult-like features in older preparations. Comparison with intracellularly recorded EPSCs revealed that most properties of ANF spike trains derive from the characteristics of presynaptic transmitter release. Pharmacological tests and waveform analysis showed that endogenous firing produces some fraction of ANF spikes, accounting for their unusual properties; the endogenous firing diminishes gradually during maturation. Paired recordings showed that ANFs contacting the same inner hair cell could have different SRs, with no correlation in their spike timing.
SIGNIFICANCE STATEMENT: The inner hair cell (IHC)/auditory nerve fiber (ANF) synapse is the first synapse of the auditory pathway. Remarkably, each IHC is the sole partner of 10-30 ANFs with a range of spontaneous firing rates (SRs). Low and high SR ANFs respond to sound differently, and both are important for encoding sound information across varying acoustical environments. Here we demonstrate SR diversification after hearing onset by afferent recordings in acutely excised rat cochlear preparations. We describe developmental changes in spike train statistics and endogenous firing in immature ANFs. Dual afferent recordings provide the first direct evidence that fibers with different SRs contact the same IHCs and do not show correlated spike timing at rest. These results lay the groundwork for understanding the differential sensitivity of ANFs to acoustic trauma.

PMID: 27733610 [PubMed - in process]

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Maturation of Spontaneous Firing Properties after Hearing Onset in Rat Auditory Nerve Fibers: Spontaneous Rates, Refractoriness, and Interfiber Correlations.

Related Articles

Maturation of Spontaneous Firing Properties after Hearing Onset in Rat Auditory Nerve Fibers: Spontaneous Rates, Refractoriness, and Interfiber Correlations.

J Neurosci. 2016 Oct 12;36(41):10584-10597

Authors: Wu JS, Young ED, Glowatzki E

Abstract
Auditory nerve fibers (ANFs) exhibit a range of spontaneous firing rates (SRs) that are inversely correlated with threshold for sounds. To probe the underlying mechanisms and time course of SR differentiation during cochlear maturation, loose-patch extracellular recordings were made from ANF dendrites using acutely excised rat cochlear preparations of different ages after hearing onset. Diversification of SRs occurred mostly between the second and the third postnatal week. Statistical properties of ANF spike trains showed developmental changes that approach adult-like features in older preparations. Comparison with intracellularly recorded EPSCs revealed that most properties of ANF spike trains derive from the characteristics of presynaptic transmitter release. Pharmacological tests and waveform analysis showed that endogenous firing produces some fraction of ANF spikes, accounting for their unusual properties; the endogenous firing diminishes gradually during maturation. Paired recordings showed that ANFs contacting the same inner hair cell could have different SRs, with no correlation in their spike timing.
SIGNIFICANCE STATEMENT: The inner hair cell (IHC)/auditory nerve fiber (ANF) synapse is the first synapse of the auditory pathway. Remarkably, each IHC is the sole partner of 10-30 ANFs with a range of spontaneous firing rates (SRs). Low and high SR ANFs respond to sound differently, and both are important for encoding sound information across varying acoustical environments. Here we demonstrate SR diversification after hearing onset by afferent recordings in acutely excised rat cochlear preparations. We describe developmental changes in spike train statistics and endogenous firing in immature ANFs. Dual afferent recordings provide the first direct evidence that fibers with different SRs contact the same IHCs and do not show correlated spike timing at rest. These results lay the groundwork for understanding the differential sensitivity of ANFs to acoustic trauma.

PMID: 27733610 [PubMed - in process]

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Brain Activity in Patients With Adductor Spasmodic Dysphonia Detected by Functional Magnetic Resonance Imaging

Publication date: Available online 13 October 2016
Source:Journal of Voice
Author(s): Asanori Kiyuna, Norimoto Kise, Munehisa Hiratsuka, Shunsuke Kondo, Takayuki Uehara, Hiroyuki Maeda, Akira Ganaha, Mikio Suzuki
ObjectivesSpasmodic dysphonia (SD) is considered a focal dystonia. However, the detailed pathophysiology of SD remains unclear, despite the detection of abnormal activity in several brain regions. The aim of this study was to clarify the pathophysiological background of SD.Study DesignThis is a case-control study.MethodsBoth task-related brain activity measured by functional magnetic resonance imaging by reading the five-digit numbers and resting-state functional connectivity (FC) measured by 150 T2-weighted echo planar images acquired without any task were investigated in 12 patients with adductor SD and in 16 healthy controls.ResultsThe patients with SD showed significantly higher task-related brain activation in the left middle temporal gyrus, left thalamus, bilateral primary motor area, bilateral premotor area, bilateral cerebellum, bilateral somatosensory area, right insula, and right putamen compared with the controls. Region of interest voxel FC analysis revealed many FC changes within the cerebellum-basal ganglia-thalamus-cortex loop in the patients with SD. Of the significant connectivity changes between the patients with SD and the controls, the FC between the left thalamus and the left caudate nucleus was significantly correlated with clinical parameters in SD.ConclusionThe higher task-related brain activity in the insula and cerebellum was consistent with previous neuroimaging studies, suggesting that these areas are one of the unique characteristics of phonation-induced brain activity in SD. Based on FC analysis and their significant correlations with clinical parameters, the basal ganglia network plays an important role in the pathogenesis of SD.



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A novel founder MYO15A frameshift duplication is the major cause of genetic hearing loss in Oman.

A novel founder MYO15A frameshift duplication is the major cause of genetic hearing loss in Oman.

J Hum Genet. 2016 Oct 13;:

Authors: Palombo F, Al-Wardy N, Ruscone GA, Oppo M, Kindi MN, Angius A, Al Lamki K, Girotto G, Giangregorio T, Benelli M, Magi A, Seri M, Gasparini P, Cucca F, Sazzini M, Al Khabori M, Pippucci T, Romeo G

Abstract
The increased risk for autosomal recessive disorders is one of the most well-known medical implications of consanguinity. In the Sultanate of Oman, a country characterized by one of the highest rates of consanguineous marriages worldwide, prevalence of genetic hearing loss (GHL) is estimated to be 6/10 000. Families of GHL patients have higher consanguinity rates than the general Omani population, indicating a major role for recessive forms. Mutations in GJB2, the most commonly mutated GHL gene, have been sporadically described. We collected 97 DNA samples of GHL probands, affected/unaffected siblings and parents from 26 Omani consanguineous families. Analyzing a first family by whole-exome sequencing, we identified a novel homozygous frameshift duplication (c.1171_1177dupGCCATCT) in MYO15A, the gene linked to the deafness locus DFNB3. This duplication was then found in a total of 8/26 (28%) families, within a 849 kb founder haplotype. Reconstruction of haplotype structure at MYO15A surrounding genomic regions indicated that the founder haplotype branched out in the past two to three centuries from a haplotype present worldwide. The MYO15A duplication emerges as the major cause of GHL in Oman. These findings have major implications for the design of GHL diagnosis and prevention policies in Oman.Journal of Human Genetics advance online publication, 13 October 2016; doi:10.1038/jhg.2016.120.

PMID: 27734841 [PubMed - as supplied by publisher]

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Speech Recognition and Parent Ratings From Auditory Development Questionnaires in Children Who Are Hard of Hearing.

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Speech Recognition and Parent Ratings From Auditory Development Questionnaires in Children Who Are Hard of Hearing.

Ear Hear. 2015 Nov-Dec;36 Suppl 1:60S-75S

Authors: McCreery RW, Walker EA, Spratford M, Oleson J, Bentler R, Holte L, Roush P

Abstract
OBJECTIVES: Progress has been made in recent years in the provision of amplification and early intervention for children who are hard of hearing. However, children who use hearing aids (HAs) may have inconsistent access to their auditory environment due to limitations in speech audibility through their HAs or limited HA use. The effects of variability in children's auditory experience on parent-reported auditory skills questionnaires and on speech recognition in quiet and in noise were examined for a large group of children who were followed as part of the Outcomes of Children with Hearing Loss study.
DESIGN: Parent ratings on auditory development questionnaires and children's speech recognition were assessed for 306 children who are hard of hearing. Children ranged in age from 12 months to 9 years. Three questionnaires involving parent ratings of auditory skill development and behavior were used, including the LittlEARS Auditory Questionnaire, Parents Evaluation of Oral/Aural Performance in Children rating scale, and an adaptation of the Speech, Spatial, and Qualities of Hearing scale. Speech recognition in quiet was assessed using the Open- and Closed-Set Test, Early Speech Perception test, Lexical Neighborhood Test, and Phonetically Balanced Kindergarten word lists. Speech recognition in noise was assessed using the Computer-Assisted Speech Perception Assessment. Children who are hard of hearing were compared with peers with normal hearing matched for age, maternal educational level, and nonverbal intelligence. The effects of aided audibility, HA use, and language ability on parent responses to auditory development questionnaires and on children's speech recognition were also examined.
RESULTS: Children who are hard of hearing had poorer performance than peers with normal hearing on parent ratings of auditory skills and had poorer speech recognition. Significant individual variability among children who are hard of hearing was observed. Children with greater aided audibility through their HAs, more hours of HA use, and better language abilities generally had higher parent ratings of auditory skills and better speech-recognition abilities in quiet and in noise than peers with less audibility, more limited HA use, or poorer language abilities. In addition to the auditory and language factors that were predictive for speech recognition in quiet, phonological working memory was also a positive predictor for word recognition abilities in noise.
CONCLUSIONS: Children who are hard of hearing continue to experience delays in auditory skill development and speech-recognition abilities compared with peers with normal hearing. However, significant improvements in these domains have occurred in comparison to similar data reported before the adoption of universal newborn hearing screening and early intervention programs for children who are hard of hearing. Increasing the audibility of speech has a direct positive effect on auditory skill development and speech-recognition abilities and also may enhance these skills by improving language abilities in children who are hard of hearing. Greater number of hours of HA use also had a significant positive impact on parent ratings of auditory skills and children's speech recognition.

PMID: 26731160 [PubMed - indexed for MEDLINE]

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