Abstract:
OBJECTIVE:This paper reports a consanguineous deaf family with three different mutations in the GJB2 gene. DESIGN:Four members of an Iranian deaf family were recruited in this study. The GJB2 coding region and exon-intron boundaries were investigated using direct sequencing. STUDY SAMPLE:The proposita was a 12-year-old girl with congenital non-syndromic hearing loss. She was born to consanguineous parents. The proposita, her parents and deaf maternal uncle were screened for GJB2 mutations. RESULTS:Sequencing demonstrated the presence of the c.176_191del and c.327_328delGGinsA mutations in the proposita, the c.176_191del mutation in her father, and the c.35delG and c.327_328delGGinsA mutations in trans in her apparently unaffected mother as well as in her congenitally deaf uncle. Follow-up pure-tone audiometry revealed moderate to severe mid- and high-frequency hearing loss in the mother. CONCLUSIONS:This study shows the complexity of genetic testing and counseling for hearing loss.
journal_name
Int J Audioljournal_title
International journal of audiologyauthors
Davoudi-Dehaghani E,Fallah MS,Shirzad T,Tavakkoly-Bazzaz J,Bagherian H,Zeinali Sdoi
10.3109/14992027.2013.850748subject
Has Abstractpub_date
2014-02-01 00:00:00pages
128-31issue
2eissn
1499-2027issn
1708-8186journal_volume
53pub_type
杂志文章abstract::Children with listening difficulties, but normal audiometry, may be diagnosed with APD. The diagnosis is typically based on poor performance on tests of perception of both non-speech and speech stimuli. However, non-speech test results correlate only weakly with evaluations of speech-in-noise processing, cognitive ski...
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
abstract:OBJECTIVE:A controlled field experiment was conducted to evaluate localization of suprathreshold gunshot reports (from blank cartridges) with four hearing protection-enhancement devices (HPEDs) in comparison to the open ear with ambient outdoor noise and in 82 dBA diesel military heavy truck noise. DESIGN:Five measure...
journal_title:International journal of audiology
pub_type: 临床试验,杂志文章
doi:10.3109/14992027.2011.631591
更新日期:2012-02-01 00:00:00
abstract:OBJECTIVE:In a clinical setting, theories of health behaviour change could help audiologists and other hearing health care professionals understand the barriers that prevent people with hearing problems to seek audiological help. The transtheoretical (stages of change) model of health behaviour change is one of these t...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992027.2017.1309466
更新日期:2017-07-01 00:00:00
abstract:OBJECTIVE:To estimate the prevalence of severe dual sensory loss (DSL) among older persons (aged ≥65 years) in the Swedish population, to identify the diagnoses that cause severe DSL, and to identify rehabilitation services in which the participants have been involved. DESIGN:A cross-sectional design was applied. Medi...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992027.2020.1783003
更新日期:2020-12-01 00:00:00
abstract:OBJECTIVE:The purpose of this study is to determine the relationship between occupational exposures and hearing among elderly Latino Americans. DESIGN:A descriptive, correlational design used for this secondary analysis with the data from the Sacramento Area Latino Study of Aging (SALSA). STUDY SAMPLE:A total of 547 ...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2014.973541
更新日期:2015-02-01 00:00:00
abstract:OBJECTIVE:This study investigated categorical loudness scaling in a large group of cochlear implant (CI) recipients. DESIGN:Categorical loudness was measured for individually determined sets of current amplitudes on apical, mid and basal electrodes of the Nucleus array. STUDY SAMPLE:Thirty adult subjects implanted wi...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992027.2017.1339129
更新日期:2017-11-01 00:00:00
abstract::This study examined group differences on a variety of measures for three groups of elderly hearing aid candidates matched for gender, age and hearing loss. These three groups were: (1) 26 candidates who declined amplification (non-adherents); (2) 24 individuals who purchased hearing aids, but subsequently returned the...
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
abstract::The effect of speech processing on articulatory-feature recognition was studied in a group of hearing-impaired listeners with high-frequency sensorineural losses. Individual difference scaling (INDSCAL) and sequential information analysis (SINFA) were applied to a set of consonant-vowel-consonant responses measured un...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992020209090419
更新日期:2002-10-01 00:00:00
abstract::In a laboratory study, we found that normal-hearing and hearing-impaired listeners preferred less than normal overall calculated loudness (according to a loudness model of Moore & Glasberg, 1997). The current study verified those results using a research hearing aid. Fifteen hearing-impaired and eight normal-hearing p...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992020500190177
更新日期:2006-01-01 00:00:00
abstract:OBJECTIVE:To measure masked detection and reaction thresholds for two reverse alarms (tonal and broadband) and compare results to available standards and psychoacoustic criteria for setting alarm levels. DESIGN:Alarm detection and reaction thresholds were adaptively measured in 80-dBA background noises without hearing...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992027.2017.1400188
更新日期:2018-02-01 00:00:00
abstract::In this study, we present a new method to derive a single-number measure of the directivity of hearing aids. The method is an extension of the conventional directivity index (DI), and is called overall directivity index (ODI). The directivities of five different hearing aid styles were compared with that of the open e...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992020209101312
更新日期:2002-01-01 00:00:00
abstract::This experiment investigated the effect of small-group versus individual hearing loss prevention (HLP) training on the attenuation performance of passive insert-type hearing protection devices (HPDs). A subject-fit (SF) methodology, which gave naive listeners access only to the instructions printed on the HPD product ...
journal_title:International journal of audiology
pub_type: 杂志文章,随机对照试验
doi:10.1080/14992020701438805
更新日期:2007-10-01 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:This study aimed to provide guidelines to optimize perception of soft speech and speech in noise for Advanced Bionics cochlear implant (CI) users. DESIGN:Three programs differing in T-levels were created for ten subjects. Using the T-level setting that provided the lowest FM-tone, sound-field threshold level...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2010.533200
更新日期:2011-04-01 00:00:00
abstract::Tympanometry using 226 Hz, 678 Hz, and 1000 Hz probe tones was undertaken on two groups of babies, age 2 to 21 weeks. A group of 104 babies with normal ABR thresholds or TEOAEs were compared with a second group of 107 babies who had evidence of temporary conductive hearing loss based on the findings of a test battery,...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992020600690951
更新日期:2006-07-01 00:00:00
abstract:OBJECTIVE:To consider the evolving concept of auditory processing disorder (APD). DESIGN:Narrative review and opinion piece. STUDY SAMPLE:Eight approaches to APD described in the literature and its definitions by ten societies or groups from around the world. RESULTS:The dominant conceptualisation of APD remains one...
journal_title:International journal of audiology
pub_type: 杂志文章,评审
doi:10.1080/14992027.2017.1409438
更新日期:2018-04-01 00:00:00
abstract:OBJECTIVE:This study surveyed youth recreational firearm users (YRFUs) regarding shooting habits, reported use of hearing protection devices (HPDs), self-assessed auditory status, and attitudes about firearm noise and hearing loss. DESIGN:A descriptive study using a 28-item survey administered by personal interview. ...
journal_title:International journal of audiology
pub_type: 杂志文章,多中心研究
doi:10.3109/14992027.2013.857437
更新日期:2014-03-01 00:00:00
abstract:OBJECTIVE:To investigate word recognition in noise in subjects treated in childhood with chemotherapy, study benefits of open-fitting hearing-aids for word recognition, and investigate whether self-reported hearing-handicap corresponded to subjects' word recognition ability. DESIGN:Subjects diagnosed with cancer and t...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2011.585667
更新日期:2011-10-01 00:00:00
abstract:OBJECTIVE:To use the self-determination theory of motivation to investigate whether different forms of motivation were associated with adults' decisions whether or not to adopt hearing aids. DESIGN:A quantitative approach was used in this cohort study. Participants completed the treatment self-regulation questionnaire...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2015.1007213
更新日期:2015-07-01 00:00:00
abstract::The speech intelligibility benefit of visual speech cues during oral communication is well-established. Therefore, an ecologically valid approach of auditory assessment should include the processing of both auditory and visual speech cues. This study describes the development and evaluation of a virtual human speaker ...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992027.2018.1511922
更新日期:2018-12-01 00:00:00
abstract:OBJECTIVE:The high energy demand of the auditory and visual pathways render these sensory systems prone to diseases that impair mitochondrial function. Primary open-angle glaucoma, a neurodegenerative disease of the optic nerve, has recently been associated with a spectrum of mitochondrial abnormalities. This study sou...
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
abstract::The objective of this study was to compare the electrically evoked compound action potentials, intra- versus post-operatively, in cochlear implant patients. In a prospective study twenty-five consecutively implanted adult patients received a multichannel cochlear implant. In all patients, electrically evoked compound ...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992020600833189
更新日期:2006-10-01 00:00:00
abstract::Tinnitus is a condition that is difficult to treat, and treatment outcomes are difficult to measure The majority of people who experience tinnitus are not troubled by it; however, the troubled minority are referred by an otorhinolaryngologist for specialist clinic help. The aim of this study was to investigate how the...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992020400050052
更新日期:2004-07-01 00:00:00
abstract:OBJECTIVE:To re-evaluate the current BSA recommendation that the test ear should be occluded during the bone-conduction procedure at frequencies above 2 kHz to prevent audible air-borne radiation. DESIGN:Pure-tone audiometry was undertaken during routine hearing tests. The audiograms of fifty-two ears met the criteria...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2015.1086029
更新日期:2015-01-01 00:00:00
abstract::The dynamics of contralateral acoustic suppression were studied using real time (millisecond resolution) distortion product otoacoustic emissions (DPOAEs) in chinchillas and humans. Latency of DPOAE suppression onset is 26 ms in chinchillas and 45 ms in humans. After onset, suppression builds over time before tending ...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992020400029996
更新日期:2005-02-01 00:00:00
abstract:OBJECTIVE:A significant relationship between dietary nutrient intake and susceptibility to acquired hearing loss is emerging. Variability in the outcomes across studies is likely related to differences in the specific metrics used to quantify nutrient intake and hearing status. Most studies have used single nutrient an...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992027.2013.780133
更新日期:2013-06-01 00:00:00
abstract::Objective: Recent changes to cochlear implant (CI) candidacy criteria have led to the inclusion of candidates with greater levels of hearing in the contralateral and/or implanted ear. This study assessed the impact of various hearing loss configurations on CI uptake rates (those assessed as eligible for CI, who procee...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992027.2020.1768445
更新日期:2020-11-01 00:00:00
abstract::The purpose of this study was to determine and to compare the overall intelligibility, articulation, resonance, and voice characteristics in children using cochlear implants (CI) and children using conventional hearing aids (HA). Nine prelingually deaf children using CI and six children with a prelingual severe hearin...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992020500189146
更新日期:2005-08-01 00:00:00
abstract::The aim of this study was to outline the psychopathological characteristics of a population of outpatients affected by tinnitus and to consider its impact on their mental state and ability to function in major areas of their lives. Seventy-five consecutive tinnitus patients were enrolled on their first visit to the ou...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.3109/14992020309056079
更新日期:2003-01-01 00:00:00
abstract::Objective: To compare the temporal processing skills and speech in noise perception of hearing-impaired individuals through channel free and multichannel hearing aids.Design: A quasi-experimental study.Study sample: Twenty-one participants with sensory neural hearing loss were involved in the study. They were subjecte...
journal_title:International journal of audiology
pub_type: 杂志文章
doi:10.1080/14992027.2019.1660005
更新日期:2019-12-01 00:00:00