Background: Binaural hearing is of utmost importance for communicating in noisy surroundings and localizing the direction of sound. Unilateral hearing loss (UHL) affects the quality of life in both childhood and adulthood, speech development, and academic achievements. Sound amplification using air-conducting hearing aids (HAs) is a common option for hearing rehabilitation of UHL. The processing time of digital HAs can significantly delay the acoustic stimulation in 3 to 10 milliseconds, which is far longer than the maximal natural interaural time difference (ITD) of 750 microseconds. This can further impair spatial localization in these patients.
Purpose: We sought to assess whether HA effects on ITD and interaural level difference (ILD) impair localization among subjects with unilateral conductive hearing loss (UCHL).
Research Design: "Normal"-hearing participants underwent localization testing in different free field settings.
Study Sample: Ten volunteers with "normal"-hearing thresholds participated.
Intervention: Repeated assessments were compared between "normal" (binaural) hearing, UCHL induced by insertion of an inactivated HA to the ear canal (conductive HL), and amplification with a HA.
Results: In UCHL mode, with HA switched-off, localization was significantly impaired compared to "normal" hearing (NH; η= 0.151). Localization error was more pronounced when sound was presented from the front and from the side of the occluded ear. When switched-on, amplification with HAs significantly improved localization for all participants compared to UCHL. Better localization with HAs was seen in high frequencies compared to low frequencies (η= 0.08, 0.03). Even with HAs, localization did not reach that of NH (η= 0.034).
Conclusion: Mild UCHL caused localization to deteriorate. HAs significantly improved sound localization, albeit the delay caused by the device processing time. Most of the improvements were seen in high-frequency sounds, representing a beneficial effect of amplification on ILD. Our results have potential clinical value in situations of mild CHL, for instance, otitis media with effusion.
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http://dx.doi.org/10.1055/a-1889-6578 | DOI Listing |
Worldwide, congenital deafness and pigmentation disorders impact millions with their diverse manifestations, and among these genetic conditions, mutations in the Microphthalmia-associated transcription factor (MITF: OMIM#156845) gene are notable for their profound effects on melanocyte development and auditory functions. This study reports a novel porcupine model exhibiting spontaneous deafness and pigmentation abnormalities reminiscent of human Waardenburg Syndrome Type 2 (WS2: OMIM#193510). Through phenotypic characterization, including coat color, skin, eye morphology, and auditory brainstem response (ABR) assessments, we identified hypopigmentation and complete deafness in mutant porcupines.
View Article and Find Full Text PDFInt J Pediatr Otorhinolaryngol
December 2024
Hacettepe University, Faculty of Medicine, Department of Opthalmology, Ankara, Turkey.
Aims And Objectives: This study aimed to investigate the presence, type, and severity of hearing losses in individuals with Duane Retraction Syndrome (DRS), and to ascertain if there are anomalies in the auditory pathways at the brainstem level in DRS, believed to arise from aberrant interaction between cranial nerves and brainstem nuclei.
Study Design: Cross-sectional observational study.
Setting: Tertiary referral centre.
Zh Nevrol Psikhiatr Im S S Korsakova
December 2024
Bochkov Research Centre for Medical Genetics, Moscow, Russia.
A fifth world case of autosomal recessive Siddiqi syndrome (SIDDIS) related to ene is presented. In a consanguineous Lezgin (a Dagestan ethnicity) family, there were two affected brothers aged 28 yrs (proband, personally examined) and 32 yrs. Whole-exome sequencing followed by familial Sanger sequencing detected a novel missence variant c.
View Article and Find Full Text PDFZh Nevrol Psikhiatr Im S S Korsakova
December 2024
Federal Center of Brain Research and Neurotechnologies, Moscow, Russia.
According to the literature, a number of anti-epileptic drugs (AEDs) have an ototoxic effect. The mechanism of hearing dysfunction due to the use of AEDs is not well known. The main clinical manifestations of the cochleotoxic effect of the drugs are: tinnitus, sensorineural hearing loss, impaired pitch perception, hyperacusis.
View Article and Find Full Text PDFBMC Musculoskelet Disord
December 2024
Physical medicine & rehabilitation research center, School of medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Background: Pompe disease is a glycogen storage disease primarily affecting striated muscles. Despite its main manifestation in muscles, patients with Pompe disease may exhibit non-muscle symptoms, such as hearing loss, suggesting potential involvement of sensory organs or the nervous system due to glycogen accumulation.
Aims: This study aimed to evaluate the presence of concomitant small and large fiber neuropathy in patients with Pompe disease.
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