scholarly journals Pneumococcal meningitis with accompanying severe hearing loss: 3D-FLAIR imaging of the inner ear and treatment

2015 ◽  
Vol 55 (2) ◽  
pp. 119-122 ◽  
Author(s):  
Naoyuki Hara ◽  
Taijun Yunoki ◽  
Satoshi Kubo ◽  
Hiroki Fujii ◽  
Kazuhiro Takamatu ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Pneumococcal Meningitis ◽  
Severe Hearing Loss ◽  
Flair Imaging ◽  
3D Flair

Inner ear histopathology in “nervous Pointer dogs” with severe hearing loss

2005 ◽  
Vol 200 (1-2) ◽  
pp. 51-62 ◽  
Author(s):  
Angélique G. Coppens ◽  
Shana Gilbert-Gregory ◽  
Sheldon A. Steinberg ◽  
Claus Heizmann ◽  
Luc Poncelet
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Severe Hearing Loss ◽  
Nervous Pointer

scholarly journals The Natural History of Labyrinthine Hemorrhage in Patients With Sudden Sensorineural Hearing Loss

2019 ◽  
Vol 98 (5) ◽  
pp. E13-E20 ◽  
Author(s):  
Xi-Hang Chen ◽  
Chao-Jun Zeng ◽  
Zhe-Ming Fang ◽  
Rong Zhang ◽  
Jin-Mei Cheng ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Endolymphatic Hydrops ◽  
Pure Tone Audiometry ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
High Signal ◽  
Mri Scans ◽  
3D Flair

To investigate the application of inner ear 3-dimensional fluid-attenuated inversion recovery (3D-FLAIR) magnetic resonance imaging (MRI) in patients with sudden sensorineural hearing loss (SSNHL) accompanied by inner ear hemorrhage. A total of 1252 SSNHL patients who were admitted from January 2010 to April 2018 were included in the study. The patients’ clinical features, complete blood counts, coagulation profiles, audiometry data, and MRI scans were retrospectively reviewed. Twenty-four patients had high labyrinth signals on inner ear 3D-FLAIR MRI (24/1252, 1.9%) that were diagnosed as inner ear hemorrhage. One patient had endolymphatic hydrops on the contralesional side. In the 24 patients, pure tone audiometry curves revealed profound deafness (19/24) and flat moderate hearing loss (5/24); most patients had associated vertigo (23/24) and tinnitus (19/24). Patients with SSNHL (N = 24) were treated. Sixteen patients had invalid improvement, 3 patients were markedly improved, 4 patients had effective treatment, and only 1 patient was cured, for a therapeutic efficacy of 33.3% (8/24). Follow-up 3D-FLAIR MRI in patients showed absorbance of labyrinthine hemorrhage and disappearance of the high signal intensity in the inner ear within 2 weeks to 4 months. Inner ear 3D-FLAIR MRI indicate that most cases of inner ear hemorrhage are spontaneous and that high labyrinth signals are absorbed within 4 months. The site of labyrinth hemorrhage is irregular and independent of hearing loss. Conventional treatment is not very effective, and an appropriate therapy for SSNHL requires further investigation.

Ear Deformities Associated with the Klippel-Feil Syndrome

1981 ◽  
Vol 90 (3) ◽  
pp. 210-216 ◽  
Author(s):  
Paul C. Windle-Taylor ◽  
Pauline J. Emery ◽  
Peter D. Phelps
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Spinal Fusion ◽  
External Ear ◽  
Severe Hearing Loss

Congenital spinal fusion, constituting the Klippel-Feil anomaly, has long been known to be associated with severe deafness in many patients. Ten such cases are described, with audiometric and tomographic assessment of the ear lesions. Of the 20 ears examined, 12 showed evidence of severe hearing loss and of these, 11 had evidence of significant inner ear dysplasia on tomography. Middle and external ear abnormalities were also demonstrated. The significance of these findings and the relation to other syndromes is discussed.

Otoacoustic Emissions in an Adult With Severe Hearing Loss

1991 ◽  
Vol 34 (3) ◽  
pp. 703-703
Author(s):  
B. A. Prieve ◽  
M. Gorga ◽  
S. T. Neely
Keyword(s):  
Hearing Loss ◽  
Otoacoustic Emissions ◽  
Severe Hearing Loss

Drug Delivery to the Inner Ear: Strategies and Their Therapeutic Implications for Sensorineural Hearing Loss

2012 ◽  
Vol 9 (3) ◽  
pp. 231-242 ◽  
Author(s):  
Teresa Rivera ◽  
Lorena Sanz ◽  
Guadalupe Camarero ◽  
Isabel Varela-Nieto
Keyword(s):  
Drug Delivery ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Hearing ◽  
Therapeutic Implications

scholarly journals Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

2021 ◽  
pp. 014556132199683
Author(s):  
Wenqi Liang ◽  
Line Wang ◽  
Xinyu Song ◽  
Fenqi Gao ◽  
Pan Liu ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Next Generation Sequencing ◽  
Inner Ear ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Transverse Diameter ◽  
Congenital Hearing Loss ◽  
Canal Stenosis ◽  
Ear Anomalies ◽  
Generation Sequencing

The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient’s hearing was rehabilitated with bilateral cochlear implantation.

scholarly journals Inner Ear and Muscle Developmental Defects in Smpx-Deficient Zebrafish Embryos

2021 ◽  
Vol 22 (12) ◽  
pp. 6497
Author(s):  
Anna Ghilardi ◽  
Alberto Diana ◽  
Renato Bacchetta ◽  
Nadia Santo ◽  
Miriam Ascagni ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Hair Cells ◽  
Muscle Fibers ◽  
Underlying Mechanism ◽  
Loss Of Function ◽  
Zebrafish Model ◽  
Developmental Defects ◽  
Inner Ear Development ◽  
Syndromic Hearing Loss

The last decade has witnessed the identification of several families affected by hereditary non-syndromic hearing loss (NSHL) caused by mutations in the SMPX gene and the loss of function has been suggested as the underlying mechanism. In the attempt to confirm this hypothesis we generated an Smpx-deficient zebrafish model, pointing out its crucial role in proper inner ear development. Indeed, a marked decrease in the number of kinocilia together with structural alterations of the stereocilia and the kinocilium itself in the hair cells of the inner ear were observed. We also report the impairment of the mechanotransduction by the hair cells, making SMPX a potential key player in the construction of the machinery necessary for sound detection. This wealth of evidence provides the first possible explanation for hearing loss in SMPX-mutated patients. Additionally, we observed a clear muscular phenotype consisting of the defective organization and functioning of muscle fibers, strongly suggesting a potential role for the protein in the development of muscle fibers. This piece of evidence highlights the need for more in-depth analyses in search for possible correlations between SMPX mutations and muscular disorders in humans, thus potentially turning this non-syndromic hearing loss-associated gene into the genetic cause of dysfunctions characterized by more than one symptom, making SMPX a novel syndromic gene.

scholarly journals Ultrasound Microbubbles Enhance the Efficacy of Insulin-Like Growth Factor-1 Therapy for the Treatment of Noise-Induced Hearing Loss

Molecules ◽  
2021 ◽  
Vol 26 (12) ◽  
pp. 3626
Author(s):  
Yi-Chun Lin ◽  
Yuan-Yung Lin ◽  
Hsin-Chien Chen ◽  
Chao-Yin Kuo ◽  
Ai-Ho Liao ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Growth Factor ◽  
Inner Ear ◽  
Outer Hair Cells ◽  
Round Window Membrane ◽  
Insulin Like Growth Factor ◽  
Noise Induced Hearing Loss ◽  
Ear Diseases ◽  
Brainstem Response ◽  
Ultrasound Microbubbles

The application of insulin-like growth factor 1 (IGF-1) to the round window membrane (RWM) is an emerging treatment for inner ear diseases. RWM permeability is the key factor for efficient IGF-1 delivery. Ultrasound microbubbles (USMBs) can increase drug permeation through the RWM. In the present study, the enhancing effect of USMBs on the efficacy of IGF-1 application and the treatment effect of USMB-mediated IGF-1 delivery for noise-induced hearing loss (NIHL) were investigated. Forty-seven guinea pigs were assigned to three groups: the USM group, which received local application of recombinant human IGF-1 (rhIGF-1, 10 µg/µL) following application of USMBs to the RWM; the RWS group, which received IGF-1 application alone; and the saline-treated group. The perilymphatic concentration of rhIGF-1 in the USM group was 1.95- and 1.67- fold of that in the RWS group, 2 and 24 h after treatment, respectively. After 5 h of 118 dB SPL noise exposure, the USM group had the lowest threshold shift in auditory brainstem response, least loss of cochlear outer hair cells, and least reduction in the number of synaptic ribbons on postexposure day 28 among the three groups. The combination of USMB and IGF-1 led to a better therapeutic response to NIHL. Two hours after treatment, the USM group had significantly higher levels of Akt1 and Mapk3 gene expression than the other two groups. The most intense immunostaining for phosphor-AKT and phospho-ERK1/2 was detected in the cochlea in the USM group. These results suggested that USMB can be applied to enhance the efficacy of IGF-1 therapy in the treatment of inner ear diseases.

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