Glutaric Aciduria Type 1 ; Does it Effect of Hearing Function?

The main objective of this study was to observe the audiological findings of patients with Glutaric Aciduria Type 1 (GA-1), which is very difficult to evaluate and diagnose due to limited scientific literature. We used an audiological test battery to evaluate the study group of 17 individuals with GA-1 diagnosis and the control group of 20 healthy individuals. Following the otoscopic examination, the audiological test battery consisting of pure-tone hearing threshold (PTA) test, immittancemeter, distortion product otoacoustic emissions (DPOAE), contralateral suppression of otoacoustic emissions, and auditory brainstem response (ABR) measurements were applied to all participants (N=37).DPOAE amplitudes and the contralateral distortion product suppression values were significantly lower in the study group compared to control group (p < 0,05). In the study group, although they had a normal hearing level, there were morphologically different ABR waves than the control group, which was considered a remarkable finding. As well as I-V interpeak latency, the absolute latencies of I, III, and V waves ABR of the study group, were observed to be prolonged significantly compared to the control group (p < 0,05). Our findings support that GA-1 disease of association with auditory damage. The use of otoacoustic emissions and ABR measurements in the audiological test battery would be useful tool in the early diagnosis and follow-up of hearing sensivity individuals with GA-1.

Evaluating plasma oxidative stress markers in prelingual profound sensorineural hearing loss

Background and Aim: The etiopathogenesis of sensorineural hearing loss (SNHL) is an ess­ential contributing factor to its morbidity, which cannot be explained entirely so far. The current study aimed to determine the oxidative stress (OS) status by comparing the total antioxidant status (TAS), total oxidant status (TOS) and oxidative stress index (OSI) values in patients with pediatric prelingual profound SNHL. We also evaluated the correlation between OS parameters and audiological test results. Methods: The study included 25 participants (9 females; age range: 6‒34 months) diagnosed with pediatric prelingual profound SNHL and 25 healthy subjects (10 females; age range: 9‒28 months). Their TAS, TOS, and OSI levels were measured in the plasma of both groups. We evaluated the correlation between OS para­meters and audiological test results in the pati­ent group. Results: We found significantly higher serum TOS levels and OSI values in the patient group (mean ± SD of TOS: 16.08 ± 1.88 μmol H2O2 eq/L, p < 0.001; mean ± SD of OSI: 1.71 ± 0.48 arbitrary units, p < 0.001), compared to the controls. Moreover, we found lower serum TAS levels in the patient group (mean ± SD of TAS: 0.99 ± 0.20 mmol Trolox eq/L), compared to the controls. There was a strong correlation between OS parameters and audiological test results of the patient group. Conclusion: We detected significantly higher TOS, OSI, and lower TAS levels in pediatric patients with SNHL, compared to the healthy subjects. The obtained data indicated that pediatric SNHL is under OS influence.

Usefulness of P300 audiological test assessment in patients with snoring for cognitive ability

<p class="abstract"><strong>Background:</strong> Snoring is a sleep disorder which often causes cognitive dysfunction, due to inadequate rhythms of sleep cycle. Commonly evaluated with polysomnography. P300 is an electrophysiological method to measure the cognitive dysfunction. Usefulness of P300 audiological test in snoring for cognitive ability is studied here.</p><p class="abstract"><strong>Methods:</strong> Prospective study with 20 cases and 30 controls. Cases were selected with simple sleep apnoea questionnaires and snoring people were selected as cases. Healthy non snoring people were selected as controls. </p><p class="abstract"><strong>Results:</strong> Significant increase in latency of P300 in snoring people (50%); more in males and in the middle aged group.</p><p class="abstract"><strong>Conclusions:</strong> P300 is an electrophysiological method to measure the cognitive dysfunction in snoring people.</p>

Evaluation of Stopping Rules for Audiological Ascending Test Procedures Using Computer Simulations

Stopping rules for ascending audiological test procedures were evaluated by Monte Carlo simulation. The stopping rules differed in the minimum number of responses required at a level, whether these responses occurred on half or a majority of the ascending series, and whether all or only the most recent ascending series were considered. For all procedures, shallow psychometric function slopes yielded estimated thresholds closer to the level giving 50% detection than did steeper slopes, which yielded estimated thresholds roughly 2.5 dB above the level corresponding to 50% on the psychometric function. Shallow slopes also resulted in decreased reliability across threshold measurements, an increased number of trials required for threshold estimates, and a higher proportion of measurements that had to be repeated. Stopping rules using a two-response criterion were faster than those using a three-response criterion, with only a small decrease in reliability. Among stopping rules using the same number of responses for criterion, differences were seen primarily in efficiency for shallow slopes, particularly for procedures using a three-response rather than a two-response criterion for stopping. Results from these simulations should be useful to standards groups.