MRL/MP-lpr/lpr Mouse as a Model of Immune-Induced Sensorineural Hearing Loss

1992 ◽  
Vol 101 (10_suppl) ◽  
pp. 82-86 ◽  
Author(s):  
Chikashi Kusakari ◽  
Koji Hozawa ◽  
Masahisa Kyogoku ◽  
Shuji Koike ◽  
Tomonori Takasaka
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Stria Vascularis ◽  
Sensorineural Hearing ◽  
Response Threshold ◽  
Auditory Brain Stem Response ◽  
Auditory Brain Stem ◽  
Hearing Acuity ◽  
Marginal Cells ◽  
Intermediate Cells

Hearing acuity and inner ear disorders of MRL/ lpr mice, bred for the study of autoimmune disease, were examined in comparison to those of BALB/c mice. The auditory brain stem response threshold of 20-week-old MRL/ lpr mice was significantly higher than that of BALB/c mice of the same age (p < .01). The pathologic changes of 20-week-old MRL/ lpr mice were characterized by the degeneration of intermediate cells, widened intercellular spaces, and immunoglobulin G deposition on the basement membrane of strial blood vessels as well as in the basal infolding of strial marginal cells, which were absent in BALB/c mice. That there were no other evident pathologic findings in the cochlea or middle ear suggests that these changes in the stria vascularis seemed to be responsible for the sensorineural hearing loss of this mouse. The MRL/ lpr mouse was thought to be a good experimental model to study the spontaneous sensorineural hearing loss caused by an immune reaction.

scholarly journals Exogenous IL-4-Expressing Bone Marrow Mesenchymal Stem Cells for the Treatment of Autoimmune Sensorineural Hearing Loss in a Guinea Pig Model

2014 ◽  
Vol 2014 ◽  
pp. 1-10 ◽  
Author(s):  
Chang-qiang Tan ◽  
Xia Gao ◽  
Lang Guo ◽  
He Huang
Keyword(s):  
Stem Cells ◽  
Bone Marrow ◽  
Mesenchymal Stem Cells ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Hearing ◽  
Scala Tympani ◽  
Auditory Brain Stem Response ◽  
Marrow Mesenchymal Stem Cells

Bone marrow mesenchymal stem cells (BMSCs) expressing recombinant IL-4 have the potential to remediate inflammatory diseases. We thus investigated whether BMSCs expressing exogenous IL-4 could alleviate autoimmune sensorineural hearing loss. BMSCs isolated from guinea pigs were transfected with recombinant lentivirus expressing IL-4. A total of 33 animals were divided into three groups. Group A received scala tympani injection of IL-4-expressing BMSCs, and Group B received control vector-expressing BMSCs, and Group C received phosphate-buffered saline. The distribution of implanted BMSCs in the inner ears was assessed by immunohistochemistry and fluorescence microscopy. Auditory brain-stem response (ABR) was monitored to evaluate the auditory changes. Following BMSCs transplantation, the threshold levels of ABR wave III decreased in Groups A and B and significant differences were observed between these two groupsP<0.05. Transplanted BMSCs distributed in the scala tympani and scala vestibuli. In some ears with hearing loss, there was a decrease in the number of spiral ganglion cells and varying degrees of endolymphatic hydrops or floccule. Following transplantation, the lentivirus-infected BMSCs migrated to the inner ear and produced IL-4. Our results demonstrate that, upon transplantation, BMSCs and BMSCs expressing recombinant IL-4 have the ability to remediate the inflammatory injury in autoimmune inner ear diseases.

Functional and morphological response of the stria vascularis following a sensorineural hearing loss

2002 ◽  
Vol 172 (1-2) ◽  
pp. 127-136 ◽  
Author(s):  
W.P.L Hellier ◽  
S.A Wagstaff ◽  
S.J O’Leary ◽  
R.K Shepherd
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Stria Vascularis ◽  
Sensorineural Hearing ◽  
Morphological Response

scholarly journals The clinical study of audiologic manifestations of type 2 diabetes mellitus in association with other diabetic microvascular complications: an observational study

2020 ◽  
Vol 7 (7) ◽  
pp. 1107
Author(s):  
Kamalesh T. N. ◽  
Sreedevi N. T. ◽  
Rakesh B. M.
Keyword(s):  
Diabetes Mellitus ◽  
Hearing Loss ◽  
Observational Study ◽  
Sensorineural Hearing Loss ◽  
Significant Relationship ◽  
Stria Vascularis ◽  
Microvascular Complications ◽  
Pure Tone Audiometry ◽  
Public Health Problem ◽  
Sensorineural Hearing

Background: Sensorineural hearing loss (SNHL) is a major clinical and public health problem. Diabetes mellitus (DM) is characterized by various microvascular and macrovascular complications. DM is associated with thickening of basal membrane of the stria vascularis capillaries on the lateral wall of cochlea and neuropathic changes which leads to SNHL.Methods: This is an observational study involving 160 participants grouped into diabetics and non-diabetics. DM was diagnosed using standard American Diabetes Association (ADA) criteria. All the participants were subjected to pure tone audiometry to detect SNHL which was graded into mild, moderate, severe and profound based on degree of decibel loss. The occurrence of SNHL was compared with the occurrence of microvascular complications among diabetics.Results: In our study, 34 (42.5%) cases and 8 (10%) of controls had SNHL. The occurrence of SNHL was higher among diabetics than non-diabetics (p <0.0001) with majority (88.23%) of cases having bilateral SNHL. Among diabetics, 26 (32.5%) had neuropathy, 23 (28.75%) had retinopathy, 21 (26.25%) had nephropathy. There was no statistically significant relationship between occurrence of SNHL and microvascular complications among diabetics (p=0.8614). The occurrence of SNHL correlated with the levels of HbA1c (p = 0.017). The occurrence of SNHL among diabetics was 42.5% as compared to only 10% among non-diabetics which was statistically significant. The occurrence of SNHL correlated with the levels of HbA1c among diabetics suggesting an association between DM and SNHL (p=0.017).Conclusions: There was increased occurrence of sensorineural hearing loss among the individuals with diabetes mellitus with significant relationship with the glycaemic index.

Significance of Auditory Brain Stem Response and Gadolinium-Enhanced Magnetic Resonance Imaging for Idiopathic Sudden Sensorineural Hearing Loss

1995 ◽  
Vol 113 (3) ◽  
pp. 271-275 ◽  
Author(s):  
Nicolas Y. Busaba ◽  
Steven D. Rauch
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Sensorineural Hearing Loss ◽  
Brain Stem ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Resonance Imaging ◽  
Auditory Brain Stem Responses ◽  
Auditory Brain Stem

Previous studies tried to correlate prognosis and response to oral corticosteroids in patients with idiopathic sudden sensorineural hearing loss to such factors as the age of the patient, presence of vertigo, shape of the audiogram, or severity of the hearing loss. However, temporal bone histopathologic evidence shows that idiopathic sudden sensorineural hearing loss may be caused by cochleitis or cochlear nerve neuritis. Herein we report results of a retrospective study of 96 consecutive patients with idiopathic sudden sensorineural hearing loss who were evaluated with auditory brain stem responses and gadolinium-enhanced magnetic resonance imaging. Results of the auditory brain stem response and magnetic resonance imaging were correlated with hearing outcome. Follow-up was available for 65 patients: 14 with abnormal and 51 with normal auditory brain stem responses. The overall rate of hearing recovery or improvement was 65% in the normal auditory brain stem response group compared with 43% in the abnormal auditory brain stem response group ( p = 0.07). Among the 38 patients treated with a tapering course of oral corticosteroids, the recovery or improvement rate was 83% for those with normal auditory brain stem responses and 56% for those with abnormal auditory brain stem responses ( p < 0.05). Of the 27 patients who did not receive steroid therapy, the improvement rate was 41% in those with normal auditory brain stem responses and 20% in those with abnormal auditory brain stem responses ( p = 0.09). Magnetic resonance imaging with gadolinium was obtained on all 14 patients with abnormal auditory brain stem responses but on none with normal auditory brain stem responses. Only 1 magnetic resonance image of 14 demonstrated an abnormality, showing a high signal intensity in the distal internal auditory canal; this resolved 6 weeks later on a follow-up magnetic resonance image. We conclude that idiopathic sudden sensorineural hearing loss patients with abnormal auditory brain stem responses have poorer hearing prognoses compared with those patients with normal auditory brain stem responses, irrespective of treatment. Idiopathic sudden sensorineural hearing loss patients with abnormal auditory brain stem responses may have cochlear neuritis causing their hearing loss or may have a more extensive involvement of their auditory system, and this “lesion” may have a lower spontaneous recovery rate and less response to therapy. Magnetic resonance imaging with gadolinium may show abnormal signal intensities along the course of the eighth nerve in patients with idiopathic sudden sensorineural hearing loss, but this is infrequent, and its prognostic implications are not clear.

scholarly journals Mengenali gangguan pendengaran pada sindrom Waardenburg

2018 ◽  
Vol 47 (2) ◽  
pp. 171
Author(s):  
Semiramis Zizlavsky ◽  
Safira Trifani Putri ◽  
Ronny Suwento
Keyword(s):  
Hearing Loss ◽  
Early Detection ◽  
Sensorineural Hearing Loss ◽  
Hearing Impairment ◽  
Clinical Manifestation ◽  
Stria Vascularis ◽  
Clinical Manifestations ◽  
Sensorineural Hearing ◽  
Waardenburg Syndrome ◽  
Autosomal Dominant Disorder

Latar belakang: Sindrom Waardenburg adalah suatu kelainan yang bersifat autosomal dominan yang ditandai oleh gangguan pendengaran sensorineural dan kelainan pigmen pada mata, rambut, dan kulit. Sindrom auditori-pigmen ini terjadi karena tidak adanya melanosit dari kulit, rambut, mata, serta stria vaskularis pada koklea. Tujuan: Memberikan informasi mengenai manifestasi klinis Sindrom Waardenburg sehingga dapat melakukan deteksi dan penanganan sedini mungkin, terutama untuk gangguan pendengaran. Kasus: Seorang anak laki-laki berusia 7 tahun dengan keterlambatan deteksi gangguan pendengaran. Orang tua anak tidak mengetahui bahwa gangguan pendengaran tersebut merupakan salah satu manifestasi sindrom Waardenburg, sehingga pada anak ini terdapat gangguan perkembangan wicara. Metode: Penelusuran literatur menghasilkan 14 jurnal, dan terdapat 2 jurnal yang relevan. Hasil: Dari 2 jurnal yang didapat, ditemukan 1 artikel penelitian dan 1 laporan kasus sindrom Waardenburg yang memaparkan berbagai manifestasi klinis yang menyertai pasien. Kesimpulan: Deteksi dini dan penatalaksanaan untuk gangguan pendengaran yang merupakan manifestasi klinis tersering dari sindrom Waardenburg, penting dilakukan karena mempengaruhi perkembangan individu dan perkembangan sosial pasien. Kata kunci: Sindrom auditori-pigmentasi, kelainan pigmen, gangguan pendengaran sensorineural, sindrom Waardenburg ABSTRACT Background: Waardenburg Syndrome (WS) is an autosomal-dominant disorder, characterized by sensorineural hearing loss and pigmentary abnormalities of the eyes, hair, and skin. Auditory-pigmentary syndromes are caused by physical absence of melanocytes from the skin, hair, eyes, and the stria vascularis of the cochlea. Purpose: To provide information regarding clinical manifestation of Waardenburg Syndrome for early detection and prompt treatment particularly for hearing impairments. Case: A 7-year-old boy with late detection of hearing impairment. Previously, his parents didn’t recognize that this condition was one manifestation of Waardenburg syndrome, so that this patient developed speech delay. Method: Searching for evidence produced 14 journals and only 2 journals were relevant. Result: Of the 2 relevant journals, there were 1 research article and 1 case report that explained several clinical manifestations of Waardenberg Syndrome. Conclusion: Early detection and management of hearing impairment as one of frequent clinical manifestation of Waardenberg Syndrome is significantly important since this condition could influence patient’s individual and social developments. Keywords: Auditory pigmentary syndrome, pigmentary abnormality, sensorineural hearing loss, Waardenburg syndrome

scholarly journals Role of the Stria Vascularis in the Pathogenesis of Sensorineural Hearing Loss: A Narrative Review

2021 ◽  
Vol 15 ◽  
Author(s):  
Wenting Yu ◽  
Shimin Zong ◽  
Peiyu Du ◽  
Peng Zhou ◽  
Hejie Li ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Stria Vascularis ◽  
Sensorineural Hearing ◽  
Sensory Impairment ◽  
Rapid Progress ◽  
Molecular Events ◽  
Underlying Mechanisms ◽  
Scientific Technology

Sensorineural hearing loss is a common sensory impairment in humans caused by abnormalities in the inner ear. The stria vascularis is regarded as a major cochlear structure that can independently degenerate and influence the degree of hearing loss. This review summarizes the current literature on the role of the stria vascularis in the pathogenesis of sensorineural hearing loss resulting from different etiologies, focusing on both molecular events and signaling pathways, and further attempts to explore the underlying mechanisms at the cellular and molecular biological levels. In addition, the deficiencies and limitations of this field are discussed. With the rapid progress in scientific technology, new opportunities are arising to fully understand the role of the stria vascularis in the pathogenesis of sensorineural hearing loss, which, in the future, will hopefully lead to the prevention, early diagnosis, and improved treatment of sensorineural hearing loss.

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