A Human Inner Ear Model for assessment of Noise Induced Hearing Loss via energy methods

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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Cytomegalovirus Isolation from a Human Inner Ear

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348947908800322 ◽

1979 ◽

Vol 88 (3) ◽

pp. 424-426 ◽

Cited By ~ 17

Author(s):

Larry E. Davis ◽

Charles G. James ◽

Frederick Fiber ◽

Leroy C. McLaren

Keyword(s):

Hearing Loss ◽

Inner Ear ◽

Congenital Deafness ◽

Cmv Infection ◽

Human Inner Ear ◽

Congenital Cmv

Cytomegalovirus (CMV) infection of the fetus has been associated with congenital deafness or hearing loss. This association has previously been based on clinical or pathological studies. We report an infant who died with the congenital CMV syndrome in which CMV was isolated from the perilymph of the inner ear providing additional evidence that this virus can infect the labyrinth.

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Isolation of sensory hair cell specific exosomes in human perilymph

10.1101/2021.04.11.439339 ◽

2021 ◽

Author(s):

Pei Zhuang ◽

Suiching Phung ◽

Athanasia Warnecke ◽

Alexandra Arambula ◽

Madeleine St. Peter ◽

...

Keyword(s):

Hearing Loss ◽

Hair Cell ◽

Sensorineural Hearing Loss ◽

Inner Ear ◽

Molecular Characterization ◽

Sensorineural Hearing ◽

Alternative Methods ◽

Sensory Hair ◽

Sensory Hair Cell ◽

Human Inner Ear

AbstractEvaluation of hearing loss patients using clinical audiometry has been unable to give a definitive cellular or molecular diagnosis, hampering the development of treatments of sensorineural hearing loss. However, biopsy of inner ear tissue without losing residual hearing function for pathologic diagnosis is extremely challenging. In a clinical setting, perilymph can be accessed, so alternative methods for molecular characterization of the inner ear may be developed. Recent approaches to improving inner ear diagnostics have been focusing on the evaluation of the proteomic or miRNA profiles of perilymph. Inspired by recent characterization and classification of many neurodegenerative diseases using exosomes which not only are produced in locally in diseased tissue but are transported beyond the blood brain barrier, we demonstrate the isolation of human inner ear specific exosomes using a novel ultrasensitive immunomagnetic nano pom-poms capture-release approach. Using perilymph samples harvested from surgical procedures, we were able to isolate exosomes from sensorineural hearing loss patients in only 2-5 μL of perilymph. By isolating sensory hair cell derived exosomes through their expression level of myosin VII, we for the first time sample material from hair cells in the living human inner ear. This work sets up the first demonstration of immunomagnetic capture-release nano pom-pom isolated exosomes for liquid biopsy diagnosis of sensorineural hearing loss. With the ability to isolate exosomes derived from different cell types for molecular characterization, this method also can be developed for analyzing exosomal biomarkers from more accessible patient tissue fluids such as plasma.

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Insights into the Pathobiology of TMPRSS3-Related Hearing Loss and Implications for Cochlear Implant Patients with TMPRSS3 Mutations.

10.21203/rs.3.rs-632257/v1 ◽

2021 ◽

Author(s):

Brady J. Tucker ◽

Yuan-Siao Chen ◽

Timothy J. Shin ◽

Ernesto Cabrera ◽

Kevin T. Booth ◽

...

Keyword(s):

Hearing Loss ◽

Inner Ear ◽

Expression Pattern ◽

Autosomal Recessive ◽

Poor Performance ◽

Auditory Neurons ◽

Spatial Expression Pattern ◽

Human Cochlea ◽

Syndromic Hearing Loss ◽

Human Inner Ear

Abstract OBJECTIVES To review the audiological outcomes after cochlear implantation (CI) for TMPRSS3-associated autosomal recessive non-syndromic hearing loss (ARNSHL) and evaluate the spatial expression pattern of TMPRSS3 within the human cochlea. METHODS Review all published cases of CI in patients with TMPRSS3-associated ARNSHL to compare postoperative consonant-nucleus-consonant (CNC) word performance to published adult CI cohorts. Protein structural modeling of TMPRSS3 variants associated with post-lingual hearing loss. Determine TMPRSS3 expression pattern in human inner ear organoids and human cochlea. RESULTS Nine articles detailed 27 patients (30 total CI ears) with TMPRSS3-associated hearing loss treated with CI. Of these, 6 cases reported prelingual onset (< 2yo) and 24 cases reported post-lingual onset (≥2yo) of hearing loss. Subjectively, 85% of cases had a favorable outcome. Objectively, the postoperative mean (SD) post-operative CNC word score was not significantly different than other adults [66.2% (25.8%) correct vs. 50.1% (12.5%); F(1,6) = 1.97, P = 0.21]. In the TMPRSS3 cohort, poor performers (CNC < 30% correct) were significantly older than good performers [49 (± 13.3) years vs. 17.4 (± 18.4) years; P < 0.01] and all harbored the A138E variant. TMPRSS3 immunostaining is restricted to the otic epithelial cells and is not expressed within auditory neurons of human cochlea and human inner ear organoids. CONCLUSIONS Patients with TMPRSS3-related hearing loss exhibit similar postoperative performance to other adult CI patients. TMPRSS3 is not expressed in human auditory neurons and the duration of hearing loss prior to CI likely contributes to poor performance.

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Histopathology of the Human Inner Ear in a Patient With Sensorineural Hearing Loss Caused by a Variant in DFNA5

Otology & Neurotology ◽

10.1097/mao.0000000000000888 ◽

2015 ◽

Vol 36 (10) ◽

pp. 1616-1621 ◽

Cited By ~ 6

Author(s):

Joseph B. Nadol ◽

Ophir Handzel ◽

Sami Amr

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Inner Ear ◽

Sensorineural Hearing ◽

Human Inner Ear

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The inner ear

Oxford Handbook of ENT and Head and Neck Surgery ◽

10.1093/med/9780199550791.003.07 ◽

2009 ◽

pp. 109-148

Author(s):

Rogan Corbridge ◽

Nicholas Steventon

Keyword(s):

Hearing Loss ◽

Inner Ear ◽

Sudden Hearing Loss ◽

Structure And Function ◽

Noise Induced Hearing Loss ◽

Hereditary Hearing Loss ◽

Syndromic Hearing Loss ◽

And Function

Structure and function of the inner ear 110 Hearing loss 114 Presbyacusis 116 Noise-induced hearing loss 118 Idiopathic sudden hearing loss 120 Autoimmune ear disease 122 Ototoxicity 124 Hereditary hearing loss 126 Syndromic hearing loss I 128 Syndromic hearing loss II 130 Non-organic hearing loss (NOHL) ...

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Noise Induced Hearing Loss: A Case Study from a Speech-Language Pathologist’s Perspective

10.5772/intechopen.96332 ◽

2021 ◽

Author(s):

Alejandro Brice

Keyword(s):

Hearing Loss ◽

Inner Ear ◽

Hearing Aids ◽

Noise Exposure ◽

Chronic Health Condition ◽

Noise Induced Hearing Loss ◽

Speech Language Pathologist ◽

The Individual ◽

The U.S

Hearing loss is very common in the United States and the most widespread disability in the U.S. Hearing loss is the third most chronic health condition in the U.S. Noise induced hearing loss (NIHL) results from damaging external noise. This injury leads to temporarily or permanently affecting sensitive inner ear structures (e.g., cochlea, organ of Corti, and hair cells). NIHL can result from a single high-level noise exposure or repeated exposures to excessively loud noises [i.e., typically 85 dBA or greater, (A weighted decibel)]. Damage to the inner ear can also result from aging (i.e., presbycusis). This case study documents the hearing loss of an otherwise healthy 21-year-old, male individual and his progressive moderate-to-severe sensorineural hearing loss over a period of 41 years. His history will be reported along with his perspective as a speech-language pathologist and speech scientist. The individual with hearing loss has adapted to wearing hearing aids over the last five years. Issues that have occurred affecting comprehension along with compensatory strategies that assisted listening and comprehension will be discussed.

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