• Genomics & Informatics
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• v.16 no.4
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• pp.20.1-20.20
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• 2018

Genetic hearing loss crosses almost all the categories of hearing loss which includes the following: conductive, sensory, and neural; syndromic and nonsyndromic; congenital, progressive, and adult onset; high-frequency, low-frequency, or mixed frequency; mild or profound; and recessive, dominant, or sex-linked. Genes play a role in almost half of all cases of hearing loss but effective treatment options are very limited. Genetic hearing loss is considered to be extremely genetically heterogeneous. The advancements in genomics have been instrumental to the identification of more than 6,000 causative variants in more than 150 genes causing hearing loss. Identification of genes for hearing impairment provides an increased insight into the normal development and function of cells in the auditory system. These defective genes will ultimately be important therapeutic targets. However, the auditory system is extremely complex which requires tremendous advances in gene therapy including gene vectors, routes of administration, and therapeutic approaches. This review summarizes and discusses recent advances in elucidating the genomics of genetic hearing loss and technologies aimed at developing a gene therapy that may become a treatment option for in the near future.

• Phonetics and Speech Sciences
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• v.2 no.4
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• pp.229-238
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• 2010

This study investigates the differences in acoustic parameters in vowel space across hearing loss, gender and vowels. The parameters include F1, F2, Euclidean Distance between vowels, and vowel triangular area comprised of /i/, /a/ and /u/. For this study, 20 hearing-impaired and normal hearing adults as a control group were asked to read 7 Korean vowels (/a, $\wedge$, o, u, w, i, $\varepsilon$/). Subjects' readings were recorded by NasalView and analyzed by Praat. Results showed that F1 were significantly higher in the hearing impaired group than in the normal hearing group, higher in the female group than in male group, and higher in low vowels than in high vowels. And the means of F2 was significantly higher in the hearing impaired group than in normal hearing group, higher in high vowels than in low vowels, and there was no difference between male and female group. Secondly, Euclidean distance between vowels was significantly shorter in the hearing-impaired group than in the normal group. Finally, acoustic vowel space area was significantly smaller in the hearing-impaired group than in the normal hearing group. The hearing-impaired group showed that front vowels tended to be backed and back vowels to be fronted.

• Phonetics and Speech Sciences
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• v.1 no.4
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• pp.177-185
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• 2009

This study investigates the differences in mean F0, intensity, jitter and shimmer across hearing aid, gender and vowels. For this study, 20 hearing-impaired adults and 20 normal hearing adults as a control group were asked to read 7 Korean vowels(/$\alpha$, $\Lambda$, o, u, ɯ, i, $\varepsilon$/). Subjects' readings were recorded by NasalView and analyzed by Praat. Results showed that the means of F0 were significantly higher in the hearing impaired group(HL) than in the normal hearing group(NH), in the female group than in male group, and in high vowels than in low vowels. Second, intensity was significantly higher in the normal hearing group(NH) than in the hearing impaired group(HL), in male group than in female group, and in low vowels than in high vowels. Third, jitter was significantly higher in the normal hearing group(NH) than in the hearing impaired group(HL), and in female group than in male group and in the back vowels than in front vowels. Finally, shimmer was significantly higher in the normal hearing group(NH) than in the hearing impaired group(HL), and in male group than in female group. In particular, the male group showed that front vowels tend to have higher shimmer than back vowels.

• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.20 no.1
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• pp.57-62
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• 2009

Background and Objectives : There are few studies reported that specifically examine the laryngeal function in patients with profound hearing loss or deafness, This study was designed to examine videostroboscopic findings and phonetic characteristics in adult patients with prelingually deaf. Materials and Method: Sixteen patients (seven males, nine females) diagnosed as prelingually deaf aged from 19 to 54 years, and were compared with a 20 normal control group with no laryngeal pathology and normal hearing group, Videostroboscopic evaluations were rated by experienced judges on various parameters describing the structure and function of the laryngeal mechanism during comfortable pitch and loudness phonations. Acoustic analysis test were done, and a nasalance test performed to measure rabbit, baby, and mother passage. CSL were measured to determine the first and two formant frequencies of vowels /a/, /i/, /u/, Statistical analysis was done using Mann-Whitney U or Wilcoxon signed ranks test. Results: Videostroboscopic findings showed phase symmetry but significantly more occurrences decrement in the amplitude of vibration, mucosal wave, irregularity of the vibration and increased glottal gap size during the closed phase of phonation, In addition, group of prelingually deaf patients were observed to have significantly more occurrences of abnormal supraglottic activities during phonation. The percentage of shimmer in the group of prelingually deaf patients were higher than in the control group. Characteristics of vowels were lower of the second formant of the vowel /i/. Nasalance in prelingually deaf patients showed normal nasality for all passages, Conclusion: Prelingually deaf patients show stroboscopic abnormal findings without any mucosal lesion, suggesting that they have considerable functional voice disorder. We suggest that prelingually deaf adults should perform vocal training for normalized laryngeal function after cochlear implantation.