Masatoki Takahashi

Additional Heterozygous 2507A>C Mutation of WFS1 in Progressive Hearing Loss at Lower Frequencies

To describe the audiological profiles in a Japanese family with autosomal dominant hereditary sensorineural hearing loss (SNHL) and to identify the causative gene.

Delayed restoration of maximum speech discrimination scores in patients with idiopathic sudden sensorineural hearing loss

OBJECTIVE To assess possible delayed recovery of the maximum speech discrimination score (SDS) when the audiometric threshold ceases to change. METHODS We retrospectively examined 20 patients with idiopathic sudden sensorineural hearing loss (ISSNHL) (gender: 9 males and 11 females, age: 24-71 years). The findings of pure-tone average (PTA), maximum SDS, auditory brainstem responses (ABRs), and tinnitus handicap inventory (THI) were compared among the three periods of 1-3 months, 6-8 months, and 11-13 months after ISSNHL onset. RESULTS No significant differences were noted in PTA, whereas an increase of greater than or equal to 10% in maximum SDS was recognized in 9 patients (45%) from the period of 1-3 months to the period of 11-13 months. Four of the 9 patients showed 20% or more recovery of maximum SDS. No significant differences were observed in the interpeak latency difference between waves I and V and the interaural latency difference of wave V in ABRs, whereas an improvement in the THI grade was recognized in 11 patients (55%) from the period of 1-3 months to the period of 11-13 months. CONCLUSION The present study suggested the incidence of maximum SDS restoration over 1 year after ISSNHL onset. These findings may be because of the effects of auditory plasticity via the central auditory pathway.

Nasopharyngeal pleomorphic adenoma presenting as otitis media with effusion: Case report and literature review☆☆☆

Most tumors arising in the nasopharynx are malignant and frequently develop otitis media with effusion (OME). On the contrary, benign nasopharyngeal tumors are very rare, and pleomorphic adenoma, which is a benign mixed tumor of the nasopharynx, is also rarely encountered. We herein report a case of nasopharyngeal pleomorphic adenoma which initially presented as OME. This tumor completely blocked the orifice of the Eustachian tube but was removed by a combination of transnasal and transoral endoscopic resection. A defect in the mucous membrane was covered with polyglycolic acid sheet and fibrin glue. Mucous membrane completely covered the exposed tubal cartilage without adhesion near the tubal orifice. OME and hearing loss completely subsided 3 months after the surgery. She was disease-free 2 years after the surgery. Use of polyglycolic acid sheet could be a feasible mesh for closure of surgical defect without scarring, and it also led to healing of OME.

Modified paraffin-embedding method for the human cochlea that reveals a fine morphology and excellent immunostaining results.

Abstract Conclusion: A modified paraffin-embedding method could be applied to histopathological and immunohistochemical studies of the human cochlea. The complementary use of molecular and immunohistochemical techniques by means of this method is thus considered to be a valuable tool for the future study of the human inner ear. Objective: To propose a new paraffin-embedding method for the morphological and immunohistochemical study of the human cochlea. Methods: Five human temporal bones were harvested at autopsy. The temporal bone specimens were fixed in 20% buffered formalin, decalcified with EDTA, cropped to a cube of approximately 15 mm, embedded in paraffin, and then cut into 6 μm thick sections. The sections were stained with hematoxylin and eosin, and immunostained with anti-prestin and anti-neurofilament antibodies. Results: Although paraffin-embedded sections cannot show the excellent morphology of the delicate membranous labyrinth obtained with celloidin, this technique successfully preserved the morphology of the cochlea, especially the organ of Corti, thereby enabling us to obtain excellent immunostaining results.

Ex vivo visualization of the mouse otoconial layer compared with micro-computed tomography.

Hypothesis Micro-computed tomography (micro-CT) is useful for assessing the 3-dimensional (3D) morphology and age-related changes of the otoconial layer. Background Wriggle mouse Sagami (WMS) is a mutant of the plasma membrane Ca2+ ATPase type2 gene (Atp2b2) with deficits in the saccular otoconia. We examined the effectiveness of micro-CT in evaluating the otoconial layer of WMS and C57BL/6J mice. Methods Otic capsules of C57BL/6J mice at different fixation time were examined using micro-CT to evaluate the effects of the fixation time on the otoconial layer. Otic capsules of Atp2b2wri/wri, Atp2b2wri/+, and Atp2b2+/+ mice at P14, P21, and the age of 3 months were used to analyze age-related changes in the otoconial layer. A series of cross-section and 3D reconstructed images of the otoconial layer were obtained. The micro-CT findings were compared with the otic capsule findings cleared in methyl salicylate using stereomicroscopy. Results Micro-CT produced high-resolution images of the otoconial layer, thereby providing information regarding the spatial configuration and 3D curvature. There were no changes between the different fixation times. In the Atp2b2wri/+ and Atp2b2+/+ mice, the saccular and utricular otoconial layers were normal among all age groups. In the Atp2b2wri/wri mice, the saccular otoconial layer decreased on P14 and was absent on P21, whereas the utricular otoconial layer disappeared at 3 months of age. Conclusion We obtained precise information regarding the mouse otoconial layer with minimum artifacts. This method is expected to improve our understanding of the physiologic function and age-related changes in otolith organs.

An Unusual Invasive Ectopic Thymoma in the Thyroid and Anterior Mediastinum

An 81-year-old woman with a 2-year history of dysphagia detected a cervical mass. Computed tomography showed a thyroid tumor extending through the superior and anterior mediastinum. Analysis of an incisional biopsy specimen revealed a thymoma. Total resection of the thyroid and mediastinal tumor was performed. The thymoma invaded the anterior tracheal wall and left brachiocephalic vein. Pathologic examination revealed thymoma type B1 concomitant with B2 and B3 (World Health Organization classification), Masaoka IVb, and T3 N2 M0-IVb, with cervical lymph node metastasis. Clinicians must be cautious during radical operations for invasive ectopic thymomas.

Increased number of IgG4-positive plasma cells in chronic rhinosinusitis

Abstract Conclusion: High levels of IgG4-positive plasma cells were observed in tissue samples from ∼30% of patients with chronic rhinosinusitis who satisfied the comprehensive diagnostic criteria for IgG4-related disease. Detection of increased numbers of IgG4-positive plasma cells in the nasal cavity or paranasal sinuses might not be sufficient to make a diagnosis of IgG4-related rhinosinusitis, and a comprehensive evaluation is required. Objectives: This study aimed to clarify the clinicopathological characteristics of IgG4-positive plasma cells in patients with chronic rhinosinusitis. Method: This study examined nasal mucosal specimens from 35 patients and assigned them to high-IgG4 and low-IgG4 groups based on infiltration of IgG4-positive plasma cells. It compared the pathological characteristics of the two groups, including the presence of fibrosis, phlebitis, hyperplasia of the nasal glands and infiltration of inflammatory cells. Results: No cases of chronic rhinosinusitis showed storiform fibrosis or obliterative phlebitis. The mean number of IgG4-positive plasma cells in samples from all patients was 29.8 ± 40.3/high-power field. Eleven of the 35 cases (31.4%) were classified as high-IgG4. Hyperplasia of the nasal glands was observed significantly more frequently in the high-IgG4 group than in the low-IgG4 group (p = .03).

Bilateral cochlear ossification in a patient with Takayasu arteritis

Abstract Takayasu arteritis (TAK) is a rare type of noninfectious, systemic vasculitis of unknown etiology. A 45-year-old female diagnosed with TAK complained of acute onset bilateral deafness. Auditory tests revealed complete deafness in both ears. Magnetic resonance imaging with fluid-attenuation inversion recovery of her temporal bone demonstrated high signal intensity in the whole cochlea and vestibule in both ears. Computed tomography carried out 10 months after the onset of bilateral deafness revealed prominent ossification localized in the area around the modiolus in both cochleae. The patient underwent cochlear implantation in both ears. The cochlear lumens were narrowed and filled with scar tissue, which hindered the insertion of an electrode in both ears. In TAK, cases associated with bilateral severe to profound SNHL where corticosteroid therapy is ineffective in improving hearing, cochlear implantation should be considered within a reasonable period to avoid the difficulty of electrode insertion associated with cochlear ossification.

Bilateral hearing impairment as the initial symptom of sympathetic ophthalmia.

IMPORTANCE Sympathetic ophthalmia (SO), a rare bilateral panuveitis following penetrating ocular trauma or ocular surgery to one eye, shares a strikingly similar ocular pathology to that of Vogt-Koyanagi-Harada disease (VKH). Audiovestibular dysfunction is a major extraocular manifestation of VKH; however, to date, only a few cases of sympathetic ophthalmia associated with hearing loss have been reported from ophthalmologists, but not otolaryngologists. Accordingly, little is known about the audiovestibular findings in patients with SO. We herein present two cases of SO with preceding bilateral hearing loss. OBSERVATIONS The patient in Case 1, an 80-year-old female, experienced acute bilateral hearing loss. Five days after the onset of hearing loss, she presented with sudden bilateral blurred vision. In Case 2, a 32-year-old female noticed acute bilateral hearing loss and also experienced acute bilateral blurred vision the subsequent day. Patient 1 had a history of a penetrating injury to the right eye 25 days before the onset of hearing loss, while patient 2 had previously undergone right vitreous surgery twice for the treatment of a myopic macular hole and retinal detachment 36 and 43 days prior to the current symptom onset. Both cases were diagnosed as SO based on ocular findings of bilateral panuveitis and the history of ocular insult. Patient 1 carried HLA-DR4, HLA-DR15, HLA-A33, HLA-A24, HLA-B44 and HLA-B52, and patient 2 carried HLA-DR4. Audiograms showed bilateral mild to moderate sensorineural hearing loss in both cases, with normal auditory brainstem responses and deteriorated distortion product otoacoustic emission amplitudes. In addition, the significant recruitment phenomenon observed in case 1 suggested a cochlear origin of the hearing loss. Both patients received corticosteroid therapy, and the cochlear signs and symptoms recovered within one month. CONCLUSIONS AND RELEVANCE This is the first report to describe the comprehensive audiovestibular findings in patients with SO. In the present study, acute bilateral hearing loss developed a couple of days prior to the onset of bilateral visual loss and auditory examinations suggested a cochlear etiology in both cases.