Satoko Usui

High prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss

OBJECTIVE Radiological and genetic examination has recently advanced for diagnosis of congenital hearing loss. The aim of this study was to elucidate the prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss (USNHL) for better management of hearing loss and genetic and lifestyle counseling. METHODS We conducted a retrospective study of charts and temporal bone computed tomography (CT) findings of 69 consecutive patients 0-15 years old with USNHL. In two cases, genetic examination was conducted. RESULTS Of these patients, 66.7% had inner-ear and/or internal auditory canal malformations. The prevalence of malformations in infants (age <1 year) was 84.6%, which was significantly higher than that in children 1-15 years old (55.8%; p<0.01). Almost half of the patients (32; 46.4%) had cochlear nerve canal stenosis; 13 of them had cochlear nerve canal stenosis alone, and in 19 it accompanied other malformations. Internal auditory canal malformations were observed in 22 subjects (31.8%), 14 (20.3%) had cochlear malformations, and 5 (7.2%) had vestibular/semicircular canal malformations. These anomalies were seen only in the affected ear, except in two of five patients with vestibular and/or semicircular canal malformations. Two patients (2.9%) had bilateral enlarged vestibular aqueducts. Mutations were found in SLC26A4 in one of the two patients with bilateral large vestibular aqueducts. The prevalence of a narrow internal auditory canal was significantly higher in subjects with cochlear nerve canal stenosis (50.0%) than in subjects with normal cochlear nerve canals (11.1%; p<0.01). There were no correlations between the type and number of malformations and hearing level. CONCLUSIONS The prevalence of inner-ear and/or internal auditory canal malformations detected by high-resolution temporal bone CT in children with USNHL was very high. Radiological and genetic examination provided important information to consider the pathogenesis and management of hearing loss. Temporal bone CT should be recommended to children with USNHL early in life. SLC26A4 mutation also should be examined in cases with bilateral enlarged vestibular aqueduct.

Secretory cell differentiation and mucus secretion in cultures of human nasal epithelial cells : Use of a monoclonal antibody to study human nasal mucin

We have developed an air-liquid interface culture system for human nasal epithelial cells that differentiate into mucociliary phenotypes in a defined serum-free medium. Dissociated cells obtained from nasal polyps were cultured on a collagen gel substrate. At confluence, the cells lost characteristics of differentiated cells, and secretory cell and ciliated cell differentiation appeared after 7 days in an air-liquid interface. After 21 days, about half of the epithelial cells were stained with Alcian blue—periodic acid—Schiff stain or monoclonal antibody HCS18, which was directed against human nasal mucin specific for epithelial secretory (goblet) cells. The quantitative examination using the antibody HCS 18 revealed that the antibody-reactive nasal mucin was secreted only on the apical side of the cultures, and interleukin-1 β and tumor necrosis factor α stimulated these mucus secretions. The culture system with an antimucin monoclonal antibody developed in this study should be useful for studying polarized mucus secretion from human nasal epithelial cells.

Biomarkers for Allergen Immunotherapy in Cedar Pollinosis

To initiate, monitor, and complete effective immunotherapy, biomarkers to predict and visualize the immune responses are needed. First, we need to identify the right candidate for immunotherapy. Secondly, the immune responses induced by immunotherapy should be monitored. For the first objective, analysis of polymorphisms of candidate genes may be helpful, but still be in development. Regarding biomarkers for immune responsese, there are numerous reports that evaluate immunotherapy-induced immune changes such as suppression of effector cells, deviation to Th1 cytokine production, and induction of regulatory T cells. No standardized methods, however, have been established. Among them, a functional assay of blocking IgG activity, the IgE-facilitated allergen binding assay, may be useful. We quantitated induced expression of an activation marker, CD203c, on basophils and found that the assay efficiently predicts sensitivity to particular allergen and severity of the allergen-induced symptoms. In patients who received rush immunotherapy for Japanese cedar pollinosis, reduction in CD203c expression after the therapy was observed, suggesting the utility of the test for monitoring immunotherapy.

Cochlear implantation in complete remission in a patient with leukemia

Patients with leukemia have an increased risk of developing sensorineural hearing loss. This is a retrospective review of a profoundly deafened patient with acute myelogenous leukemia who underwent cochlear implantation. The 26-year-old patient was successfully implanted with a Nucleus cochlear implant in the complete remission after peripheral blood stem cell transplantation. To date, with a follow-up of 1 year, the patient has not experienced any complication and has regained useful open-set speech perception. To our knowledge, this is the first reported case of successful cochlear implantation in a patient deafened by acute myelogenous leukemia.

Independent exercise for glottal incompetence to improve vocal problems and prevent aspiration pneumonia in the elderly: a randomized controlled trial:

Objectives: To evaluate the effect of a self-controlled vocal exercise in elderly people with glottal closure insufficiency. Design: Parallel-arm, individual randomized controlled trial. Methods: Patients who visited one of 10 medical centers under the National Hospital Organization group in Japan for the first time, aged 60 years or older, complaining of aspiration or hoarseness, and endoscopically confirmed to have glottal closure insufficiency owing to vocal cord atrophy, were enrolled in this study. They were randomly assigned to an intervention or a control group. The patients of the intervention group were given guidance and a DVD about a self-controlled vocal exercise. The maximum phonation time which is a measure of glottal closure was evaluated, and the number of patients who developed pneumonia during the six months was compared between the two groups. Results: Of the 543 patients enrolled in this trial, 259 were allocated into the intervention group and 284 into the control; 60 of the intervention group and 75 of the control were not able to continue the trial. A total of 199 patients (age 73.9 ±7.25 years) in the intervention group and 209 (73.3 ±6.68 years) in the control completed the six-month trial. Intervention of the self-controlled vocal exercise extended the maximum phonation time significantly (p < 0.001). There were two hospitalizations for pneumonia in the intervention group and 18 in the control group, representing a significant difference (p < 0.001). Conclusion: The self-controlled vocal exercise allowed patients to achieve vocal cord adduction and improve glottal closure insufficiency, which reduced the rate of hospitalization for pneumonia significantly. Clinical Trial.gov Identifier-UMIN000015567

Two Cases of Tuberculosis of the Parotid Gland.

Tuberculosis of the parotid gland is not a common disease. We reported here two cases of tuberculosis of the parotid gland. The first case is a 78-year-old man who had a left parotid gland mass for 10 years. We suspected pleomorphic adenoma from the preoperative findings of MRI and performed a parotidectomy. Postoperative pathological findings showed a Warthins tumor of the parotid gland with tuberculosis. We realized that this case was very rare.The second case is a 76-year-old woman who had had a left parotid gland mass for 1 month. The mass had rapidly enlarged and adhered to the skin, in addition to the skin becoming reddish. She complained of tenderness at the mass. We suspected a malignant tumor of the parotid gland and performed surgical resections with the skin. Postoperative pathological findings showed tuberculosis of the parotid gland.Tuberculosis is one of the most important of the re-emerging infectious diseases in the world. We should always consider tuberculosis, because early diagnosis and treatment are mandatory.