Monogenic Causes of Low-Frequency Non-Syndromic Hearing Loss
Background: Low-frequency non-syndromic hearing loss (LFNSHL) is a rare form of hearing loss (HL). It is defined as HL at low frequencies ( ≤2,000 Hz) resulting in a characteristic ascending audiogram. LFNSHL is usually diagnosed postlingually and is progressive, leading to HL affecting other frequencies as well. Sometimes it occurs with tinnitus. Around half of the diagnosed prelingual HL cases have a genetic cause and it is usually inherited in an autosomal recessive mode. Postlingual HL caused by genetic changes generally has an autosomal dominant pattern of inheritance and its incidence remains unknown.Summary: To da...
Source: Audiology and Neurotology - April 28, 2023 Category: Audiology Source Type: research
Hearing Loss Related to Gene Mutations in Distal Renal Tubular Acidosis
Conclusions: LVAS is common in patients with dRTA and may influence the type and severity of hearing loss in these patients. The possibility of both congenital and late-onset and progressive hearing loss should be considered in dRTA patients. A regular audiological follow-up is essential for the early detection of a possible late-onset or progressive hearing loss in these patients.Audiol Neurotol (Source: Audiology and Neurotology)
Source: Audiology and Neurotology - April 28, 2023 Category: Audiology Source Type: research
Audiovestibular Symptoms of Severe Acute Respiratory Syndrome Coronavirus-2 Infection
Audiol Neurotol (Source: Audiology and Neurotology)
Source: Audiology and Neurotology - April 26, 2023 Category: Audiology Source Type: research
Influence of Speech Recognition Ability on Acceptable Noise Level for Mandarin (Chinese) Speakers with Normal Hearing
Conclusion: Speech recognition in noise appears to play an important role for listeners with normal hearing in deciding their ANLs. Additionally, ANL can predict speech performance (r-squared = 53 –61%) in the degraded noise condition.Audiol Neurotol (Source: Audiology and Neurotology)
Source: Audiology and Neurotology - April 25, 2023 Category: Audiology Source Type: research
A Novel Biallelic Variant in < i > CDH23 < /i > Gene in a Family with Atypical USH1D Manifestation: A Literature Review and Investigation of Genotype-Phenotype Correlation
Conclusion: By reporting a novel damaging variant, we expand the mutational spectrum of theCDH23 gene that leads to atypical USH1D. Also, reviewing the literature shows that, contrary to previous claims, different genotypes occur in theCDH23 gene allelic disorders, and there is no clear-cut genotype-phenotype correlation.Audiol Neurotol (Source: Audiology and Neurotology)
Source: Audiology and Neurotology - April 21, 2023 Category: Audiology Source Type: research
Augmented Virtual Reality in Vestibular Assessment: A Dynamic Gait Application
Conclusion: The accurate quantification of gait, movement along the walking axis, and deviation from the normality using HL2 provide an initial evidence for its useful adoption as a valuable tool in gait and mobility assessment.Audiol Neurotol (Source: Audiology and Neurotology)
Source: Audiology and Neurotology - April 18, 2023 Category: Audiology Source Type: research
Direct Elicitation of Cortical Auditory Evoked Potentials by Electrical Stimulation and Their Use to Verify the Most Comfortable Level of Stimulation in Cochlear Implant Users
Conclusion: eCAEPs can readily be elicited in the majority of adult CI users and show normal waveform characteristics at stimulation levels corresponding to MCL, as well as at basal, medial, and apical electrode stimulation sites. Neither the latencies nor amplitudes of eCAEPs are confounded by variables of age, duration of deafness prior to CI surgery, or the laterality of hearing loss. eCAEPs are a useful, objective method evaluate sound perception in CI users.Audiol Neurotol (Source: Audiology and Neurotology)
Source: Audiology and Neurotology - March 23, 2023 Category: Audiology Source Type: research
Event-Related Potentials of Single-Sided Deaf Cochlear Implant Users: Using a Semantic Oddball Paradigm in Noise
Conclusion: The inconsistency between the behavioural and neural results suggests that EEG may not be a reliable measure of cognitive effort. This rationale is further supported by different explanations used in past studies to explain N2N4 and P3b effects. Future studies should look to alternative measures of auditory processing (e.g., pupillometry) to gain a deeper understanding of the underlying auditory processes that facilitate speech-in-noise intelligibility.Audiol Neurotol (Source: Audiology and Neurotology)
Source: Audiology and Neurotology - March 20, 2023 Category: Audiology Source Type: research
Test-Retest Reliability of the Coordinate Response Measure in Adults with Normal Hearing or Cochlear Implants
Conclusions: NH adults had significantly lower CRM SRTs than the CI recipients;t (31.16) = −23.91,p #x3c; 0.001. CRM had better replicability, stability and lower variability for the CI adults compared to NH adults.Audiol Neurotol 2023;28:84 –93 (Source: Audiology and Neurotology)
Source: Audiology and Neurotology - February 22, 2023 Category: Audiology Source Type: research
Test-Retest Reliability of the Coordinate Response Measure in Adults with Normal Hearing or Cochlear Implants
Conclusions: NH adults had significantly lower CRM SRTs than the CI recipients;t (31.16) = −23.91,p #x3c; 0.001. CRM had better replicability, stability and lower variability for the CI adults compared to NH adults.Audiol Neurotol (Source: Audiology and Neurotology)
Source: Audiology and Neurotology - February 22, 2023 Category: Audiology Source Type: research
The Relationship between the Characteristics of Tinnitus and the Hearing Curative Effect of Sudden Deafness
Background: Tinnitus is the most common complication of sudden deafness. There are many studies on tinnitus and tinnitus as a prognostic factor for sudden deafness.Summary: We collected 285 cases (330 ears) of sudden deafness to investigate the relationship between tinnitus psychoacoustic characteristics and the hearing curative effective rate. The hearing curative effective rate was analyzed and compared between the patients whether it is accompanied by tinnitus, with different tinnitus frequency and different tinnitus loudness.Key Messages: Patients with tinnitus frequency (125 –2,000 Hz) and no tinnitus have better he...
Source: Audiology and Neurotology - February 21, 2023 Category: Audiology Source Type: research
Residual Hearing Does Not Influence the Effectiveness of Beamforming when Using a Cochlear Implant in Conjunction with Contralateral Routing of Signals
Discussion: We recommend the use of BB over MB for CI-CROS users. Residual hearing in the CROS ear is not a limiting factor for fitting a CROS device, although a bimodal option should be considered.Audiol Neurotol (Source: Audiology and Neurotology)
Source: Audiology and Neurotology - February 15, 2023 Category: Audiology Source Type: research
