Chapter 26 AUDITORY PROCESSING IN SENSORINEURAL HEARING LOSS

2006 ◽  
2020 ◽  
Author(s):  
Sarineh Keshishzadeh ◽  
Markus Garrett ◽  
Sarah Verhulst

AbstractOver the past decades, different types of auditory models have been developed to study the functioning of normal and impaired auditory processing. Several models can simulate frequency-dependent sensorineural hearing loss (SNHL), and can in this way be used to develop personalized audio-signal processing for hearing aids. However, to determine individualized SNHL profiles, we rely on indirect and non-invasive markers of cochlear and auditory-nerve (AN) damage. Our progressive knowledge of the functional aspects of different SNHL subtypes stresses the importance of incorporating them into the simulated SNHL profile, but has at the same time complicated the task of accomplishing this on the basis of non-invasive markers. In particular, different auditory evoked potential (AEP) types can show a different sensitivity to outer-hair-cell (OHC), inner-hair-cell (IHC) or AN damage, but it is not clear which AEP-derived metric is best suited to develop personalized auditory models. This study investigates how simulated and recorded AEPs can be used to derive individual AN- or OHC-damage patterns and personalize auditory processing models. First, we individualized the cochlear-model parameters using common methods of frequency-specific OHC-damage quantification, after which we simulated AEPs for different degrees of AN-damage. Using a classification technique, we determined the recorded AEP metric that best predicted the simulated individualized CS profiles. We cross-validated our method using the dataset at hand, but also applied the trained classifier to recorded AEPs from a new cohort to illustrate the generalisability of the method.


2017 ◽  
Vol 28 (01) ◽  
pp. 080-090
Author(s):  
Danielle Mercer ◽  
Annette Hurley ◽  
Fern Tsien

AbstractXeroderma pigmentosum (XP) is a rare autosomal recessive condition characterized by extreme sensitivity to ultraviolet light. Individuals with XP lack the ability to repair DNA (deoxyribonucleic acid) damage caused by ultraviolet radiation, leading to sunburn and increased susceptibility to skin cancers. Approximately 25% of patients also exhibit neural degeneration, which includes progressive mental deterioration, cortical thinning, and sensorineural hearing loss.Herein, we describe the audiological and genetic findings in a patient with XP subtype D with neural degeneration and hearing loss.This is a case report of a patient with XP subtype D, type 1 diabetes, and some clinical features typical of Charcot-Marie-Tooth disease.We obtained audiological evaluations over a course of 11 yr, including serial audiograms, auditory processing disorders evaluations, and electrophysiological testing.Hearing sensitivity has progressed from a unilateral mild high-frequency sensorineural hearing loss to a bilateral sloping moderate to severe/profound sensorineural hearing loss. In addition to the dramatic decline in hearing sensitivity, the patient demonstrates global auditory processing deficits, indicating a central component to his hearing loss.These findings emphasize the importance of the contribution of audiological evaluations to the diagnosis of a genetic disorder. Periodic evaluations of hearing sensitivity and auditory processing can provide information on disease progression in patients with XP with neural degeneration.


2019 ◽  
Author(s):  
Lorna Halliday ◽  
Stuart Rosen ◽  
Outi Tuomainen ◽  
Axelle Calcus

Psychophysical thresholds were measured for 8-16 year-old children with mild-to-moderate sensorineural hearing loss (MMHL; N = 46) on a battery of auditory processing tasks that included measures designed to be predominantly reliant upon frequency selectivity, and sensitivity to temporal fine structure (TFS) or envelope cues. Children with MMHL who wore hearing aids were tested in both unaided and aided conditions, and all were compared to a group of normally hearing (NH) age-matched controls. Children with MMHL performed more poorly than NH controls on tasks considered to be dependent upon frequency selectivity, sensitivity to TFS, and speech discrimination (/bɑ/-/dɑ/), but not on tasks measuring sensitivity to envelope cues. Auditory processing deficits remained regardless of age, were observed in both unaided and aided conditions, and could not be attributed to differences in nonverbal IQ or attention between groups. However, better auditory processing for children with MMHL was predicted by better audiometric thresholds and, for aided tasks only, higher levels of maternal education. These results suggest that, as for adults with MMHL, children with MMHL may show deficits in their frequency selectivity and sensitivity to TFS, but that sensitivity to envelope cues may remain intact.


2021 ◽  
Vol 25 ◽  
pp. 233121652098840 ◽  
Author(s):  
Sarineh Keshishzadeh ◽  
Markus Garrett ◽  
Sarah Verhulst

Over the past decades, different types of auditory models have been developed to study the functioning of normal and impaired auditory processing. Several models can simulate frequency-dependent sensorineural hearing loss (SNHL) and can in this way be used to develop personalized audio-signal processing for hearing aids. However, to determine individualized SNHL profiles, we rely on indirect and noninvasive markers of cochlear and auditory-nerve (AN) damage. Our progressive knowledge of the functional aspects of different SNHL subtypes stresses the importance of incorporating them into the simulated SNHL profile, but has at the same time complicated the task of accomplishing this on the basis of noninvasive markers. In particular, different auditory-evoked potential (AEP) types can show a different sensitivity to outer-hair-cell (OHC), inner-hair-cell (IHC), or AN damage, but it is not clear which AEP-derived metric is best suited to develop personalized auditory models. This study investigates how simulated and recorded AEPs can be used to derive individual AN- or OHC-damage patterns and personalize auditory processing models. First, we individualized the cochlear model parameters using common methods of frequency-specific OHC-damage quantification, after which we simulated AEPs for different degrees of AN damage. Using a classification technique, we determined the recorded AEP metric that best predicted the simulated individualized cochlear synaptopathy profiles. We cross-validated our method using the data set at hand, but also applied the trained classifier to recorded AEPs from a new cohort to illustrate the generalizability of the method.


2004 ◽  
Vol 15 (01) ◽  
pp. 006-016 ◽  
Author(s):  
Karin Neijenhuis ◽  
Hans Tschur ◽  
Ad Snik

The application of auditory processing tests to patients with sensorineural hearing loss is controversial. Several studies have shown that it is difficult to separate peripheral from central hearing processes. In the present study, a Dutch auditory processing test battery was administered to 24 subjects with mild, relatively flat, symmetrical sensorineural hearing loss. Tests were administered twice; the second time, the presentation level in four out of the six tests was adjusted according to the speech reception threshold. The scores of the hearing-impaired subjects were significantly poorer than those of the subjects with normal hearing on five out of the six tests, even with the adjusted presentation level. Significant correlations were found between test scores and PTA (pure-tone average); scores on words-in-noise, filtered-speech and binaural-fusion tests were additionally corrected according to PTA. In contrast to previous studies in the literature, the present dichotic-digit and pattern-recognition tests were greatly influenced by mild hearing loss. Therefore, this auditory processing test battery cannot readily be used to diagnose central auditory processing disorders in patients with flat sensorineural hearing loss. At least, both adjustment of presentation level and additional correction are needed.


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