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Journal Ear Hear

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Diagnostic Yield of Targeted Hearing Loss Gene Panel Sequencing in a Large German Cohort With a Balanced Age Distribution from a Single Diagnostic Center: An Eight-year Study.

Anke Tropitzsch, Thore Schade-Mann, Philipp Gamerdinger, Saskia Dofek, Björn Schulte, Martin Schulze, Florian Battke, Sarah Fehr, Saskia Biskup, Andreas Heyd, Marcus Müller, Hubert Löwenheim, Barbara Vona, Martin Holderried,

<h4>Objectives</h4>Hereditary hearing loss exhibits high degrees of genetic and clinical heterogeneity. To elucidate the population-specific and age-related genetic and clinical spectra of hereditary hearing loss, we investigated the sequencing data of causally associated hearing loss genes in a large cohort of hearing-impaired probands with a balanced age distribution from a ... Read more >>

Ear Hear (Ear and hearing)
[2021, :]

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The Importance of Extended High-Frequency Speech Information in the Recognition of Digits, Words, and Sentences in Quiet and Noise.

Sigrid Polspoel, Sophia E Kramer, Bas van Dijk, Cas Smits,

In pure-tone audiometry, hearing thresholds are typically measured up to 8 kHz. Recent research has shown that extended high-frequency (EHF; frequencies >8 kHz) speech information improves speech recognition. However, it is unclear whether the EHF benefit is present for different types of speech material. This study assesses the added value ... Read more >>

Ear Hear (Ear and hearing)
[2021, :]

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Understanding Self-reported Hearing Disability in Adults With Normal Hearing.

Aryn M Kamerer, Sara E Harris, Judy G Kopun, Stephen T Neely, Daniel M Rasetshwane,

<h4>Objectives</h4>Despite a diagnosis of normal hearing, many people experience hearing disability (HD) in their everyday lives. This study assessed the ability of a number of demographic and auditory variables to explain and predict self-reported HD in people regarded as audiologically healthy via audiometric thresholds.<h4>Design</h4>One-hundred eleven adults (ages 19 to 74) ... Read more >>

Ear Hear (Ear and hearing)
[2021, :]

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An Economic Evaluation of Australia's Newborn Hearing Screening Program: A Within-Study Cost-Effectiveness Analysis.

Rajan Sharma, Yuanyuan Gu, Kompal Sinha, Teresa Y C Ching, Vivienne Marnane, Lisa Gold, Melissa Wake, Jing Wang, Bonny Parkinson,

Hearing loss is one of the most prevalent congenital disorders among children. Many countries have implemented universal newborn hearing screening (UNHS) for the early diagnosis and treatment of hearing loss. Despite widespread implementation, the value for money of UNHS is unclear due to lack of cost and outcomes data from ... Read more >>

Ear Hear (Ear and hearing)
[2021, :]

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Acceptable Noise Levels and Preferred Signal-to-noise Ratios for Speech and Music.

Donguk Lee, James D Lewis, Patti M Johnstone, Patrick N Plyler,

<h4>Objectives</h4>The objective of this study was to evaluate the influence of music versus speech on a listener's signal-to-noise ratio (SNR) preferences when listening in a noise background. SNR preferences were quantified using acceptable noise level (ANL) and preferred SNR metrics. The measurement paradigm for ANL allows the listener to adjust ... Read more >>

Ear Hear (Ear and hearing)
[2021, :]

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Cost-Effectiveness of Screening Preschool Children for Hearing Loss in Australia.

Mutsa Gumbie, Bonny Parkinson, Harvey Dillon, Ross Bowman, Rachel Song, Henry Cutler,

<h4>Objectives</h4>While all newborns in Australia are tested for congenital hearing loss through universal newborn hearing screening programs, some children will acquire hearing loss in their first five years of life. Delayed diagnosed or undiagnosed hearing loss in children can have substantial immediate- and long-term consequences. It can significantly reduce school ... Read more >>

Ear Hear (Ear and hearing)
[2021, :]

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Genotype-Phenotype Correlation Study in a Large Series of Patients Carrying the p.Pro51Ser (p.P51S) Variant in COCH (DFNA9) Part II: A Prospective Cross-Sectional Study of the Vestibular Phenotype in 111 Carriers.

Sebastien P F JanssensdeVarebeke, Julie Moyaert, Erik Fransen, Britt Bulen, Celine Neesen, Katrien Devroye, Raymond van de Berg, Ronald J E Pennings, Vedat Topsakal, Olivier Vanderveken, Guy Van Camp, Vincent Van Rompaey,

<h4>Introduction</h4>DFNA9 is characterized by adult-onset hearing loss and evolution toward bilateral vestibulopathy (BVP). The genotype-phenotype correlation studies were conducted 15 years ago. However, their conclusions were mainly based on symptomatic carriers and the vestibular data exclusively derived from the horizontal (lateral) semicircular canal (SCC). The last decade was marked by ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1525-1543]

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Adult Auditory Brainstem Implant Outcomes and Three-Dimensional Electrode Array Position on Computed Tomography.

Dana Egra-Dagan, Isabeau van Beurden, Samuel R Barber, Christine L Carter, Mary E Cunnane, M Christian Brown, Barbara S Herrmann, Daniel J Lee,

<h4>Objectives</h4>Factors contributing to auditory brainstem implant (ABI) outcomes are poorly understood. The aims of this study are to (1) characterize ABI electrode array position on postoperative imaging and (2) determine if variability in position is related to perceptual outcomes.<h4>Design</h4>Retrospective cohort study. Subjects were selected from the adult ABI recipient population ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1741-1754]

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The Relationship Between Disorders of Bone Metabolism and Benign Paroxysmal Positional Vertigo: A Systematic Review.

Ambrose Lee, Fatemeh Hassannia, Michael J Bergin, Khalid Al Zaabi, Priyanka Misale, John Alexander Rutka,

<h4>Objectives</h4>Several studies have reported an association between benign paroxysmal positional vertigo (BPPV) and bone mineral density or serum vitamin D levels. The aim of this review is to provide further clarification regarding the relationship between BPPV and calcium metabolism.<h4>Design</h4>PubMed and MEDLINE databases were systematically reviewed to identify all English language ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1462-1471]

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Personalizing Transient Noise Reduction Algorithm Settings for Cochlear Implant Users.

H Christiaan Stronks, Annemijn L Tops, Phillipp Hehrmann, Jeroen J Briaire, Johan H M Frijns,

<h4>Objectives</h4>Speech understanding in noise is difficult for patients with a cochlear implant. One common and disruptive type of noise is transient noise. We have tested transient noise reduction (TNR) algorithms in cochlear implant users to investigate the merits of personalizing the noise reduction settings based on a subject's own preference.<h4>Design</h4>The ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1602-1614]

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Hearing Aid Noise Reduction Lowers the Sustained Listening Effort During Continuous Speech in Noise-A Combined Pupillometry and EEG Study.

Lorenz Fiedler, Tirdad Seifi Ala, Carina Graversen, Emina Alickovic, Thomas Lunner, Dorothea Wendt,

<h4>Objectives</h4>The investigation of auditory cognitive processes recently moved from strictly controlled, trial-based paradigms toward the presentation of continuous speech. This also allows the investigation of listening effort on larger time scales (i.e., sustained listening effort). Here, we investigated the modulation of sustained listening effort by a noise reduction algorithm as ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1590-1601]

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Cytomegalovirus-A Risk Factor for Childhood Hearing Loss: A Systematic Review.

Bénédicte Vos, Dorie Noll, JoAnne Whittingham, Marie Pigeon, Marlene Bagatto, Elizabeth M Fitzpatrick,

<h4>Objective</h4>Permanent hearing loss is an important public health issue in children with consequences for language, social, and academic functioning. Early hearing detection, intervention, and monitoring are important in mitigating the impact of permanent childhood hearing loss. Congenital cytomegalovirus (CMV) infection is a leading cause of hearing loss. The purpose of ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1447-1461]

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Cutting Through the Noise: Noise-Induced Cochlear Synaptopathy and Individual Differences in Speech Understanding Among Listeners With Normal Audiograms.

Mishaela DiNino, Lori L Holt, Barbara G Shinn-Cunningham,

Following a conversation in a crowded restaurant or at a lively party poses immense perceptual challenges for some individuals with normal hearing thresholds. A number of studies have investigated whether noise-induced cochlear synaptopathy (CS; damage to the synapses between cochlear hair cells and the auditory nerve following noise exposure that ... Read more >>

Ear Hear (Ear and hearing)
[2021, :]

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Evaluating the Association Between Hearing Loss and Falls in Adults With Vestibular Dysfunction or Nonvestibular Dizziness.

Ryan J Huang, Carl F Pieper, Heather E Whitson, Douglas B Garrison, Juliessa M Pavon, Kristal M Riska,

<h4>Objectives</h4>Although emerging evidence suggests that hearing loss (HL) is an independent risk factor for falls, it is unclear how HL may impact falls risk in adults with vestibular dysfunction and nonvestibular dizziness. The purpose of this study was to characterize the impact of HL on falls in patients with vestibular ... Read more >>

Ear Hear (Ear and hearing)
[2021, :]

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Listening Difficulties in Children With Normal Audiograms: Relation to Hearing and Cognition.

Lauren Petley, Lisa L Hunter, Lina Motlagh Zadeh, Hannah J Stewart, Nicholette T Sloat, Audrey Perdew, Li Lin, David R Moore,

<h4>Objectives</h4>Children presenting at audiology services with caregiver-reported listening difficulties often have normal audiograms. The appropriate approach for the further assessment and clinical management of these children is currently unclear. In this Sensitive Indicators of Childhood Listening Difficulties (SICLiD) study, we assessed listening ability using a reliable and validated caregiver questionnaire ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1640-1655]

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Effect of Test Realism on Speech-in-noise Outcomes in Bilateral Cochlear Implant Users.

Javier Badajoz-Davila, Jörg M Buchholz,

<h4>Objectives</h4>First, to evaluate the effect of laboratory-based test realism on speech intelligibility outcomes of cochlear implant users. Second, to conduct an exploratory investigation of speech intelligibility of cochlear implant users, including bilateral benefit, under realistic laboratory conditions.<h4>Design</h4>For the first goal, the authors measured speech intelligibility scores of 15 bilateral cochlear ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1687-1698]

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Auditory Brainstem Response Detection Using Machine Learning: A Comparison With Statistical Detection Methods.

Richard M McKearney, Steven L Bell, Michael A Chesnaye, David M Simpson,

<h4>Objectives</h4>The primary objective of this study was to train and test machine learning algorithms to be able to detect accurately whether EEG data contains an auditory brainstem response (ABR) or not and recommend suitable machine learning methods. In addition, the performance of the best machine learning algorithm was compared with ... Read more >>

Ear Hear (Ear and hearing)
[2021, :]

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Genotype-phenotype Correlation Study in a Large Series of Patients Carrying the p.Pro51Ser (p.P51S) Variant in COCH (DFNA9): Part I-A Cross-sectional Study of Hearing Function in 111 Carriers.

Sebastien P F JanssensdeVarebeke, Julie Moyaert, Erik Fransen, Britt Bulen, Celine Neesen, Katrien Devroye, Raymond van de Berg, Ronald J E Pennings, Vedat Topsakal, Olivier Vanderveken, Guy Van Camp, Vincent Van Rompaey,

<h4>Introduction</h4>DFNA9 is characterized by adult-onset progressive sensorineural hearing loss (SNHL) and vestibular impairment. More than 15 years ago, genotype-phenotype correlation studies estimated the initial age of hearing deterioration in the fourth to fifth decade (ranging from 32 to 43 years). However, these analyses were based on relatively limited numbers of ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1508-1524]

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Comparative Analysis of Cortical Auditory Evoked Potential in Cochlear Implant Users.

Dayse Távora-Vieira, Giacomo Mandruzzato, Marek Polak, Belinda Truong, Alex Stutley,

<h4>Objectives</h4>The primary goal of the study was to investigate electrical cortical auditory evoked potentials (eCAEPs) at maximum comfortable level (MCL) and 50% MCL on three cochlear implant (CI) electrodes and compare them with the acoustic CAEP (aCAEPs), in terms of the amplitude and latency of the P1-N1-P2 complex. This was ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1755-1769]

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Ecological Momentary Assessment: A Field Evaluation of Subjective Ratings of Speech in Noise.

Lorienne M Jenstad, Gurjit Singh, Michael Boretzki, Anita DeLongis, Elmar Fichtl, Rachel Ho, Myron Huen, Vincent Meyer, Flora Pang, Ellen Stephenson,

<h4>Purpose</h4>As hearing rehabilitation research evolves to include both retrospective and momentary assessment outcome measures, it is important to understand how in-the-moment contextual factors influence subjective ratings. We aimed to determine, over a 4-week period of participants responding to ecological momentary assessments (EMAs) in their own environments, whether: (1) participants will ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1770-1781]

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Evaluation of Extended-Wear Hearing Aids as a Solution for Intermittently Noise-Exposed Listeners With Hearing Loss.

LaGuinn P Sherlock, Trevor T Perry, Douglas S Brungart,

<h4>Objectives</h4>Many individuals with noise-related hearing loss continue working in environments where they are periodically exposed to high levels of noise, which increases their risk for further hearing loss. These individuals often must remove their hearing aids in operational environments because of incompatibility with the mandated personal protective equipment, thus reducing ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1544-1559]

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Development of Masked Speech Detection Thresholds in 2- to 15-year-old Children: Speech-Shaped Noise and Two-Talker Speech Maskers.

Angela Yarnell Bonino, Michael E Ramsey, Elizabeth S Pancoast, Eric A Vance,

<h4>Objectives</h4>On the basis of the data from school-aged children, there is consistent evidence that there is a prolonged course of auditory development for perceiving speech embedded in competing background sounds. Furthermore, age-related differences are prolonged and pronounced for a two-talker speech masker compared to a speech-shaped noise masker. However, little ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1712-1726]

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Cochlear Synaptopathy due to Mutations in OTOF Gene May Result in Stable Mild Hearing Loss and Severe Impairment of Speech Perception.

Rosamaria Santarelli, Pietro Scimemi, Marco Costantini, María Domínguez-Ruiz, Montserrat Rodríguez-Ballesteros, Ignacio Del Castillo,

<h4>Objectives</h4>Congenital profound hearing loss with preserved cochlear outer hair cell activity (otoacoustic emissions and cochlear microphonic) is the most common phenotype associated with mutations in the OTOF gene. The aim of this study was to investigate the pathophysiological mechanisms behind the auditory dysfunction in five patients (2 adults and 3 ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1627-1639]

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Predicting Hearing Aid Satisfaction in Adults: A Systematic Review of Speech-in-noise Tests and Other Behavioral Measures.

Alyssa Davidson, Nicole Marrone, Bryan Wong, Frank Musiek,

<h4>Objectives</h4>Adults with hearing loss report a wide range of hearing aid satisfaction that does not significantly correlate to degree of hearing loss. It is not clear which auditory behavioral factors do contribute to hearing aid satisfaction. While poor speech understanding in noise is known to contribute to dissatisfaction, there are ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1485-1498]

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The Presence of Another Individual Influences Listening Effort, But Not Performance.

Hidde Pielage, Adriana A Zekveld, Gabrielle H Saunders, Niek J Versfeld, Thomas Lunner, Sophia E Kramer,

<h4>Objectives</h4>The aim of this study was to modify a speech perception in noise test to assess whether the presence of another individual (copresence), relative to being alone, affected listening performance and effort expenditure. Furthermore, this study assessed if the effect of the other individual's presence on listening effort was influenced ... Read more >>

Ear Hear (Ear and hearing)
[2021, 42(6):1577-1589]

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