Hearing impairment in Stickler syndrome.
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Audiometry↗
Biomedical subjects
Y M Szymko
Publications and source records attributed to Y M Szymko.
Enhanced cochlear responses after sound exposure.
Alternating potentials produced in Hensen's cells of Mongolian gerbils by sinusoidal stimuli were enhanced or depressed after exposure to broad-band sound of moderately high intensity, depending on exposure- and stimulus intensities. Since Hensen's cell responses have been shown to be identical in phase and directly proportional in magnitude to outer hair cell (OHC) responses (Oesterle, E.C., Dallos, P., 1989, J. Acoust. Soc. Am. 86 (3), 1013-1032.; Zwislocki, J.J., Slepecky, N.B., Cefaratti, L., Smith, R.L., 1992, Hear. Res. 57, 175-194), it was assumed that these changes were reflections of changes in OHC receptor potentials, which were of main interest. The indirect method of intracellularly recording the Hensen's cell potentials rather than OHC potentials was used to minimize damage to the organ of Corti and reduce technical difficulties associated with repeated recordings from OHCs. Continuous magnitude and phase transfer functions (TFs) were obtained before and after the exposure over a range of sound pressure levels (SPLs) extending from 40-90 dB by using frequency sweeps ranging from 0.125-18 kHz. Cochlear microphonic (CM) TFs were also acquired over the same frequency and intensity ranges for monitoring purposes. The exposure stimuli were set at 80, 86, 90 or 100 dB SPL for periods ranging from 10-40 min. When response enhancement occurred, it was most clearly seen in the peak of the transfer function determined at 90 dB SPL. Enhancement ranged from approximately 12-230% of the original peak. In contrast, control Hensen's cell recordings obtained over periods of up to 130 min revealed great response stability. In all reliable recordings, response enhancement was associated with a phase lead or no phase change. The strongest exposure stimuli tended to produce sensitivity loss accompanied by phase lag at the lower SPLs, in agreement with previous work in this laboratory (Zhang and Zwislocki, 1995). In some preparations, both sensitivity loss at lower SPLs and enhancement at higher SPLs occurred simultaneously, suggesting involvement of two different mechanisms.
Acoustic Stimulation↗
Stiffness changes in chick hair bundles following in vitro overstimulation.
1. The in vitro effect of intense stimulation on the micromechanical stiffness of hair cell sensory hair bundles was studied at three locations on the chick basilar papilla. Threshold levels of hair bundle motion, produced by a water jet stimulus, were examined before and after exposure to a 300 Hz water jet stimulus set at 25 dB above the pre-exposure threshold level. 2. Threshold levels of motion were systematically examined in 8 unexposed control cells. The level of water jet stimulus needed to achieve the detection threshold of motion remained constant in these cells when periodically tested over a 36.5-min interval. 3. Post-exposure changes in the motion detection threshold of hair bundles were examined in 82 hair bundles, and a number of effects were identified: 2.4% of the hair bundles showed no threshold changes; 31.7% of the hair bundles had threshold shifts which indicated an increase in stiffness; 18.3% exhibited a threshold shift that indicated a decrease in hair bundle stiffness, but with no recovery; and 47.6% had thresholds that indicated a decrease in hair bundle stiffness with recovery to pre-exposure levels within 16-18 min. 4. The results suggest that chick hair bundles exhibit complex and varied responses to overstimulation which are very different from that seen in the mammal.
Animals↗
Stiffness of hair bundles in the chick cochlea.
The stiffness of hair bundles from isolated chick cochlear hair cells was measured in tissue culture medium. A water jet was used to deflect fiberglass fibers, quartz fibers, and hair bundles of isolated hair cells. A voltage-displacement curve was generated for a water jet ramp stimulus applied to miniature fiberglass and quartz fibers. Fiber displacements were measured using video image subtraction techniques. A force-voltage calibration curve was then derived for the fibers by modelling them as cantilever beams subjected to point forces at the tips. A voltage-displacement curve was then generated for isolated hair cell stereociliary bundles using the same procedure as for the fibers. A corresponding force-displacement curve was derived for isolated hair cells under water jet stimulation by correlating maximum ramp voltage from the hair cell's voltage-displacement curve to a corresponding force applied to a fiber from the fiberglass fiber calibration curve. The stiffness of the hair bundle, which is the slope of the hair cell's force-displacement curve, was then calculated using Hooke's law, assuming the force was distributed along the entire length of the hair bundle. The mean stiffness value was 5.04 +/- 2.68 x 10(-4) N/m for 14 hair cells, and was in close agreement with previously reported stiffness values of several investigators utilizing different animal models and procedures.
Animals↗
The structural and functional consequences of acoustic injury in the cochlea and peripheral auditory system: a five year update.
This presentation considers important developments and new trends related to acoustic injury in the peripheral auditory system reported during the past 5 years. The discussion begins with the effect overstimulation has on the "active" cochlear process, and the associated loss in receptive field (tuning curve) selectivity. Exposure to intense sound also changes the structure and function of the tectorial membrane, sensory hair bundles, tip links, and intracellular organelles. All of these injuries may change the way in which energy is delivered to the transduction channels of the hair cell. Important new evidence describing the quantitative relation between hair cell loss and permanent hearing loss is reviewed, and the possibility that specific exposure conditions cause unique lesions to the inner or outer hair cells is explored. Finally, the importance of hair cell regeneration in the chick cochlea, changes in the CNS following acoustic injury, and the cochlear vascular system are considered.
Animals↗
The design, calibration, and use of a water microjet for stimulating hair cell sensory hair bundles.
The design, calibration, and use of a noninvasive, noncontact device for stimulating hair cell hair bundles in vitro are described. This device employed a piezoelectric crystal, driven at high frequencies, to generate sinusoidal pressure in a contained fluid volume. The pressure was propagated to the tip of a glass micropipette and the oscillating water jet stimulus produced at the tip was used to stimulate sensory hair bundles. The movements of glass microbeads, caught in the oscillating pressure field of the water jet, provided a means of calibrating this stimulus. The linearity of the jet, its waveform and frequency response, the influence of pipette shape and tip diameter, as well as models to explain the operation of the water jet, are described. The use of this stimulus for measuring hair bundle micromechanics at high frequencies is then demonstrated.
Animals↗