Loss of cargo binding in the human myosin VI deafness mutant (R1166X) increases actin filament binding.

Loss of cargo binding in the human myosin VI deafness mutant (R1166X) increases actin filament binding.

Biochem J. 2016 Jul 29;

Authors: Arden SD, Tumbarello DA, Butt T, Kendrick-Jones J, Buss F

Abstract
Mutations in myosin VI have been associated with autosomal recessive (DFNB37) and autosomal dominant (DFNA22) deafness in humans. Here, we characterise a myosin VI nonsense mutation (R1166X) that was identified in a family with hereditary hearing loss in Pakistan. This mutation leads to deletion of the C-terminal 120 amino acids of the myosin VI cargo-binding domain, which includes the WWY binding motif for the adaptor proteins LMTK2, Tom1 as well as Dab2. Interestingly, compromising myosin VI vesicle binding ability by expressing myosin VI with the R1166X mutation or with single point mutations in the adaptor binding sites leads to increased F-actin-binding of this myosin in vitro and in vivo As our results highlight the importance of cargo attachment for regulating actin binding to the motor domain, we perform a detailed characterisation of adaptor protein binding and identify single amino acids within myosin VI required for binding to cargo adaptors. We not only show that the adaptor proteins can directly interact with the cargo-binding tail of myosin VI, but our in vitro studies also suggest that multiple adaptor proteins can bind simultaneously to non-overlapping sites in the myosin VI tail. In conclusion, our characterisation of the human myosin VI deafness mutant (R1166X) suggests that defects in cargo binding may leave myosin VI in a primed/activated state with an increased actin-binding ability.

PMID: 27474411 [PubMed - as supplied by publisher]

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Femoral derotation osteotomy in adults with cerebral palsy

Publication date: September 2016
Source:Gait & Posture, Volume 49
Author(s): C. Putz, S.I. Wolf, A. Geisbüsch, M. Niklasch, L. Döderlein, T. Dreher
BackgroundInternal rotation gait constitutes a complex gait disorder in bilateral spastic cerebral palsy (BSCP) including static torsional and dynamic components resulting in lever arm dysfunction. Although femoral derotation osteotomy (FDO) is a standard procedure to correct increased femoral anteversion in children, unpredictable outcome has been reported. The effect of FDO when it is done as part of single-event multilevel surgery (SEMLS) in adulthood has not been investigated.MethodsIn this study mid-term data of 63 adults with BSCP and internal rotation gait, undergoing SEMLS including FDO were analyzed pre- and 1.7 years postoperatively by clinical examination and 3D-instrumented gait analysis. All legs were categorized as the more or less involved side to consider asymmetry. The mean hip rotation in stance preoperatively and the intraoperative derotation was correlated with the difference pre- and postoperatively.ResultsThe group as a whole experienced the following results postoperatively: improved mean hip rotation in stance (p=0.0001), mean foot progression angle (p=0.0001) and a significant improvement of the clinical parameter: passive internal and external hip rotation, midpoint and anteversion (p=0.0001) for both legs separately. With regard to the less and more involved side, clinical and kinematic parameters showed comparable significant changes (p=0.0001). The anteversion improved significantly in proximal compared to distal FDO (p=0.03).ConclusionThis study emphasizes an overall good correction of internal rotation gait in adults with bilateral involvement after FDO. However, the results are more predictable in adults compared to studies reporting outcome after FDO in children.



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Daily changes of individual gait patterns identified by means of support vector machines

Publication date: September 2016
Source:Gait & Posture, Volume 49
Author(s): F. Horst, F. Kramer, B. Schäfer, A. Eekhoff, P. Hegen, B.M. Nigg, W.I. Schöllhorn
Despite the common knowledge about the individual character of human gait patterns and about their non-repeatability, little is known about their stability, their interactions and their changes over time. Variations of gait patterns are typically described as random deviations around a stable mean curve derived from groups, which appear due to noise or experimental insufficiencies. The purpose of this study is to examine the nature of intrinsic inter-session variability in more detail by proving separable characteristics of gait patterns between individuals as well as within individuals in repeated measurement sessions. Eight healthy subjects performed 15 gait trials at a self-selected speed on eight days within two weeks. For each trial, the time-continuous ground reaction forces and lower body kinematics were quantified. A total of 960 gait patterns were analysed by means of support vector machines and the coefficient of multiple correlation. The results emphasise the remarkable amount of individual characteristics in human gait. Support vector machines results showed an error-free assignment of gait patterns to the corresponding individual. Thus, differences in gait patterns between individuals seem to be persistent over two weeks. Within the range of individual gait patterns, day specific characteristics could be distinguished by classification rates of 97.3% and 59.5% for the eight-day classification of lower body joint angles and ground reaction forces, respectively. Hence, gait patterns can be assumed not to be constant over time and rather exhibit discernible daily changes within previously stated good repeatability. Advantages for more individual and situational diagnoses or therapy are identified.



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Femoral derotation osteotomy in adults with cerebral palsy

Publication date: September 2016
Source:Gait & Posture, Volume 49
Author(s): C. Putz, S.I. Wolf, A. Geisbüsch, M. Niklasch, L. Döderlein, T. Dreher
BackgroundInternal rotation gait constitutes a complex gait disorder in bilateral spastic cerebral palsy (BSCP) including static torsional and dynamic components resulting in lever arm dysfunction. Although femoral derotation osteotomy (FDO) is a standard procedure to correct increased femoral anteversion in children, unpredictable outcome has been reported. The effect of FDO when it is done as part of single-event multilevel surgery (SEMLS) in adulthood has not been investigated.MethodsIn this study mid-term data of 63 adults with BSCP and internal rotation gait, undergoing SEMLS including FDO were analyzed pre- and 1.7 years postoperatively by clinical examination and 3D-instrumented gait analysis. All legs were categorized as the more or less involved side to consider asymmetry. The mean hip rotation in stance preoperatively and the intraoperative derotation was correlated with the difference pre- and postoperatively.ResultsThe group as a whole experienced the following results postoperatively: improved mean hip rotation in stance (p=0.0001), mean foot progression angle (p=0.0001) and a significant improvement of the clinical parameter: passive internal and external hip rotation, midpoint and anteversion (p=0.0001) for both legs separately. With regard to the less and more involved side, clinical and kinematic parameters showed comparable significant changes (p=0.0001). The anteversion improved significantly in proximal compared to distal FDO (p=0.03).ConclusionThis study emphasizes an overall good correction of internal rotation gait in adults with bilateral involvement after FDO. However, the results are more predictable in adults compared to studies reporting outcome after FDO in children.



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Daily changes of individual gait patterns identified by means of support vector machines

Publication date: September 2016
Source:Gait & Posture, Volume 49
Author(s): F. Horst, F. Kramer, B. Schäfer, A. Eekhoff, P. Hegen, B.M. Nigg, W.I. Schöllhorn
Despite the common knowledge about the individual character of human gait patterns and about their non-repeatability, little is known about their stability, their interactions and their changes over time. Variations of gait patterns are typically described as random deviations around a stable mean curve derived from groups, which appear due to noise or experimental insufficiencies. The purpose of this study is to examine the nature of intrinsic inter-session variability in more detail by proving separable characteristics of gait patterns between individuals as well as within individuals in repeated measurement sessions. Eight healthy subjects performed 15 gait trials at a self-selected speed on eight days within two weeks. For each trial, the time-continuous ground reaction forces and lower body kinematics were quantified. A total of 960 gait patterns were analysed by means of support vector machines and the coefficient of multiple correlation. The results emphasise the remarkable amount of individual characteristics in human gait. Support vector machines results showed an error-free assignment of gait patterns to the corresponding individual. Thus, differences in gait patterns between individuals seem to be persistent over two weeks. Within the range of individual gait patterns, day specific characteristics could be distinguished by classification rates of 97.3% and 59.5% for the eight-day classification of lower body joint angles and ground reaction forces, respectively. Hence, gait patterns can be assumed not to be constant over time and rather exhibit discernible daily changes within previously stated good repeatability. Advantages for more individual and situational diagnoses or therapy are identified.



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Femoral derotation osteotomy in adults with cerebral palsy

Publication date: September 2016
Source:Gait & Posture, Volume 49
Author(s): C. Putz, S.I. Wolf, A. Geisbüsch, M. Niklasch, L. Döderlein, T. Dreher
BackgroundInternal rotation gait constitutes a complex gait disorder in bilateral spastic cerebral palsy (BSCP) including static torsional and dynamic components resulting in lever arm dysfunction. Although femoral derotation osteotomy (FDO) is a standard procedure to correct increased femoral anteversion in children, unpredictable outcome has been reported. The effect of FDO when it is done as part of single-event multilevel surgery (SEMLS) in adulthood has not been investigated.MethodsIn this study mid-term data of 63 adults with BSCP and internal rotation gait, undergoing SEMLS including FDO were analyzed pre- and 1.7 years postoperatively by clinical examination and 3D-instrumented gait analysis. All legs were categorized as the more or less involved side to consider asymmetry. The mean hip rotation in stance preoperatively and the intraoperative derotation was correlated with the difference pre- and postoperatively.ResultsThe group as a whole experienced the following results postoperatively: improved mean hip rotation in stance (p=0.0001), mean foot progression angle (p=0.0001) and a significant improvement of the clinical parameter: passive internal and external hip rotation, midpoint and anteversion (p=0.0001) for both legs separately. With regard to the less and more involved side, clinical and kinematic parameters showed comparable significant changes (p=0.0001). The anteversion improved significantly in proximal compared to distal FDO (p=0.03).ConclusionThis study emphasizes an overall good correction of internal rotation gait in adults with bilateral involvement after FDO. However, the results are more predictable in adults compared to studies reporting outcome after FDO in children.



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Daily changes of individual gait patterns identified by means of support vector machines

Publication date: September 2016
Source:Gait & Posture, Volume 49
Author(s): F. Horst, F. Kramer, B. Schäfer, A. Eekhoff, P. Hegen, B.M. Nigg, W.I. Schöllhorn
Despite the common knowledge about the individual character of human gait patterns and about their non-repeatability, little is known about their stability, their interactions and their changes over time. Variations of gait patterns are typically described as random deviations around a stable mean curve derived from groups, which appear due to noise or experimental insufficiencies. The purpose of this study is to examine the nature of intrinsic inter-session variability in more detail by proving separable characteristics of gait patterns between individuals as well as within individuals in repeated measurement sessions. Eight healthy subjects performed 15 gait trials at a self-selected speed on eight days within two weeks. For each trial, the time-continuous ground reaction forces and lower body kinematics were quantified. A total of 960 gait patterns were analysed by means of support vector machines and the coefficient of multiple correlation. The results emphasise the remarkable amount of individual characteristics in human gait. Support vector machines results showed an error-free assignment of gait patterns to the corresponding individual. Thus, differences in gait patterns between individuals seem to be persistent over two weeks. Within the range of individual gait patterns, day specific characteristics could be distinguished by classification rates of 97.3% and 59.5% for the eight-day classification of lower body joint angles and ground reaction forces, respectively. Hence, gait patterns can be assumed not to be constant over time and rather exhibit discernible daily changes within previously stated good repeatability. Advantages for more individual and situational diagnoses or therapy are identified.



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'Open access' and the International Journal of Audiology.

'Open access' and the International Journal of Audiology.

Int J Audiol. 2016 Jul 29;:1-2

Authors:

PMID: 27472297 [PubMed - as supplied by publisher]

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'Open access' and the International Journal of Audiology.

'Open access' and the International Journal of Audiology.

Int J Audiol. 2016 Jul 29;:1-2

Authors:

PMID: 27472297 [PubMed - as supplied by publisher]

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Cost-benefit analysis of targeted hearing directed early testing for congenital cytomegalovirus infection.

Related Articles

Cost-benefit analysis of targeted hearing directed early testing for congenital cytomegalovirus infection.

Int J Pediatr Otorhinolaryngol. 2015 Dec;79(12):2090-3

Authors: Bergevin A, Zick CD, McVicar SB, Park AH

Abstract
OBJECTIVES: In this study, we estimate an ex ante cost-benefit analysis of a Utah law directed at improving early cytomegalovirus (CMV) detection.
STUDY DESIGN: We use a differential cost of treatment analysis for publicly insured CMV-infected infants detected by a statewide hearing-directed CMV screening program.
METHODS: Utah government administrative data and multi-hospital accounting data are used to estimate and compare costs and benefits for the Utah infant population.
RESULTS: If antiviral treatment succeeds in mitigating hearing loss for one infant per year, the public savings will offset the public costs incurred by screening and treatment. If antiviral treatment is not successful, the program represents a net cost, but may still have non-monetary benefits such as accelerated achievement of diagnostic milestones.
CONCLUSIONS: The CMV education and treatment program costs are modest and show potential for significant cost savings.

PMID: 26432541 [PubMed - indexed for MEDLINE]

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Whole-exome sequencing as a diagnostic tool for distal renal tubular acidosis.

Related Articles

Whole-exome sequencing as a diagnostic tool for distal renal tubular acidosis.

J Pediatr (Rio J). 2015 Nov-Dec;91(6):583-9

Authors: Pereira PC, Melo FM, De Marco LA, Oliveira EA, Miranda DM, Simões e Silva AC

Abstract
OBJECTIVE: Distal renal tubular acidosis (dRTA) is characterized by metabolic acidosis due to impaired renal acid excretion. The aim of this study was to demonstrate the genetic diagnosis of four children with dRTA through use of whole-exome sequencing.
METHODS: Two unrelated families were selected; a total of four children with dRTA and their parents, in order to perform whole-exome sequencing. Hearing was preserved in both children from the first family, but not in the second, wherein a twin pair had severe deafness. Whole-exome sequencing was performed in two pooled samples and findings were confirmed with Sanger sequencing method.
RESULTS: Two mutations were identified in the ATP6V0A4 and ATP6V1B1 genes. In the first family, a novel mutation in the exon 13 of the ATP6V0A4 gene with a single nucleotide change GAC → TAC (c.1232G>T) was found, which caused a substitution of aspartic acid to tyrosine in position 411. In the second family, a homozygous recurrent mutation with one base-pair insertion (c.1149_1155insC) in exon 12 of the ATP6V1B1 gene was detected.
CONCLUSION: These results confirm the value of whole-exome sequencing for the study of rare and complex genetic nephropathies, allowing the identification of novel and recurrent mutations. Furthermore, for the first time the application of this molecular method in renal tubular diseases has been clearly demonstrated.

PMID: 26208211 [PubMed - indexed for MEDLINE]

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'Open access' and the International Journal of Audiology.

'Open access' and the International Journal of Audiology.

Int J Audiol. 2016 Jul 29;:1-2

Authors:

PMID: 27472297 [PubMed - as supplied by publisher]

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'Open access' and the International Journal of Audiology.

'Open access' and the International Journal of Audiology.

Int J Audiol. 2016 Jul 29;:1-2

Authors:

PMID: 27472297 [PubMed - as supplied by publisher]

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‘Open access’ and the International Journal of Audiology

10.1080/14992027.2016.1211765<br/>

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‘Open access’ and the International Journal of Audiology

10.1080/14992027.2016.1211765<br/>

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‘Open access’ and the International Journal of Audiology

10.1080/14992027.2016.1211765<br/>

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‘Open access’ and the International Journal of Audiology

10.1080/14992027.2016.1211765<br/>

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‘Open access’ and the International Journal of Audiology

10.1080/14992027.2016.1211765<br/>

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You Too Could Record the Earth

We have recently talked about making soundscapes from photographs and a group of professional recording artists joining forces to document the exquisite sounds of nature. The real world, no doubt, is much more sonically diverse and full. Is there a way to capture the characteristic sounds of the world we live in? Is there then a way for me to travel sonically to hear what you hear in another part of the world?

Tweet

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Sound localization in the presence of multiple reflections using a binaurally integrated cross-correlation/auto-correlation mechanism

A precedence effect model is described that can use a binaural signal to robustly localize a sound source in the presence of multiple reflections for the frontal horizontal plane. The model also estimates a room impulse response from a running binaural signal and determines the spatial locations and delays of early reflections, without any prior or additional knowledge of the source. A dual-layer cross-correlation/auto-correlation algorithm is used to determine the interaural time difference of the direct sound source component and to estimate a binaural activity pattern.

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Concurrent validity and reliability of using ground reaction force and center of pressure parameters in the determination of leg movement initiation during single leg lift

Publication date: Available online 29 July 2016
Source:Gait & Posture
Author(s): Daniela Aldabe, Pedduzzi Marcelo Castro, Stephan Milosavljevic, Dawn Melanie Bussey
Postural adjustment evaluations during single leg lift requires the initiation of heel lift (T1) identification. T1 measured by means of motion analyses system is the most reliable approach. However, this method involves considerable workspace, expensive cameras, and time processing data and setting up laboratory. The use of ground reaction forces (GRF) and centre of pressure (COP) data is an alternative method as its data processing and setting up is less time consuming. Further, kinetic data is normally collected using frequency samples higher than 1000Hz whereas kinematic data are commonly captured using 50 to 200Hz. This study describes the concurrent-validity and reliability of GRF and COP measurements in determining T1, using a motion analysis system as reference standard. Kinematic and kinetic data during single leg lift were collected from ten participants. GRF and COP data were collected using one and two force plates. Displacement of a single heel marker was captured by means of ten Vicon^© cameras. Kinetic and kinematic data were collected using a sample frequency of 1000Hz. Data were analysed in two stages: identification of key events in the kinetic data, and assessing concurrent validity of T1 based on the chosen key events with T1 provided by the kinematic data. The key event presenting the least systematic bias, along with a narrow 95% CI and limits of agreement against the reference standard T1, was the Baseline COPy event. Baseline COPy event was obtained using one force plate and presented excellent between-tester reliability.



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Concurrent validity and reliability of using ground reaction force and center of pressure parameters in the determination of leg movement initiation during single leg lift

Publication date: Available online 29 July 2016
Source:Gait & Posture
Author(s): Daniela Aldabe, Pedduzzi Marcelo Castro, Stephan Milosavljevic, Dawn Melanie Bussey
Postural adjustment evaluations during single leg lift requires the initiation of heel lift (T1) identification. T1 measured by means of motion analyses system is the most reliable approach. However, this method involves considerable workspace, expensive cameras, and time processing data and setting up laboratory. The use of ground reaction forces (GRF) and centre of pressure (COP) data is an alternative method as its data processing and setting up is less time consuming. Further, kinetic data is normally collected using frequency samples higher than 1000Hz whereas kinematic data are commonly captured using 50 to 200Hz. This study describes the concurrent-validity and reliability of GRF and COP measurements in determining T1, using a motion analysis system as reference standard. Kinematic and kinetic data during single leg lift were collected from ten participants. GRF and COP data were collected using one and two force plates. Displacement of a single heel marker was captured by means of ten Vicon^© cameras. Kinetic and kinematic data were collected using a sample frequency of 1000Hz. Data were analysed in two stages: identification of key events in the kinetic data, and assessing concurrent validity of T1 based on the chosen key events with T1 provided by the kinematic data. The key event presenting the least systematic bias, along with a narrow 95% CI and limits of agreement against the reference standard T1, was the Baseline COPy event. Baseline COPy event was obtained using one force plate and presented excellent between-tester reliability.



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Concurrent validity and reliability of using ground reaction force and center of pressure parameters in the determination of leg movement initiation during single leg lift

Publication date: Available online 29 July 2016
Source:Gait & Posture
Author(s): Daniela Aldabe, Pedduzzi Marcelo Castro, Stephan Milosavljevic, Dawn Melanie Bussey
Postural adjustment evaluations during single leg lift requires the initiation of heel lift (T1) identification. T1 measured by means of motion analyses system is the most reliable approach. However, this method involves considerable workspace, expensive cameras, and time processing data and setting up laboratory. The use of ground reaction forces (GRF) and centre of pressure (COP) data is an alternative method as its data processing and setting up is less time consuming. Further, kinetic data is normally collected using frequency samples higher than 1000Hz whereas kinematic data are commonly captured using 50 to 200Hz. This study describes the concurrent-validity and reliability of GRF and COP measurements in determining T1, using a motion analysis system as reference standard. Kinematic and kinetic data during single leg lift were collected from ten participants. GRF and COP data were collected using one and two force plates. Displacement of a single heel marker was captured by means of ten Vicon^© cameras. Kinetic and kinematic data were collected using a sample frequency of 1000Hz. Data were analysed in two stages: identification of key events in the kinetic data, and assessing concurrent validity of T1 based on the chosen key events with T1 provided by the kinematic data. The key event presenting the least systematic bias, along with a narrow 95% CI and limits of agreement against the reference standard T1, was the Baseline COPy event. Baseline COPy event was obtained using one force plate and presented excellent between-tester reliability.



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Taste Disturbance Due to Cochlear Implant Stimulation.

Objective: To characterize stimulation of taste fibers in the facial nerve following cochlear implantation. Patient: A 34-year old presented with reversible dysgeusia following activation of a cochlear implant. Intervention: Reprogramming targeted to specific offending electrodes reduced symptom intensity. Computed tomography demonstrated dehiscence of the bone separating the labyrinthine segment of the facial nerve and the basal turn of the cochlea in proximity to the electrode array. Results: Dysgeusia was attributed to stimulation of taste fibers in the facial nerve by electrodes 13 to 16 of the cochlear implant array located in the superior-most portion of the basal turn. Conclusions: Dysgeusia following cochlear implant activation has not previously been reported. This likely results from stimulation of taste fibers through dehiscence of the bone separating the labyrinthine segment of the Fallopian canal and the basal turn of the cochlea. While in some cases of apparent dehiscence there may be thin bone present, recognition of this potential anatomic feature may influence the choice of which ear and which electrode design to implant. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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The Outcomes of Surgery and Chemoradiotherapy for Temporal Bone Cancer.

Objective: We aimed to evaluate the prognostic factors and efficacy of treatment modalities for patients with temporal bone cancer, and to determine if definitive chemoradiotherapy (CRT) for advanced-stage disease can provide a substitute for highly invasive surgeries. Study Design: Retrospective case series. Setting: Tertiary referral center. Patients: Sixty-six patients with previously untreated squamous cell carcinoma of the temporal bone treated with curative intent between April 1997 and March 2015. Intervention: Surgery alone, radiotherapy (RT) alone, surgery followed by RT or definitive CRT. Main Outcome Measure: The overall survival (OS) rate. Results: The 5-year OS rate for each T classification was 100% for T1, 76.2% for T2, 55.6% for T3, and 36.7% for T4. Univariable and multivariable analysis showed that T classification was an independent predictor of the OS rate (hazard ratio 5.66; 95% confidence interval 1.51-27.0; p = 0.015). Analysis by treatment modality revealed that the 5-year OS rate for patients with T1-2 was 100% for surgery and 81.3% for RT alone. The rate for patients with T3-4 was 52.1% for definitive CRT and 55.6% for surgery followed by RT with or without chemotherapy. Conclusions: Patients with T1-2 benefited from surgical intervention without significant morbidity or mortality. Our findings also suggested that definitive CRT might be appropriate as the first-line treatment for T3-4, especially in cases with unresectable tumors. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Vestibular Schwannomas: Do Linear and Volumetric Parameters on MRI Correlate With Hearing Loss?.

Purpose: To determine if two-dimensional and volumetric imaging parameters in vestibular schwannomas (VS) correlate with hearing loss at presentation. Study Design: Retrospective. Methods: Forty-one cases of pathologically confirmed sporadic VS were analyzed. Maximal tumor dimensions in anteroposterior (AP), coronal (ML), and craniocaudal (CC) dimensions were obtained along with tumor-fundus distance and internal auditory canal (IAC) porus diameters. Volumetric analysis was done on 37 cases. Tumors volumes were calculated through both 3-D volumetric and ABC/2 [AP x ML x CC/2] methodology. With the 3-D method, total tumor volume (TTV), and cerebellopontine angle (CPA) tumor volume were separately calculated and IAC volumes obtained by subtracting CPA tumor volumes from TTV. Pure tone average (PTA) and speech discrimination scores (SDS) were correlated with tumor dimensions. Results: Non-volumetric analysis was performed on 41 tumors. The AP and ML dimensions correlated with both PTA and SDS (p

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The Yield of Multigene Testing in the Management of Pediatric Unilateral Sensorineural Hearing Loss.

Objective: To explore the role and yield of multigene evaluation in children recently diagnosed with unilateral sensori-neural hearing loss (SNHL). Study Design: Retrospective cohort study. Setting: Tertiary level pediatric institution. Patients: Sixty children diagnosed with unilateral SNHL between January 2005 and December 2015. Intervention: Targeted mutation analysis was performed in 51 children using DNA microarray for genotyping 11 different genes known to be correlated with nonsyndromic SNHL. Main Outcome Measure: Multigene testing results. Results: Average age at diagnosis was 4.3 years. All children appeared to have nonsyndromic hearing loss (HL). HL was categorized as mild (17 children), moderate (17 children), severe (7 children), or profound (19 children). Genetic testing was performed in 51 (85%) children. Sixteen children (31.3%) were tested positive to connexin 26 (GJB2). One patient (2%) from this subgroup was homozygous and 15 were heterozygous. The average age of GJB2 positive children was 6 years and their average pure tone audiometry (PTA) was 75 dB. Computed tomography (CT) and/or magnetic resonance imaging (MRI) scans were performed in 43 children (71.66%). Out of the children who had a scan, 27 patients (62.8%) had negative findings on either CT or MRI scans. Sixteen (37.2%) patients had positive scan finding/s. Conclusions: Significant proportion of children with unilateral SNHL may have positive genetic testing while the vast majority of these children present with heterozygous mutations of connexin 26 (GJB2). Findings suggest that genetic evaluation has a role as a complementary modality in HL evaluation for pediatric unilateral SNHL although it may not be necessary to analyze for various abnormalities other than connexin 26 when practising in a limited resources environment. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Vestibular Impairment in Dementia.

Objective: Recent studies suggest an association between vestibular and cognitive function. The goal of the study was to investigate whether vestibular function was impaired in individuals with mild cognitive impairment (MCI) and Alzheimer's disease (AD) compared with cognitively normal individuals. Study Design: Cross-sectional study. Setting: Outpatient memory clinic and longitudinal observational study unit. Patients: Older individuals >=55 years with MCI or AD. Age, sex, and education-matched normal controls were drawn from the Baltimore Longitudinal Study of Aging (BLSA). Intervention: Saccular and utricular function was assessed with cervical and ocular vestibular-evoked myogenic potentials (c- and oVEMPs) respectively, and horizontal semicircular canal function was assessed with video head impulse testing. Main Outcome Measures: Presence or absence of VEMP responses, VEMP amplitude, and vestibular ocular reflex (VOR) gain were measured. Results: Forty-seven individuals with cognitive impairment (MCI N = 15 and AD N = 32) underwent testing and were matched with 94 controls. In adjusted analyses, bilaterally absent cVEMPs were associated with an over three-fold odds of AD (OR 3.42, 95% CI 1.33-8.91, p = 0.011). One microvolt increases in both cVEMP and oVEMP amplitudes were associated with decreased odds of AD (OR 0.28, 95% CI 0.09-0.93, p = 0.038 and OR 0.92, 95% CI 0.85-0.99, p = 0.036, respectively). There was no significant difference in VOR gain between the groups. Conclusions: These findings confirm and extend emerging evidence of an association between vestibular dysfunction and cognitive impairment. Further investigation is needed to determine the causal direction for the link between peripheral vestibular loss and cognitive impairment. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Response to "Drug Diffusion to the Apex of the Human Cochlea?".

No abstract available

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Treatment Outcomes for Single Modality Management of Glomus Jugulare Tumors With Stereotactic Radiosurgery.

Objectives: The objectives were to evaluate the audiological outcomes, response of symptoms, and response of tumor volume in patients with glomus jugulare tumors treated solely with single fraction gamma knife radiosurgery. Study Design: Single institution retrospective review. Setting: Academic, tertiary referral center. Patients: The diagnosis code for glomus jugulare was used to identify patients. Only those who underwent gamma knife radiosurgery were included. Those previously treated with any modality were excluded. A total of 12 patients were included for the tumor response and symptom response data and 7 of those were included in the audiometric analysis. Main Outcomes Measures: Audiometric data at most recent follow-up compared with presentation, subjective improvement in pulsatile tinnitus, and change in tumor volume at most recent follow-up compared with pretreatment. Results: The average time to most recent follow-up was 27.6 months. There was no significant change in pure-tone average or word recognition. Pulsatile tinnitus completely resolved or improved in 80% of patients. Cranial neuropathies were stable or improved. A single patient experienced facial nerve paresis 2 years after treatment, which resolved with steroid treatment. Tumor control was 100% and the average change in tumor volume was a decrease of 37%. Conclusion: Single modality gamma knife radiosurgery treatment of glomus jugulare tumors seems to be safe. Treatment results in decreased tumor volume and improved pulsatile tinnitus in most patients. There was no significant progression of hearing loss after treatment. Lower cranial nerve function remains stable in all patients. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Music-induced Hearing Loss in Children, Adolescents, and Young Adults: A Systematic Review and Meta-analysis.

Objective: Exposure to loud music has increased significantly because of the current development of personal music players and mobile phones. The aim of this study was to provide an overview of music-induced hearing loss and its symptoms in children. Data Sources: The search was performed in the databases Embase, Medline (OvidSP), Web-of-science, Scopus, Cinahl, Cochrane, PubMed publisher, and Google Scholar. Only articles written in English were included. Study Selection: Articles describing hearing levels and music exposure in children were used, published from 1990 until April 2015. Data Extraction: The quality of the studies was assessed on reporting, validity, power, and the quality of audiometric testing. Data Synthesis: Data of each publication was extracted into spreadsheet software and analyzed using best evidence synthesis. Conclusion: The prevalence of increased hearing levels (>15 dB HL) was 9.6%, and high-frequency hearing loss was found in 9.3%. The average hearing thresholds were 4.79 dB HL at low frequencies (0.5, 1, and 2 kHz) and 9.54 dB HL at high frequencies (3, 4, and 6 kHz). Most studies reported no significant association between pure-tone air thresholds and exposure to loud music. However, significant changes in hearing thresholds and otoacoustic emissions, and a high tinnitus prevalence suggest an association between music exposure and hearing loss in children. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Cochlear Implants in Adults: Effects of Age and Duration of Deafness on Speech Recognition.

Objective: Unexplained outcome variability exists among adults with cochlear implants (CIs). Two significant predictors are age and duration of deafness, with older patients and those with longer durations of deafness generally demonstrating poorer speech recognition. However, these factors are often highly correlated. Thus, it is unclear whether poorer outcomes should be attributed primarily to age-related declines or to the experience of auditory deprivation. Our aim was to examine the effects of aging and duration of hearing loss on outcomes for postlingually deaf adults with CIs. Study Design: Retrospective review of adults who received CIs from 1983 to 2014. Setting: Tertiary adult CI program. Patients: Sixty-four adult patients with postlingual hearing loss beginning after age 12 years, full electrode insertion, normal cochlear anatomy, and availability of postoperative outcome measures. Intervention: Cochlear implantation with 12 months or greater of device use. Main Outcome Measures: Postoperative pure-tone averages (0.5, 1, 2, and 3 kHz) and recognition of words in sentences (Hearing in Noise Test and AzBio). Results: Age at postoperative testing had a negative partial correlation with AzBio scores, when controlling for duration of deafness, whereas duration of deafness had a positive partial correlation with AzBio scores, when controlling for age. No other effects were identified. Conclusion: Older age at testing was associated with poorer recognition of words in difficult sentences, suggesting that cognitive aging may negatively impact CI outcomes. Further studies are needed to examine how a long duration of auditory deprivation affects CI outcomes. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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The Role of Bacteriologic Studies in Predicting Recurrent Mastoiditis in 456 Patients.

Objectives: To evaluate the effect of microbiologic growth on the recurrence rate of mastoiditis. Materials and Methods: A historical prospective study on all cases of children with acute mastoiditis (AM) admitted between 2000 and 2015, was performed. The following data were collected in the recurrent AM (rAM) group: age, sex, and medical history, antibiotic therapy before hospitalization, clinical presentation at admission including symptoms and signs, blood test results, computed tomography (CT) imaging, microbiological findings, treatment, complications, outcome, and follow-up duration. Results: Four hundred fifty six consecutive hospitalizations because of mastoiditis were identified; 22 patients were found to have rAM. The median age at first episode in the rAM group was 1 year (range, 1-11 years), and the median period from one episode to the next was 2 months. There were 15 men and 7 women. The right ear was involved in 11 cases in the first and in the second episode. The rate of Pseudomonas aeruginosa growth in the rAM group was significantly higher compared with the AM group (40.9 and 8.9%, respectively) (p = 0.001) as was found in anerobic growth (15 vs. no growth) (p

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A Review of Temporal Bone CT Imaging With Respect to Pediatric Bone-anchored Hearing Aid Placement.

Objective: Bone-anchored hearing aid has been shown to be effective in hearing rehabilitation for conductive loss or single-sided deafness. Current FDA guidelines allow implantation in patients over 5 years old. This guideline is at least partially due to concern for thickness of bone stock at the implant site. We aim to investigate whether temporal bone thickness should be a deterrent to implantation in those younger than five. Study Design: A retrospective review of high-resolution temporal bone computed tomographies (CTs) comparing measurements between ears with chronic disease and controls. Setting: Single institution tertiary care center. Patients: One hundred patients between 1 and 5.99 years had temporal bone CTs performed between 2000 and 2009. Patients with chronic ear disease were identified by ICD-9 code, as well as confirmation by review of the imaging. Intervention(s): None. Main Outcome Measures: Temporal bone thickness was measured on axial CT slices at a point 1 cm posterior to the sigmoid sinus, at the superior margin of the bony canal. Results: Average thickness was greater than 3 mm in all age groups. No significant difference was found between age groups, or between normal ears and ears with chronic disease (3.5 mm versus 3.3 mm, p = 0.21) when compared individually. Conclusion: This data shows pediatric temporal bone thickness is frequently greater than the recommended 3 mm, even in patients as young as one. Anatomically, concerns regarding temporal bone thickness in patients younger than five could be reliably addressed with imaging typically obtained in workup of hearing loss. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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The Use of an Ultrasonic Serrated Knife in Transcanal Excision of Exostoses.

Objective: To describe a novel approach for excision of exostoses and evaluate the outcomes of transcanal excision of exostoses using ultrasonic serrated knife-assistance versus microosteotomes only. Study Design: Retrospective analysis. Setting: Academic Tertiary Care Center. Patients/Interventions: Patients receiving a transcanal excision of exostoses between 2007 and 2016. Main Outcome Measures: The outcomes and postoperative complications of 138 patients who received transcanal microosteotome only technique were compared to 10 patients who received transcanal excision of exostoses using ultrasonic serrated knife-assistance (Stryker Corporation, Kalamazoo, MI). Results: A total of 175 ears in 128 patients underwent a transcanal microosteotome only technique. Of these, 11 (6.3%) developed an intraoperative tympanic membrane perforation. One developed anterior canal mobilization requiring prolonged (3 wk) packing. One hundred thirty-five ears (77%) were well healed by the 3-week postoperative visit. All but one ear was well healed by the 6-week visit. In comparison, 11 ears in 10 patients were treated for exostoses using a transcanal approach with the use of ultrasonic serrated knife and microosteotomes. One ear canal (the first in the series) was not well healed by the 3-week postoperative visit due to suspected thermal injury. All patients had well-healed ear canals by 6 weeks, and there were no complications. Conclusion: This is the first series to report the use of an ultrasonic serrated knife in the transcanal excision of exostoses. When compared with a transcanal microosteotome only excision of exostoses, the addition of the Sonopet seems to result in a similarly low complication rate. Sonopet can allow for more controlled transcanal excision of broad-based exostoses in patients with severe obstruction. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Effect of Elevated Intracranial Pressure on Amplitudes and Frequency Tuning of Ocular Vestibular Evoked Myogenic Potentials Elicited by Bone-Conducted Vibration.

Objective: Recently, it could be demonstrated that an increased intracranial pressure causes a modulation of the air conducted sound evoked ocular vestibular evoked myogenic potential (oVEMP). The mechanism for this modulation is not resolved and may depend on a change of either receptor excitability or sound energy transmission. Design: oVEMPs were elicited in 18 healthy subjects with a minishaker delivering 500 and 1000 Hz tone bursts, in supine and tilted positions. Results: The study could confirm the frequency tuning of oVEMP. However, at neither stimulus frequency could a modulating effect of increased intracranial pressure be observed. Conclusion: These data suggest that the observed modulation of the oVEMP response by an increased intracranial pressure is primarily due to the effect of an increased intralabyrinthine pressure onto the stiffness of the inner ear contents and the middle ear-inner ear junction. Future studies on the effect of intracranial pressure on oVEMP should use air-conducted sound and not bone-conducted vibration. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Effect of Elevated Intracranial Pressure on Amplitudes and Frequency Tuning of Ocular Vestibular Evoked Myogenic Potentials Elicited by Bone-Conducted Vibration.

Objective: Recently, it could be demonstrated that an increased intracranial pressure causes a modulation of the air conducted sound evoked ocular vestibular evoked myogenic potential (oVEMP). The mechanism for this modulation is not resolved and may depend on a change of either receptor excitability or sound energy transmission. Design: oVEMPs were elicited in 18 healthy subjects with a minishaker delivering 500 and 1000 Hz tone bursts, in supine and tilted positions. Results: The study could confirm the frequency tuning of oVEMP. However, at neither stimulus frequency could a modulating effect of increased intracranial pressure be observed. Conclusion: These data suggest that the observed modulation of the oVEMP response by an increased intracranial pressure is primarily due to the effect of an increased intralabyrinthine pressure onto the stiffness of the inner ear contents and the middle ear-inner ear junction. Future studies on the effect of intracranial pressure on oVEMP should use air-conducted sound and not bone-conducted vibration. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Effect of Elevated Intracranial Pressure on Amplitudes and Frequency Tuning of Ocular Vestibular Evoked Myogenic Potentials Elicited by Bone-Conducted Vibration.

Objective: Recently, it could be demonstrated that an increased intracranial pressure causes a modulation of the air conducted sound evoked ocular vestibular evoked myogenic potential (oVEMP). The mechanism for this modulation is not resolved and may depend on a change of either receptor excitability or sound energy transmission. Design: oVEMPs were elicited in 18 healthy subjects with a minishaker delivering 500 and 1000 Hz tone bursts, in supine and tilted positions. Results: The study could confirm the frequency tuning of oVEMP. However, at neither stimulus frequency could a modulating effect of increased intracranial pressure be observed. Conclusion: These data suggest that the observed modulation of the oVEMP response by an increased intracranial pressure is primarily due to the effect of an increased intralabyrinthine pressure onto the stiffness of the inner ear contents and the middle ear-inner ear junction. Future studies on the effect of intracranial pressure on oVEMP should use air-conducted sound and not bone-conducted vibration. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Continuing the Unique Journey - New Products in the Widex Unique Family

Widex believes that every patient in every situation is absolutely unique: hence, the name Widex UNIQUE. On a daily basis, patients explain to us the settings in which they would like to hear better and where they are struggling. Everyone has different lifestyles, family dynamics, and a myriad of activities that they enjoy. With the UNIQUE product line, Widex has designed hearing aids to fit and to be appropriate for as many people in as many situations as possible.

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Continuing the Unique Journey - New Products in the Widex Unique Family

Widex believes that every patient in every situation is absolutely unique: hence, the name Widex UNIQUE. On a daily basis, patients explain to us the settings in which they would like to hear better and where they are struggling. Everyone has different lifestyles, family dynamics, and a myriad of activities that they enjoy. With the UNIQUE product line, Widex has designed hearing aids to fit and to be appropriate for as many people in as many situations as possible.

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Continuing the Unique Journey - New Products in the Widex Unique Family

Widex believes that every patient in every situation is absolutely unique: hence, the name Widex UNIQUE. On a daily basis, patients explain to us the settings in which they would like to hear better and where they are struggling. Everyone has different lifestyles, family dynamics, and a myriad of activities that they enjoy. With the UNIQUE product line, Widex has designed hearing aids to fit and to be appropriate for as many people in as many situations as possible.

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Recovery of ambulation activity across the first six months post-stroke

Publication date: September 2016
Source:Gait & Posture, Volume 49
Author(s): Niruthikha Mahendran, Suzanne S. Kuys, Sandra G. Brauer
Stroke survivors commonly adopt sedentary activity behaviours by the chronic phase of recovery. However, the change in activity behaviours from the subacute to chronic phase of stroke is variable. This study explored the recovery of ambulation activity (volume and bouts) at one, three and six months after hospital discharge post-stroke. A total of 42 stroke survivors were recruited at hospital discharge and followed up one, three and six months later. At follow-up, ambulation activity was measured over four days using the ActivPAL™ accelerometer. Measures included volume of activity and frequency and intensity of ambulation activity bouts per day. Linear mixed effects modelling was used to determine changes over time. There was wide variation in activity. Total step counts across all time points were below required levels for health benefits (mean 4592 SD 3411). Most activity was spread across short bouts. While most number of bouts was of low intensity, most time was spent in moderate intensity ambulation across all time points. Daily step count and time spent walking and sitting/lying increased from one month to three and six months. The number of and time spent in short and medium duration bouts increased from one to six months. Time in long duration bouts increased at three months only. Time spent in moderate intensity ambulation increased over time. No change was observed for any other measures. In future, it would be valuable to identify strategies to increase engagement in activity behaviours to improve health outcomes after stroke.



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Recovery of ambulation activity across the first six months post-stroke

Publication date: September 2016
Source:Gait & Posture, Volume 49
Author(s): Niruthikha Mahendran, Suzanne S. Kuys, Sandra G. Brauer
Stroke survivors commonly adopt sedentary activity behaviours by the chronic phase of recovery. However, the change in activity behaviours from the subacute to chronic phase of stroke is variable. This study explored the recovery of ambulation activity (volume and bouts) at one, three and six months after hospital discharge post-stroke. A total of 42 stroke survivors were recruited at hospital discharge and followed up one, three and six months later. At follow-up, ambulation activity was measured over four days using the ActivPAL™ accelerometer. Measures included volume of activity and frequency and intensity of ambulation activity bouts per day. Linear mixed effects modelling was used to determine changes over time. There was wide variation in activity. Total step counts across all time points were below required levels for health benefits (mean 4592 SD 3411). Most activity was spread across short bouts. While most number of bouts was of low intensity, most time was spent in moderate intensity ambulation across all time points. Daily step count and time spent walking and sitting/lying increased from one month to three and six months. The number of and time spent in short and medium duration bouts increased from one to six months. Time in long duration bouts increased at three months only. Time spent in moderate intensity ambulation increased over time. No change was observed for any other measures. In future, it would be valuable to identify strategies to increase engagement in activity behaviours to improve health outcomes after stroke.



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Recovery of ambulation activity across the first six months post-stroke

Publication date: September 2016
Source:Gait & Posture, Volume 49
Author(s): Niruthikha Mahendran, Suzanne S. Kuys, Sandra G. Brauer
Stroke survivors commonly adopt sedentary activity behaviours by the chronic phase of recovery. However, the change in activity behaviours from the subacute to chronic phase of stroke is variable. This study explored the recovery of ambulation activity (volume and bouts) at one, three and six months after hospital discharge post-stroke. A total of 42 stroke survivors were recruited at hospital discharge and followed up one, three and six months later. At follow-up, ambulation activity was measured over four days using the ActivPAL™ accelerometer. Measures included volume of activity and frequency and intensity of ambulation activity bouts per day. Linear mixed effects modelling was used to determine changes over time. There was wide variation in activity. Total step counts across all time points were below required levels for health benefits (mean 4592 SD 3411). Most activity was spread across short bouts. While most number of bouts was of low intensity, most time was spent in moderate intensity ambulation across all time points. Daily step count and time spent walking and sitting/lying increased from one month to three and six months. The number of and time spent in short and medium duration bouts increased from one to six months. Time in long duration bouts increased at three months only. Time spent in moderate intensity ambulation increased over time. No change was observed for any other measures. In future, it would be valuable to identify strategies to increase engagement in activity behaviours to improve health outcomes after stroke.



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Collagen type IV-related nephropathies in Portugal: pathogenic COL4A3 and COL4A4 mutations and clinical characterization of 25 families.

Related Articles

Collagen type IV-related nephropathies in Portugal: pathogenic COL4A3 and COL4A4 mutations and clinical characterization of 25 families.

Clin Genet. 2015 Nov;88(5):456-61

Authors: Nabais Sá MJ, Storey H, Flinter F, Nagel M, Sampaio S, Castro R, Araújo JA, Gaspar MA, Soares C, Oliveira A, Henriques AC, da Costa AG, Abreu CP, Ponce P, Alves R, Pinho L, Silva SE, de Moura CP, Mendonça L, Carvalho F, Pestana M, Alves S, Carvalho F, Oliveira JP

Abstract
Pathogenic mutations in genes COL4A3/COL4A4 are responsible for autosomal Alport syndrome (AS) and thin basement membrane nephropathy (TBMN). We used Sanger sequencing to analyze all exons and splice site regions of COL4A3/COL4A4, in 40 unrelated Portuguese probands with clinical suspicion of AS/TBMN. To assess genotype-phenotype correlations, we compared clinically relevant phenotypes/outcomes between homozygous/compound heterozygous and apparently heterozygous patients. Seventeen novel and four reportedly pathogenic COL4A3/COL4A4 mutations were identified in 62.5% (25/40) of the probands. Regardless of the mutated gene, all patients with ARAS manifested chronic renal failure (CRF) and hearing loss, whereas a minority of the apparently heterozygous patients had CRF or extrarenal symptoms. CRF was diagnosed at a significantly younger age in patients with ARAS. In our families, the occurrence of COL4A3/COL4A4 mutations was higher, while the prevalence of XLAS was lower than expected. Overall, a pathogenic COL4A3/COL4A4/COL4A5 mutation was identified in >50% of patients with fewer than three of the standard diagnostic criteria of AS. With such a population background, simultaneous next-generation sequencing of all three genes may be recommended as the most expedite approach to diagnose collagen IV-related glomerular basement membrane nephropathies.

PMID: 25307543 [PubMed - indexed for MEDLINE]

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Growth rate of vestibular schwannoma.

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Growth rate of vestibular schwannoma.

J Clin Neurosci. 2016 Jul 20;

Authors: Paldor I, Chen AS, Kaye AH

Abstract
Vestibular schwannoma (VS) is the most common tumor in the extra-axial posterior fossa compartment in adults. Growth rate is paramount to decision making regarding treatment and follow up of these tumors. We conducted a comprehensive review of the literature to answer four questions: What percentage of newly diagnosed VS will grow on follow-up? What factors correlate to tumor growth? What is the "normal" growth rate for sporadic VS? What factors characterize VS with rapid growth? Thirty-seven reports, with more than 4000 patients, fit our review criteria. One third of newly diagnosed VS will grow on follow-up of 1-3years. However, after 5years, up to one half will grow. Patient age and sex do not influence growth of VS. Hearing loss and vertigo at presentation do not predict tumor growth. It is unclear whether balance disturbance or tinnitus predict tumor growth. Tumor size and location do not predict tumor growth. Growth in the first year of observation is a strong predictor of tumor growth. The average growth rate of a VS is 0.99-1.11mm/year. However, the expected growth rate for VS that have been shown to grow at first follow-up is 3mm/year. Factors that may predict tumor growth of above 4mm/year are cystic and hemorrhagic features in the tumor, and hormonal treatment. VS grow at an average 1mm/year. VS that have been shown to grow at first follow-up should be considered for treatment, unless contraindicated. Long term follow-up is recommended for VS.

PMID: 27450283 [PubMed - as supplied by publisher]

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Growth rate of vestibular schwannoma.

Related Articles

Growth rate of vestibular schwannoma.

J Clin Neurosci. 2016 Jul 20;

Authors: Paldor I, Chen AS, Kaye AH

Abstract
Vestibular schwannoma (VS) is the most common tumor in the extra-axial posterior fossa compartment in adults. Growth rate is paramount to decision making regarding treatment and follow up of these tumors. We conducted a comprehensive review of the literature to answer four questions: What percentage of newly diagnosed VS will grow on follow-up? What factors correlate to tumor growth? What is the "normal" growth rate for sporadic VS? What factors characterize VS with rapid growth? Thirty-seven reports, with more than 4000 patients, fit our review criteria. One third of newly diagnosed VS will grow on follow-up of 1-3years. However, after 5years, up to one half will grow. Patient age and sex do not influence growth of VS. Hearing loss and vertigo at presentation do not predict tumor growth. It is unclear whether balance disturbance or tinnitus predict tumor growth. Tumor size and location do not predict tumor growth. Growth in the first year of observation is a strong predictor of tumor growth. The average growth rate of a VS is 0.99-1.11mm/year. However, the expected growth rate for VS that have been shown to grow at first follow-up is 3mm/year. Factors that may predict tumor growth of above 4mm/year are cystic and hemorrhagic features in the tumor, and hormonal treatment. VS grow at an average 1mm/year. VS that have been shown to grow at first follow-up should be considered for treatment, unless contraindicated. Long term follow-up is recommended for VS.

PMID: 27450283 [PubMed - as supplied by publisher]

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Capturing contextual effects in spectro-temporal receptive fields

Publication date: Available online 27 July 2016
Source:Hearing Research
Author(s): Johan Westö, Patrick J.C. May
Spectro-temporal receptive fields (STRFs) are thought to provide descriptive images of the computations performed by neurons along the auditory pathway. However, their validity can be questioned because they rely on a set of assumptions that are probably not fulfilled by real neurons exhibiting contextual effects, that is, nonlinear interactions in the time or frequency dimension that cannot be described with a linear filter. We used a novel approach to investigate how a variety of contextual effects, due to facilitating nonlinear interactions and synaptic depression, affect different STRF models, and if these effects can be captured with a context field (CF). Contextual effects were incorporated in simulated networks of spiking neurons, allowing one to define the true STRFs of the neurons. This, in turn, made it possible to evaluate the performance of each STRF model by comparing the estimations with the true STRFs. We found that currently used STRF models are particularly poor at estimating inhibitory regions. Specifically, contextual effects make estimated STRFs dependent on stimulus density in a contrasting fashion: inhibitory regions are underestimated at lower densities while artificial inhibitory regions emerge at higher densities. The CF was found to provide a solution to this dilemma, but only when it is used together with a generalized linear model. Our results therefore highlight the limitations of the traditional STRF approach and provide useful recipes for how different STRF models and stimuli can be used to arrive at reliable quantifications of neural computations in the presence of contextual effects. The results therefore push the purpose of STRF analysis from simply finding an optimal stimulus toward describing context-dependent computations of neurons along the auditory pathway.



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Dopamine agonists for preventing future miscarriage in women with idiopathic hyperprolactinemia and recurrent miscarriage history.

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Dopamine agonists for preventing future miscarriage in women with idiopathic hyperprolactinemia and recurrent miscarriage history.

Cochrane Database Syst Rev. 2016 Jul 25;7:CD008883

Authors: Chen H, Fu J, Huang W

Abstract
BACKGROUND: Hyperprolactinemia is the presence of abnormally high circulating levels of prolactin. Idopathic hyperprolactinemia is the term used when no cause of prolactin hypersecretion can be identified and it is causally related to the development of miscarriage in pregnant women, especially women who have a history of recurrent miscarriage. A possible mechanism is that high levels of prolactin affect the function of the ovaries, resulting in a luteal phase defect and miscarriage. A dopamine agonist is a compound with high efficacy in lowering prolactin levels and restoring gonadal function.
OBJECTIVES: To assess the effectiveness and safety of different types of dopamine agonists in preventing future miscarriage given to women with idiopathic hyperprolactinemia and a history of recurrent miscarriage.
SEARCH METHODS: We searched the Cochrane Pregnancy and Childbirth Group's Trials Register (30 June 2016) and reference lists of retrieved studies.
SELECTION CRITERIA: Randomized controlled trials (RCTs) in all languages examining the effect of dopamine agonists on preventing future miscarriage. Women who had idiopathic hyperprolactinemia with a history of recurrent miscarriages were eligible for inclusion in this review. Comparisons planned included: dopamine agonists alone versus placebo/no treatment; and dopamine agonists combined with other therapy versus other therapy alone.
DATA COLLECTION AND ANALYSIS: Two review authors independently assessed a single trial for inclusion, evaluated trial quality and extracted data. Data were checked for accuracy.
MAIN RESULTS: One study (recruiting 48 women with idiopathic hyperprolactinemia) met our inclusion criteria; 46 women (42 pregnancies - 4/46 women did not conceive during the study period) were included in the analysis. The study compared the use of a dopamine agonist (bromocriptine, 2.5 mg to 5.0 mg/day until the end of the ninth week of gestation) versus a no-treatment control. The study was judged as being at a high risk of bias. It was not possible to carry out meta-analysis due to insufficient data.The study reported both of this review's primary outcomes of miscarriage and live birth. Results from this single study suggest that, compared to no treatment, oral bromocriptine was effective in preventing future miscarriage (risk ratio (RR) 0.28, 95% confidence interval (CI) 0.09 to 0.87, 46 participants (low-quality evidence)) in women with idiopathic hyperprolactinemia. There was no clear difference with regard to the other primary outcome of live births (RR 1.50, 95% CI 0.93 to 2.42, 46 participants (very low-quality evidence)).There was no difference with regard to this review's secondary outcome of conception (RR 0.92, 95% CI 0.77 to 1.09, 46 participants (very low-quality evidence)) between the group of women who received dopamine (21 out of 24 women conceived) and women in the no-treatment group (21 out of 22 women conceived). The included study only reported the serum prolactin levels in pregnant women and therefore the data could not be analyzed in this review. No other secondary outcomes relevant to this review were reported; adverse effects for women (nausea, vomiting, headache, vertigo, fatigue, hypotension, arrhythmia, and psychotic symptoms) and infants (birth defects, low birthweight, and developmental disabilities) were not reported.We downgraded the quality of the evidence for risk of bias in the one trial contributing outcome data (no description of allocation concealment, lack of blinding and possible reporting bias) and for imprecision (all effect estimates were based on small sample size, miscarriage was based on few events, and the 95% CIs of live birth and conception cross the line of no effect).
AUTHORS' CONCLUSIONS: Currently, there is insufficient evidence (from a single randomized trial with a small sample size, and judged to be at high risk of bias) to evaluate the effectiveness of dopamine agonists for preventing future miscarriage in women with idiopathic hyperprolactinemia and a history of recurrent miscarriage. We assessed outcomes using GRADE methodology. Miscarriage was assessed as low quality due to risk of bias concerns in the one trial contributing data (no description of allocation concealment, lack of blinding and possible reporting bias) and to imprecision (effect estimates were based on small sample size and few events). Live births and conception were assessed as of very low quality due to the same risk of bias concerns in study design and to imprecision (with a wide 95% CI consistent with either benefit or harm), and a small sample size. There were no data relating to adverse effects of the intervention for either the mother or her baby.Futher high-quality research in this area is warranted. There is a need for well-designed, larger RCTs to confirm and extend the findings of the trial reviewed here. Many questions remain unanswered. Some important considerations for future research include, the need for well-designed RCTs with large sample sizes, and for those studies to consider important outcomes (including adverse effects for both the mother and her baby). Future studies should examine the effectiveness and safety of various dopamine agonists including bromocriptine, cabergoline and quinagolide.

PMID: 27455388 [PubMed - as supplied by publisher]

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Capturing contextual effects in spectro-temporal receptive fields

Publication date: Available online 27 July 2016
Source:Hearing Research
Author(s): Johan Westö, Patrick J.C. May
Spectro-temporal receptive fields (STRFs) are thought to provide descriptive images of the computations performed by neurons along the auditory pathway. However, their validity can be questioned because they rely on a set of assumptions that are probably not fulfilled by real neurons exhibiting contextual effects, that is, nonlinear interactions in the time or frequency dimension that cannot be described with a linear filter. We used a novel approach to investigate how a variety of contextual effects, due to facilitating nonlinear interactions and synaptic depression, affect different STRF models, and if these effects can be captured with a context field (CF). Contextual effects were incorporated in simulated networks of spiking neurons, allowing one to define the true STRFs of the neurons. This, in turn, made it possible to evaluate the performance of each STRF model by comparing the estimations with the true STRFs. We found that currently used STRF models are particularly poor at estimating inhibitory regions. Specifically, contextual effects make estimated STRFs dependent on stimulus density in a contrasting fashion: inhibitory regions are underestimated at lower densities while artificial inhibitory regions emerge at higher densities. The CF was found to provide a solution to this dilemma, but only when it is used together with a generalized linear model. Our results therefore highlight the limitations of the traditional STRF approach and provide useful recipes for how different STRF models and stimuli can be used to arrive at reliable quantifications of neural computations in the presence of contextual effects. The results therefore push the purpose of STRF analysis from simply finding an optimal stimulus toward describing context-dependent computations of neurons along the auditory pathway.



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Capturing contextual effects in spectro-temporal receptive fields

Publication date: Available online 27 July 2016
Source:Hearing Research
Author(s): Johan Westö, Patrick J.C. May
Spectro-temporal receptive fields (STRFs) are thought to provide descriptive images of the computations performed by neurons along the auditory pathway. However, their validity can be questioned because they rely on a set of assumptions that are probably not fulfilled by real neurons exhibiting contextual effects, that is, nonlinear interactions in the time or frequency dimension that cannot be described with a linear filter. We used a novel approach to investigate how a variety of contextual effects, due to facilitating nonlinear interactions and synaptic depression, affect different STRF models, and if these effects can be captured with a context field (CF). Contextual effects were incorporated in simulated networks of spiking neurons, allowing one to define the true STRFs of the neurons. This, in turn, made it possible to evaluate the performance of each STRF model by comparing the estimations with the true STRFs. We found that currently used STRF models are particularly poor at estimating inhibitory regions. Specifically, contextual effects make estimated STRFs dependent on stimulus density in a contrasting fashion: inhibitory regions are underestimated at lower densities while artificial inhibitory regions emerge at higher densities. The CF was found to provide a solution to this dilemma, but only when it is used together with a generalized linear model. Our results therefore highlight the limitations of the traditional STRF approach and provide useful recipes for how different STRF models and stimuli can be used to arrive at reliable quantifications of neural computations in the presence of contextual effects. The results therefore push the purpose of STRF analysis from simply finding an optimal stimulus toward describing context-dependent computations of neurons along the auditory pathway.



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Capturing contextual effects in spectro-temporal receptive fields

Publication date: Available online 27 July 2016
Source:Hearing Research
Author(s): Johan Westö, Patrick J.C. May
Spectro-temporal receptive fields (STRFs) are thought to provide descriptive images of the computations performed by neurons along the auditory pathway. However, their validity can be questioned because they rely on a set of assumptions that are probably not fulfilled by real neurons exhibiting contextual effects, that is, nonlinear interactions in the time or frequency dimension that cannot be described with a linear filter. We used a novel approach to investigate how a variety of contextual effects, due to facilitating nonlinear interactions and synaptic depression, affect different STRF models, and if these effects can be captured with a context field (CF). Contextual effects were incorporated in simulated networks of spiking neurons, allowing one to define the true STRFs of the neurons. This, in turn, made it possible to evaluate the performance of each STRF model by comparing the estimations with the true STRFs. We found that currently used STRF models are particularly poor at estimating inhibitory regions. Specifically, contextual effects make estimated STRFs dependent on stimulus density in a contrasting fashion: inhibitory regions are underestimated at lower densities while artificial inhibitory regions emerge at higher densities. The CF was found to provide a solution to this dilemma, but only when it is used together with a generalized linear model. Our results therefore highlight the limitations of the traditional STRF approach and provide useful recipes for how different STRF models and stimuli can be used to arrive at reliable quantifications of neural computations in the presence of contextual effects. The results therefore push the purpose of STRF analysis from simply finding an optimal stimulus toward describing context-dependent computations of neurons along the auditory pathway.



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Capturing contextual effects in spectro-temporal receptive fields

Publication date: Available online 27 July 2016
Source:Hearing Research
Author(s): Johan Westö, Patrick J.C. May
Spectro-temporal receptive fields (STRFs) are thought to provide descriptive images of the computations performed by neurons along the auditory pathway. However, their validity can be questioned because they rely on a set of assumptions that are probably not fulfilled by real neurons exhibiting contextual effects, that is, nonlinear interactions in the time or frequency dimension that cannot be described with a linear filter. We used a novel approach to investigate how a variety of contextual effects, due to facilitating nonlinear interactions and synaptic depression, affect different STRF models, and if these effects can be captured with a context field (CF). Contextual effects were incorporated in simulated networks of spiking neurons, allowing one to define the true STRFs of the neurons. This, in turn, made it possible to evaluate the performance of each STRF model by comparing the estimations with the true STRFs. We found that currently used STRF models are particularly poor at estimating inhibitory regions. Specifically, contextual effects make estimated STRFs dependent on stimulus density in a contrasting fashion: inhibitory regions are underestimated at lower densities while artificial inhibitory regions emerge at higher densities. The CF was found to provide a solution to this dilemma, but only when it is used together with a generalized linear model. Our results therefore highlight the limitations of the traditional STRF approach and provide useful recipes for how different STRF models and stimuli can be used to arrive at reliable quantifications of neural computations in the presence of contextual effects. The results therefore push the purpose of STRF analysis from simply finding an optimal stimulus toward describing context-dependent computations of neurons along the auditory pathway.



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Academy Responds to Consumer Reports Hearing Aid Guide 2016

Like Consumer Reports, the American Academy of Audiology places a high priority on providing individuals with hearing loss and their families with easily accessible, accurate information that will address common questions and guide them to appropriate intervention. Because we share a common goal of directing those with hearing loss to meaningful resources that will improve outcomes and satisfaction, we were compelled to draw attention to several inaccuracies in the recent publication that may be misleading.

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Quantifying Six-Minute Walk Induced Gait Deterioration with Inertial Sensors in Multiple Sclerosis Subjects

Publication date: Available online 27 July 2016
Source:Gait & Posture
Author(s): Matthew M. Engelhard, Sriram Raju Dandu, Stephen D. Patek, John C. Lach, Myla D. Goldman
BackgroundThe six-minute walk (6MW) is a common walking outcome in multiple sclerosis (MS) thought to measure fatigability in addition to overall walking disability. However, direct evidence of 6MW induced gait deterioration is limited by the difficulty of measuring qualitative changes in walking.ObjectivesThis study aims to (1) define and validate a measure of fatigue-related gait deterioration based on data from body-worn sensors; and (2) use this measure to detect gait deterioration induced by the 6MW.MethodsGait deterioration was assessed using the Warp Score, a measure of similarity between gait cycles based on dynamic time warping (DTW). Cycles from later minutes were compared to baseline cycles in 89 subjects with MS and 29 controls. Correlation, corrected (partial) correlation, and linear regression were used to quantify relationships to walking and fatigue outcomes.ResultsWarp Scores rose between minute 3 and minute 6 in subjects with mild and moderate disability (p<0.001). Statistically significant correlations (p<0.001) to the MS walking scale (MSWS-12), modified fatigue impact scale (MFIS) physical subscale, and cerebellar and pyramidal functional system scores (FSS) were observed even after controlling for walking speed. Regression of MSWS-12 scores on Warp Scores and walking speed explained 73.9% of response variance. Correlations to individual MSWS-12 and MFIS items strongly suggest a relationship to fatigability.ConclusionThe Warp Score has been validated in MS subjects as an objective measure of fatigue-related gait deterioration. Progressive changes to gait cycles induced by the 6MW often appeared in later minutes, supporting the importance of sustained walking in clinical assessment.



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Reliability and minimum detectable change of the gait profile score for post-stroke patients

Publication date: Available online 26 July 2016
Source:Gait & Posture
Author(s): Gisele Francini Devetak, Suzane Ketlyn Martello, Juliana Carla de Almeida, Katren Pedroso Correa, Dielise Debona Iucksch, Elisangela Ferretti Manffra
The objectives of this work were (i) to determine Gait Profile Score (GPS) for hemiparetic stroke patients, (ii) to evaluate its reliability within and between sessions, and (iii) to establish its minimal detectable change (MDC). Seventeen hemiparetic patients (mean age 54.9±10.5years; 9 men and 8 women; 6 hemiparetic on the left side and 11 on the right side; mean time after stroke 6.1±3.5months) participated in 2 gait assessment sessions within an interval of 2–7 days. Intra-session reliability was obtained from the intraclass correlation coefficient (ICC) between the three strides of each session. Inter-session reliability was estimated by the ICC from the averages of that three strides. GPS value of non paretic lower limb (NPLL) (13.9±2.4°) was greater than that of paretic lower limb (PLL) (12.0±2.8°) and overall GPS (GPS_O) was 13.7±2.5°. The Gait Variable Scores (GVS), GPS and GPS_O exhibited intra-session ICC values between 0.70 and 0.99, suggesting high intra-day stability. Most of GVS exhibited excellent inter-session reliability (ICC between 0.81 and 0.93). Only hip rotation, hip abduction of PLL exhibited moderate reliability with ICC/MDC values of 0.57/10.0° and 0.71/3.1°, respectively. ICC/MDC values of GPS were 0.92/2.3° and 0.93/1.9° for PLL and NPLL, respectively. GPS_O exhibited excellent test-retest reliability (ICC=0.95) and MDC of 1.7°. Given its reliability, the GPS has proven to be a suitable tool for therapeutic assessment of hemiparetic patients after stroke.



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Quantifying Six-Minute Walk Induced Gait Deterioration with Inertial Sensors in Multiple Sclerosis Subjects

Publication date: Available online 27 July 2016
Source:Gait & Posture
Author(s): Matthew M. Engelhard, Sriram Raju Dandu, Stephen D. Patek, John C. Lach, Myla D. Goldman
BackgroundThe six-minute walk (6MW) is a common walking outcome in multiple sclerosis (MS) thought to measure fatigability in addition to overall walking disability. However, direct evidence of 6MW induced gait deterioration is limited by the difficulty of measuring qualitative changes in walking.ObjectivesThis study aims to (1) define and validate a measure of fatigue-related gait deterioration based on data from body-worn sensors; and (2) use this measure to detect gait deterioration induced by the 6MW.MethodsGait deterioration was assessed using the Warp Score, a measure of similarity between gait cycles based on dynamic time warping (DTW). Cycles from later minutes were compared to baseline cycles in 89 subjects with MS and 29 controls. Correlation, corrected (partial) correlation, and linear regression were used to quantify relationships to walking and fatigue outcomes.ResultsWarp Scores rose between minute 3 and minute 6 in subjects with mild and moderate disability (p<0.001). Statistically significant correlations (p<0.001) to the MS walking scale (MSWS-12), modified fatigue impact scale (MFIS) physical subscale, and cerebellar and pyramidal functional system scores (FSS) were observed even after controlling for walking speed. Regression of MSWS-12 scores on Warp Scores and walking speed explained 73.9% of response variance. Correlations to individual MSWS-12 and MFIS items strongly suggest a relationship to fatigability.ConclusionThe Warp Score has been validated in MS subjects as an objective measure of fatigue-related gait deterioration. Progressive changes to gait cycles induced by the 6MW often appeared in later minutes, supporting the importance of sustained walking in clinical assessment.



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Reliability and minimum detectable change of the gait profile score for post-stroke patients

Publication date: Available online 26 July 2016
Source:Gait & Posture
Author(s): Gisele Francini Devetak, Suzane Ketlyn Martello, Juliana Carla de Almeida, Katren Pedroso Correa, Dielise Debona Iucksch, Elisangela Ferretti Manffra
The objectives of this work were (i) to determine Gait Profile Score (GPS) for hemiparetic stroke patients, (ii) to evaluate its reliability within and between sessions, and (iii) to establish its minimal detectable change (MDC). Seventeen hemiparetic patients (mean age 54.9±10.5years; 9 men and 8 women; 6 hemiparetic on the left side and 11 on the right side; mean time after stroke 6.1±3.5months) participated in 2 gait assessment sessions within an interval of 2–7 days. Intra-session reliability was obtained from the intraclass correlation coefficient (ICC) between the three strides of each session. Inter-session reliability was estimated by the ICC from the averages of that three strides. GPS value of non paretic lower limb (NPLL) (13.9±2.4°) was greater than that of paretic lower limb (PLL) (12.0±2.8°) and overall GPS (GPS_O) was 13.7±2.5°. The Gait Variable Scores (GVS), GPS and GPS_O exhibited intra-session ICC values between 0.70 and 0.99, suggesting high intra-day stability. Most of GVS exhibited excellent inter-session reliability (ICC between 0.81 and 0.93). Only hip rotation, hip abduction of PLL exhibited moderate reliability with ICC/MDC values of 0.57/10.0° and 0.71/3.1°, respectively. ICC/MDC values of GPS were 0.92/2.3° and 0.93/1.9° for PLL and NPLL, respectively. GPS_O exhibited excellent test-retest reliability (ICC=0.95) and MDC of 1.7°. Given its reliability, the GPS has proven to be a suitable tool for therapeutic assessment of hemiparetic patients after stroke.



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Quantifying Six-Minute Walk Induced Gait Deterioration with Inertial Sensors in Multiple Sclerosis Subjects

Publication date: Available online 27 July 2016
Source:Gait & Posture
Author(s): Matthew M. Engelhard, Sriram Raju Dandu, Stephen D. Patek, John C. Lach, Myla D. Goldman
BackgroundThe six-minute walk (6MW) is a common walking outcome in multiple sclerosis (MS) thought to measure fatigability in addition to overall walking disability. However, direct evidence of 6MW induced gait deterioration is limited by the difficulty of measuring qualitative changes in walking.ObjectivesThis study aims to (1) define and validate a measure of fatigue-related gait deterioration based on data from body-worn sensors; and (2) use this measure to detect gait deterioration induced by the 6MW.MethodsGait deterioration was assessed using the Warp Score, a measure of similarity between gait cycles based on dynamic time warping (DTW). Cycles from later minutes were compared to baseline cycles in 89 subjects with MS and 29 controls. Correlation, corrected (partial) correlation, and linear regression were used to quantify relationships to walking and fatigue outcomes.ResultsWarp Scores rose between minute 3 and minute 6 in subjects with mild and moderate disability (p<0.001). Statistically significant correlations (p<0.001) to the MS walking scale (MSWS-12), modified fatigue impact scale (MFIS) physical subscale, and cerebellar and pyramidal functional system scores (FSS) were observed even after controlling for walking speed. Regression of MSWS-12 scores on Warp Scores and walking speed explained 73.9% of response variance. Correlations to individual MSWS-12 and MFIS items strongly suggest a relationship to fatigability.ConclusionThe Warp Score has been validated in MS subjects as an objective measure of fatigue-related gait deterioration. Progressive changes to gait cycles induced by the 6MW often appeared in later minutes, supporting the importance of sustained walking in clinical assessment.



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Reliability and minimum detectable change of the gait profile score for post-stroke patients

Publication date: Available online 26 July 2016
Source:Gait & Posture
Author(s): Gisele Francini Devetak, Suzane Ketlyn Martello, Juliana Carla de Almeida, Katren Pedroso Correa, Dielise Debona Iucksch, Elisangela Ferretti Manffra
The objectives of this work were (i) to determine Gait Profile Score (GPS) for hemiparetic stroke patients, (ii) to evaluate its reliability within and between sessions, and (iii) to establish its minimal detectable change (MDC). Seventeen hemiparetic patients (mean age 54.9±10.5years; 9 men and 8 women; 6 hemiparetic on the left side and 11 on the right side; mean time after stroke 6.1±3.5months) participated in 2 gait assessment sessions within an interval of 2–7 days. Intra-session reliability was obtained from the intraclass correlation coefficient (ICC) between the three strides of each session. Inter-session reliability was estimated by the ICC from the averages of that three strides. GPS value of non paretic lower limb (NPLL) (13.9±2.4°) was greater than that of paretic lower limb (PLL) (12.0±2.8°) and overall GPS (GPS_O) was 13.7±2.5°. The Gait Variable Scores (GVS), GPS and GPS_O exhibited intra-session ICC values between 0.70 and 0.99, suggesting high intra-day stability. Most of GVS exhibited excellent inter-session reliability (ICC between 0.81 and 0.93). Only hip rotation, hip abduction of PLL exhibited moderate reliability with ICC/MDC values of 0.57/10.0° and 0.71/3.1°, respectively. ICC/MDC values of GPS were 0.92/2.3° and 0.93/1.9° for PLL and NPLL, respectively. GPS_O exhibited excellent test-retest reliability (ICC=0.95) and MDC of 1.7°. Given its reliability, the GPS has proven to be a suitable tool for therapeutic assessment of hemiparetic patients after stroke.



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Hearing test may identify autism risk

Researchers have identified an inner ear deficiency in children with Autism that may impact their ability to recognize speech.

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Hearing test may identify autism risk

Researchers have identified an inner ear deficiency in children with Autism that may impact their ability to recognize speech.

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Hearing test may identify autism risk

Researchers have identified an inner ear deficiency in children with Autism that may impact their ability to recognize speech.

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Seasonal variations in auditory processing in the inferior colliculus of Eptesicus fuscus

Publication date: Available online 26 July 2016
Source:Hearing Research
Author(s): Kimberly E. Miller, Kaitlyn Barr, Mitchell Krawczyk, Ellen Covey
Eptesicus fuscus is typical of temperate zone bats in that both sexes undergo marked seasonal changes in behavior, endocrine status, and reproductive status. Acoustic communication plays a key role in many seasonal behaviors. For example, males emit specialized vocalizations during mating in the fall, and females use different specialized vocalizations to communicate with infants in late spring. Bats of both sexes use echolocation for foraging during times of activity, but engage in little sound-directed behavior during torpor and hibernation in winter. Auditory processing might be expected to reflect these marked seasonal changes.To explore the possibility that seasonal changes in hormonal status could drive functional plasticity in the central auditory system, we examined responses of single neurons in the inferior colliculus throughout the year.The average first-spike latency in females varied seasonally, almost doubling in spring compared to other times of year. First-spike latencies in males remained relatively stable throughout the year. Latency jitter for both sexes was higher in winter and spring than in summer or fall.Females had more burst responders than other discharge patterns throughout the year whereas males had more transient responders at all times of year except fall, when burst responses were the predominant type. The percentage of simple discharge patterns (sustained and transient) was higher in males than females in the spring and higher in females than males in the fall.In females, the percentage of shortpass duration-tuned neurons doubled in summer and remained elevated through fall and early winter. In males, the percentage of shortpass duration-tuned cells increased in spring and the percentage of bandpass duration-tuned cells doubled in the fall.These findings suggest that there are clear seasonal changes in basic response characteristics of midbrain auditory neurons in Eptesicus, especially in temporal response properties and duration sensitivity. Moreover, the pattern of changes is different in males and females, suggesting that hormone-driven plasticity adjusts central auditory processing to fit the characteristics of vocalizations specific to seasonal behavioral patterns.



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Acoustic input impedance of the avian inner ear measured in ostrich (Struthio camelus)

Publication date: Available online 26 July 2016
Source:Hearing Research
Author(s): Pieter G.G. Muyshondt, Peter Aerts, Joris J.J. Dirckx
In both mammals and birds, the mechanical behavior of the middle ear structures is affected by the mechanical impedance of the inner ear. In this study, the aim was to quantify the acoustic impedance of the avian inner ear in the ostrich, which allows us to determine the effect on columellar vibrations and middle ear power flow in future studies. To determine the inner ear impedance, vibrations of the columella were measured for both the quasi-static and acoustic stimulus frequencies. In the frequency range of 0.3-4 kHz, we used electromagnetic stimulation of the ossicle and a laser Doppler vibrometer to measure the vibration response. At low frequencies, harmonic displacements were imposed on the columella using piezo stimulation and the resulting force response was measured with a force sensor. From these measurement data, the acoustic impedance of the inner ear could be determined. A simple RLC model in series of the impedance measurements resulted in a stiffness reactance of KIE = 0.20·10¹² Pa/m³, an inertial impedance of MIE = 0.652·10⁶ Pa·s²/m³, and a resistance of RIE = 1.57·10⁹ Pa·s/m. We found that values of the inner ear impedance in the ostrich are one to two orders in magnitude smaller than what is found in mammal ears.



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