• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.3
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• pp.341-347
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• 2003

Smith-Magenis syndrome (SMS) is a clinically recognizable multiple congenital anomaly and mental retardation syndrome caused by an interstitial deletion of chromosome 17 p11.2. Physical features include short stature, characteristic facial appearance: flattened mid-face, down-turned mouth, prominent and often rosy cheeks; prominent jaw in older children and adults, chronic ear infections, hearing impairment, eye problems, including: strabismus (an eye which turns in or out) and myopia (nearsightedness), hoarse voice, short fingers and toes, heart defects or murmurs, problems related to the urinary system, scoliosis (curvature of the spine), an unusual gait (walking pattern), and decreased sensitivity to pain. Behavioral and developmental characteristics include speech delay and articulation problems, developmental delay, learning disability, mental retardation, hyperactivity, self-injury, including: head banging; hand biting; picking at skin, sores and nails; pulling off finger- and toenails; inserting foreign objects into ears, nose, or other body orifices, explosive outbursts, prolonged tantrums, destructive and aggressive behavior, excitability, arm hugging or hand squeezing when excited. This report is the case of a Korean 3-year-3-month old male with Smith-Magenis syndrome referred from local clinic for the treatment of dental caries. The patient was treated by physical restraint after prophylatic administration of antibiotic(Amoxacillin 50mg/kg).

• Archives of Craniofacial Surgery
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• v.11 no.1
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• pp.53-57
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• 2010

Purpose: The radial forearm fasciocutaneous free flap is currently considered as the ideal free flap for reconstruction of mucosal and soft tissue defects of the palate. But the availability of stably attached oral and nasal mucosal lining is needed. In addition to this, for better operation field, operating convenience and esthetics, we planned a prelaminated radial forearm free flap. Methods: A 64-year-old male patient was admitted due to a $4{\times}4.5cm$ full through defect in the middle of the hard palate caused by peripheral T cell lymphoma with actinomycosis. In the first stage, the radial forearm flap was elevated, tailored to fit the hard palate defect, and then it positioned up-side down with split thickness skin graft. Two weeks later, the prelaminated radial forearm free flap was re-elevated and transferred to the palatal defect. One side covered with grafted skin was used to line the nasal cavity, and the other side (the cutaneous portion of the radial forearm flap) was used to line the oral cavity. Results: The prelamination procedure was relatively easy and useful. The skin graft was well taken to the flap. After 2nd stage operation, the flap survived uneventfully. There was no prolapse of the inset flap into the oral cavity and the cutaneous portion of the flap was mucosalized. The procedure was very successful and the patient can enjoy normal rigid diet and speech. Conclusion: The use of prelaminated radial forearm free flap for hard palate reconstruction is an excellent method to restore oral function. Based upon the result of this case, microvascular free flap transfer with prelaminated procedure is a valid alternative to the prosthetic obturator for palatal defect that provides an improved quality of life. It should be considered as an integral component of head and neck cancer therapy and rehabilitation.

• Clinical and Experimental Pediatrics
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• v.49 no.12
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• pp.1301-1307
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• 2006

Purpose : Hearing loss is one of the most common birth defects, and early detection and intervention positively impact language/speech and cognitive development. It has been reported that NICU graduates have a high incidence of hearing loss. So we investigated the incidence, risk factors and clinical outcome of hearing loss in NICU graduates. Methods : This study involved neonatal auditory brainstem response (ABR) testing of newborn infants who graduated from the NICU of Kyungpook National University Hospital during a 3-year period (between July 2002 and June 2005) and subsequent follow-up of these infants. Results : ABR evaluations were performed on 474 infants. Of these infants, 64 showed abnormal ABR (13.5 percent). Of 128 ears from these 64 infants, two ears (1.6 percent) and 10 ears (7.8 percent) were classified as severe and profound hearing loss, respectively. The infants with abnormal ABR had higher incidence of prematurity, low birth weight, very low birth weight, neonatal asphyxia, cranio-facial malformation and amikacin treatment over 15 days (P<0.05). In infants with hyperbilirubinemia, the peak level of serum bilirubin, duration of phototherapy and exchange transfusion were not associated with the higher incidence of hearing loss. Follow-up ABR evaluation was performed on 15 infants with abnormal ABR at $8.8{\pm}4.4months$. In follow-up ABR, 80.0% showed improvement or normalization of threshold sensitivity. Conclusion : NICU graduates exhibit high risk for hearing loss. Systemic and effective hearing assessment program is needed for these high risk infants.

• Journal of Biomedical Engineering Research
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• v.21 no.6
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• pp.615-621
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• 2000

A social phobia is an anxiety disorder characterized by extreme fear and phobic avoidance of social and performance situations. Medications or cognitive-behavior methods have been mainly used in treating it. These methods have some shortcomings such as being inefficient and difficult to apply to treatment. Lately the virtual rcality technology has been applied to dcal with the anxiety disorders in order to compcnsate for these defects. A virtual environment provides a patient with stimuli which cvokes a phobia. and the patient's exposure to the virtual phobic situation make him be able to overcome it. In this study, we suggested the public speaking simulator based on a personal computer for the treatment of social phobia. The public speaking simulator was composed of a position sensor. head mount display and audio system. And a virtual environment for the treatment was suggested to be a seminar room where 8 avatars are sitting. The virtual environment includes a tracking system the trace a participant's head-movement using a HMD with position sensor and 3D sound is added to the virtual environment so that he might fcel it realistic. We also made avatars' motion and facial expression change in reaction to a participant's speech. The goal of developing public speaking simulator is to apply to treat fear of public speaking efficiently and economically. In a future study. we should get more information about immergence and treatment efficiency by clinical test and apply it to this simulator.

• Korean Journal of Cognitive Science
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• v.23 no.4
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• pp.425-455
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• 2012

Multi-dimensional verbal short-term memory mechanisms are largely divided into the phonological channel and the lexical-semantic channel. The former is called phonological short-term memory and the latter is called semantic short-term memory. Phonological short-term memory is further segmented into the phonological input buffer and the phonological output buffer. In this study, the language performance of each of three patients with similar levels of conduction aphasia was analyzed in terms of multi-dimensional verbal short-term memory. To this end, three patients with conduction aphasia were instructed to perform four different aspects of language tasks that are spontaneous speaking, repetition, spontaneous writing, and dictation in both word and sentence level. Moreover, the patients' phonological memories and semantic short-term memories were evaluated using digit span tests and verbal learning tests. As a result, the three subjects exhibited various types of performances and error responses in the four aspects of language tests, and the short-term memory tests also did not produce identical results. The language performance of three patients with conduction aphasia can be explained according to whether the defects occurred in the semantic short-term memory, phonological input buffer and/or phonological output buffer. In this study, the relations between language and multi-dimensional verbal short-term memory were discussed based on the results of language tests and short-term memory tests in patients with conduction aphasia.

• Korean Journal of Cleft Lip And Palate
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• v.4 no.1
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• pp.13-26
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• 2001

Cleft lip and/or palate is the congenital orofacial malformation most commonly occurred in humans, The disease is multifactorial and is probably caused by genetic and/or environmental factors, So, there are many problems in research concerning etiology and in treatment of the disease, Even the most practiced and sophisticated methods of surgical repair are necessarily followed by scar contraction and fibrosis, which result in skeletal defects, dental abnormalities, cosmetic disfigurement, and speech impairment, As a result, Fetal surgery can be considered but practiced rarely when the deformity is not fatal to life, And treatment of cleft palate is performed in the form of medicine projection into uterus in animal experiments, Many studies show that growth factor and its receptor emerge from the developing palate; and the epidermal growth factor receptors have a important role in craniofacial development and in palatal fusion, The palatal morphogenesis of the avine is different from the mammal's; it takes the form of physiologic cleft palate, Recently, cleft palate fusion experiment was performed when the avine were in the period of palate formation through the exogenous TGF-β3 addition, and it showed that the exogenous TGF-β3 makes fusion of divided palate through certain process when cleft palate is occurred in palatal formation, In this study, I had the conformation of the fusion of cleft palate through the addition of TGF-β in case of chicken embryo, and observed the effect of TGF-β in EGF receptor distribution, And the following is the results of this study, 1. In case of the TGF-βl and β3 addition group, there was the decrease of EGFR(Epidermal Growth Factor Receptor) immunoreactivity in mesenchymal cells beneath the medial edge epithelium and also in epithelial mesenchymal interface which is between medial edge epithelium and nasal septum in 72 hours, 2, The immunoreactivity of the control group resembles that of normal chicken embryo palate in development, 3. In the view through fluorescence confocal microscopy, there was confluence in TGF-β3 addition group, This shows that the confluence induced by exogenous TGF-β3 is related to EGFR expression in palate of chicken embryo, which is a physiologic cleft palate model.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.2
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• pp.87-92
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• 2015

Mucopolysaccharidosis (MPS) is an inherited disease entity associated with lysosomal enzyme deficiencies. MPS type 2, also known as Hunter syndrome, has a characteristic morphology primarily involving x-l inked recessive defects and iduronate-2-sulfatase gene mutation. The purpose of this case report is to provide important clues to help pediatricians identify Hunter syndrome patients earlier (i.e., before the disease progresses). A 30-month-old boy showed developmental delay and decreased speech ability. Physical examinations revealed a flat nose and extensive Mongolian spots. Brain magnetic resonance images (MRIs) showed bilateral multiple patchy T2 hyperintense lesions in the periventricular and deep white matter, several cyst-like lesions in the body of the corpus callosum, and diffuse brain atrophy, which were in keeping with the diagnosis. Based on these findings, the patient was suspected of having MPS. In the laboratory findings, although the genetic analysis of IDS (Iduronate-2-sulfatase) did not show any pathogenic variant, the enzymatic activity of IDS was not detected. We could confirm the diagnosis of MPS, because other sulfatases, such as ${\alpha}$-L-iduronidase, were detected in the normal range. Early enzymatic replacement therapy is essential and has a relatively good prognosis. Therefore, early diagnosis should be made before organ damage becomes irreversible, and brain MRIs can provide additional diagnostic clues to help distinguish the disorder.

• Journal of the Institute of Electronics Engineers of Korea SC
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• v.47 no.3
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• pp.48-55
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• 2010

Hearing impairment is one of the most common birth defects among infants. Significant bilateral hearing impairment have profound effects on speech and language development. But it can be prevented, if a hearing impairment is identified and treated in its early stage. ABR (auditory brainstem response) is useful screening tool for new born hearing test. However, the interpretation of conventional ABR should be done by a experienced audiologist and testing takes some time. Therefore, A-ABR(automated ABR) which detect ABR peak automatically have been developed recently. In contrast to A-ABR researches became active in overseas, there has been little study in Korea. In this study, we have developed a portable A-ABR system based on the results of our previous study. For the evaluation of the developed system, the clinical trials were performed on adults and infants. As a results, it showed good sensitivity (94.4%) and specificity (92.2%), and accuracy (93.0%) between clinical diagnosis and the developed A-ABR test.

• Korean Journal of Cleft Lip And Palate
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• v.3 no.1
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• pp.23-31
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• 2000

Objective: When an alveolar cleft is too large to close with adjacent mucobuccal flaps or large secondary fistula following a primary bilateral palatoplasty exists, a one-stage procedure for bone grafting becomes challenging. In such a case, we used the tongue flap to repair the fistula and cleft alveolus in the first stage, and bone grafting to the cleft defect was performed in the second stage several months later. The purpose of this paper is to report our experiences with the use of an anteriorly-based Y-shaped tongue flap to fit the palatal and labial alveolar defects and the ultimate result of the bone graft. Patients: A series of 14 patients underwent surgery of this type from January 1994 to December 1998.The average age of the patients was 15.8 years old (range: 5 to 28 years old). The mean period of follow-up following the 2nd stage bone raft operation was 45.9 months (range: 9 to 68 months). In nine of the 14 cases, the long-fork type of a Yshaped tongue flap was used for extended coverage of the labial side alveolar defects with the palatal fistula in the remaining cases the short-forked design was used. Results: All cases demonstrated a good clinical result after the initial repair of cleft alveolus and palatal fistula. There was no fistula recurrence, although Partial necrosis of distal margin in long-forked tongue flap was occurred in one case. Furthermore, the bone graft, which was performed an average of 8 months after the tongue flap repair, was always successful. Occasionally, the transferred tongue tissue was bulging and interfering with the hygienic care of nearby teeth; however, these problems were able to be solved with proper contour-pasty performed afterwards. No donor site complications such as sensory disturbance, change in taste, limitations in tongue movement, normal speech impairments or tongue disfigurement were encountered. Conclusion: This two-stage reconstruction of a bilateral cleft alveolus using a Y-shaped tongue flap and iliac bone graft was very successful. It may be indicated for a bilateral cleft alveolus patient where the direct closure of the cleft defect with adjacent tissue or the buccal flap is not easy due to scarred fibrotic mucosa and/or accompanied residual palatal fistula.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.4
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• pp.721-728
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• 2004

Velopharyngeal closure is a sphincter mechanism between the activities of the soft palate, lateral pharyngeal wall and the posterior pharyngeal wall, which divides the oral and nasal cavity. It participates in physiological activities such as swallowing, breathing and speech. It is called a velopharyngeal dysfunction when this mechanism malfunctions. The causes of this dysfunction are defects in (1) length, function, posture of the soft palate, (2) depth and width of the nasopharynx and (3) activity of the posterior and lateral pharyngeal wall. The purposes of this study are to analyze the nasopharynx of cleft palate patients using cephalometry and to evaluate the degree of hypernasality using nasometry to find its relationship with velopharyngeal dysfunction. The following results were obtained : 1. In cephalometry, there were significant differences in soft palate length, soft palate thickness, nasopharyngeal depth, nasopharyngeal area, and adequate ratio between two groups. 2. In nasometry, there were significant differences between two groups in vowel /o/ and sentences including oral consonants. 3. In cleft palate patients, though no general correlation was found between Anatomic VPI and nasalance scores, vowel /i/ and sentences including oral consonants were slightly correlated. In conclusion, cephalometry and nasometer results were significantly different between the two groups. Though in the cleft palate group, Anatomic VPI and nasalance scores, which are indices for velopharyngeal closure, excluding the vowel /i/ and sentences including oral consonants show generally no significance.