The sources and consequences of a sensorineural hearing loss are diverse. While several approaches have aimed at disentangling the physiological and perceptual consequences of different etiologies, hearing deficit characterization and rehabilitation have been dominated by the results from pure-tone audiometry. Here, we present a novel approach based on data-driven profiling of perceptual auditory deficits that attempts to represent auditory phenomena that are usually hidden by, or entangled with, audibility loss. We hypothesize that the hearing deficits of a given listener, both at hearing threshold and at suprathreshold sound levels, result from two independent types of "auditory distortions." In this two-dimensional space, four distinct "auditory profiles" can be identified. To test this hypothesis, we gathered a data set consisting of a heterogeneous group of listeners that were evaluated using measures of speech intelligibility, loudness perception, binaural processing abilities, and spectrotemporal resolution. The subsequent analysis revealed that distortion type-I was associated with elevated hearing thresholds at high frequencies and reduced temporal masking release and was significantly correlated with elevated speech reception thresholds in noise. Distortion type-II was associated with low-frequency hearing loss and abnormally steep loudness functions. The auditory profiles represent four robust subpopulations of hearing-impaired listeners that exhibit different degrees of perceptual distortions. The four auditory profiles may provide a valuable basis for improved hearing rehabilitation, for example, through profile-based hearing-aid fitting.
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http://dx.doi.org/10.1177/2331216520973539 | DOI Listing |
FASEB J
January 2025
Laboratory of Molecular Pharmacology, Biosignal Research Center, Kobe University, Kobe, Japan.
DFNA1 (deafness, nonsyndromic autosomal dominant 1), initially identified as nonsyndromic sensorineural hearing loss, has been associated with an additional symptom: macrothrombocytopenia. However, the timing of the onset of hearing loss (HL) and thrombocytopenia has not been investigated, leaving it unclear which occurs earlier. Here, we generated a knock-in (KI) DFNA1 mouse model, diaphanous-related formin 1 (DIA1), in which Aequorea coerulescens green fluorescent protein (AcGFP)-tagged human DIA1(p.
View Article and Find Full Text PDFMov Disord
January 2025
Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Objective: Pathogenic variants in B-cell receptor-associated protein (BCAP31) are associated with X-linked, deafness, dystonia and cerebral hypomyelination (DDCH) syndrome. DDCH is congenital and non-progressive, featuring severe intellectual disability (ID), variable dysmorphism, and sometimes associated with shortened survival. BCAP31 encodes one of the most abundant chaperones, with several functions including acting as a negative regulator of endoplasmic reticulum (ER) calcium ion (Ca) concentration.
View Article and Find Full Text PDFTher Adv Ophthalmol
January 2025
Department of Ophthalmology, Zealand University Hospital, Roskilde, Denmark.
Background: Age-related macular degeneration (AMD) is a prevalent cause of irreversible vision loss among the elderly. The prevalence and detailed characteristics of Charles Bonnet syndrome (CBS) remain largely unexplored in patients with geographic atrophy (GA) secondary to AMD.
Objectives: To investigate the prevalence and characteristics of CBS in patients with GA secondary to AMD.
Cureus
December 2024
Ear Nose and Throat, Bahrain Defense Force Hospital, Royal Medical Services, Manama, BHR.
Objective: The aim of this study was to assess hearing level of preschoolers with delayed speech in order to detect any underlying hearing loss Methods: In this research we targeted preschool children with speech delay, who have not been previously diagnosed with any medical or psychological illnesses. A total of 54 preschool speech-delayed children were audiologically assessed in our clinic in the past year. The age at time of referral ranged from two to 7.
View Article and Find Full Text PDFNatl J Maxillofac Surg
November 2024
Department of ENT, Jubilee Mission Medical College and Research Institute, Thrissur, Kerala, India.
Background And Objectives: Serous otitis media (SOM), also called otitis media with effusion (OME) or glue ear, is a collection of non-purulent fluid within the middle ear space. Children with cleft palate are more prone to develop this condition. This is caused by impaired eustachian tube function in cleft palate.
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