Ultrastructural Changes in Skeletal Muscle of Infants with Mitochondrial Respiratory Chain Complex I Defects

BACKGROUND AND PURPOSE: The pathogenesis of mitochondrial disease (MD) involves the disruption of cellular energy metabolism, which results from defects in the mitochondrial respiratory chain complex (MRC). We investigated whether infants with MRC I defects showed ultrastructural changes in skeletal muscle. METHODS: Twelve infants were enrolled in this study. They were initially evaluated for unexplained neurodegenerative symptoms, myopathies, or other progressive multiorgan involvement, and underwent muscle biopsies when MD was suspected. Muscle tissue samples were subjected to biochemical enzyme assays and observation by transmission electron microscopy. We compared and analyzed the ultrastructure of skeletal muscle tissues obtained from patients with and without MRC I defects. RESULTS: Biochemical enzyme assays confirmed the presence of MRC I defects in 7 of the 12 patients. Larger mitochondria, lipid droplets, and fused structures between the outer mitochondrial membrane and lipid droplets were observed in the skeletal muscles of patients with MRC I defects. CONCLUSIONS: Mitochondrial functional defects in MRC I disrupt certain activities related to adenosine triphosphate synthesis that produce changes in the skeletal muscle. The ultrastructural changes observed in the infants in this study might serve as unique markers for the detection of MD.

Congenital Middle Ear Cholesteatoma in Children; Retrospective Review of 35 Cases

Congenital middle ear cholesteatoma (CMEC) is a rare disease entity in otolaryngology. However, we try to assess the characteristic features and recurrences of CMEC in pediatric patients according to stages, and to determine the value of preoperative computed tomography (CT) scan. Retrospective review of 35 cases of CMEC under the age of 15 yr that had been treated at the tertiary referral center from 1995 through 2006. The main outcome measures were CT findings, surgical findings, recurrence rate and hearing assessment. Preoperative CT scan accurately predicted the extent of the cholesteatoma seen during surgery in 30/35 (85.7%). The recurrence rate of CMEC was 5.7% (2/35) and all of recurred cases were stage IV. In recurred cases, cholesteatomas were extended to sinus tympani and facial recess at revisional operation as well as initial operation. So we concluded that preoperative CT scan is essential in defining the extent of existing pathology. The intraoperative CMEC extension and location influence the outcome of surgery. In the higher stages, careful eradication of disease, particularly in the region of sinus tympani and facial recess is recommended.

The Role of Inflammatory Mediators in the Pathogenesis of Otitis Media and Sequelae

This review deals with the characteristics of various inflammatory mediators identified in the middle ear during otitis media and in cholesteatoma. The role of each inflammatory mediator in the pathogenesis of otitis media and cholesteatoma has been discussed. Further, the relation of each inflammatory mediator to the pathophysiology of the middle and inner ear along with its mechanisms of pathological change has been described. The mechanisms of hearing loss including sensorineural hearing loss (SNHL) as a sequela of otitis media are also discussed. The passage of inflammatory mediators through the round window membrane into the scala tympani is indicated. In an experimental animal model, an application of cytokines and lipopolysaccharide (LPS), a bacterial toxin, on the round window membrane induced sensorineural hearing loss as identified through auditory brainstem response threshold shifts. An increase in permeability of the blood-labyrinth barrier (BLB) was observed following application of these inflammatory mediators and LPS. The leakage of the blood components into the lateral wall of the cochlea through an increase in BLB permeability appears to be related to the sensorineural hearing loss by hindering K+ recycling through the lateral wall disrupting the ion homeostasis of the endolymph. Further studies on the roles of various inflammatory mediators and bacterial toxins in inducing the sensorineumral hearing loss in otitis media should be pursued.

Role of Inflammatory Mediators in the Pathogenesis of Otitis Media / 대한이비인후과학회지

No Abstract available.

A Case of Congenital Inner Ear Anomaly Combined with Cerebrospinal Leak Using Hydroxyapatite Cement for Treatment / 대한이비인후과학회지

Cerebrospinal fluid otorrhea is a rare disease entity, especially when brought on by spontaneous causes. The site of leakage should be identified to treat the disorder effectively. Various localizing modality was suggested in the literatures. Intrathecal gadolinium-enhanced magnetic resonance imaging identifies the small leakage site easily and correctly. Hydroxyapatite cement is a calcium phosphate-based material that forms dense paste mixed with water or sodium phosphate solution. Hydroxyapatite cement is remarkable for its excellent biocompatability in interacting with the native bone to osseointergrate with the surrounding bone. We report on our experience of successfully treating a patient with the cerebrospinal fluid leak from the congenital inner ear anomaly using the hydroxyapatite cement.

Magnetic Resonance Imaging of the Mastoid Cavity and the Middle Ear: Prevalence and Clinical Significance of Incidental Abnormal Findings in a Non-ENT Adult and Pediatric Population / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The purpose of this study is to determine the prevalence of abnormalities in the mastoid cavity and middle ear in a non-ENT population, and to correlate with clinical data. SUBJECTIVES AND METHOD: Cross-sectional study. We prospectively evaluated a total of 100 adults and 30 children undergoing MRI scan for suspected intracranial pathology from May to July 2003. Patients who had previous history of mastoid/middle ear surgery and were suffering otitis media were excluded. The T2-weighted images were reviewed. The abnormalities found on MRI were divided into 1) mastoid cavity abnormality, and 2) middle ear abnormality. All patients were asked to complete a questionnaire pertaining to symptoms of mastoid/middle ear pathology and history of previous otitis media. Also, their ears were examined carefully by otoscope or otomicroscope. RESULTS: In both groups, most of abnormalities were found in the mastoid cavity. Analysis of the clinical data revealed that abnormal MRI findings of the mastoid cavity were significantly correlated to clinically significant mastoid/middle ear diseases in adults. CONCLUSION: Incidental MRI abnormalities in mastoid cavity and middle ear detected in a non-ENT population were relatively uncommon, compared to incidental paranasal sinus abnormalities. However, clinicians should remember the possibility of the pathologies that demand active treatment among these abnormalities, especially when high signal abnormality is found in the mastoid cavity of adults.

Efficacy of Adenoidectomy in Preventing Recurrence of Otitis Media with Effusion / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Previous studies of the efficacy of adenoidectomy in otitis media with effusion have yielded conflicting results. The aim of this study was to investigate the efficacy of adenoidectomy and influences of other associated risk factors on the recurrence of otitis media with effusion (OME). SUBJECTS AND METHOD: A retrospective analysis was carried out on 441 ears of 266 patients who underwent tympanostomy tube (T-tube) insertions from January 1990 to December 2000. Patients were grouped according to the surgical procedures they underwent; T-tube insertion only, T-tube insertion and adenoidectomy and T-tube insertion and adenotonsillectomy. In addition to the type of surgical procedure, the time elapsed to the extrusion of T-tube, age, paranasal sinusitis, the nature of effusion, and adenoid size were chosen as factors affecting the recurrence of otitis media with effusion. The recurrence of OME, the reinsertion of T-tube, and the number of T-tube insertions during each patients' follow-up period were defined as the dependent variables. Statistical analyses were performed by multiple logistic regression and cumulative multiple logistic regression methods. RESULTS: A significant benefit was observed with adenoidectomy in preventing recurrence of OME (p<0.001), which was not enhanced by tonsillectomy. The effect of adenoidectomy was independent of adenoid size. Earlier extrusion of tympanostomy tubes was strongly correlated with the recurrence of otitis media with effusion (p<0.0001). OME tended to recur if the patients had younger age. CONCLUSION: Performing adenoidectomy at the time of the insertion of tympanostomy tube substantially reduces the recurrence of otitis media with effusion and the likelihood of reinsertion of T-tube.

Two Cases of Hypoglossal Neurilemmoma in the Submandibular Space / 대한이비인후과학회지

Neurilemmomas are tumors arising from the myelin-producing Schwann cells of the peripheral nervous system. Neurilemmomas of the 12th cranial nerve are rare, with most originating intracranially. The rarity of such pathology in the submandibular space makes proper preoperative diagnosis difficult, and is often mistaken to be an inflammatory mass, a malignant lymph node or a salivary gland tumor. The preoperative diagnosis of hypoglossal nerve neurilemmoma in this region is very important in that the patient must be warned of the morbidity of sacrificing the nerve during surgical excision and that the surgeon might be prepared for nerve graft. Therefore, we report recent experience of 2 cases of hypoglossal nerve neurilemmomas occurring in the submandibular area with a review of literature focusing on radiological findings and preoperative differential diagnosis.

Characterization of Th2 Cytokines, IL-5, IL-10 and IL-13, in Otitis Media with Effusion: Comparison of Allergy and Non Allergy / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: While it has been reasonably well demonstrated that allergy is a risk factor for patients of otitis media with effusion (OME), the causal mechanism is not well understood. Although Th2 cytokines are known to mediate allergic inflammatory reactions, there have been no reports about the presence of other Th2 cytokines in the middle ear effusions of OME patients except for IL-4. The aim for this study was to determine whether Th2 cytokines, IL-5, IL-10 and IL-13, were present in the middle ear effusions, and whether these cytokines were present in higher concentrations in the effusions of allergic patients than of non-allergic patients. MATERIALS AND METHOD: Thirty ears of twenty nine patients with persistent OME were assayed for the cytokines using enzyme-linked immunosorbent assay (ELISA). Ten of these patients, which are 11 ears, were confirmed allergy positive by multiple allergosorbent test (MAST). The statistical differences in the positive rates of each cytokines between the allergy and the non-allergy groups were assessed by chi 2 test or Fisher's exact test, and the statistical differences in the measured concentrations of each cytokines between the two groups were assessed by Mann-Whitney U test. RESULTS: The positive rates of each cytokines and the level of IL-5 were not statistically different between the allergy and the non-allergy groups. However, the levels of IL-10 and IL-13 in the non-allergy group were significantly higher than in the allergy group. CONCLUSION: The higher levels of IL-10 and IL-13 of the non-allergy group suggest that more active anti-inflammatory cellular responses exist in the middle ear of the non-allergic patients with OME than of the allergic patients, or that at least the different inflammatory processes exist between the two groups with regard to the pathogenesis of OME.

A Case of Inflammatory Pseudotumor of the Temporal Bone with Extraocular Muscle Palsy / 대한이비인후과학회지

Inflammatory pseudotumors are benign tumors rarely that occurs in the head and neck. We present the case of a 39-year-old man presenting extraocular muscle palsy with inflammatory pseudotumor affecting the temporal bone including mastoid, middle ear and petrosal apex. At computed tomography and magnetic resonance imaging (MRI) scan, the lesion appeared as an enhancing mass of soft tissue occupying the majority of the mastoid bone and petrosal apex causing diplopia. The patient was treated with conservative surgical excision (a open cavity mastoidectomy and tympanoplasty) and postoperative steroid therapy. Microscopic examination showed predominantly mature plasma cells. The patient remains free of disease 8 months after treatment. We believe this is the first case report of inflammatory pseudotumor affecting the middle ear and mastoid involving the petrous apex.

A Case of Multifocal Mucosa-associated Lymphoid Tissue Lymphoma Involving Ethmoid Sinus, Hard Palate and Cheek / 대한이비인후과학회지

Mucosa-associated lymphoid tissue (MALT) is a specialized form of lymphiod tissue that may be acquired at sites in response to chronic inflammation. MALT lymphoma is a type of low-grade, B-cell lymphoma within the spectrum of non-Hodgkin's extranodal lymphomas, and may arise within several sites in the head and neck. Because of the differences in the natural history, prognosis, and therapy of MALT lymphoma and other lymphomas, the immunohistochemical and molecular biology studies should be considered in the diagnosis of MALT lymphoma in cases where atypical lymphoid infiltrates into the head and neck. We present a case of advanced staged multifocal MALT lymphoma, which has disseminated into the bone marrow, involving mucosal sites of the ethmoid sinus, hard palate and cheek, but not the gastrointestinal tract, with a review of relevant literature.

A Case of Multiple Myeloma Involving Temporal Bone / 대한이비인후과학회지

Multiple myeloma (MM) is a systemic manifestation of plasma cell tumor. It shows anemia, bone pain and some pathologic fracture. MM is a relatively well known disease, but temporal bone involvement is very rare. In 1979, Lavine first reported the temporal bone involvement in MM. Since then, only a few cases of temporal involvement in MM were reported in literature. In Korea, temporal bone involvement in MM has not been reported yet. It is very difficult to diagnose MM of temporal bone in the early stage, since it presents general, nonspecific otologic symptoms and it is difficult to carry out pathological confirmation in the temporal bone. In most of reported cases, patients are already diagnosed with MM. when they are presented with MM of temporal bone. If MM is found in the temporal area, it is considered to be in the terminal stage of disease, but treatment for it is the same as that for the usual MM. We experienced a case with intractable otalgia and suddenly developed facial palsy, which was later confirmed to be a MM after a pathologic review and a systemic evaluation. We report this rare case with a review of literatures.

Efficacy of Adenoidectomy on Pediatric Chronic Rhinosinusitis

BACKGROUND AND OBJECTIVES: Adenoid vegetation can induce chronic rhinosinusitis in children by obstruction of the nasopharynx or as a reservoir for bacteria. The aim of this study is to determine the efficacy of adenoidectomy in chronic pediatric rhinosinusitis. Materials and Methods: Symptoms of chronic rhinosinusitis were evaluated in 17 patients who underwent an adenoidectomy between November 2001 and July 2002. Patients' age ranged from 4 to 11 years old. Caregivers of the patients were asked about symptoms of chronic rhinosinusitis, which were nasal obstruction, rhinorrhea, postnasal drainage, headache, cough and sputum, by a 10-graded visual analogue scale of 6 items at preoperative and postoperative 2, 3, 4, 5, 6, 8, 10 and 12 weeks, respectively. We assessed the grades of plain radiographic (Waters' view) findings at postoperative 1, 2 and 3 months consecutively. RESULTS: Symptoms of chronic rhinosinusitis were significantly improved following adenoidectomy. Significant improvement of symptom scores began at postoperative 2 weeks on the average. Plain radiographic findings were significantly improved at postoperative 1 month. After postoperative 7 months, 'Cure' was reported in 9 (53%) of 17 patients. Conclusions: In majority of cases, symptoms and radiographic findings of chronic rhinosinusitis in children are improved by adenoidectomy. Adenoidectomy should be considered as a first-line surgical option for pediatric chronic rhino-sinusitis.

Assessment of Olfactory Function after Endoscopic Sinus Surgery in Chronic Rhinosinusitis Patients and Clinical Validity of Cross-Cultrual Smell Identification Test / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Endoscopic sinus surgery (ESS) improves olfactory dysfunction due to chronic rhinosinusitis. Cross-Cultural Smell Identification test (CC-SIT) is known to be one of the useful methods to assess the olfactory dysfunction. We assessed the influence of ESS on olfactory function by using symptom scores and CC-SIT, and determined the clinical validity of CC-SIT. MATERIALS AND METHODS: 151 patients with chronic rhinosinusitis who had received ESS were evaluated. Prior to and three months following the surgery, each patient was requested to mark a 5-point scale ranging from normosmia to anosmia. Preoperative and postoperative CC-SITs were performed for 20 patients who complained of olfactory dysfunction. To evaluate the clinical validity of CC-SIT, we assessed the correlations between CC-SIT scores, symptom scores and CT scores using the Lund-Mackay CT scoring system. RESULTS: There were a significant decrease in the symptom scores and an increase in the CC-SIT scores after ESS. However, normosmia was achieved in only 38.5% (25/65) of the patients determined by symptom scores and 23.5% (4/17) by CC-SITs. There were significant correlations between CC-SIT scores, symptom scores and CT scores (r=-0.416, r=-0.357, r=0.510, p<0.05). CONCLUSION: Although the average postoperative olfactory ability after ESS was in the hyposmic range, ESS could improve the olfactory ability in patients with chronic rhinosinusitis. The 5-point scale and the CC-SIT scores reflected well the severity of olfactory dysfunction in patients with chronic rhinosinusitis, and therefore, we recommend them as convenient and practical tools to assess olfactory ability before and after ESS.

The Clinical Significance of Lund-Mackay CT Staging System in Assessing the Severity of Chronic Rhinosinusitis / 대한이비인후과학회지

Backgrounds and Objectives : Although the sinus CT (computed tomography) scan has emerged as the preferred method for assessing the extent of chronic rhinosinusitis, the relationship between sinus CT findings and symptom severity in chronic rhinosinusitis has not been established yet. The Lund-Mackay CT staging systems were known as one of the most prevalent methods in assessing the severity of chronic rhinosinusitis. We aimed to determine the relationship between the severity of the Lund- Mackay CT scores and symptom severity, and to determine the relationship between the laterality and symptom severity in chronic rhinosinusitis. MATERIALS AND METHODS: 108 patients who fit the eligiblity and exclusion criteria were evaluated. We scored each CT scan on the Lund-Mackay system and let the patients complete the questionnaires for the rhinosinusitis symptoms. The correlation between the CT scores and the severity of patients' symptoms was assessed. Then, we divided the patients into 4 groups according to the grades of CT scores. And in each group, we subgrouped them according to the CT score differences between the right and left sides. Statistical analyses of symptom scores between subgroups in each group were performed. RESULTS: The CT scores graded by the Lund-Mackay system and symptom scores were correlated significantly (rho=0.365, p< or = 0.0001). According to the laterality of chronic rhinosinusitis, there were no statistically significant differences between CT scores and symptom scores in the subgroups of each group. CONCLUSION: The CT score by the Lund-Mackay system was correlated with the patients-based reports of symptom severity regardless of the laterality of chronic rhinosinusitis. We therefore recommend that the Lund-Mackay system to be adopted as the standard method of measuring the extent of chronic rhinosinusitis.

Congenital Middle Ear Cholesteatoma / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Congenital middle ear cholesteatoma (CMEC) is a keratinous mass behind an intact tympanic membrane. CMEC does not have a history of instrumentation and is less common than acquired one. Many theories have been put forward to explain the pathophysiology of CMEC, however, none of these so far have been convincingly proven. This clinical study was performed to investigate the characteristic features of CMEC and to evaluate the correlation between pathophysiology and CMEC by retrospectivly reviewing the cases. MATERIALS AND METHOD: The medical records of patients who underwent otologic procedures at the hospitals of the Catholic university from January 1993 to September 1998 have been reviewed. They were ten males and four females, ranging in age from 4 to 59 (mean age 18). RESULTS: Three of the 14 patients had the lesions isolated to the anterosuperior quadrant of the mesotympanum which were cystic, easily removed and did not affect hearing. The others had more serious condition with extension into the posterior mesotympanum, which were large, often too extensive to indicate a formative site, and causing ossicular damage. CONCLUSION: CMEC presents in two distinctive forms according to the site of formation: the anterosuperior and posterior mesotympanum. The review suggest that the pathophysiology of posterior lesions may be different from anterior ones. For early diagnosis of CMEC, screening program should be carried out in children to prevent the more extensive diseases.

The Non-Squamous Cell Tumors of the Larynx / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The majority of laryngeal tumors are known to be squamous cell carcinoma. Non-squamous cell tumors of the larynx represent various spectrum of diseases with different prognosis and rationales of management from squamous cell carcinoma. Therapeutic options to these tumors depend on their histopathological characteristics and clinical behavior. To assess the unique aspects of the non-squamous cell tumors which can lead to their diagnosis and to provide considerations of management options and prognostic variables. MATERIALS AND METHODS: Eight non-squamous cell tumors of the larynx including 6 malignant tumors (two malignant lymphoma, single instances of adenoid cystic carcinoma, malignant schwanoma, osteosacoma, and spindle cell carcinoma) and two benign tumor (hemangioma, spindle cell lipoma) during the 7-year period from 1990 to 1996 were reviewed retrospectively. RESULTS: The frequently presenting symptoms were hoarseness, dyspnea, sore throat, and lump sensation in the throat. The most common origin site was supraglottis (50%), followed by glottis (38%) and subglottis (12%). All non-squamous malignancies beside non-Hodgkin's lymphoma were treated by surgery and postoperative radiotherapy. Two benign tumors were treated with only surgery. The mean follow-up period was 37.4 months and all patients have survivied with no evidence of recurrence. CONCLUSION: Our experience suggests that non-squamous cell tumors are a group of various pathological types and it may be possible to achieve better results with combined treatment including surgery.

A Case of Heterotopic Brain Tissue in the Soft Palate / 대한이비인후과학회지

Heterotopic brain tissue is a very rare developmental anomaly in which isolated brain tissue is found outside the cranial cavity. In the majority of cases, the nasal cavity is the most common site of involvement, and only 10 cases which were developed in the palate have been reported in the international literature. Recently, we experienced a case in which heterotropic brain tissue, a congenital tumor-like mass, was observed in the soft palate. This was considered initially as hemangioma under MRI study, and under this impression, we observed this lesion to regress spontaneously for two years. Surgical excisoin was performed for diagnostic and therapeutic purpose since the mass have had no interval change. Histopathological diagnosis of positive immunohistochemical reaction was performed for the heterotropic brain tissue using S-100 protein and glial fibrillary acidic protein (GFAP). At the present time, the patient has no evidence of the disease, recurrence nor complication. We present this case with a review of literature.