scholarly journals Daple deficiency causes hearing loss in adult mice by inducing defects in cochlear stereocilia and apical microtubules

Author(s):  
Yoshiyuki Ozono ◽  
Atsushi Tamura ◽  
Shogo Nakayama ◽  
Elisa Herawati ◽  
Yukiko Hanada ◽  
...  

Abstract The V-shaped arrangement of hair bundles on cochlear hair cells is critical for the auditory sensing. However, regulation of hair bundle arrangements is not fully understood. Recently, defects in hair bundle arrangement were reported in postnatal Dishevelled-associating protein (Daple)-deficient mice. Here, we found that adult Daple-/- mice exhibited hearing disturbances over a broad frequency range through auditory brainstem response testing. Consistently, distorted patterns of hair bundles were detected in almost all regions, more typically in the basal region of the cochlear duct. In adult Daple-/- mice, apical microtubules were irregularly aggregated, and the number of microtubules attached to plasma membranes was decreased. Similar phenotypes were manifested upon nocodazole treatment in a wild type cochlea culture without affecting the microtubule structure of the kinocilium. These results indicate critical roles of Daple in hair bundle arrangement, through the orchestration of apical microtubule distribution, and thereby in hearing, especially at high frequencies.

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yoshiyuki Ozono ◽  
Atsushi Tamura ◽  
Shogo Nakayama ◽  
Elisa Herawati ◽  
Yukiko Hanada ◽  
...  

AbstractThe V-shaped arrangement of hair bundles on cochlear hair cells is critical for auditory sensing. However, regulation of hair bundle arrangements has not been fully understood. Recently, defects in hair bundle arrangement were reported in postnatal Dishevelled-associating protein (ccdc88c, alias Daple)-deficient mice. In the present study, we found that adult Daple−/− mice exhibited hearing disturbances over a broad frequency range through auditory brainstem response testing. Consistently, distorted patterns of hair bundles were detected in almost all regions, more typically in the basal region of the cochlear duct. In adult Daple−/− mice, apical microtubules were irregularly aggregated, and the number of microtubules attached to plasma membranes was decreased. Similar phenotypes were manifested upon nocodazole treatment in a wild type cochlea culture without affecting the microtubule structure of the kinocilium. These results indicate critical role of Daple in hair bundle arrangement through the orchestration of apical microtubule distribution, and thereby in hearing, especially at high frequencies.


2020 ◽  
Vol 131 ◽  
pp. 109861 ◽  
Author(s):  
David B. Behrman ◽  
Jessica L. Bishop ◽  
Jeremy Godsell ◽  
Brian Shirley ◽  
Sarah Storey ◽  
...  

2003 ◽  
Vol 14 (10) ◽  
pp. 556-562 ◽  
Author(s):  
Susan A. Small ◽  
David R. Stapells

Behavioral thresholds were measured from 31 adults with normal hearing for 500, 1000, 2000, and 4000 Hz brief tones presented using a B-71 bone oscillator. Three occlusion conditions were assessed: ears unoccluded, one ear occluded, and both ears occluded. Mean threshold force levels were 67, 54, 49, and 41 dB re:1μN peak-to-peak equivalent in the unoccluded condition for 500, 1000, 2000, and 4000 Hz, respectively (corrected for air-conduction pure-tone thresholds). A significant occlusion effect was observed for 500 and 1000 Hz stimuli. These thresholds may be used as the 0 dB nHL (normalhearing level) for brief-tone bone-conduction stimuli for auditory brainstem response testing.


2007 ◽  
Vol 18 (10) ◽  
pp. 863-871 ◽  
Author(s):  
Nancy Vaughan ◽  
Kenneth James ◽  
Daniel McDermott ◽  
Susan Griest ◽  
Stephen Fausti

A number of studies have found statistically significant delays in auditory brainstem latencies of patients with diabetes compared with non-diabetic controls. However, the mechanisms ascribed as responsible for the latency differences between diabetic and non-diabetic patients vary among studies, and the latency differences, while significant, are small. In this 5-year prospective study, auditory brainstem response testing was conducted with 416 non-diabetic and 375 diabetic veterans from the Portland Veterans Affairs Medical Center as part of a larger study. Patients with diabetes had significantly delayed latencies of Wave III and V in the right ear and significantly prolonged interpeak I-III and I-V latencies in both ears. Stimulus polarity difference yielded slightly different results. None of the diabetes-related clinical characteristics were associated with the latency differences between the subject groups after accounting for hearing loss and age. Effect size was calculated and clinical significance of these differences is discussed. Un número de estudios han encontrado retardos estadísticamente significativos en las latencias auditivas del tallo cerebral en pacientes diabéticos comparados con controles no diabéticos. Sin embargo, los mecanismos atribuidos como responsables de las diferencias en las latencias entre pacientes diabéticos y no diabéticos varían entre estudios, y las diferencias en las latencias, aunque significativas, fueron pequeñas. En este estudio prospectivo a 5 años, se condujeron las pruebas de respuestas auditivas del tallo cerebral en 416 veteranos no diabéticos y 375 diabéticos, del Centro Médico de Asuntos de Veteranos de Portland, como parte de un estudio mayor. Los pacientes con diabetes tuvieron latencias significativamente retardadas para las ondas III y V en el oído derecho, y latencias inter-pico I-III y I-V significativamente retardadas en ambos oídos. Diferencias en la polaridad del estímulo rindieron resultados levemente diferentes. Ninguna de las características clínicas relacionadas con la diabetes se asoció con las diferencias en las latencias entre los grupos de sujetos, luego de considerar la pérdida auditiva y la edad. Se calculó el efecto del tamaño y se discutió el significado clínico de estas diferencias.


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