Abstract:
:Objective: The primary goal of this study was to characterise the cochlear and neural components of hearing loss in a large cohort of people with Charcot-Marie Tooth neuropathy who reported hearing difficulties.Design: A full complement of audiologic measures including behavioral, physiologic and subjective assessments were administered.Study sample: Seventy-nine participants completed the study. Forty-four people had CMT1, 27 had CMT2 and four had CMT-INT.Results: Pure tone average was related to age and the absence of high-frequency DPOAE energy, suggesting a strong cochlear component. Acoustic reflexes were often elevated or absent and many participants exhibited abnormal ABR waveforms, suggesting additional neural hearing loss components. Participants with an abnormal or absent ABR wave V exhibited poorer speech perception abilities. There was an association between a prolonged ABR wave I latency and an abnormal or absent ABR wave V with a higher Charcot-Marie Tooth Neuropathy Score (indicating greater disability).Conclusions: The hearing abilities of people with CMT are highly variable. While there were strong neural hearing loss components, speech perception abilities were not disproportionately affected in most participants. Therefore, a hearing aid trial is recommended. ABR responses may be a useful tool for monitoring the progression of CMT over time.
journal_name
Int J Audioljournal_title
International journal of audiologyauthors
Giuliani N,Holte L,Shy M,Grider Tdoi
10.1080/14992027.2019.1633022subject
Has Abstractpub_date
2019-12-01 00:00:00pages
902-912issue
12eissn
1499-2027issn
1708-8186journal_volume
58pub_type
杂志文章abstract::Objective: This clinical note describes the Individualised - Active Communication Education (I-ACE) programme designed to improve problem solving and self-management in adults with hearing impairment. Design: The I-ACE was offered to adult clients seeking help for the first time and effects were measured for participa...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992027.2019.1587180
更新日期:2019-08-01 00:00:00
abstract:OBJECTIVE:A study was conducted to determine whether modifications to input compression and input frequency response characteristics can improve music-listening satisfaction in cochlear implant users. DESIGN:Experiment 1 compared three pre-processed versions of music and speech stimuli in a laboratory setting: origina...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2014.986689
更新日期:2015-06-01 00:00:00
abstract:OBJECTIVE:To investigate the effect of consecutive 1000-Hz tympanometry testing on admittance measures, and examine test-retest reliability. DESIGN:Repeated measures with eight trials of 1000-Hz Y(a) and B(a)/G(a) tympanometry, respectively, in two ears of each subject, followed by repeated 226-Hz tympanometry. STUDY...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2014.955888
更新日期:2015-04-01 00:00:00
abstract:OBJECTIVE:The current study investigates the extent to which the linguistic complexity of three commonly employed speech recognition tests and second language proficiency influence speech recognition thresholds (SRTs) in noise in non-native listeners. DESIGN:SRTs were measured for non-natives and natives using three G...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2015.1063715
更新日期:2015-01-01 00:00:00
abstract:OBJECTIVE:The purpose of this review was to evaluate the current literature on phosphoribosylpyrophosphate synthetase 1 (PRPS1)-related diseases and their consequences on hearing function. DESIGN:A literature search of peer-reviewed, published journal articles was conducted in online bibliographic databases. STUDY SA...
journal_title:International journal of audiology
pub_type: 杂志文章,评审
doi:10.3109/14992027.2012.736032
更新日期:2013-01-01 00:00:00
abstract::Age-related effects on several parameters of hearing aid provision, comprising the importance of various hearing aid attributes, the main reason for dissatisfaction, and a measure for aided performance, were examined. When comparing two subgroups of younger and elderly hearing aid users, a significant difference occur...
journal_title:International journal of audiology
pub_type: 杂志文章,多中心研究
doi:
更新日期:2003-07-01 00:00:00
abstract:OBJECTIVE:To clarify the impact of hearing aids on mental health, social engagement, cognitive function, and physical health outcomes in older adults with hearing impairment. DESIGN:We assessed hearing handicap (hearing handicap inventory for the elderly; HHIE-S), cognition (mini mental state exam, trail making, audit...
journal_title:International journal of audiology
pub_type: 杂志文章
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2014.900195
更新日期:2014-09-01 00:00:00
abstract::The effect of inverting electrode location and stimulus perceptibility on the presence of the mismatch negativity (MMN) evoked response was investigated in 20 normal-hearing adult females. Perceptible and imperceptible frequency contrasts were employed in an oddball paradigm. The perceptible contrast consisted of an 1...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992020802283241
更新日期:2008-11-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
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更新日期:2008-11-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992020601145310
更新日期:2007-04-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
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abstract:OBJECTIVE:Labyrinthine concussion due to a postauricular gunshot wound has not been well reported. DESIGN:Retrospective chart review. STUDY SAMPLE:We describe an otherwise healthy 22-year-old male who received a gunshot wound to the left mastoid and subsequently reported hearing loss and rotational vertigo. RESULTS:...
journal_title:International journal of audiology
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2013.798687
更新日期:2013-09-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992020309078338
更新日期:2003-03-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章,随机对照试验
doi:10.3109/14992027.2012.705903
更新日期:2013-12-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2010.503664
更新日期:2010-12-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992027.2016.1226520
更新日期:2016-12-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:
更新日期:2004-02-01 00:00:00
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pub_type: 杂志文章
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更新日期:2021-01-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992020802572635
更新日期:2009-04-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992020309080053
更新日期:2003-10-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
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更新日期:2020-03-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2013.821208
更新日期:2013-11-01 00:00:00
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journal_title:International journal of audiology
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doi:10.3109/14992027.2015.1117663
更新日期:2016-07-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2013.824117
更新日期:2013-11-01 00:00:00
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journal_title:International journal of audiology
pub_type: 临床试验,杂志文章,随机对照试验
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更新日期:2004-03-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2012.723143
更新日期:2013-01-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992027.2016.1214756
更新日期:2016-11-01 00:00:00
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journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2011.609185
更新日期:2012-01-01 00:00:00