• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.22 no.2
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• pp.126-132
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• 2011

Background and Objectives : Vocal fold paresis is a clinical condition and considered as a continuum of neurologic dysfunction encompassing partial denervation and variable degrees and patterns of reinnervation. Its incidence, clinical presentation, significance are incompletely understood and still debated. This study describes the clinical, electromyographic findings in patients who presented with complaints of dysphonia and whose laryngoscopic finding revealed vocal fold paresis. Materials and Method : 47 patients (male : 25, female : 22) who referred to Ewha Womans University Medical Center Voice clinic for evaluation of vocal complaints were enrolled in this study. All patients had undergone a through history and physical examination including strobovideoscopic and laryngoscopic examination. Patients with in the history and/or laryngoscopic examination suggestive of vocal fold paresis were evaluated by laryngeal electromyography (LEMG). Results : Of these patients, 23 (48.9%) were found to have evidence of neuropathy on LEMG. There was no significant difference in voice symptoms and laryngoscopic findings between two groups of patients with evidence of neuropathy and who show normal findings on LEMG. Conclusion : LEMG can clinically help to guide the evaluation and management of vocal fold paresis. Due to some limitations of LEMG, laryngoscopic findings and clinical correlations should also be considered when diagnosing the vocal fold paresis.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1982.05a
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• pp.11.2-12
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• 1982

The major advancement in phonosurgery due to recent development of laryngomicrosurgery enabled more accurate diagnosis and treatment of patient with voice disorders. Among large proportion of voice disordered patients, prominent linear furrow running parallel along the free edge of vocal cord extending from the vocal process to anterior commissure can be seen as well as incomplete closure during phonation. These cases were illustrated and coined as sulcus vocalis by Salvi in 1901, since then other similar paper was reported in Europe and Japan, but has not been reported in Korea. The exact etiology and therapeutic methods of sulcus vocalis has not been elaborated. At Department of Otolaryngology of Yonsei University College of Medicine a series of voice analysis were performed among those 35 patients with sulcus vocalis visited to Vocal Dynamics Laboratory from May, 1981 to March, 1982. Following is the result of clinical statistical investgation and therapeutic modality. 1) The incidance of sulcus vocalis among 290 patients with voice disorder visited to Vocal Dynamics Laboratory was approximately 12%(35 cases). 2) Onset of this voice disorder was most frequent among patient under 10 year-old groups; 19 cases (54%) followed by second decade, third decade groups in decreasing frequency respectably. 3) The etiology of sulcus vocalis was mostly unknown. The sequelae after measle (4 cases) and severe upper respiratory infection (3 cases) and congenital deformity (2 cases) were the possible causes of sulcus vocalis. 4) These patients were involved bilaterally in 25 cases (71%), left side only in 8 cases (23%) and right side only in 2 cases (6%). 5) Almost all patients complained hoarseness and 7 patients were suffering from chronic laryngitis. 6) In aerodynamic analysis, Maximal Phonation Time was decreased in 20 cases (57%), Phonation Quotient was increased in 22 cases (63%) and Mean Air Flow Rate was increased in 23 cases (66%). 7) Among them, 33 cases were analyzed with stroboscopy. The findings were as follows; incomplete glottic closure during phonation in 31 cases (93%), regular vocal cord movement in whole cases, asymmetric cord movement in 4 cases (12%), decreased amplitude in 5 cases (21%) and small mucosal wave in 24 cases (73%). 8) Intracordal Teflon injection in 5 cases and Sulcusectomy in 1 cases were performed as therapeutic management, however, the therapeutic results were not effective except one case with Teflon injection.

• Phonetics and Speech Sciences
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• v.15 no.4
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• pp.61-69
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• 2023

Sulcus vocalis is characterized by incomplete closure of the vocal folds, with a high mean airflow rate (MFR) as a distinctive feature. The MFR is measured using two aerodynamic analysis methods [the maximum sustained phonation protocol (MXPH) and voicing efficiency protocol (VOEF)] of the phonatory aerodynamic system (PAS), and the results may vary depending on the method. This study compared the differences in MFR before and after treatment (microsurgery and voice therapy) according to the MXPH and VOEF of the PAS in 30 patients with sulcus vocalis. Additionally, we examined whether there were differences in the subjective voice evaluation (voice handicap index, VHI), perceptual voice evaluation (GRBS), and fundamental frequency (F0) before and after treatment. The results showed significant differences between the two methods, both before and after treatment, in patients with sulcus vocalis. However, there were no significant differences by methods in the changes before and after treatment. The VHI and GRBS scores significantly decreased after treatment; however, F0 showed no significant differences before and after treatment. This study indicates that when evaluating MFR changes in patients with sulcus vocalis, it is acceptable to use either aerodynamic analysis (MXPH or VOEF).

• Proceedings of the KOR-BRONCHOESO Conference
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• 1983.05a
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• pp.8.2-9
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• 1983

Recently, the laryngomicrosurgery has been done for the removal of benign laryngeal mass and for the improvement of voice. For the evaluation of the effect of the treatment, there must be any objective method such as aerodynamic study, vocal fold vibration study, acoustic analysis, psycho-acoustic evaluation and the neuro-muscular study. The authors evaluated the phoniatric effect of the laryngomicrosurgery for the patients of 15 laryngeal polyps and 9 laryngeal nodules, who received pre-op. and post-op. vocal function study from Jun. 1981 to Mar. 1983. The results obtained were as follows ; 1) The post-op. mean value of the maximum phonation time was increased 40 % in the unilateral polyps, 62 % in the bilateral nodules and 18 % in the unilateral nodules. 2) The post-op. mean value of the phonation quotient was decreased 25 % in comparison with pre-op. value in the case of the bilateral polyps, 26 % in the unilateral polyps, 55 % in the bilateral nodules and 12 % in the unilateral nodules. 3) The post-op. mean value of the mean air flow rate was decreased 27 % in comparison with the pre-op. value in the case of the bilateral polyps, 25 % in the unilateral polyps, 65 % in the bilateral nodules, 25 % in the unilateral nodules. 4) The glottic chink of the 10 cases of polyps among the 11 cases were disappeared, and the glottic chink of the 5 cases of nodules among 7 cases were also disappeared after surgery. 5) The pre-op. hoarseness of the 10 cases of polyps among the pre-op. hoarseness of the 11 cases of polyps were changed to clear and the 3 cases of nodules were also changed to clear.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.3 no.1
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• pp.4-14
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• 2003

Maple syrup urine disease or branched chain ketoacidurias caused by a deficiency in activity of the branched-chain ${\alpha}$-keto acid dehydrogenase(BCKD) complex. This metabolic block results in the accumulation of the branched-chain amino acids(BCAAs) leucine, isoleucine and valine, and the corresponding branched chain ${\alpha}$-keto acids (BCKAs). Based on the clinical presentation and biochemical responses to thiamine administration, MSUD patients can be divided into five phenotypes : classic, intermediate, intermittent, thiamine responsive and dihydrolipoyl dehydrogenase(E3)-deficient. Classic MSUD has a neonatal onset of encephalopathy, and is the most severe ad most common form. Variant forms of MSUD generally have the initial symptoms by 2 years of age. The majority of untreated classic patients die within the early months of life from recurrent metabolic crisis and neurologic deterioration. Treatment involves both longterm dietary management and aggressive intervention during acute metabolic decompensation. We report here our experience of longterm diet therapy and treatment of acute metabolic decompensation of a case of classic MSUD.

• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.29 no.2
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• pp.70-75
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• 2018

The office-based, un-sedated vocal fold injection laryngoplasty has re-emerged in the past decade as an appealing alternative to microsuspension laryngoscopic procedure which is conducted under general anesthesia. The trend toward vocal fold injection laryngoplasty in an in-office setting was possible due to technological evolution for visualization and new injection materials. However, invisibility of the injection needle has been the main shortcomings of cricothyroid approach. The purpose of this review is to provide an up-to-date review of office-based, trans-cricothyroid membrane approach injection laryngoplasty technique under local anesthesia and efforts made to increase the preciseness of amount and location of the injected materials in the management of glottic insufficiency. A review of variable efforts undertaken to maximize the result of cricothyroid approach by technically increasing accuracy of the location of needle tip was done. With the proper patient selection and utilization of the new technologies, office-based and un-sedated vocal fold injection laryngoplasty via cricothyroid approach can be redeem as a main-stay in the management of glottic insufficiency, such as vocal fold paralysis, vocal fold paresis, vocal fold atrophy and vocal fold scar.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.3
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• pp.148-154
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• 2016

A urea cycle disorder is a condition caused by a defect of the enzymes in the urea cycle, and deficiency of ornithine transcarbamylase (OTC), which converts carbamoyl phosphate and ornithine into citrulline, is the most common type of the disorder. OTC deficiency induces the accumulation of precursors of urea, ammonia, and glutamine, leading to neurological symptoms including hypotonia, respiratory failure, seizure, lethargy, and coma and sometimes to death. Because OTC deficiency is inherited in an X-linked manner, typical symptoms such as vomiting, poor feeding, and lethargy appear mainly in male neonates. We recently had a case that presented with neonatal onset lethargy, vomiting, and apnea in a 4-day-old boy. He was diagnosed with OTC deficiency by biochemical phenotype, including hyperammonemia and an increased orotic acid level in the urine. Genetic analysis of the OTC gene showed a novel mutation c.780_781insCAGGCAGTGT (p.Ile261Glnfs*35). He was treated for hyperammonemia using continuous venovenous hemofiltration (CVVH) at 118 hours after birth. After 4 days of CVVH, his consciousness and blood ammonia concentration were normalized, and he was discharged at the age of 53 days. At around 12 months of age, bilateral femur fractures and osteomyelitis occurred in this patient. Two months after the fracture, he died of septic shock, insulin-resistant hyperglycemia, and multi-organ failure.

• Journal of Chest Surgery
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• v.36 no.6
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• pp.444-447
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• 2003

Sleeve pneumonectomy can be a method of treatment in a selected patient with bronchogenic carcinoma involving carina. A 64 years old male with a history of mitral valve replacement via midsternotomy 13 years ago and resection of papilloma of the vocal cord 2 years ago. The patient was admitted due to blood-tinged sputum. Bronchoscopy and computerized tomogram of the chest revealed 3.5 cm mass at lower margin of the trachea and totally obstructing the left main bronchus. A biopsy revealed squamous cell carcinoma. He underwent left sleeve pneumonectomy through sequential bilateral thoracotomy without cardiopulmonary bypass, and the pathologic stage was T4N0M0 stage IIIB. The patient is being followed through the outpatient clinic in good general condition.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.2
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• pp.98-100
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• 2015

Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is a rare mitochondrial fatty-acid oxidation disorder that is inherited as an autosomal recessive pattern. SCAD deficiency is caused by mutations in the ACADS gene (Acyl-CoA Dehydrogenase, Short-chain, OMIM #606885), which encodes SCAD, the mitochondrial enzyme that catalyzes the first reaction in the beta-oxidation of fatty acids four to six carbons in length. Here, we describe one Korean pediatric case of SCAD deficiency, which was diagnosed during newborn screening through tandem mass spectrometry. An increased concentration of butyrylcarnitine was detected on the newborn screening test, and the urine organic acid analysis showed increased urinary excretion of ethylmalonic acid. The patient has been asymptomatic and has shown normal growth and development by 8 months of age without any intervention during follow-up period.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.54-59
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• 2014

Adrenoleukodystrophy is an X-linked genetic disease resulting from mutations in the ABCD1 gene. Cerebral adrenoleukodystrophy is one of the phenotypes of adrenoleukodystrophy and shows progressive demyelination of brain white matter and adrenal insufficiency. We report a nine year old male who presented with rapidly progressive cognitive and neurologic deterioration. He had abnormal findings in brain imaging and elevated very long chain fatty acid level in serum. Mutation analysis of ABCD1 revealed a c. 1252C>T (p.Arg418Trp) mutation which was previously known but not reported in Korea.