• Title/Summary/Keyword: ear type

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The Spectrum of GJB2 Mutations in Korean Patients with Genetic Hearing Loss: a Functional Study and Study of Cell Growth Control by Dominant Type of GJB2 Mutants

  • Jin, Hyun-Seok;Kim, Jong-Bae;Go, Sang-Hee;Lee, Mi-Young;Jung, Sung-Chul;Park, Hyun-Young;Park, Hong-Joon;Koo, Soo-Kyung
    • Biomedical Science Letters
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    • v.12 no.4
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    • pp.311-318
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    • 2006
  • The GJB2 mutation is mostly recessive in non-syndromic hearing loss, but specific mutations display a dominant type and syndromic hearing impairment. Both U54K and R75Q mutations present a dominant type in pedigrees with associated skin disorders. The purpose of this study was to investigate whether two GJB2 mutations can exhibit a dominant-negative effect on the growth abrogation and the gap junctional intercellular communication capacity exerted by wild-type connexin 26. A specific mutant region of GJB2 showed a loss of gap junction activity and a dominant negative effect on wild-type GJB2. The two mutants exerted a dominant-negative effect on the GJIC capacity and have independently effected GJB2 regulated growth of Hela cells; however, they have no dominant-negative growth effect on wild-type GJB2. It is proposed that the different mechanisms of the dominant-negative effect on wild-type GJB2 involve cell growth and GJIC function. This study describes mutations found in Korean deaf patients and that are typical of other east Asian regions.

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Considerations for the Management of Cryptotia Based on the Experience of 34 Patients

  • Kim, Seok-Kwun;Yoon, Chung-Min;Kim, Myung-Hoon;Kim, Min-Su;Lee, Keun-Cheol
    • Archives of Plastic Surgery
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    • v.39 no.6
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    • pp.601-605
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    • 2012
  • Background Cryptotia is a congenital ear deformity in which the upper pole appears buried beneath the mastoid skin. Cryptotia is a common auricular malformation among Asians. The aim of this paper is to examine the surgical techniques for and complications of 34 cryptotic patients. Methods Surgery was performed for 34 cryptotic deformities (January 2005 to January 2012). Twenty-two patients (64.7%) were classified as having type I cryptotia, and 12 patients (37.5%) type II cryptotia. Among the type I cryptotia patients, 8 patients had mild deformity and 14 severe deformity. Among the type II cryptotia patients, 10 patients had mild deformity and 2 severe deformity. Results The mild deformities were corrected via Z-plasty, V-Y plasty, full-thickness skin graft, and transposition flap, while the severe deformities were corrected via cartilage graft or Medporfor the spread of cartilage adhesion of antihelix. There were two cases of reinvagination in the autologous cartilage graft group. Implant exposure occurred with Medpor (two cases). There were two cases of hypertrophic scar on the previous surgical wound with Medpor. There were no complications in the 18 patients who had mild deformities. Conclusions The type I cryptotia patients had more severe deformities than the type II cryptotia patients. As most of the type II cryptotia patients had only mild deformities, their deformities were corrected without using autologous conchal cartilage graft or Medpor, except for two patients. Through more case analyses, researchers should make an effort to identify methods for recurrence and prevention of complication.

Structure-Activity Relationships of Polyhydroxyursane-type Triterpenoids on the Cytoprotective and Anti-inflammatory Effects

  • Jung, Hyun-Ju;Nam, Jung-Hwan;Lee, Kyung-Tae;Lee, Yong-Sup;Choi, Jong-Won;Kim, Won-Bae;Chung, Won-Yoon;Park, Kwang-Kyun;Park, Hee-Juhn
    • Natural Product Sciences
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    • v.13 no.1
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    • pp.33-39
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    • 2007
  • Eleven polyhydroxyursane triterpenoids (PHUTs) were tested to determine their cytoprotective, immunosuppressive and anti-inflammatory effects. To compare the bioactivities of $19{\alpha}$-hydroxyursane-type triterpenoids {23-hydroxytormentic acid (6), its methyl ester (7), tormentic acid (8), niga-ichigoside $F_1$ (9),euscaphic acid (10) and kaji-ichigoside $F_1$ (11)} of the Rosaceae crude drugs (Rubi Fructus and Rosa rugosae Radix) with PHUTs possessing no $19{\alpha}-hydroxyl$ of Centella asiatica (Umbelliferae), the four PHUTs, asiaticoside (1), madecassoside (2), asiatic acid (3), and madecassic acid (4) were isolated from C. asiatica and 23-hydroxyursolic acid (5) from Cussonia bancoensis. Cytoprotective effects were assessed by measuring cell viabilities against cisplatin-induced cytotoxocity in $LLC-PK_1$, cells (proximal tubule, pig kidney) to determine whether these agents have protective effects against nephrotoxicity caused by cisplatin. The inhibitory effect of 11 PHUTS on nitric oxide (NO) and prostaglandin $E_2\;(PGE_2)$ were evaluated by measuring nitrite accumulation in lipopolysaccharide (LPS)-induced macrophage RAW 264.7 cells, and their anti-inflammatory effects were tested in 12-O-tetradecanoylphorbol-13-acetate (TPA)-induced mouse ear edema model. Six MHUTs (compounds 1, 2, 4, 6, 10, and 11) exhibited higher cell viabilities during cisplatin-induced cytotoxicity testing even at a concentration of $200\;{\mu}g/ml$ than cisplatin only-treated group, suggesting that ese compounds have the potentcytoprotective efffcts. Compounds 1 and 3 of the C. asiatica and niga-ichigoside $F_1$ exhibited no inhibitory effect on NO and/or $PGE_2$ production whereas other PHUTs produced mild to significant NO and/or $PGE_2$ production.The four compounds (2, 5, 9, and 10) potently inhibited mouse ear edema induced by TPA whereas two compounds (1 and 3) had no activity in this test. These results suggest that many PHUTs are potentchemopreventives. Structure-activity relationship (SAR) was also discussed in each assay with regard to the significant role of OHs at the position of 2, 3, 6, 19, and 23 and to the glycoside linkage at the 28-carboxyl.

The Design of an Infrared Transcutaneous Control Unit for Totally Implantable Middle Ear System (완전 이식형 인공중이를 위한 체외 및 체내 제어시스템 구현)

  • 정의성;강호경;박일용;윤영호;김민규;송병섭;원철호;조진호
    • Journal of the Institute of Electronics Engineers of Korea SC
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    • v.41 no.5
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    • pp.71-78
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    • 2004
  • An infrared remote control-type transcutaneous control device using a $\mu$-processor is design for the totally implantable middle ear system. An infrared light transmission model for the tissue of skin was introduced and then a radiant intensity and the required current of the infrared light emitting diode(IR LED) driving circuit at transmission part were calculated for the external control device. And the transmission part generates IR signal by the system's own data protocol which prevents interferences from other infrared remote controls of the household appliances. The control part of the implanted device was designed to analyze functions of the received infrared(IR) signal that indicate the power ON/OFF and volume UP/DOWN. After the system is implemented, a data transmission experiments using 4 mm thickness of porcine skin were carried out. From the experiment, it was verified that the infrared control signal was transmitted to receiving module of the implemented system without any error.

Development of Bovine Nuclear Transfer Embryos Using Life-span Extended Donor Cells Transfected with Foreign Gene

  • Hwang, Seongsoo;Choi, Eun Joo;You, Seungkwon;Choi, Yun-Jaie;Min, Kwan-Sik;Yoon, Jong-Taek
    • Asian-Australasian Journal of Animal Sciences
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    • v.19 no.11
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    • pp.1574-1579
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    • 2006
  • This study was performed to determine the developmental potentials of nuclear transfer (NT) embryos using life-span extended cells transfected with a foreign gene as donor cells. A life-span extended bovine embryonic fibroblast cell line was transfected with an expression vector in which the human type II collagen (BOMAR) and ear fibroblasts were used as a donor cell. Cytogenetic analysis was performed to analyze the chromosomal abnormality of donor cells. The fusion rate of 1.8 kV/cm for $15{\mu}sec$ given twice was significantly higher than that of other groups (p<0.05) and the embryos lysed were significantly higher after 1.8 kV/cm for $20{\mu}sec$ given once compared to other groups (p<0.01). The blastocyst development in the ear cell group was statistically significant compared to both BOMAR groups (p<0.01). Both BOMAR groups cultured more than 40 passages (>40 passages) had a lower number of chromosomes; however, fresh granulosa cell (GC) and BOMAR groups cultured less than 20 passages had normal chromosome numbers. Both >40 passages BOMAR groups had numerous obscure debris in metaphase spreads. The transfected foreign gene was expressed in all BOMAR groups, but not in the GC group. Based on these results, the lower developmental potential of NT embryos using life-span extended donor cells transfected with a foreign gene might be a cause of chromosomal abnormality in donor cells.

Genetical and Pathological Studies on the Mutant Mice as an Animal Model for Deafness Disease

  • Lee, Jeong-Woong;Lee, Eun-Ju;Lee, Hoon-Taek;Chung, Kil-Saeng;Ryoo, Zae-Young
    • Proceedings of the KSAR Conference
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    • 2001.03a
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    • pp.48-48
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    • 2001
  • A new neurological mutant has been found in the ICR outbred strain mouse. Affected mice display profound deafness and a head-tossing and bidirectional circling behavior, showing an autosomal recessive mode of inheritance. It was, therefore, named cir/Kr with the gene symbol cir. The auditory tests identified clearly the hearing loss of the cir mice when compared to wild type mice. Pathological studies confirmed the developmental defects in the middle ear, cochlea, cochlear nerve, and semicircular canal areas, which were correlated to the abnormal behavior observed in the cir mice. Thus, cir mice may be useful as a model for studying inner ear abnormalities and deafness. We have constructed a genetic linkage map by positioning 14 microsatellite markers across the (cir) region and intraspecific backcross between cir and C57BL/6J mice. The cir mouse harbors an autosomal recessive mutation on mouse chromosome 9. The cir gene was mapped to a region between D9Mit116 and D9Mit38 Estimated distances between cir and D9Mit116, and between cir and D9Mit38 are 0.7 and 0.2 cM, respectively. The gene in order was defines : centromere-D9Mit182-D9Mit51/D9Mit79/D9Mit310-D9Mit212/D9Mit184-D9Mit116-cir-D9Mit38-D9Mit20-D9Mit243-D9Mit16-D9Mit55/D9Mit125-D9Mit281. The mouse map location of the cir locus appears to be in a region homologous to human 3q21. Our present date suggest that the nearest flanking marker D9Mit38 provides a useful anchor for the isolation of the cir gene in a yeast artificial chromosome contig.

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A Case of Pituitary Dwarfism in a Dog: Improvement of Alopecia with Oral Melatonin (개에서 뇌하수체 왜소증의 증례: 멜라토닌의 경구 투약에 의한 탈모의 개선)

  • Oh, Seungkuk;Baek, Dae-Seung;Kim, Yunhye;Kim, Namsoo;Kim, Jonghoon;Kim, Namjung;Chung, Tae-Ho;Kim, Ju-Min;Park, Jinho;Park, Chul
    • Journal of Veterinary Clinics
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    • v.31 no.4
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    • pp.313-315
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    • 2014
  • A 13-month old, intact female, miniature Schnauzer dog was presented with history of dermatitis, pruritus and pain of ear pinnae with delayed puberty. The basal serum growth hormone level was 0.01 ng/ml, which was much lower than the reference range (1.80-2.00 ng/ml). On the xylazine stimulation test, the dog was no responsive to administration of xylazine. The dog was diagnosed as isolated type pituitary dwarfism. Treatment was aimed to improve alopecia with oral melatonin. After initiation of treatment, alopecia of the ear pinnae has been improved.

Low-Level Officials' Costumes as Illustrated in Tamna-Sullyeokdo at the Era of King Sukjong of the Joseon Dynasty (조선 숙종조 탐라순력도를 통해 본 하급관원 복식)

  • Lee, Joo-Young;Jang, Hyun-Joo
    • Journal of the Korean Society of Costume
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    • v.57 no.6 s.115
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    • pp.172-183
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    • 2007
  • The purpose of this study is to examine the dress and its ornaments of low-level officials shown in Tamna-Sullyeokdo, the paintings of the inspection tour of Jeju Island. The low-level officials Include low-level soldiers-sungjungun, gichigun, foot soldiers, cavalries, gunroe, ilsu, artillerymen, najang, and sawon-, musicians, servants, and kisaengs in government employ. 1. Different types of low-level soldiers tended to wear different kinds of clothes that is worn under an outer garment and to wear different coats and trousers depending on an outer garment. That is, sungjunggun, gichigun, foot soldiers, and cavalries were jeonbok over a jeogori and baji. On the other hand, gunroe, ilsu, artillerymen, and najang were houi or jakui over a jeogori and baji. However, Sawon simply were Jeonbok or armer or they wore Cheolik in formal occasions. 2. Musicians wore a jeonlib, a type of hat, decorated with an ear of barley and a red houi over a Jungchimak, a man's outer coat with large sleeves. Musicians wore colorful red clothes in order to add to the amusement during military trainings or banquets. 3. Servants in government employ had their hair in plaits and wore a jeonbok over a Jungchimak or baji and jeogori. However, when they dance during banquets, they were cheolik and jeonlib decored with/without an ear of barley or red flowers. 4. Gisaengs in government employ wore a gache on their head and wonsam, a woman's full dress during big occasions. They, on the other hand, wore a jeogoli and chima for regular occasions.

Studies on the Desalinization in Reclaimed Tidal Lands (III) -by the Soil Improving Materials- (간척지제감에 관한 시험(III) -토양 개량제에 의한 제염시험-)

  • 정두호
    • Magazine of the Korean Society of Agricultural Engineers
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    • v.12 no.4
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    • pp.2084-2089
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    • 1970
  • This research was experimented to clarity the effects of the calcareous materials which were utilized to improve the soil in reclaimed tidal land. It is located at the Kang-Hwa polder. Kil-sang myun, Kang-Hwa Gun, Kyung-gi Do. The results obtained are as follows 1. Comparing with the yields of rice in each treatment, it is noticed that the plot treated with ca-humated 1.0% gets the most effect to improve the soil, and the next ones are in the order of the plots treated with raw straw, gypsum and calcium hydroxide. 2. Treating the calcium or organic matter in soil is evaluated to increase the yields of rice in paddy fields of tidal land, because of increasing the number of ear per plant, the number of grain per ear, the weight of 1,000 grains and somthing like that. 3. Adding calcium or organic matter into soil, it makes a good condition for the growth of rice, because of pomoting the formation of soil structure of simple grain type. 4. To improve the soil in reclaimed tidal land, we can separately treat calcium, calcium hydroxide and raw straw in soil, but it is found out the fact that the method of treatment of calcareous materials together with organic matter is more effective.

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A Physical Cochlear Model for Transducer Performance Evaluation of Implantable Hearing Aid with Round Window Driver (정원창 구동기의 진동체 성능 평가를 위한 내이 물리모델)

  • Shin, Dong Ho;Lim, Hyung Gyu;Jung, Eui Sung;Seong, Ki Woong;Lee, Jyung Hyun;Cho, Jin Ho
    • Journal of Sensor Science and Technology
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    • v.22 no.2
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    • pp.150-155
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    • 2013
  • Recently, various hearing aids are developed to overcome hearing loss. There are available hearing aids, such as air conduction hearing aid, implantable middle ear hearing aid and so on. But air conduction hearing aid is inconvenience caused by howling, and ossicle chain driving type implantable middle ear hearing aid has some week point due to problem of possible nercobiosis of coupling spot along incus long process. In recent years, in order to improve these shortcomings round window (RW) driving hearing aid has been paying attention. In this paper, the physical cochlear model is proposed for a performance evaluation of the RW driving hearing aids of a transducer. In order to verify an experiment proposed on a performance of physical cochlear model, the transducer which has ossicles characteristics is used. By measuring and comparing the frequency characteristics of transducer with ossicles and human temporal bone, performance of physical cochlear model was verified. As from the result of experiment, it is expected that an implemented cochlear model is useful for evaluating characteristics of RW transducer.