• Journal of Audiology & Otology
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• v.25 no.1
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• pp.49-54
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• 2021

Type II mucopolysaccharidosis (MPS II) commonly known as Hunter syndrome, is a rare X-linked lysosomal storage disorder caused by iduronate-2-sulfatase deficiency, which in turn causes otorhinolaryngological manifestations, including sensorineural hearing loss (SNHL). Previously, the median survival age of patients with MPS was approximately 13.4 years. However, in the era of enzyme replacement therapy and other multidisciplinary care modalities, the life expectancy has increased. Herein, we report a rare case of an adolescent with MPS II who underwent SNHL treatment with cochlear implantation (CI). Based on unexpected findings of mastoid emissary veins and overgrowth of the vessels around the temporal bone, CI was performed using the transmeatal approach instead of the conventional transmastoid method, to avoid damage to the vessels. The average hearing threshold after CI was 35 dB and no surgical complications were encountered. Adolescent MPS II may present vessel abnormalities, which can reduce the success rate of surgery. In patients with MPS II with SNHL, CI should be performed under careful monitoring of vessel overgrowth. Moreover, with regard to feasibility of CI in adolescent patients with MPS II with SNHL, surgical techniques such as the transmeatal approach should be selected based on adequate assessment of the case.

• Speech Sciences
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• v.10 no.4
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• pp.181-187
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• 2003

This paper introduces a new zinc coated copper wire electrode with coiled cone shape which has low surface resistance and tolerance to the motion artifact for promontory stimulation electrically auditory brainstem responses (PSEABR). Auditory brainstem responses (ABR) can be used to predict hearing threshold level with a great deal of accuracy particularly for a young child who cannot cooperate mechanically and some hearing impaired who are exaggerating a hearing loss for economic compensation. While severe profound sensorineural hearing losses may not be implemented by auditory potentials, PSEABR is proven as a useful tool even for some sensorineural related hearing impaired. It was shown that PSEABR gives the electrical stimuli to promontory of the cochlear instead of giving acoustic stimuli. For this reason, PSEABR can be used as an alternative for cochlear implantation, and can also be used as an optimal device selection and neural information for MAP. It was found that the role of electrode is very important in PSEABR. Even though this cone-shaped electrode was applied in animal experiments, waveforms are well produced by PSEABR. Thus, it was concluded that cone-shaped electrode turned out to be a useful preoperative audiological evaluation tool in deciding time for cochlear implantation surgery.

• Phonetics and Speech Sciences
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• v.13 no.2
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• pp.103-109
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• 2021

In this study, we investigated longitudinal music perception of adult cochlear implant (CI) users and how acoustic stimulation with CI affects their music performance. A total of 163 participants' data were analyzed retrospectively. 96 participants were using acoustic stimulation with CI and 67 participants were using electrical stimulation only via CI. The music performance (melody identification, appreciation, and satisfaction) data were collected pre-implantation, 1-year, and 2-year post-implantation. Mixed repeated measures of ANOVA and pairwise analysis adjusted by Tukey were used for the statistics. As result, in both groups, there were significant improvements in melody identification, music appreciation, and music satisfaction at 1-year, and 2-year post-implantation than a pre-implantation, but there was no significant difference between 1 and 2 years in any of the variables. Also, the group of acoustic stimulation with CI showed better perception skill of melody identification than the CI-only group. However, no differences found in music appreciation and satisfaction between the two groups, and possible explanations were discussed. In conclusion, acoustic and/or electrical hearing devices benefit the recipients in music performance over time. Although acoustic stimulation accompanied with electrical stimulation could benefit the recipients in terms of listening skills, those benefits may not extend to the subjective acceptance of music. These results suggest the need for improved sound processing mechanisms and music rehabilitation.

• Journal of radiological science and technology
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• v.22 no.2
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• pp.27-32
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• 1999

Cochlear implant poses a contraindication to the magnetic resonance imaging(MRI) process, because MRI generates artifacts, inducing an electrical current and causing device magnetization. CT is relatively expensive and the metal electrodes scatter the image. Post-implantation radiological studies using anterior-posterior transorbital, submental-vertex and lateral views, the intracochlear electrodes are not well displayed. Therefore, the authors developed a special view, which we call the cochlear view. The patient is sitting in front of a vertical device. Then the midsagittal plane is adjusted to form an angle of $15^{\circ},\;30^{\circ}$, and $45^{\circ}$ with the film. The flexion of the neck is adjusted to make the infraorbitomeatal line(IOML) is parallel with the transverse axis of the film. The central ray is directed to exit from the skull at point which is 3.0 cm anterior and 2.0 cm superior to the EAM(external auditory meatus). Results have shown that single radiography of the cochlear view provides sufficient information to demonstrate the position of the electrodes array and the depth of insertion in cochlear. Radiography of the cochlear view in angle of $45^{\circ}$ is an excellent image. The cochlear view gives the greatest amount of medical information with the least radiation and lowest medical cost. It can be widely used in all cochlear implant clinics.

• MALSORI
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• no.52
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• pp.49-60
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• 2004

The purpose of this paper was to evaluate the effect of hearing level on fundamental frequencies in children. Participants totaled sixty children divided by three groups: congenitally deafened children with cochlear implantation(CI), congenitally deafened children with hearing aids(HA), and children with normal hearing(NH). Fundamental frequencies were measured during the sustained phonation of a vowel /a/. There was statistically significant difference of fundamental frequencies across the groups(p<.01). In post hoc analysis, HA and NH group showed statistically significant difference, but CI group didn't showed significant differences with two groups. In correlation analysis between F0 and the chronological age, there were significant negative tendencies in CI and NH group, but not in HA group. The characteristics of fundamental frequency in CI group were found similar to NH group than HA group in this study. Therefore the results of this study suggest that the hearing level is one of the influencing factors to the fundamental frequency of children.

• Korean Journal of Audiology
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• v.25 no.3
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• pp.138-145
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• 2021

Background and Objectives: This study aimed to evaluate the audiologic results after cochlear implantation (CI) in older patients and the degree of improvement in their quality of life (QoL). Subjects and Methods: Patients over 65 years old who underwent CI at implant center in Bozyaka Training and Research Hospital were included in this study (n=54; 34 males and 20 females). The control group was patient over 65 years old with normal hearing (n=54; 34 males and 20 females). We administered three questionnaires [World Health Organization Quality of Life-BREF (WHOQOL-BREF), World Health Organization Quality of Life-OLD (WHOQOL-OLD)], and Geriatric Depression Scale (GDS) to evaluate the QoL, CIrelated effects on activities of daily life, and social activities in all the subjects. Moreover, correlations between speech recognition and the QoL scores were evaluated. The duration of implant use and comorbidities were also examined as potential factors affecting QoL. Results: The patients had remarkable improvements (the mean score of postoperative speech perception 75.7%) in speech perception after CI. The scores for the WHOQOL-OLD and WHOQOL-BREF questionnaire responses were similar in both the study and control groups, except those for a two subdomains (social relations and social participation). The patients with longer-term CI had higher scores than those with short-term CI use. In general, the changes in GDS scores were not significant (p<0.05). Conclusions: The treatment of hearing loss with CI conferred significant improvement in patient's QoL (p<0.01). The evaluation of QoL can provide multidimensional insights into a geriatric patient's progress and, therefore, should be considered by audiologists.

• Journal of Audiology & Otology
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• v.25 no.3
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• pp.138-145
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• 2021

Background and Objectives: This study aimed to evaluate the audiologic results after cochlear implantation (CI) in older patients and the degree of improvement in their quality of life (QoL). Subjects and Methods: Patients over 65 years old who underwent CI at implant center in Bozyaka Training and Research Hospital were included in this study (n=54; 34 males and 20 females). The control group was patient over 65 years old with normal hearing (n=54; 34 males and 20 females). We administered three questionnaires [World Health Organization Quality of Life-BREF (WHOQOL-BREF), World Health Organization Quality of Life-OLD (WHOQOL-OLD)], and Geriatric Depression Scale (GDS) to evaluate the QoL, CIrelated effects on activities of daily life, and social activities in all the subjects. Moreover, correlations between speech recognition and the QoL scores were evaluated. The duration of implant use and comorbidities were also examined as potential factors affecting QoL. Results: The patients had remarkable improvements (the mean score of postoperative speech perception 75.7%) in speech perception after CI. The scores for the WHOQOL-OLD and WHOQOL-BREF questionnaire responses were similar in both the study and control groups, except those for a two subdomains (social relations and social participation). The patients with longer-term CI had higher scores than those with short-term CI use. In general, the changes in GDS scores were not significant (p<0.05). Conclusions: The treatment of hearing loss with CI conferred significant improvement in patient's QoL (p<0.01). The evaluation of QoL can provide multidimensional insights into a geriatric patient's progress and, therefore, should be considered by audiologists.

• Speech Sciences
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• v.11 no.2
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• pp.51-67
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• 2004

The objective of this study was to investigate the effects in auditory performance and vocal development of Onomatopoeia and Mimetic Word Productive Training Program in prelingually deafened children with cochlear implantation. The effects were measured with Lip-profile (Listening progress profile: LiP), the number of utterances, vocal developmental level and phonetic inventory. Subjects were four children with cochlear implants who were able to detect speech sounds and environmental sounds. The Onomatopoeia and Mimetic word Productive Training Program was made up of 3 steps with 24 Onomatopoeia and Mimetic words. This study was pre and post design. The results of the study were as follows: First, after Onomatopoeia and Mimetic word Productive Training Program was treated, LiP score was significantly higher. Second, after this program was treated, the number of utterances and emergence of both canonical and postcanonical utterances were increased. Emergence of vowel and consonant Features were increased and diversified. In conclusion, Onomatopoeia and Mimetic Word Productive Training Program appeared to facilitate efficient auditory performance and vocal development.

• Phonetics and Speech Sciences
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• v.5 no.4
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• pp.71-78
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• 2013

The purpose of this study was to compare the acoustic voice outcomes of children with cochlear implant to those of children with normal hearing. Participants were 41 children using unilateral cochlear implant (18 males and 23 females), and children with normal hearing from the same age and sex. Mean age of implantation was approximately 3 years old, mean duration of implant use was 4 years in CI group. Acoustic analyses were performed using MDVP of CSL. Speech samples were 3 sustained vowels, /a, i, u/. 9 parameters (F0, Fhi, Flo, Jitter, Shimmer, vF0, vAm, NHR, and SPI) were analyzed. Children with CI did not show the significant differences in those parameters after the vowel /a/ phonation. Meanwhile, there were significantly different results in F0, Fhi, vF0, and SPI after /i, u/ phonation. These results revealed that differences of voice characteristics in children with CI compare to children with NH persist regarding vowel context. It suggests that high vowels would recommend as speech samples for acoustic evaluation. Futhermore perceptual analysis and speech therapy for phonation control would be necessary for children with CI.

• Speech Sciences
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• v.11 no.3
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• pp.201-211
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• 2004

This paper introduces a development of an electrical stimulator for auditory stimulation. The electrical stimulator is useful in neurotological diagnosis, audiological evaluation, candidate selection for cochlear implantation, optimal device selection and decision making of MAP strategy for severe-to-profound hearing impaired persons. The development was based on sound parameters of auditory brainstem responses and auditory electrophysiological characteristic such as effective firing of auditory nerve and recording evoked potentials during refractory period of neuron. Besides pulse parameter could adjustable by programming for more varied electrical stimulation evoked response audiometry. Using the electrical stimulator, electrical square pulse was applied to promontory, and electrically evoked auditory brainstem response and electrically middle latency response were successfully recorded in cats.