Pediatric Auditory Brainstem Implantation: Surgical, Electrophysiologic, and Behavioral Outcomes.

Objectives: The objectives of this study were to demonstrate the safety of auditory brainstem implant (ABI) surgery and document the subsequent development of auditory and spoken language skills in children without neurofibromatosis type II (NFII).

Design: A prospective, single-subject observational study of ABI in children without NFII was undertaken at the University of North Carolina at Chapel Hill. Five children were enrolled under an investigational device exemption sponsored by the investigators. Over 3 years, patient demographics, medical/surgical findings, complications, device mapping, electrophysiologic measures, audiologic outcomes, and speech and language measures were collected.

Results: Five children without NFII have received ABIs to date without permanent medical sequelae, although 2 children required treatment after surgery for temporary complications. All children wear their device daily, and the benefits of sound awareness have developed slowly. Intra-and postoperative electrophysiologic measures augmented surgical placement and device programming. The slow development of audition skills precipitated limited changes in speech production but had little impact on growth in spoken language.

Conclusions: ABI surgery is safe in young children without NFII. Benefits from device use develop slowly and include sound awareness and the use of pattern and timing aspects of sound. These skills may augment progress in speech production but progress in language development is dependent upon visual communication. Further monitoring of this cohort is needed to better delineate the benefits of this intervention in this patient population.

Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Rescue of Outer Hair Cells with Antisense Oligonucleotides in Usher Mice Is Dependent on Age of Treatment

Abstract

              <p>The absence of functional outer hair cells is a component of several forms of hereditary hearing impairment, including Usher syndrome, the most common cause of concurrent hearing and vision loss. Antisense oligonucleotide (ASO) treatment of mice with the human Usher mutation, <em>Ush1c</em> c.216G&gt;A, corrects gene expression and significantly improves hearing, as measured by auditory-evoked brainstem responses (ABRs), as well as inner and outer hair cell (IHC and OHC) bundle morphology. However, it is not clear whether the improvement in hearing achieved by ASO treatment involves the functional rescue of outer hair cells. Here, we show that <em>Ush1c</em> c.216AA mice lack OHC function as evidenced by the absence of distortion product otoacoustic emissions (DPOAEs) in response to low-, mid-, and high-frequency tone pairs. This OHC deficit is rescued by treatment with an ASO that corrects expression of <em>Ush1c</em> c.216G&gt;A. Interestingly, although rescue of inner hairs cells, as measured by ABR, is achieved by ASO treatment as late as 7 days after birth, rescue of outer hair cells, measured by DPOAE, requires treatment before post-natal day 5. These results suggest that ASO-mediated rescue of both IHC and OHC function is age dependent and that the treatment window is different for the different cell types. The timing of treatment for congenital hearing disorders is of critical importance for the development of drugs such ASO-29 for hearing rescue.</p><br /><br />

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Auditory sequential accumulation of spectral information

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Publication date: Available online 11 October 2017
Source:Hearing Research
Author(s): Yi Shen
In many listening situations, information about the spectral content of a target sound may be distributed over time, and estimating the target spectrum requires efficient sequential processing. Listeners’ ability to estimate the spectrum of a random-frequency, six-tone complex was investigated and the spectral content of the complex was revealed using a sequence of bursts. Whether each of the six tones was presented within each burst was determined at random according to a presentation probability. In separate conditions, the presentation probabilities (p) ranged from 0.2 to 1, the total number of bursts varied from 1 to 16, and the inter-burst interval was either 0 or 200 ms. To evaluate the information acquired by the listener, the burst sequence was followed, after a 500-ms silent interval, by the six-tone complex acting as an informational masker and the listener was required to detect a pure-tone target presented simultaneously with the masker. Greater performance in this task indicates more accurate estimation of the spectrum of the complex by the listener. Evidence for integration of information across bursts was observed, and the integration process did not significantly depend on inter-burst interval.

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Local dynamic stability during treadmill walking can detect children with developmental coordination disorder

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Publication date: January 2018
Source:Gait & Posture, Volume 59
Author(s): Merete B. Speedtsberg, Sofie B. Christensen, Jan Stenum, Thomas Kallemose, Jesper Bencke, Derek J. Curtis, Bente R. Jensen
ObjectiveDevelopmental coordination disorder (DCD) is an innate impairment of motor coordination that affects basic locomotion and balance. This study investigated local dynamic stability of trunk accelerations during treadmill walking as an objective evaluation of gait stability and the sensitivity and specificity of this measure to discriminate children with DCD from typically developing children.MethodEight children with DCD and ten age- and gender-matched typically developing children (TD) walked four minutes on a treadmill. Trunk accelerations in vertical, medio-lateral and anterior-posterior directions were recorded with a sternum mounted accelerometer at 256Hz. Short term local dynamic stability (λs), root mean square (RMS) and relative root mean square (RMSR) were calculated from measures of orthogonal trunk accelerations. Receiver operating characteristic curve (ROC) analysis was performed to discriminate between groups based on short term local dynamic stability.Resultsλs was significantly greater in children with DCD in the main movement direction (AP) (DCD: 1.69±0.17 λs; TD:1.41±0.17 λs; p=0.005), indicating reduced local dynamic stability. RMS and RMSR accelerations showed no difference between children with DCD and TD children in any direction. The ROC analysis of λs in separate directions and in two dimensions showed an excellent accuracy of discriminating between children with DCD and TD children. Anterior-posterior direction in combination with medio-lateral or vertical showed best performance with an area under the curve (AUC) of 0.91.ConclusionWe have shown that children with developmental coordination disorder have general reduced local dynamic stability and that the short term Lyapunov exponent has good power of discrimination between DCD and TD.

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Combining valgus knee brace and lateral foot wedges reduces external forces and moments in osteoarthritis patients

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Publication date: January 2018
Source:Gait & Posture, Volume 59
Author(s): Amir Ali Jafarnezhadgero, Anderson S. Oliveira, Seyed Hamed Mousavi, Morteza Madadi-Shad
Osteoarthritis progression can be related to the external knee adduction and flexion moments during walking. Lateral foot wedges and knee braces have been used as treatment for osteoarthritis, but little is known about their influence on knee joint moments generated in the sagittal and frontal planes. Therefore, the aim of the present study was determine the effects of the isolated and combined use of valgus knee brace and lateral wedge foot orthotic on peak forces and moments during gait in knee osteoarthritis patients. Twenty four males (age: 62.1±2.0years) with varus alignment, symptomatic medial compartment knee osteoarthritis participated in this study. Subjects walked over ground at preferred speed in four conditions: (1) no assistive device (control); (2) using lateral wedges, (3) using knee braces, and (4) using both lateral wedges and knee braces. Ground reaction forces (GRF) and moments, as well as lower limb kinematics were recorded. Peak GRF, vertical loading rate, free moment, external knee adduction and flexion moments were compared across conditions. The concurrent use of lateral wedge and knee brace reduced the first peak GRF in the vertical (6%, p=0.002), anterior-posterior (30%, p=0.028) and medial-lateral directions (44%, p=0.029). Moreover, the use of these devices reduced the peak external knee adduction moment (25%, p=0.019), but not the external flexion moment and free moment (p>0.05). The combined use of lateral wedges and knee braces can reduce medial-lateral knee joint loading, but despite reduced peak forces in the sagittal plane, these device do not reduce joint moments.

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Virtual reality balance training for elderly: Similar skiing games elicit different challenges in balance training

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Publication date: January 2018
Source:Gait & Posture, Volume 59
Author(s): Aijse W. de Vries, Gert Faber, Ilse Jonkers, Jaap H. Van Dieen, Sabine M.P. Verschueren
BackgroundVirtual Reality (VR) balance training may have advantages over regular exercise training in older adults. However, results so far are conflicting potentially due to the lack of challenge imposed by the movements in those games. Therefore, the aim of this study was to assess to which extent two similar skiing games challenge balance, as reflected in center of mass (COM) movements relative to their Functional Limits of Stability (FLOS).MethodsThirty young and elderly participants performed two skiing games, one on the Wii Balance board (Wiiski), which uses a force plate, and one with the Kinect sensor (Kinski), which performs motion tracking. During gameplay, kinematics were captured using seven opto-electronical cameras. FLOS were obtained for eight directions. The influence of games and trials on COM displacement in each of the eight directions, and maximal COM speed, were tested with Generalized Estimated Equations.ResultsIn all directions with anterior and medio-lateral, but not with a posterior component, subjects showed significantly larger maximal %FLOS displacements during the Kinski game than during the Wiiski game. Furthermore, maximal COM displacement, and COM speed in Kinski remained similar or increased over trials, whereas for Wiiski it decreased.ConclusionsOur results show the importance of assessing the movement challenge in games used for balance training. Similar games impose different challenges, with the control sensors and their gain settings playing an important role. Furthermore, adaptations led to a decrease in challenge in Wiiski, which might limit the effectiveness of the game as a balance-training tool.

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Constraints specific influences of vision, touch and surface compliance in postural dynamics

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Publication date: January 2018
Source:Gait & Posture, Volume 59
Author(s): I-Chieh Lee, Matheus M. Pacheco, Karl M. Newell
Studies that have manipulated vision and touch in posture usually emphasize the prescriptive closed-loop function of the information to reduce the amount of postural motion. In contrast, we examine here the hypothesis that the standard sensory manipulations to maintain quiet stance also change in specific ways the constraints on the task goal and the emergent movement organization. Twelve participants were instructed to maintain quiet postural stance under three sensory factors: surface compliance (foam/no foam), visual information (open/closed eyes) and tactile information (finger touch/no finger touch). The standard deviation of center of pressure (COP) motion decreased with the presence of vision, touch and rigid surface. The correlation dimension showed that the manipulation of touch and vision produced different attractor dynamics that also interacted with surface compliance. Vision decreased the correlation dimension in the foam surface while the touch manipulation increased dimension in the rigid surface. The sensory information manipulations changed the qualitative properties of the attractor dynamics as well as the quantitative properties of the amount of postural motion providing evidence for the specific nature of the postural organization across information conditions.

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VERIFICATION OF AN IMPROVED HIP JOINT CENTER PREDICTION METHOD

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Publication date: Available online 10 October 2017
Source:Gait & Posture
Author(s): Emily J. Miller, Kenton R. Kaufman
In motion analysis, the hip joint center (HJC) is used to define the proximal location of the thigh segment and is also the point about which hip moments are calculated. The HJC cannot be palpated; its location must be calculated. Functional methods have been proposed but are difficult to perform by some clinical populations. Therefore, regression methods are utilized, but yield large errors in estimating the HJC location. These prediction methods typically utilize the anterior and posterior superior iliac spines, where excessive adipose tissue makes correctly locating difficult. A new regression method (Hara) utilizes leg length and has been shown to improve HJC location in cadavers and less error than previous pelvic based regression methods, such as those proposed by Harrington et al. This study compared the accuracy of the HJC location calculated with both of the Harrington methods and the Hara method. The coronal knee angle was calculated for each method using a static motion analysis trial, and compared to the tibiofemoral angle measured on a gold standard digital full-leg coronal radiograph. This study demonstrated that the Hara method was more accurate than either of the Harrington methods The mean error between the gold standard x-ray measurement and the motion analysis calculation for the Harrington (stepwise and LOOCV), the Harrington (linear regression), and Hara regression methods, respectively were 6.0°, 4.0°, and 1.8°. Accurately modeling the HJC is critical for data interpretation and patient care. This study confirmed that the Hara HJC regression method is valid in an in-vivo setting.

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Effects of surgical lesions on choline acetyltransferase activity in the cat cochlea

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Publication date: Available online 10 October 2017
Source:Hearing Research
Author(s): Mark Frilling, Gregory J. Wiet, Donald A. Godfrey, Judy A. Parli, Jon D. Dunn, C. David Ross
Although it is well established that the choline acetyltransferase (ChAT, the enzyme for acetylcholine synthesis) in the mammalian cochlea is associated with its olivocochlear innervation, the distribution of this innervation in the cochlea varies somewhat among mammalian species. The quantitative distribution of ChAT activity in the cochlea has been reported for guinea pigs and rats. The present study reports the distribution of ChAT activity within the organ of Corti among the three turns of the cat cochlea and the effects of removing olivocochlear innervation either by a lateral cut aimed to totally transect the left olivocochlear bundle or a more medial cut additionally damaging the superior olivary complex on the same side. Similarly to results for guinea pig and rat, the distribution of ChAT activity in the cat outer hair cell region showed a decrease from base to apex, but, unlike in the guinea pig and rat, the cat inner hair cell region did not. As in the rat, little ChAT activity was measured in the outer supporting cell region. As previously reported for whole cat cochlea and for rat cochlear regions, transection of the olivocochlear bundle resulted in almost total loss of ChAT activity in the hair cell regions of the cat cochlea. Lesions of the superior olivary complex resulted in loss of ChAT activity in the inner hair cell region of all cochlear turns only on the lesion side but bilateral losses in the outer hair cell region of all turns. The results are consistent with previous evidence that virtually all cholinergic synapses in the mammalian cochlea are associated with its olivocochlear innervation, that the olivocochlear innervation to the inner hair cell region is predominantly ipsilateral, and that the olivocochlear innervation to the outer hair cells is bilateral.

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A Subjective Rating Scale for Initial Assessment of Sudden Unilateral Sensorineural Hearing Loss

Objective: To examine the value of a subjective numerical rating scale (NRS) in the initial evaluation of patients suspected of suffering from unilateral sudden sensorineural hearing loss (SSNHL) until a formal audiogram is available. Study Design: Prospective noncontrolled clinical study. Methods: Thirty-one consecutive patients referred to the emergency department due to suspected unilateral SSNHL and with no other aural pathology by history or physical examination were enrolled. Patients were asked to characterize the severity of their hearing loss using an NRS of 1 (normal hearing) to 6 (complete deafness). SSNHL was defined as an SNHL of at least 30 dB over 3 consecutive frequencies that occurred in 3 days or less. A formal audiogram was obtained subsequently as soon as available. Results: Twenty-four patients were treated with steroids and met the audiometric criteria of SSNHL. All scored their NRS as 3 or more. None of the 7 patients whose NRS grades were ≤2.5 met the criteria for SSNHL. Two patients were treated with steroids although their hearing did not meet the audiometric criteria for SSNHL as the hearing loss was limited to 2 consecutive frequencies. The NRS score for both was Conclusion: In addition to the patient’s history and physical examination, a NRS can be a useful tool in the preliminary assessment of patients suspected of having SSNHL until audiometry becomes available. In the scale of 1-6, an NRS score of 3 or more reliably predicts the need to treat the patient with steroids according to the accepted criteria.
Audiol Neurotol 2017;22:154-159

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