• Korean Journal of Audiology
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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.

• 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.

• The Journal of Korean Academy of Sensory Integration
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• v.19 no.1
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• pp.13-23
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• 2021

Objective : This study examined the effects of vestibular sensory integration on the sensory processing, problem behavior, and auditory behavior in children who had cochlear Implants. Methods : This single-subject AB study was conducted for 11 weeks from August to November 2019 with three children with cochlear implants. A pre-test measure of each child's sensory processing and auditory behavior was recorded with the baseline on operationally defined behavioral problems as four incidents during 40 minutes of free play with an occupational therapist. The treatment period was 15 40-minute sessions of vestibular sensory integration. Behavioral problems were monitored and recorded during each treatment session. Once treatment was complete, a post-test was conducted on the children's sensory processing and auditory behavior. Results : The sensory processing and auditory behavior of all three participants improved after the vestibular-focused sensory integration, and diverse problem behaviors showed decreasing trends during the interventions. Conclusion : Vestibular sensory integration leads to positive changes in the sensory processing, problem behavior, and auditory behavior of children with cochlear implants.

• 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.

• Proceedings of the KOSOMBE Conference
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• v.1992 no.05
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• pp.133-138
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• 1992

Evoked otoacoustic emission (e-OAE) signals are measured from adult and analyzed by computer. Stimulation and detection are repeated and averaged 1000 times for noise cancellation. e-OAE signals are analyzed on frequency domain and time domain. The frequency domain analysis reveals that frequency of stimulus and emission has lineal relationship in 50 dB input sound amplitude. This result altos the cross correlation method to be applied for latency calculation. As the stimulus frequency grows higher, the latency tine is shorter and the gain or emission signal becomes greater. We introduced two mathmatical functions to identify these latecy and gain. These results can be utilized for cochlear modeling.

• Proceedings of the KSPS conference
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• 2007.05a
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• pp.274-277
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• 2007

The purpose of this study is 1) to describe the phoneme inventories of cochlear implant(CI) children and 2) to describe their utterances using narrow phonetic transcription method. All the subjects had more than 2 year-experience with CI and showed more than 87% open-set sentence perception abilities. Average consonant accuracy was 81.36% and it was improved up to 87.41% when distortion errors were not counted. They showed different error patterns from hearing aid users. The prominent error pattern was weakening of consonants.

• BMB Reports
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• v.50 no.10
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• pp.487-495
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• 2017

Mammalian inner ear comprises of six sensory organs; cochlea, utricle, saccule, and three semicircular canals. The cochlea contains sensory epithelium known as the organ of Corti which senses sound through mechanosensory hair cells. Mammalian inner ear undergoes series of morphogenesis during development beginning thickening of ectoderm nearby hindbrain. These events require tight regulation of multiple signaling cascades including FGF, Wnt, Notch and Bmp signaling. In this review, we will discuss the role of newly emerging signaling, FGF signaling, for its roles required for cochlear development.

• Journal of Biomedical Engineering Research
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• v.15 no.4
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• pp.439-446
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• 1994

Two-Dimensional modelling of the Cochlear biomechanics is presented in this paper. The Laplace partial differential equation which represents the fluld mechanics of the Cochlea has been transformed into two-dimensional electrical transmission line. The procedure of this transformation is explained in detail. The comparison between one and two dimensional models is also presented. This electrical modelling of the basilar membrane (BM) is clearly useful for the next approach to the further development of active elements which are essenclal in the producing of the sharp tuning of the BM. This paper shows that two-dimension model is qualitatively better than one-dimensional model both in amplitude and phase responses of the BM displacement. The present model is only for frequency response. However because the model is electrical, the two-dimensional transmission line model can be extended to time response without any difficult.

• Journal of Biomedical Engineering Research
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• v.5 no.1
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• pp.19-24
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• 1984

In this study, the general characteristics, and theories of cochlear concerning with hearing are examined. Also the digital method is studied in order to analyze with microcomputer on the model equation of basiliar membrane in the cochlear derived from 3-dimensional rectangular block model which is studied by Boer. The method is illustrated for the amplitude characteristics of basiliar momtrane wave velocity. The results obtained are as follows; 1. In the magnitude characteristics, the velocity gradually increases from the stapes, shows the maximum magnitude, and then rapidly decreases to the Helicotrema. 2. The characteristics of 3-dimensional model is located between 1-and 2- dimensional models in the velocity characteristics coefficients, magnitude characteristics, and the pattern of 2-dimensional model shoves the different features from the 1-dimensional and 3-dimensional rectangular block model. 3. In the 3-dimensional rectangular block model, the characteristics of the waveform and the maximum resonant point are same whether Z(X) is linear or nonlinear.

• Proceedings of the KOSOMBE Conference
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• v.1998 no.11
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• pp.161-162
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• 1998

In this paper, a speech processor which is the most important part of the cochlear implant is developed, to recover auditory ability for the sensorineural disorders who have damaged for their inner ear. This system consists of the analog and digital signal processing part, of which functions is the pre-processing and the main processing, respectively. The main processing is peformed in DSP processor (TMS320C31-40) by using S/W. Because the program is used in this system, it is possible to cope with the individual status of the patients, very easily.