National Institute of Sensory Organs

Sensory, Japan

National Institute of Sensory Organs

Sensory, Japan
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Jin Y.,University of Tokyo | Jin Y.,Yanbian University | Kondo K.,University of Tokyo | Ushio M.,University of Tokyo | And 5 more authors.
Cell and Tissue Research | Year: 2013

The way that the development of the inner ear innervation is regulated by various neurotrophic factors and/or their combinations at different postnatal developmental stages remains largely unclear. Moreover, survival and neuritogenesis in deafferented adult neurons is important for cochlear implant function. To address these issues, developmental changes in the responsiveness of postnatal rat spiral ganglion neurons (SGNs) to neurotrophin-3 (NT-3), brain-derived neurotrophic factor (BDNF) and leukemia inhibitory factor (LIF) were examined by using a dissociated cell culture system. SGNs at postnatal day (P) 0, P5 and P20 (young adult) were cultured with the addition of NT-3, BDNF, or LIF or of a combination of NT-3 and BDNF (N + B) or of NT-3, BDNF and LIF (ALL factors). SGNs were analyzed for three parameters: survival, longest neurite length (LNL) and neuronal morphology. At P0, SGNs required exposure to N + B or ALL factors for enhanced survival and the ALL factors combination showed a synergistic effect much greater than the sum of the individual factors. At P5, SGNs responded to a wider range of treatment conditions for enhanced survival and combinations showed only an additive improvement over individual factors. The survival percentage of untreated SGNs was highest at P20 but combinations of neurotrophic factors were no more effective than individual factors. LNL of each SGN was enhanced by LIF alone or ALL factors at P0 and P5 but was suppressed by NT-3, BDNF and N + B at P5 in a dose-dependent manner. The LNL at P20 was enhanced by ALL factors and suppressed by N + B. Treatment with ALL factors increased the proportion of SGNs that had two or more primary neurites in all age groups. These findings suggest that NT-3, BDNF, LIF and their combinations predominantly support different ontogenetic events at different developmental stages in the innervation of the inner ear. © 2012 Springer-Verlag Berlin Heidelberg.

Watanabe K.,National Tokyo Medical Center | Tsunoda K.,National Institute of Sensory Organs | Mizuno Y.,National Institute of Sensory Organs | Akiyama K.,National Tokyo Medical Center | Noda T.,National Tokyo Medical Center
JAMA Ophthalmology | Year: 2013

Objective: To determine the relationship between the morphology of the fovea and visual acuity in patients with an untreated idiopathic epiretinal membrane (ERM). Methods: We examined 52 eyes of 45 patients diagnosed with an ERM. The morphology of the foveal area was determined by spectral-domain optical coherence tomography. The relationships between the best-corrected visual acuity (BCVA) and 8 optical coherence tomography features, central retinal thickness, cone outer segment tip (COST) line, photoreceptor inner/outer segment (IS/OS) junction line, foveal bulge of the IS/OS line, external limiting membrane, inner limiting membrane, foveal pit, and ERM over the foveal center, were evaluated. Results: Multiple regression analysis showed that intact COST line, IS/OS junction line, and external limiting membrane independently and significantly contributed to the BCVA. The standardized partial regression coefficient β was 0.415 for the COST line, 0.287 for the IS/OS junction line, and 0.247 for the external limiting membrane. However, the other features, eg, foveal bulge, inner limiting membrane, foveal pit, and ERM, were not significantly associated with the BCVA. The central retinal thickness was significantly correlated with the BCVA (r2=0.274; P<.01). Conclusions: At an early stage of an ERM, only the photoreceptor structures are significantly associated with the BCVA, and the appearance of the COST line was most highly associated. Detailed examinations of the photoreceptor structures using optical coherence tomography may help find photoreceptor dysfunction in cases of idiopathic ERM. © 2013 American Medical Association. All rights reserved.

Tsunoda K.,National Institute of Sensory Organs | Watanabe K.,National Tokyo Medical Center | Akiyama K.,National Tokyo Medical Center | Usui T.,Akiba Eye Clinic | Noda T.,National Tokyo Medical Center
Ophthalmology | Year: 2012

Objective: To report the optical coherence tomography (OCT) findings in eyes with vitreomacular traction (VMT) or with an epiretinal membrane (ERM). Design: Retrospective case series. Participants: Fifty-four eyes of 45 consecutive patients with subjective visual disturbances resulting from VMT or idiopathic ERM were studied. Methods: The morphologic features of the photoreceptor layer at the foveal center were determined and the central foveal thickness (CFT) was measured by spectral-domain (SD) OCT. Main Outcome Measures: The morphologic characteristics of the foveal region observed by SD OCT. Results: A roundish or diffuse highly reflective region was observed between the photoreceptor inner segment/outer segment junction line and the cone outer segment tip line at the center of the fovea. This highly reflective region was present in 7 of 7 cases of VMT and 30 of 47 cases of ERM. In the ERM cases, the mean CFT of the cases with the highly reflective region was significantly thicker than that in cases without it. The highly reflective region disappeared when the inward traction on the fovea was released surgically or spontaneously. Conclusions: The highly reflective region is a characteristic sign observed in the OCT images of eyes with VMT and ERM, and it has been termed the cotton ball sign after its appearance. The presence of the cotton ball sign indicates an inward traction on the fovea and may be a predictor of visual impairment. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. © 2012 American Academy of Ophthalmology.

Matsunaga T.,National Institute of Sensory Organs | Mutai H.,National Institute of Sensory Organs | Namba K.,National Institute of Sensory Organs | Morita N.,Teikyo University | Masuda S.,National Mie Hospital
Acta Oto-Laryngologica | Year: 2013

Conclusion: PAX3 genetic analysis increased the diagnostic accuracy for Waardenburg syndrome type I (WS1). Analysis of the three-dimensional (3D) structure of PAX3 helped verify the pathogenicity of a missense mutation, and multiple ligation-dependent probe amplification (MLPA) analysis of PAX3 increased the sensitivity of genetic diagnosis in patients with WS1. Objectives: Clinical diagnosis of WS1 is often difficult in individual patients with isolated, mild, or non-specific symptoms. The objective of the present study was to facilitate the accurate diagnosis of WS1 through genetic analysis of PAX3 and to expand the spectrum of known PAX3 mutations. Methods: In two Japanese families with WS1, we conducted a clinical evaluation of symptoms and genetic analysis, which involved direct sequencing, MLPA analysis, quantitative PCR of PAX3, and analysis of the predicted 3D structure of PAX3. The normal-hearing control group comprised 92 subjects who had normal hearing according to pure tone audiometry. Results: In one family, direct sequencing of PAX3 identified a heterozygous mutation, p.I59F. Analysis of PAX3 3D structures indicated that this mutation distorted the DNA-binding site of PAX3. In the other family, MLPA analysis and subsequent quantitative PCR detected a large, heterozygous deletion spanning 1759-2554 kb that eliminated 12-18 genes including a whole PAX3 gene. © 2013 Informa Healthcare.

Fujinami K.,National Institute of Sensory Organs | Tsunoda K.,National Institute of Sensory Organs | Hanazono G.,National Institute of Sensory Organs | Hanazono G.,Kikkoman General Hospital | And 5 more authors.
Archives of Ophthalmology | Year: 2011

Objective: To characterize fundus autofluorescence (FAF) images of eyes with autosomal dominant occult macular dystrophy (OMD). Methods: All patients received a comprehensive ophthalmologic examination for diagnosis of OMD. We evaluated the FAF images in 13 eyes of 7 patients with autosomal dominant OMD by confocal scanning laser ophthalmoscopy with excitation at 488 nm and emission more than 500 nm. Results: The FAF images showed unspecific weak foveal hyperfluorescence in 4 eyes of 2 patients; one showed a thin hyperfluorescence in the temporal fovea bilaterally and the other showed weak hyperfluorescence in the fovea bilaterally. The optical coherence tomographic images showed abnormalities of the photoreceptor inner segment-outer segment line and cone outer segment tip line in all patients. However, 5 patients had normal FAF images regardless of morphological abnormalities of the photoreceptor. Conclusions: Fundus autofluorescence is a useful method to acquire additional information of photoreceptor/retinal pigment epithelium function in eyes with OMD. Fundus autofluorescence will be also helpful for the differential diagnosis of eyes with OMD vs eyes with other dystrophies that have a distinctive FAF pattern. ©2011 American Medical Association. All rights reserved.

Suzuki W.,National Institute of Sensory Organs | Suzuki W.,RIKEN | Suzuki W.,National Institute of Neuroscience | Tsunoda K.,National Institute of Sensory Organs | And 4 more authors.
Investigative Ophthalmology and Visual Science | Year: 2013

Purpose. To investigate the properties and origin of retinal intrinsic signals by functional optical coherence tomography (fOCT) in macaque retinas. Methods. We modified a spectral domain OCT system to be able to give short-duration flashes or continuous light stimulation to the retina of three adult macaque monkeys (Macaca mulatta) under general anesthesia. Changes in the intensities of the OCT signals following the stimulus were determined. Results. Stimulus-evoked decreases or increases in the OCT signals were observed in the photoreceptor inner segment ellipsoids and outer segments, respectively. Experiments with focal and colored stimuli confirmed that these fOCT signals originated from the photoreceptors. No diffuse changes in the OCT signals were detected in the inner retinal layers; however, there were slow changes in small discrete areas where the retinal vessels were located. The polarity of the fOCT signals in the inner retinal layer was dependent on each activated region, and one of the possible sources of the reflectance changes was the light-scattering changes of the retinal vessels. Conclusions. The fOCT signals in the macaque retina consist of at least three components: light-scattering changes from the photoreceptor inner segment ellipsoids, light-scattering changes from the outer segments, and slow light-scattering changes from the blood vessels in the inner retina. This technique has the potential of mapping local neuronal activity three-dimensionally and may help in the diagnosis of retinal disorders of different retinal origins. © 2013 The Association for Research in Vision and Ophthalmology, Inc.

Although children may dislike and/or resist oral and pharyngeal examination with a tongue depressor, they enjoy lollipops on sticks, eating with spoons, forks, and chopsticks, and brushing their teeth. Many reports have noted this apparent contradiction, since paediatric patients are often treated after toothbrushes or chopsticks penetrate the pharyngeal wall. We therefore developed a novel device to observe the inside of the mouth without using a flashlight, tongue depressor or head mirror. We previously developed the AWS for tracheal intubation through the mouth for anaesthesia and emergency situations, along with a new device to observe the inside of the oral cavity simultaneously. We have developed a new attachment to the AWS for observations inside the oral cavity and pharynx. Our newly developed oral and pharyngeal examination system is a useful tool for diagnostic examinations and may also enable treatment without causing discomfort or distress to patients and their families.

Masuda T.,National Institute of Sensory Organs | Masuda T.,Nihon University | Kaga K.,National Institute of Sensory Organs
Acta Oto-Laryngologica | Year: 2014

Conclusions: When vestibular function is reduced in the rotational chair test in children with severe hearing loss, the vestibular function may be acquired later due to maturing vestibular sensory cells and vestibular nerve of the inner ear along with physical growth. Objectives: To examine the relationship between acquisition of motor function and vestibular function in children with bilateral severe hearing loss. Methods: A total of 97 children under 4 years old with hearing loss defined as a hearing threshold of both ears greater than 80 dB were included in this study. For evaluation of vestibular function, a damped-rotational chair test was performed and the horizontal nystagmus was recorded using electronystagmography (ENG). Results: Head control and independent walking were delayed in 28 of 97 children with severe hearing loss. Reduced response to the rotational chair test was observed in 16 of 97 children (16.5%), with 11 of these children having inner ear anomalies and reduced vestibular function. Of the 10 children who were followed up by the rotational chair test, 2 children with idiopathic congenital hearing loss without inner ear anomalies (100%) and 6 of 8 children with bilateral inner ear anomalies (75%) showed more obvious nystagmus during rotation compared with the initial examination. © 2014 Informa Healthcare.

Tsunoda K.,National Institute of Sensory Organs
Nihon rinsho. Japanese journal of clinical medicine | Year: 2013

Miyake's disease (occult macular dystrophy: OMD) was first described by Miyake et al. to be a hereditary macular dystrophy without visible fundus abnormalities. Patients with OMD are characterized by a progressive decrease of visual acuity with normal appearing fundus and normal fluorescein angiograms. The important signs of OMD are normal full-field electroretinograms (ERGs) but abnormal focal macular ERGs. In 2010, we found that dominant mutations in the RP1L1 gene were responsible for OMD by a linkage analysis of two OMD families, and recently, the same mutations were known to cause OMD in non-Japanese patients. Here, we describe how this disorder has been discovered and the causative gene was found by Miyake's group, together with the detailed characteristics of OMD.

Scholl H.P.N.,Wilmer Eye Institute | Birch D.G.,University of Texas Southwestern Medical Center | Iwata T.,National Institute of Sensory Organs | Miller N.R.,Wilmer Eye Institute | And 2 more authors.
Archives of Ophthalmology | Year: 2012

Objective: To characterize the phenotype of a white patient with occult macular dystrophy (OMD) and her clinically unaffected family members and to determine whether similar mutations were present in the RP1L1 gene in this family. Occult macular dystrophy is a rare macular dystrophy with central cone dysfunction hidden behind a normal fundus appearance that has been attributed to a mutation in the retinitis pigmentosa 1-like 1 (RP1L1) gene in 4 Japanese families. Methods: In this observational cross-sectional study of 1 white family with OMD, patients meeting the clinical criteria for OMD and their family members were evaluated by use of multifocal electroretinography, the Farnsworth D-15 color vision test, automated perimetry, spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence, and fundus photography. Fluorescein angiography was performed only on the proband. Members of this family were screened for genetic mutations in the RP1L1 gene. Results: In the family studied, the clinically affected proband was noted to have loss of the foveal outer segments and absence of bowing of the inner segment/outer segment junction on SD-OCT scans. In addition, 1 clinically unaffected family member also demonstrated loss of the foveal photoreceptor outer segments and, therefore, decreased bowing of the inner segment/outer segment junction on SD-OCT scans. The fundus autofluorescence images of the eyes of the proband and her family members were normal. Although mutations in the RP1L1 gene have been identified in sporadic and autosomal dominant OMD pedigrees, no mutations in the RP1L1 gene were found in any of the participants. Conclusions: Loss of the outer segments of foveal photoreceptors can be detected and quantified by use of SDOCT in patients with OMD. Similar findings are present in some clinically unaffected family members and may represent subclinical manifestations of the disease. Although mutations in the RP1L1 gene have been described in several Japanese families with OMD, there were no such mutations in this white family of European descent, which suggests that inherited OMD is a genetically heterogeneous disorder. ©2012 American Medical Association. All rights reserved.

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