Effect of perinatal biotin deficiency on auditory pathway of the Wistar-Albino rats.

Aim: Severe biotin deficiency associated with biotinidase enzyme deficiency in newborns is seen as severe neurological problems and hearing loss. However, the effect on the infant of deficiencies in the maternal diet during pregnancy are not clear. Material and methods: The study included 16 female Wistar albino rats and 4 male Wistar albino rats, that were mated and then the females were separated into 4 groups. At 40 days after the birth, 3 pups were selected from each group, and these 12 pups were evaluated with DPOAE and ABR electrophysiologically and the cochlea was examined ultrastructurally with electron microscopy. Results: In the DPOAE evaluation, At 8000 and 11,000 Hz, the signal-noise ratios in the B-N and B-B groups were statistically significantly higher (p < .05). In ABR, lengthening of the latency periods was determined in all the waves at both 8 and 16 kHz in the B-B group. When the IPL periods were examined, lengthening in IPL 1-5 was statistically significant in the B-B group only at 8 kHz. Conclusions: Biotin can be said to have an effect on hearing pathways. However, specifically where on the hearing pathways that biotin is involved has not been clarified.

Sensorineural hearing loss in ß-thalassemia patients treated with iron chelation.

The predictive value of pure-tone audiometry (PTA) in the early detection of ototoxicity has been questioned, particularly in the higher frequencies. Otoacoustic emissions testing appears to be more sensitive to cochlear insult than conventional PTA. We conducted a cross-sectional descriptive study to compare the efficacy of distortion-product otoacoustic emissions (DPOAE) testing with that of PTA as a method of audiologic monitoring. Our study group was made up of 159 patients (318 ears)-69 males (43.4%) and 90 females (56.6%), aged 5 to 61 years (mean: 23.59 ± 12.55). All patients had been diagnosed with either ß-thalassemia major (BTM) or ß-thalassemia intermedia (BTI), and all had received at least 1 year of treatment within the previous year with an iron chelator-either deferasirox, desferrioxamine (deferoxamine in the United States), deferiprone, or a combination of desferrioxamine and deferiprone. PTA and DPOAE evaluations were performed by the same audiologist using the same audiometer for all patients. In the right ears, the overall incidence of ototoxicity as manifested by sensorineural hearing loss was 39.0% on PTA and 22.0% on DPOAE testing; in the left ears, the corresponding figures were 27.7 and 19.5%, respectively. There were no statistically significant differences in the incidence of ototoxicity between the BTM and BTI groups with any of the four different drug regimens on PTA (p = 0.765, p = 0.378, p = 0.265, and p = 0.579, respectively) or on DPOAE testing (p = 0.890, p = 0.263, p = 0.390, and p = 0.340, respectively). Based on these data, we found no significant difference between PTA and DPOAE testing in their ability to detect ototoxicity. We conclude that periodic testing with both PTA and DPOAE is necessary for patients with suspected ß-thalassemia in order arrive at a prompt diagnosis and initiate timely management.

[The effect of operation success on middle ear ventilation in patients with nasal septal deviation]. / Nazal septum deviyasyonlu hastalarda ameliyat basarisinin orta kulak ventilasyonu üzerine etkisi.

OBJECTIVES: This study aims to evaluate the effect of septal deviation causing nasal obstruction and septal surgery on middle ear ventilation. PATIENTS AND METHODS: Twenty-five patients over 18 years of age (6 females, 19 males; mean age 24.7±6.1 years; range 18 to 38 years) who underwent septoplasty due to septal deviation with a normal baseline ear function were included in the study. Nasal obstruction was evaluated preoperatively and at three months postoperatively using visual analog scale and nasal obstruction symptom evaluation (NOSE) scale. Tympanometric tests were performed preoperatively and on seventh day, in the first month and third month postoperatively. RESULTS: There was a statistically significant difference in the mean NOSE score (p=0.00). The mean preoperative NOSE score was 55.5±16.4, while the mean postoperative (at 3 months) NOSE score was 19.0±7.7. The difference in preoperative and postoperative (at 3 months) NOSE scores was statistically significant (p=0.00). In the comparison of the preoperative and postoperative tympanometry results, no statistically significant difference was observed in middle ear pressures (p=0.375). There was no statistically significant relationship between postoperative NOSE scores and middle ear pressures (r=-0.10; p=0.943). CONCLUSION: Our study results showed that the success of septoplasty operation and operation success does not affect the ventilation and pressure of the middle ear significantly.

Comparison of pure tone audiometry and auditory steady-state responses in subjects with normal hearing and hearing loss.

The objective of this study is to compare pure tone audiometry and auditory steady-state response (ASSR) thresholds in normal hearing (NH) subjects and subjects with hearing loss. This study involved 23 NH adults and 38 adults with hearing loss (HI). After detection of behavioral thresholds (BHT) with pure tone audiometry, each subject was tested for ASSR responses in the same day. Only one ear was tested for each subject. The mean pure tone average was 9 ± 4 dB for NH group and 57 ± 14 for HI group. There was a very strong correlation between BHT and ASSR measurements in HI group. However, the correlation was weaker in the NH group. The mean differences of pure tone average of four frequencies (0.5, 1, 2, and 4 kHz) and ASSR threshold average of same frequencies were 13 ± 6 dB in NH group and 7 ± 5 dB in HI group and the difference was significant (P = 0.01). It was found that 86% of threshold difference values were less than 20 dB in NH group and 92% of threshold difference values were less than 20 dB in HI group. In conclusion, ASSR thresholds can be used to predict the configuration of pure tone audiometry. Results are more accurate in HI group than NH group. Although ASSR can be used in cochlear implant decision-making process, findings do not permit the utilization of the test for medico-legal reasons.