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1.
Stem Cell Res ; 67: 103017, 2023 03.
Article in English | MEDLINE | ID: mdl-36630839

ABSTRACT

We report the establishment of two human induced pluripotent stem cell (iPSC) lines from individuals without auditory disorders. Extensive audiometry tests were performed to confirm normal hearing. The generated iPSC lines expressed pluripotency genes and showed differentiation capability into the three germ layers. The iPSC lines will be used as controls for pathological analysis and drug screening for ear disorders.


Subject(s)
Induced Pluripotent Stem Cells , Humans , Induced Pluripotent Stem Cells/metabolism , Cell Differentiation/genetics
2.
Ear Nose Throat J ; : 1455613221112346, 2022 Jun 29.
Article in English | MEDLINE | ID: mdl-35768166

ABSTRACT

OBJECTIVES: To evaluate the effectiveness of sound therapy using hearing aids in patients experiencing tinnitus with acquired unilateral sensorineural hearing loss. METHODS: A total of 97 patients with unilateral tinnitus with acquired ipsilateral sensorineural hearing loss were included. Evaluation involved self-report questionnaires administered at entry, 3 months after treatment, and 1 year after treatment. RESULTS: The mean Tinnitus Handicap Inventory score before treatment decreased significantly (p < 0.01) at 3 months (50.0 ± 24.5-12.7 ± 16.2), and 1 year (53.3 ± 25.5-8.79 ± 13.9), after treatment. Moreover, the visual analog scale score decreased significantly at 3 months (loudness, 69.6 ± 21.9-29.1 ± 27.2; annoyance, 71.1 ± 24.7-22.7 ± 25.5) and 1 year (loudness, 69.8 ± 22.0-21.1 ± 26.1; annoyance, 72.7 ± 25.6-19.4 ± 27.6). Approximately 80-90% of patients noticed improvements in tinnitus annoyance and loudness, as determined by their responses to the questionnaires of subjective symptom improvement. CONCLUSIONS: Hearing aids are remarkably effective for patients with unilateral tinnitus with acquired ipsilateral sensorineural hearing loss.

3.
Acta Otolaryngol ; 141(8): 773-779, 2021 Aug.
Article in English | MEDLINE | ID: mdl-34369276

ABSTRACT

BACKGROUND: The effectiveness of hearing aids for tinnitus patients without hearing loss in conversation-comprehension range and inconvenience in daily communication is unknown. OBJECTIVE: We aimed to evaluate their therapeutic effect. MATERIALS AND METHODS: We included 91 patients with average hearing levels <30 dBHL. To determine the effects, self-reported questionnaires were used. Data were collected at the entry and 3 months (n = 91) and 1 year (n = 70) after treatment initiation. RESULTS: All scores (mean ± standard deviation) decreased significantly (p < 0.0001) from before treatment to 3 months and 1 year after treatment: tinnitus handicap inventory score: from 56 ± 21 to 17 ± 17 and 13 ± 15; visual analogue scale (VAS) score for tinnitus loudness: from 71 ± 20 to 31 ± 26 and 28 ± 29; VAS score for tinnitus annoyance: 79 ± 20 to 27 ± 25 and 26 ± 30, respectively. Approximately 90% of patients noticed improvements in tinnitus annoyance and loudness, as determined by the questionnaires regarding subjective symptom improvement. CONCLUSIONS: Hearing aids may be useful for tinnitus patients without hearing loss and inconvenience in daily communication. SIGNIFICANCE: Our findings provide a treatment option for tinnitus patients with an average hearing level of <30 dBHL.


Subject(s)
Hearing Aids , Tinnitus/therapy , Adult , Aged , Aged, 80 and over , Audiometry, Pure-Tone , Female , Hearing , Humans , Male , Middle Aged , Self Report , Treatment Outcome
4.
J Int Adv Otol ; 17(1): 42-45, 2021 Jan.
Article in English | MEDLINE | ID: mdl-33605220

ABSTRACT

OBJECTIVES: The tinnitus functional index (TFI) is becoming a new international gold standard for tinnitus assessment. This study aimed to evaluate the efficacy of hearing aids for chronic tinnitus, using the Japanese version of the TFI, while focusing on its subscales. MATERIALS AND METHODS: This is a retrospective study. A total of 21 patients with chronic tinnitus were included. All participants were treated with hearing aids for at least 12 months. They answered the TFI and tinnitus handicap inventory (THI) at the first visit and 12 months later. We analyzed each TFI subscale score and the overall scores of TFI and THI. RESULTS: The overall TFI score decreased significantly after treatment (p=0.005) with moderate effect size (d=0.70). The scores of the intrusive, sense of control, sleep, and emotional subscales decreased significantly after the treatment. Large effect sizes were found in the intrusive and sense of control subscales (d=1.33 and d=1.25, respectively). CONCLUSION: Hearing aids are highly effective for improving the intrusive and sense of control subscales in patients with tinnitus. Identifying better treatments for the small effect size subscales and combining these with the use of hearing aids could achieve a higher therapeutic effect with better outcomes.


Subject(s)
Hearing Aids , Tinnitus , Factor Analysis, Statistical , Humans , Retrospective Studies , Severity of Illness Index , Surveys and Questionnaires
5.
Auris Nasus Larynx ; 48(5): 815-822, 2021 Oct.
Article in English | MEDLINE | ID: mdl-33461856

ABSTRACT

OBJECTIVE: This study aimed to assess the effectiveness of our sound therapy with appropriate hearing aid fitting and periodic hearing aid adjustment in patients with chronic tinnitus. METHODS: We conducted a retrospective study. The study included 490 individuals who received treatment with hearing aids for chronic tinnitus at least for 3 months. To determine the effects of tinnitus on patients' quality of life, the participants completed a series of questionnaires, including the Tinnitus Handicap Inventory (THI), Visual Analogue Scale (VAS) for loudness and annoyance, and questionnaires of subjective symptom improvement. Data were collected at entry and 3 months and 1 year after treatment initiation. RESULTS: All 490 participants completed the questionnaires at 3 months; however, only 312 completed them at 1 year. The mean ± standard deviation THI score before treatment decreased significantly at 3 months (490 participants: 53 ± 25 to 11 ± 16 and 312 participants: 55 ± 24 to 12 ± 16) and 1 year (55 ± 24 to 9 ± 14) (P < 0.01). The mean VAS score for tinnitus loudness before treatment decreased significantly at 3 months (490 participants: 70 ± 22 to 25 ± 27 and 312 participants: 71 ± 22 to 27 ± 26) and 1 year (71 ± 22 to 21 ± 28) (P < 0.01). In addition, the mean VAS score for tinnitus annoyance before treatment decreased significantly at 3 months (490 participants: 75 ± 26 to 20 ± 26 and 312 participants: 75 ± 25 to 23 ± 27) and 1 year (75 ± 25 to 17 ± 26) (P < 0.01). Approximately 80% of patients noticed improvements in their tinnitus annoyance and loudness, as determined by their responses to the questionnaires of subjective symptom improvement. CONCLUSION: The results of this study suggest that treatment with sound therapy may ameliorate the symptoms of chronic tinnitus associated with hearing loss.


Subject(s)
Acoustic Stimulation/methods , Hearing Aids , Patient Education as Topic , Tinnitus/rehabilitation , Adult , Aged , Aged, 80 and over , Female , Hearing Loss, Sensorineural/complications , Hearing Loss, Sensorineural/physiopathology , Hearing Loss, Sensorineural/rehabilitation , Humans , Male , Middle Aged , Quality of Life , Tinnitus/complications , Tinnitus/physiopathology , Treatment Outcome , Young Adult
6.
Eur Arch Otorhinolaryngol ; 278(11): 4243-4249, 2021 Nov.
Article in English | MEDLINE | ID: mdl-33386435

ABSTRACT

PURPOSE: To evaluate tinnitus and its management in patients with vestibular schwannoma (VS) who underwent surgery, we investigate the effect of surgical approach or residual hearing on tinnitus severity and the effects of intervention for tinnitus including educational counseling, sound therapy using hearing aids (HAs), and medication (selective serotonin reuptake inhibitors, and SSRIs). METHODS: Seventy-one subjects of VS patients who underwent surgery were included. Their tinnitus severity was evaluated using the Japanese version of the Tinnitus Handicap Inventory (THI). The relationships between postoperative THI scores and surgery types or residual hearing levels were examined. We also examined longitudinal changes in THI scores and the efficacy of the intervention. RESULTS: Surgery approach, hearing preservation or hearing loss surgery, and residual hearing levels were not significantly related to the postoperative tinnitus severity. In 71 cases, 45 cases did not require any management for tinnitus. On the contrary, 26 patients had at least one episode of tinnitus distress (THI score was greater than or equal to 18). Educational counseling alone was found to be effective in 17 cases out of the 26 cases, and the remaining 9 cases required more intervention than educational counseling alone. We selected sound therapy with HA for 7 cases and administration of SSRI for 2 cases, which was found to be highly effective in 8 cases. CONCLUSION: Based on the present study, we consider that appropriate management may be possible for tinnitus in the majority of VS patients who underwent surgery.


Subject(s)
Deafness , Hearing Aids , Neuroma, Acoustic , Tinnitus , Hearing , Humans , Neuroma, Acoustic/complications , Neuroma, Acoustic/surgery , Tinnitus/etiology , Tinnitus/therapy
7.
Eur Arch Otorhinolaryngol ; 278(11): 4251-4258, 2021 Nov.
Article in English | MEDLINE | ID: mdl-33389010

ABSTRACT

PURPOSE: To determine pre- and post-treatment factors that are useful for predicting the prognosis of hearing improvement in idiopathic sudden sensorineural hearing loss (ISSHL). METHODS: This retrospective study included 332 patients with ISSHL. Patients received intravenous steroid treatment (prednisolone sodium succinate; 120 mg/day followed by dose tapering). Complete recovery of hearing levels was defined as a final pure-tone audiometry of ≤ 20 dB HL or the same level as the contralateral ear. Patients' age; sex; side of hearing loss; initial hearing level; days from onset to treatment; presence of vertigo, diabetes, and hypertension; and hearing improvement on days 3-4 and 6-7 after treatment initiation were analyzed as potential prognostic factors. RESULTS: Overall, 109 patients (32%) had complete recovery. Results of the multivariate logistic regression model identified age (odds ratio [OR] = 0.974), initial hearing level (OR = 0.949), vertigo (OR = 0.409), and hearing improvement on days 6-7 after treatment initiation (OR = 1.11) as significant independent predictors of complete recovery. Age ≥ 60 years, initial hearing level ≥ 72.5 dB HL, and vertigo contributed to poor prognosis. Patients without these three factors and a hearing improvement of ≥ 10 dB HL on days 6-7 post-treatment had a complete recovery rate of 80%. Only 1.5% of the patients with 2-3 of these factors and a hearing improvement of < 10 dB HL on days 6-7 after treatment initiation achieved complete recovery. CONCLUSION: Age, initial hearing level, vertigo, and hearing improvement on days 6-7 after treatment initiation were independent predictors of hearing recovery in ISSHL.


Subject(s)
Hearing Loss, Sensorineural , Hearing Loss, Sudden , Audiometry, Pure-Tone , Glucocorticoids , Hearing , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/drug therapy , Hearing Loss, Sudden/diagnosis , Hearing Loss, Sudden/drug therapy , Humans , Middle Aged , Prognosis , Retrospective Studies , Treatment Outcome , Vertigo/diagnosis , Vertigo/drug therapy
8.
Psychiatr Q ; 92(1): 249-257, 2021 03.
Article in English | MEDLINE | ID: mdl-32613523

ABSTRACT

In Japan, there have been no examinations of tinnitus with respect to personality traits, and only a few studies have investigated sleep disorders in tinnitus. Understanding the association between these can aid in selecting and developing effective treatment options for patients with tinnitus. This study aimed to clarify the relationship between tinnitus severity, personality, and sleep disorders, in patients with chronic tinnitus in Japan. We retrospectively evaluated the personality factors of 56 patients presenting with tinnitus using the NEO Five-Factor Inventory (NEO-FFI), which examines five personality characteristics, including Neuroticism, Extraversion, Openness, Agreeableness, and Conscientiousness. We evaluated tinnitus severity, sleep disorders, depression, and anxiety using the Tinnitus Handicap Inventory, Pittsburgh Sleep Quality Index (PSQI), Self-Rating Depression Scale, and State-Trait Anxiety Inventory, respectively. Neuroticism and tinnitus severity were positively correlated (r = 0.548, p < 0.001). Sleep disorders were observed in 73.2% of patients; however, there was no correlation between the PSQI score and tinnitus severity or between PSQI score and scores of the five NEO-FFI items. Sleep disorders are frequently experienced by patients with chronic tinnitus, regardless of tinnitus severity or personality. Understanding the relationship between personality and tinnitus severity could lead to better management of tinnitus.


Subject(s)
Personality , Sleep Wake Disorders/complications , Sleep Wake Disorders/physiopathology , Tinnitus/complications , Tinnitus/psychology , Adult , Aged , Aged, 80 and over , Female , Humans , Japan , Male , Middle Aged , Retrospective Studies , Sleep Wake Disorders/diagnosis , Tinnitus/physiopathology , Young Adult
9.
J Am Acad Audiol ; 32(9): 556-561, 2021 10.
Article in English | MEDLINE | ID: mdl-35176798

ABSTRACT

BACKGROUND: There are several types of silicone domes in receiver in the canal hearing aids (RICs), which have different occlusion levels. However, the frequency characteristics of each type of silicone dome are unclear. PURPOSE: This study aimed to determine the frequency characteristics of three types of silicone domes (open domes, double-type power domes, and tulip domes) in RICs. RESEARCH DESIGN: This is an interventional study. STUDY SAMPLE: In total, 11 participants with a normal sense of hearing were prospectively enrolled. INTERVENTION: Participants were fitted with hearing aids, which were adjusted to similar settings, and only the silicone domes were changed. The acoustic gain of hearing aids was adjusted to 20 dB in the range of 250 Hz to 4 kHz using the 2-cc coupler. DATA COLLECTION AND ANALYSIS: We measured the real-ear aided gain (REAG) for each type of silicone dome. In each frequency, we statistically compared the REAG of each type of dome. Acoustic gain using the Open Fit coupler of each type of dome was also measured. RESULTS: The REAG was obtained with no leakage with open domes only at 2 kHz, and with tulip domes and double-type power domes in the range of 1 to 2 kHz. Double-type power domes obtained significantly higher REAG than tulip domes at 250 and 500 Hz. Under the Open Fit coupler, all types obtained higher acoustic gain than the REAG. CONCLUSION: This study provides the analysis of the frequency characteristics of silicone domes in RICs. The highest degree of occlusion was observed in double-type power domes, followed by tulip domes, and the lowest was observed in open domes.


Subject(s)
Hearing Aids , Acoustics , Ear , Hearing Tests , Humans , Silicones
11.
Acta Otolaryngol ; 140(9): 736-740, 2020 Sep.
Article in English | MEDLINE | ID: mdl-32493099

ABSTRACT

Background: Individuals with 22q11.2 deletion syndrome (22q11.2DS) exhibit various phenotypes.Objective: To compare the clinical and otorhinolaryngological features of Japanese patients with 22q11.2DS with those of patients reported in Western literature.Materials and methods: We retrospectively assessed the medical records of 17 Japanese patients with 22q11.2DS and compared our findings with previously reported findings in Western literature.Results: Hearing loss was the most frequent complaint (n = 8, 47%), followed by articulation disorders and/or nasopharyngeal closure failure (n = 4, 24%) and language development delay (n = 2, 12%). Ten patients (59%) had hearing loss regardless of the chief complaint (total 15 ears - mild, 9; moderate, 6). Four patients had bilateral hearing loss. One patient (6%) underwent tympanostomy tube placement for refractory exudative otitis media, another (6%) underwent myringoplasty, and three patients (18%) underwent tympanoplasties for chronic otitis media or middle ear malformation. Previous studies in Western countries reported similar results in terms of frequency of hearing loss, severity of hearing loss, and the percentage of middle ear malformations.Conclusions: The otorhinolaryngological characteristics of Japanese patients with 22q11.2DS were similar to those in Western countries. Hearing loss was primarily caused by disorders like otitis media and middle ear malformation.Significance: Our findings may aid treatment planning for Asian patients with 22q11.2DS.


Subject(s)
DiGeorge Syndrome/complications , Hearing Loss/etiology , Adolescent , Adult , Child , Child, Preschool , Female , Humans , Infant , Japan , Male , Otitis Media/etiology , Retrospective Studies , Velopharyngeal Insufficiency/complications , Western World
13.
Int J Audiol ; 58(3): 167-173, 2019 03.
Article in English | MEDLINE | ID: mdl-30700182

ABSTRACT

OBJECTIVE: The tinnitus functional index (TFI), a new self-report questionnaire, was developed for the measurement of tinnitus severity and treatment-related changes. The original English version has been translated into several languages. The aim of this study was to evaluate the reliability and validity of the Japanese version of the TFI. DESIGN: The English version of the TFI was translated into Japanese using a translation-back translation method. Participants completed a series of questionnaires including the TFI, Tinnitus Handicap Inventory (THI), Self-rating Depression Scale, State-Trait Anxiety Inventory, Visual Analogue Scale, and the questionnaire of subjective symptom improvement. We examined internal consistency, reproducibility, convergent and discriminant validity, and effect sizes. STUDY SAMPLE: One hundred and forty-three patients with subjective tinnitus were included. RESULTS: The Japanese version of the TFI had high internal consistency and reproducibility, and good convergent and discriminant validity. At six months after the start of treatment, the effect sizes for the TFI were somewhat larger than those for the THI. CONCLUSIONS: The Japanese version of the TFI is appropriate for measuring tinnitus severity and treatment-related changes. The reliability and validity of this version are comparable to those of the original English version of the TFI.


Subject(s)
Severity of Illness Index , Tinnitus , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Outcome Assessment, Health Care , Reproducibility of Results , Young Adult
14.
BMC Pediatr ; 18(1): 171, 2018 05 23.
Article in English | MEDLINE | ID: mdl-29792164

ABSTRACT

BACKGROUND: Waardenburg syndrome type 1 (WS1) can be distinguished from Waardenburg syndrome type 2 (WS2) by the presence of dystopia canthorum. About 96% of WS1 are due to PAX3 mutations, and SOX10 mutations have been reported in 15% of WS2. CASE PRESENTATION: This report describes a patient with WS1 who harbored a novel SOX10 nonsense mutation (c.652G > T, p.G218*) in exon 3 which is the penultimate exon. The patient had mild prodromal neurological symptoms that were followed by severe attacks of generalized seizures associated with delayed myelination of the brain. The immature myelination recovered later and the neurological symptoms could be improved. This is the first truncating mutation in exon 3 of SOX10 that is associated with neurological symptoms in Waardenburg syndrome. Previous studies reported that the neurological symptoms that associate with WS are congenital and irreversible. These findings suggest that the reversible neurological phenotype may be associated with the nonsense mutation in exon 3 of SOX10. CONCLUSIONS: When patients of WS show mild prodromal neurological symptoms, the clinician should be aware of the possibility that severe attacks of generalized seizures may follow, which may be associated with the truncating mutation in exon 3 of SOX10.


Subject(s)
Mutation , Seizures/etiology , Waardenburg Syndrome/complications , Waardenburg Syndrome/genetics , Exons , Humans , Infant , Male , SOXE Transcription Factors/genetics , Waardenburg Syndrome/diagnosis
15.
Auris Nasus Larynx ; 44(4): 489-492, 2017 Aug.
Article in English | MEDLINE | ID: mdl-27552828

ABSTRACT

OBJECTIVE: To report the treatment efficacy of continual intratympanic steroid injection (ITSI) therapy in a patient with refractory sensorineural hearing loss accompanied by relapsing polychondritis. PATIENT: A 49-year-old female diagnosed with relapsing polychondritis at the age of 45 years and who had been treated with corticosteroids and immunosuppressants developed sensorineural hearing loss in the left ear. INTERVENTION: Her unilateral hearing loss did not recover despite receiving one cyclophosphamide pulse treatment, one methylprednisolone pulse treatment, and weekly leukapheresis. Thus, we decided to initiate weekly ITSI therapy. MAIN OUTCOME MEASURE: Pure tone audiometry. RESULTS: A week after the first ITSI treatment, the patient's hearing improved. We continued ITSI therapy and attempted to extend the interval between treatments, but her hearing worsened when ITSI therapy was delivered at 2- to 3-week intervals. Thus, we returned ITSI therapy to once per week for 21 months (total of 71 treatments). She experienced no adverse events, like tympanic perforation, and 1 year after terminating the therapy, her hearing remained stable and did not worsen. CONCLUSIONS: Continual, weekly ITSI therapy was effective in treating sensorineural hearing loss accompanied by relapsing polychondritis. ITSI therapy may be a promising treatment option for sensorineural hearing loss caused by autoimmune disease.


Subject(s)
Dexamethasone/analogs & derivatives , Glucocorticoids/administration & dosage , Hearing Loss, Sensorineural/drug therapy , Hearing Loss, Unilateral/drug therapy , Polychondritis, Relapsing/drug therapy , Audiometry, Pure-Tone , Dexamethasone/administration & dosage , Female , Hearing Loss, Sensorineural/complications , Hearing Loss, Sensorineural/physiopathology , Hearing Loss, Unilateral/complications , Hearing Loss, Unilateral/physiopathology , Humans , Injection, Intratympanic , Middle Aged , Polychondritis, Relapsing/complications , Treatment Outcome
16.
Nihon Jibiinkoka Gakkai Kaiho ; 119(5): 721-6, 2016 May.
Article in Japanese | MEDLINE | ID: mdl-27459817

ABSTRACT

BACKGROUND: Preoperative diagnosis of lymph node metastasis from thyroid carcinoma is usually confirmed by using fine needle aspiration cytology (FNAC) when thyroid carcinoma is suspected based on the clinical findings. However, the result of FNAC sometimes leads to a false negative, especially in cases of hypocellular lesions such as metastases with cystic change. Thyroglobulin measurement in fine needle aspirates (FNA-Tg) has been shown to be a useful technique to detect the protein specifically secreted by thyroid follicular cells. Elevated FNA-Tg levels in an extra-thyroidal lesion means that the lesion comprises thyroid-originated tissue, most of which suggests the metastasis from thyroid carcinoma. Thus, FNA-Tg is expected to improve the sensitivity of FNAC for the aforementioned purpose. PATIENTS AND METHODS: From 2008 to 2012, 49 extra-thyroidal lesions from 43 patients with thyroid carcinoma were examined using both FNAC and FNA-Tg, followed by surgical resection with a histopathological diagnosis. The results were retrospectively reviewed and analyzed. RESULTS: Among 49 lesions, 47 were metastatic lymph nodes from thyroid carcinoma (46 papillary carcinoma and one follicular carcinoma), one was a metastatic lymph node from submandibular gland adenocarcinoma, and one was an ectopic thyroid gland. In the 47 cases of thyroid carcinoma, the sensitivity of FNAC was 57.4% (27/47), whereas that of FNA-Tg was 76.6% (36/47). When both methods were combined, the sensitivity increased to 93.6% (44/47). Metastasis from submandibular gland adenocarcinoma was considered to be an example of a false positive from FNAC, whereas an ectopic thyroid gland was an FNA-Tg false positive. Three lesions were negative for both FNAC and FNA-Tg, although metastases were suspected by imaging studies and confirmed by histopathological diagnosis, which were consistent with examples of a false negative from both FNAC and FNA-Tg findings. CONCLUSIONS: FNAC reflects whether the lesion has malignant cells, whereas FNA-Tg reflects whether the lesion has thyroid-originated tissue that specifically secrets thyroglobulin. Therefore, FNAC and FNA-Tg are considered to be complementary to each other for the preoperative diagnosis of lymph node metastasis from thyroid carcinoma. FNA-Tg was validated to improve the preoperative diagnostic sensitivity especially when combined with FNAC, however, it is attended with the possibility of a false positive or negative finding, which requires caution in interpretation of the findings.


Subject(s)
Neck/pathology , Thyroglobulin/analysis , Thyroid Neoplasms/pathology , Adolescent , Adult , Aged , Aged, 80 and over , Biopsy, Fine-Needle , Female , Humans , Lymphatic Metastasis , Male , Middle Aged , Thyroid Neoplasms/chemistry , Young Adult
17.
Neuroreport ; 27(11): 833-6, 2016 08 03.
Article in English | MEDLINE | ID: mdl-27341211

ABSTRACT

Wolfram syndrome is an autosomal recessive disorder of the neuroendocrine system, known as DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy and Deafness) syndrome, and considered an endoplasmic reticulum disease. Patients show mutations in WFS1, which encodes the 890 amino acid protein wolframin. Although Wfs1 knockout mice develop diabetes, their hearing level is completely normal. In this study, we examined the expression of wolframin in the cochlea of a nonhuman primate common marmoset (Callithrix jacchus) to elucidate the discrepancy in the phenotype between species and the pathophysiology of Wolfram syndrome-associated deafness. The marmoset cochlea showed wolframin immunoreactivity not only in the spiral ligament type I fibrocytes, spiral ganglion neurons, outer hair cells, and supporting cells, but in the stria vascularis basal cells, where wolframin expression was not observed in the previous mouse study. Considering the absence of the deafness phenotype in Wfs1 knockout mice, the expression of wolframin in the basal cells of primates may play an essential role in the maintenance of hearing. Elucidating the function of wolframin protein in the basal cells of primates would be essential for understanding the pathogenesis of hearing loss in patients with Wolfram syndrome, which may lead to the discovery of new therapeutics.


Subject(s)
Cochlea/metabolism , Membrane Proteins/metabolism , Animals , Callithrix , Cochlea/anatomy & histology , Connexin 26/metabolism , Female , Male , Myosin VIIa , Myosins/metabolism , Phenotype
18.
Nihon Jibiinkoka Gakkai Kaiho ; 118(7): 860-6, 2015 Jul.
Article in Japanese | MEDLINE | ID: mdl-26427126

ABSTRACT

The evaluation and management of vertigo in children varies among institutional and medical specialties. The aim of this study was to describe the characteristics of vertigo in children presenting at a national pediatric center. Patients < 16 years old presenting with vertigo to the department of otolaryngology at a national center for child health and development from April 2004 to October 2009 were included (N = 77; 42 males and 35 females; average age, 8.7 ± 3.4 years) in this study. The most common diagnoses were vestibular migraine (VM; N = 21), benign paroxysmal vertigo (BPV; N =16), unilateral vestibulopathy (N = 12), and psychogenic vertigo (N = 8). Significant differences were observed in the frequency of the diagnoses between children aged older and younger than 7 years: BPV was most common in children < 7 years of age (p < 0.01) and VM was most common in ≥ 27 years of age (p < 0.05). Because obtaining adequate information from children for making a correct diagnosis is sometimes difficult, acquiring sufficient information from the parents is important. In addition, getting the parents to record the nystagmus during a vertigo attack with a digital camera or cellular phone can be useful because observing the nystagmus recorded on the video is helpful for making a diagnosis. Furthermore, the parents are participating in their child's care by attempting to record the attack, strengthening the relationship between the parents and the child. The incidence of psychogenic vertigo is low (less than 10%). Therefore, although physicians have recently tended to define the disorder as psychogenic when no objective abnormality is found in a patient, making a diagnosis of psychogenic vertigo is not recommended. Because vertigo can sometimes make a child anxious, delivering the correct diagnosis and treatment at the early stage is important for preventing anxiety in affected children.


Subject(s)
Vertigo/diagnosis , Adolescent , Child , Female , Humans , Male , Retrospective Studies , Tokyo/epidemiology , Vertigo/epidemiology
19.
Nihon Jibiinkoka Gakkai Kaiho ; 118(3): 206-12, 2015 Mar.
Article in Japanese | MEDLINE | ID: mdl-26349336

ABSTRACT

INTRODUCTION: We commonly use data obtained prior to cervical lymph node biopsy for the diagnosis of malignant lymphoma. Based on such data, we can confirm whether a biopsy should be performed in some cases. Currently, the parameters used to indicate a presurgery examination prior to biopsy have been very few. So, we retrospectively analyzed cases of cervical lymph node biopsy. Moreover, we examined the usefulness of clinicobiological data obtained prior to cervical lymph node biopsy to evaluate various factors related to the diagnosis of malignant lymphoma. METHODS: This study included 77 patients for whom the initial diagnostic impression was malignant lymphoma before surgery. Of the 77 cases, 40 were diagnosed as having malignant lymphoma and 37 were diagnosed as having non-malignant lymphoma. We performed a case-controlled study to evaluate the differences in clinicobiological data between malignant and non-malignant lymphoma in terms of the following parameters: (1) age, (2) sex, (3) number of white blood cells, (4) white blood cell lymphocyte count, (5) percentage of white blood cell lymphocytes, (6) percentage of eosinophils (%), (7) percentage of monocytes (%), (8) atypical lymphocytes (%), (9) hemoglobin level, (10) lactate dehydrogenase level, (11) C-reactive protein level, (12) soluble interleukin-2 receptor (IL-2R) level, and (13) cytological findings. We used multivariate and univariate analyses to study the data statistically. RESULTS: The following 5 factors were found to be significant in a Wilcoxon t-test for malignant lymphoma: percentage of white blood cell lymphocytes, sIL-2R level, age, white blood cell lymphocyte count, and cytological findings; these factors were also significant when examined using a Pearson χ2 test. The other factors did not differ significantly between the malignant and non-malignant lymphomas. The percentage of white blood cell lymphocytes and the cytological findings were identified as significant independent factors for the diagnosis of malignant lymphoma in a multivariate analysis, whereas the other factors were not found to be significant. CONCLUSION: Based on the results of the univariate and multivariate analyses performed in the present study, the decline in the percentage of white blood cell lymphocytes and the cytological findings obtained prior to cervical lymph node biopsy are significant indicators of malignant lymphoma.


Subject(s)
Lymph Nodes/pathology , Lymphoma/pathology , Age Distribution , Biomarkers/blood , Biopsy , Female , Humans , Male , Middle Aged , Neck/pathology
20.
Auris Nasus Larynx ; 42(4): 318-21, 2015 Aug.
Article in English | MEDLINE | ID: mdl-25717026

ABSTRACT

OBJECTIVE: Closure of a tracheoesophageal puncture (TEP) formed during voice prosthesis implantation may sometimes be required. We report a new method of closure that we have termed the RESET method. METHODS: We used the RESET method for four patients. An initial incision was made at the mucocutaneous junction of the tracheostoma, and the trachea and esophagus were separated. The TEP was sectioned, and the tracheal and esophageal sides were sutured into separate layers. A pedicled fascia flap lifted from the sternocleidomastoid muscle was sandwiched between the trachea and the esophagus, and subsequently secured. RESULTS: The TEP was closed in all patients, and no complications were observed. CONCLUSION: The RESET method, using a pedicled fascia flap with stable perfusion, is a simple, reliable method of TEP closure.


Subject(s)
Esophagus/surgery , Fascia/transplantation , Laryngectomy/rehabilitation , Larynx, Artificial , Neck Muscles , Surgical Flaps , Trachea/surgery , Wound Closure Techniques , Aged , Device Removal/methods , Female , Humans , Male , Middle Aged , Prosthesis Failure , Prosthesis Implantation/methods , Retrospective Studies , Treatment Outcome
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