Congenital Nonprofound Bilateral Sensorineural Hearing Loss in Children: Comprehensive Characterization of Auditory Function and Hearing Aid Benefit

Berninger, Erik; Drott, Maria; Romanitan, Mircea; Tranebjærg, Lisbeth; Hellström, Sten

doi:10.3390/audiolres12050054

Cited by 3 publications

(2 citation statements)

References 84 publications

(134 reference statements)

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“…Fifty percent of the MRI scans showed an absence of a cochlear nerve in the IE ( n = 7/14), while 29% showed inner ear malformations ( n = 4/14) ( Table 1 ). The malformation incidence was 86% ( n = 6/7) in subjects with profound uSNHL, and also considerably high in subjects with non-profound uSNHL (43%, n = 3/7), in stark contrast to a diagnostic yield of 0% ( n = 0/8) for congenital non-profound and alleged non-syndromic bilateral SNHL, also recruited through Region Stockholm’s UNHS program [ 26 ].…”

Section: Resultsmentioning

confidence: 99%

A Prospective Study of Genetic Variants in Infants with Congenital Unilateral Sensorineural Hearing Loss

Johansson

Karltorp

Asp

et al. 2023

JCM

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Children with unilateral sensorineural hearing loss (uSNHL) have a high risk of speech-language delays and academic difficulties. Still, challenges remain in the diagnosis of uSNHL. With a prospective cross-sectional design, 20 infants were consecutively recruited from a universal newborn hearing screening program and invited to genetic testing. Eighteen of the subjects agreed to genetic testing, 15 subjects with OtoSCOPE® v.9 screening 224 genes, and four subjects underwent targeted testing, screening for chromosomal abnormalities or 105–137 gene mutations. The genetic results were described together with the 20 infants’ previously published auditory profiles and imaging results. Genetic causes for the uSNHL were found in 28% of subjects (5/18) including CHARGE syndrome (CHD7), autosomal recessive non-syndromic hearing loss (GJB2), Townes–Brocks syndrome (SALL1), Pendred Syndrome (SLC26A4) and Chromosome 8P inverted duplication and deletion syndrome. In subjects with comorbidities (malformation of fingers, anus, brain, and heart), 100% were diagnosed with a genetic cause for uSNHL (3/3 subjects), while 13% (2/15 subjects) were diagnosed without comorbidities observed at birth (p = 0.002). Genetic testing for congenital uSNHL is currently efficient for alleged syndromes, whereas genetic variants for non-syndromic congenital uSNHL need further research.

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Section: Resultsmentioning

confidence: 99%

A Prospective Study of Genetic Variants in Infants with Congenital Unilateral Sensorineural Hearing Loss

Johansson

Karltorp

Asp

et al. 2023

JCM

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“…The shift in the auditory threshold for detecting a pure tone relative to a normal ear is commonly used International Journal of Intelligent Engineering and Systems, Vol. 16 to quantify hearing loss [9]. Because of this, there are many different kinds of hearing aids with various features and functions to meet other demands.…”

Section: Introductionmentioning

confidence: 99%

Aquila Based Adaptive Filtering for Hearing Aid with Optimized Performance

2023

IJIES

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In recent days, one of the most serious health problems in humans is hearing loss. For helping the hearing impairment, the Hearing Aid (HA) system is used, but it may lack in various aspects such as delay, matching error, power consumption, low complexity and unwanted noise. Many techniques were introduced to overcome these issues, but they still have problems. Therefore, this research uses a novel Aquila-based Adaptive Filter (AbAF) for improving the HA system with the optimized parameters of filter. Here, the incorporation of the Aquila optimizer along with the adaptive filter is considered as the key novelty of this present work. The main motive of this proposed model is to filter the noisy features more possible range for improve the HA performance. Hence, the Aquila agent removes the unwanted noise by updating the adaptive processor's filter coefficient and optimizing the filter parameters such as matching error, delay, complexity, and bandwidth and power consumption. Moreover, the suggested model is designed and executed in the MATLAB platform. Finally, the performance parameters were estimated and compared with the past studies to validate the performance improvement. The presented model scored the reduced Matching error as 0.3% and reduced power consumption as 10%, which quite lower than the compared models.

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Click evoked otoacoustic emissions in occupational exposure to lead, concentrations of selected essential elements and markers of oxidative stress

Wąsik,

Lisowska,

Słota

et al. 2024

Eur Arch Otorhinolaryngol

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Purpose This study focused on the selected markers of oxidative stress, impact of elevated lead levels on long-term hearing quality. We investigated whether the presence of certain essential minerals might provide protection to the auditory system against the effects of lead (and cadmium) compounds. Methods The research group included 280 male employees of the zinc and lead smelter, which was divided into: L-Pb—low blood lead concentration (PbB) subgroup, H-Pb—high PbB subgroup. Hearing tests were performed using the click evoked otoacoustic emission (CEOAE). Results Zinc protoporphyrin level was significantly higher in the H-Pb subgroup by 68%. Cd concentration was significantly higher in H-Pb by 33%. The Ca concentration was significantly lower in the H-Pb by − 2%. Selected oxidative stress markers concentration were significantly higher in the H-Pb group: malondialdehyde (MDA) by 4%, and lipofuscin (LPS) by 9%. In the CEOAE results showed statistically significant differences between the L-Pb and H-Pb subgroups. Larger negative changes in otoemission amplitude were observed in H-Pb subgroup. All otoemission results showed a statistically significant negative correlation with age, time of work, MDA concentration, and with PbB. Selected CEOAE parameters showed a significant negative correlation with cadmium blood concentration (CdB), and a positive correlation with Ca and Zn. Conclusion Elevated blood lead content in occupational exposure is associated with an increase in MDA and LPS concentration, which negatively correlates with CEOAE parameters. This suggests an important role of oxidative stress in the long-term deterioration of hearing.

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Congenital Nonprofound Bilateral Sensorineural Hearing Loss in Children: Comprehensive Characterization of Auditory Function and Hearing Aid Benefit

Cited by 3 publications

References 84 publications

A Prospective Study of Genetic Variants in Infants with Congenital Unilateral Sensorineural Hearing Loss

A Prospective Study of Genetic Variants in Infants with Congenital Unilateral Sensorineural Hearing Loss

Aquila Based Adaptive Filtering for Hearing Aid with Optimized Performance

Click evoked otoacoustic emissions in occupational exposure to lead, concentrations of selected essential elements and markers of oxidative stress

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