20Q: Hearing Loss and Its Comorbidities

From the Desk of Gus MuellerTell me if you’ve heard this one before.  Your Monday morning patient is in his 50s, has a history of some noise exposure (occasional hunting), and states that over the past few years he is starting to have trou

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20Q: Hearing Loss and Its Comorbidities

From the Desk of Gus MuellerTell me if you’ve heard this one before.  Your Monday morning patient is in his 50s, has a history of some noise exposure (occasional hunting), and states that over the past few years he is starting to have trou

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20Q: Hearing Loss and Its Comorbidities

From the Desk of Gus MuellerTell me if you’ve heard this one before.  Your Monday morning patient is in his 50s, has a history of some noise exposure (occasional hunting), and states that over the past few years he is starting to have trou

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A movie where the hero has tinnitus… and it’s good?

Baby Driver is a movie about a getaway car driver who has tinnitus. From what I can tell, it’s the first Hollywood movie where the hero has tinnitus. Seeing tinnitus sufferers represented onscreen is a really wonderful thing, and humanizes us further.

Currently, the film has an 86% rating on MetaCritic, a review aggregator. The reviews are in, and it’s great!

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Mutations in KARS cause early-onset hearing loss and leukoencepha lopathy: Potential pathogenic mechanism.

Related Articles

Mutations in KARS cause early-onset hearing loss and leukoencepha lopathy: Potential pathogenic mechanism.

Hum Mutat. 2017 Sep 08;:

Authors: Zhou XL, He LX, Yu LJ, Wang Y, Wang XJ, Wang ED, Yang T

Abstract
Leukoencephalopathies are a broad class of common neurologic deterioration for which the etiology remain unsolved in many cases. In a Chinese Han family segregated with sensorineural hearing loss and leukoencephalopathy, candidate pathogenic variants were identified by targeted next-generation sequencing of 144 genes associated with deafness and 108 genes with leukoencephalopathy. Novel compound heterozygous mutations p.R477H and p.P505S were identified in KARS, which encodes lysyl-tRNA synthetase (LysRS), as the only candidate causative variants. These two mutations were functionally characterized by enzymatic assays, immunofluorescence, circular dichroism analysis and gel filtration chromatography. Despite no alteration in the dimer-tetramer oligomerization and cellular distribution by either mutation, the protein structure was notably influenced by the R477H mutation, which subsequently released the protein from the multiple-synthetase complex (MSC). Mutant LysRSs with the R477H and P505S mutations had decreased tRNA(Lys) aminoacylation and displayed a cumulative effect when introduced simultaneously. Our studies showed that mutations in KARS lead to a newly defined subtype of leukoencephalopathy associated with sensorineural hearing impairment. The combined effect of reduced aminoacylation and release of LysRS from the MSC likely underlies the pathogenesis of the KARS mutations identified in this study. This article is protected by copyright. All rights reserved.

PMID: 28887846 [PubMed - as supplied by publisher]

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Longitudinal Changes in Electrically Evoked Auditory Event-Related Potentials in Children With Auditory Brainstem Implants: Preliminary Results Recorded Over 3 Years.

Objectives: This preliminary study aimed (1) to assess longitudinal changes in electrically evoked auditory event-related potentials (eERPs) in children with auditory brainstem implants (ABIs) and (2) to explore whether these changes could be accounted for by maturation in the central auditory system of these patients. Design: Study participants included 5 children (S1 to S5) with an ABI in the affected ear. The stimulus was a train of electrical pulses delivered to individual ABI electrodes via a research interface. For each subject, the eERP was repeatedly measured in multiple test sessions scheduled over up to 41 months after initial device activation. Longitudinal changes in eERPs recorded for each ABI electrode were evaluated using intraclass correlation tests for each subject. Results: eERPs recorded in S1 showed notable morphological changes for five ABI electrodes over 41 months. In parallel, signs or symptoms of nonauditory stimulation elicited by these electrodes were observed or reported at 41 months. eERPs could not be observed in S2 after 9 months of ABI use but were recorded at 12 months after initial stimulation. Repeatable eERPs were recorded in S3 in the first 9 months. However, these responses were either absent or showed remarkable morphological changes at 30 months. Longitudinal changes in eERP waveform morphology recorded in S4 and S5 were also observed. Conclusions: eERP responses in children with ABIs could change over a long period of time. Maturation of the central auditory system could not fully account for these observed changes. Children with ABIs need to be closely monitored for potential changes in auditory perception and unfavorable nonauditory sensations. Neuroimaging correlates are needed to better understand the emergence of nonauditory stimulation over time in these children. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Longitudinal Changes in Electrically Evoked Auditory Event-Related Potentials in Children With Auditory Brainstem Implants: Preliminary Results Recorded Over 3 Years.

Objectives: This preliminary study aimed (1) to assess longitudinal changes in electrically evoked auditory event-related potentials (eERPs) in children with auditory brainstem implants (ABIs) and (2) to explore whether these changes could be accounted for by maturation in the central auditory system of these patients. Design: Study participants included 5 children (S1 to S5) with an ABI in the affected ear. The stimulus was a train of electrical pulses delivered to individual ABI electrodes via a research interface. For each subject, the eERP was repeatedly measured in multiple test sessions scheduled over up to 41 months after initial device activation. Longitudinal changes in eERPs recorded for each ABI electrode were evaluated using intraclass correlation tests for each subject. Results: eERPs recorded in S1 showed notable morphological changes for five ABI electrodes over 41 months. In parallel, signs or symptoms of nonauditory stimulation elicited by these electrodes were observed or reported at 41 months. eERPs could not be observed in S2 after 9 months of ABI use but were recorded at 12 months after initial stimulation. Repeatable eERPs were recorded in S3 in the first 9 months. However, these responses were either absent or showed remarkable morphological changes at 30 months. Longitudinal changes in eERP waveform morphology recorded in S4 and S5 were also observed. Conclusions: eERP responses in children with ABIs could change over a long period of time. Maturation of the central auditory system could not fully account for these observed changes. Children with ABIs need to be closely monitored for potential changes in auditory perception and unfavorable nonauditory sensations. Neuroimaging correlates are needed to better understand the emergence of nonauditory stimulation over time in these children. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Longitudinal Changes in Electrically Evoked Auditory Event-Related Potentials in Children With Auditory Brainstem Implants: Preliminary Results Recorded Over 3 Years.

Objectives: This preliminary study aimed (1) to assess longitudinal changes in electrically evoked auditory event-related potentials (eERPs) in children with auditory brainstem implants (ABIs) and (2) to explore whether these changes could be accounted for by maturation in the central auditory system of these patients. Design: Study participants included 5 children (S1 to S5) with an ABI in the affected ear. The stimulus was a train of electrical pulses delivered to individual ABI electrodes via a research interface. For each subject, the eERP was repeatedly measured in multiple test sessions scheduled over up to 41 months after initial device activation. Longitudinal changes in eERPs recorded for each ABI electrode were evaluated using intraclass correlation tests for each subject. Results: eERPs recorded in S1 showed notable morphological changes for five ABI electrodes over 41 months. In parallel, signs or symptoms of nonauditory stimulation elicited by these electrodes were observed or reported at 41 months. eERPs could not be observed in S2 after 9 months of ABI use but were recorded at 12 months after initial stimulation. Repeatable eERPs were recorded in S3 in the first 9 months. However, these responses were either absent or showed remarkable morphological changes at 30 months. Longitudinal changes in eERP waveform morphology recorded in S4 and S5 were also observed. Conclusions: eERP responses in children with ABIs could change over a long period of time. Maturation of the central auditory system could not fully account for these observed changes. Children with ABIs need to be closely monitored for potential changes in auditory perception and unfavorable nonauditory sensations. Neuroimaging correlates are needed to better understand the emergence of nonauditory stimulation over time in these children. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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