Objective: To investigate imaging and audiology features of temporal bone and analyze the classification and prevalence of inner ear abnormalities in children with sensorineural hearing loss.
Method: Children who were diagnosed with sensorineural hearing loss were examined by high resolution CT and the inner ear fluid of MRI. And each chart was retrospectively reviewed to determine the imaging and audiology features.
Result: There were 125 patients(232 ears) found with inner ear malformation in 590 children with SNHL. About 21.71% of the inner ear malformation occurred in severe and profound hearing loss ears, and 12.85% occurred in r moderate hearing loss ears. The inner ear malformation rate in normal hearing ears were 13.59%.
Conclusion: CT and MRI examinations of temporal bone are important diagnostic tools to indentify inner ear malformations. Inner ear malformations are almost bilateral and hearing loss are profoud. Cochleo-vestibular malformations and large vestibular aqueduct are the 2 most frequent deformities. Among the children with SNHL, deformity rate in the severe and profound hearing loss ears is higher than that in moderate hearing loss ear. Inner ear malformations can exist in people with normal hearing.
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Nat Commun
December 2024
The Solomon H. Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD, 21205, USA.
Deafness is the most common form of sensory impairment in humans and frequently caused by defects in hair cells of the inner ear. Here we demonstrate that in male mice which model recessive non-syndromic deafness (DFNB6), inactivation of Tmie in hair cells disrupts gene expression in the neurons that innervate them. This includes genes regulating axonal pathfinding and synaptogenesis, two processes that are disrupted in the inner ear of the mutant mice.
View Article and Find Full Text PDFJ Otol
July 2024
Department of Neurology, Ningbo No.2 Hospital, Ningbo, Zhejiang, 315010, PR China.
Objective: To evaluate the plasma levels of the otoconial proteins, otoconin-90 and otolin-1, in individuals diagnosed with vestibular neuritis (VN) and determine the feasibility of using these proteins as biomarkers for VN.
Methods: In this preliminary study, 30 patients diagnosed with VN and 70 healthy individuals were recruited and followed to confirm whether they had benign paroxysmal positional vertigo (BPPV) during the following time. The recorded data included measurements of height, weight, and history of diabetes mellitus or hypertension.
Front Neurol
December 2024
Department of Surgical Sciences, Otorhinolaryngology and Head and Neck Surgery, Uppsala University, Uppsala, Sweden.
Background: There still exists controversy about whether the healthy human middle ear mucosa is sterile or if it may harbor a diverse microbiome. Considering the delicacy of the human round window membrane (RWM), different mechanisms may exist for avoiding inner ear pathogen invasion causing sensorineural deafness. We re-analyzed archival human RWMs using light and transmission electron microscopy after decalcification to determine if bacteria are present in clinically normal human middle ears.
View Article and Find Full Text PDFLin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
January 2025
To explore the value of high resolution computed tomography(HRCT) combined with Magnetic Resonance Imaging(MRI) in the diagnosis of inner ear malformation. HRCT and MRI data of 82 patients with inner ear malformations were analyzed retrospectively. HRCT MPR and CPR reconstruction of the inner ear structure, facial nerve canal and oblique sagittal MRI reconstruction of the internal auditory canal were performed.
View Article and Find Full Text PDFLin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
January 2025
This study aimed to compare the effects of cochlear implantation(CI) on vestibular function in patients with large vestibular aqueduct syndrome(LVAS) and in patients with extremely severe deafness with normal inner ear structure. A total of 28 LVAS patients and 28 patients with normal inner ear structure who suffered from extremely severe deafness were selected. The parameters of caloric tests, bone conduction evoked cervical vestibular-evoked myogenic potentials(cVEMP), bone conduction evoked ocular vestibular-evoked myogenic potentials(oVEMP) and video head impulse tests(v-HIT) were compared between the two groups before and after CI.
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