Patients with Autoimmune Hearing Loss Have Antibodies That Bind to Inner Ear Tissues of Laboratory Animals

Aim. Study of pathological changes in the inner ear in the modeling of sensorineural hearing loss in laboratory animals.Materials and methods. A pilot study involving 27 laboratory animals − white outbred rats – was conducted. Modeling of sensorineural hearing loss was conducted by exposing them for 10 days to broadband noise of 90 dB, vibration and simultaneous immobilization of laboratory animals divided into 3 groups according to exposure conditions. The development of hearing loss was confirmed by the results of the registration of delayed caused emissions and emissions at the frequency of distortion products. After removing rats from the experiment histological medication from animals cochlear was produced and they were studied by the method of light microscopy.Results. In animals exposed to noise and vibration effects on the background of immobilization, the formation of persistent sensorineural hearing loss was achieved, which was confirmed by objective registration of violations of auditory function, and also by the results of morphological study of cochlear of the temporal bones of animals. More pronounced histological changes were noted in animals exposed to noise and vibration on the background of immobilization compared with the group of rats which were exposed only to noise impact and immobilization without applying vibration. Dystrophic and destructive changes in the structures of the spiral organ, signs of apoptotic way of cell death in the inner ear were detected. In addition, pronounced changes occurred in the spiral ganglia.Conclusion. Modeling of sensorineural hearing loss in laboratory animals on the basis of noise and vibration exposure in terms of immobilization leads to the formation of persistent sensorineural hearing loss, as evidenced by functional and morphological methods. Pathological changes in the inner ear show themselves through dystrophic and destructive changes in the spiral organ, including apoptosis of cells, and especially in the spiral ganglia. The use of this noise-vibration model of hearing loss can be a promising basis for future studies of drugs for the treatment of sensorineural hearing loss.

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

Current Drug Delivery ◽

10.2174/156720112800389098 ◽

2012 ◽

Vol 9 (3) ◽

pp. 231-242 ◽

Cited By ~ 28

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

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Hearing loss in inner ear and systemic autoimmune disease: A systematic review of post‐cochlear implantation outcomes

Laryngoscope Investigative Otolaryngology ◽

10.1002/lio2.563 ◽

2021 ◽

Author(s):

Jonathan Lee ◽

Kirsty Biggs ◽

Jameel Muzaffar ◽

Manohar Bance ◽

Peter Monksfield

Keyword(s):

Systematic Review ◽

Hearing Loss ◽

Autoimmune Disease ◽

Inner Ear ◽

Cochlear Implantation ◽

Systemic Autoimmune Disease

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Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

Ear Nose & Throat Journal ◽

10.1177/0145561321996839 ◽

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.

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

International Journal of Molecular Sciences ◽

10.3390/ijms22126497 ◽

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.

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

Molecules ◽

10.3390/molecules26123626 ◽

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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