Treatment Of Hearing Loss Research Articles

Additional comments on foot reflexology treatment for sensorineural hearing loss in infant.

Currently, treatment options for infant sensorineural hearing loss (SNHL) are limited. This article describes a novel case of SNHL in an infant successfully treated with foot reflexology, along with observed brain activity changes before and after treatment, as indicated by functional magnetic resonance imaging. Hence, this commentary discusses the case and our viewpoints regarding foot reflexology for treating SNHL.

Over-the-Counter Hearing Aids Versus Traditional Hearing Aids in Patients With Mild-to-Moderate Hearing Loss: Protocol for a Noninferiority Randomized Controlled Trial.

With the aging of society, the prevalence of hearing loss (HL) is increasing. Currently, approximately 5% of the global population has HL, and this number is projected to reach 7 million by 2050. Although hearing aids (HAs) are the primary treatment for HL, their use is limited by barriers such as high costs and social stigma. To address these limitations, over-the-counter (OTC) HAs have been introduced, but their effectiveness and drawbacks require further investigation. This study aims to conduct a noninferiority randomized controlled trial comparing OTC HAs with traditional HAs to assess the clinical effectiveness of OTC HAs. We designed a noninferiority randomized controlled trial comparing OTC HAs and traditional HAs in adults with mild-to-moderate HL. A total of 64 participants (32 per group) will be recruited. Randomization will be performed using block randomization (block sizes of 2 or 4) with an equal allocation ratio. The study will include 2 types of HAs: an OTC HA (Jabra Enhance Pro) and a traditional HA (LiNX Quattro LE561-DRW) by GN ReSound A/S. OTC HAs will be self-fitted using a smartphone app, while traditional HAs will be fitted by a licensed audiologist using the National Acoustics Laboratories-Non-Linear Prescription, second generation. Assessments, including functional gain, real-ear measurement, speech audiometry, and questionnaires, will be conducted at 6-month intervals over the course of 3 visits. Statistical analysis will compare the 2 outcomes, focusing on functional gain, to determine noninferiority. This study is scheduled to begin in August 2024 and has not yet recruited any participants. The study will be conducted over 2 years, from August 2024 to July 2026. Each participant will have 2 follow-up visits at 6-month intervals, making the total follow-up period 1 year. Since 2022, the introduction of OTC HAs has revolutionized access to these devices. Researchers, clinicians, and the general public are keen to evaluate the clinical effectiveness of OTC HAs, as more individuals will likely use them for HL. This increased usage will provide valuable real-world data to understand the benefits and limitations of OTC HAs. Monitoring the outcomes and user feedback will provide insights into their effectiveness and impact on hearing rehabilitation. PRR1-10.2196/59894.

State-of-the-Art on the Impact of Bimodal Acoustic Stimulation on Speech Perception in Noise in Adults: A Systematic Review.

Bimodal stimulation (BS), which combines the use of a cochlear implant (CI) in one ear and a hearing aid (HA) in the opposite ear, is an established strategy to treat hearing loss by exploiting the unique capabilities of each device. CIs stimulate the auditory nerve by bypassing damaged hair cells, while HAs amplify sounds by requiring a functional hearing residual. The aim of this systematic review is to investigate the advantages and disadvantages of BS such as speech perception in noise. We examined clinical studies published from October 2020 to July 2024, following the PRISMA guidelines, focusing on the advantages and disadvantages of BS on speech perception in noise in adulthood. BS in adult patients significantly improves speech perception in quiet and noisy environments, especially for those with increased residual hearing. Unilateral CIs and BS perform similarly in quiet conditions, but BS significantly improves speech discrimination in noisy environments if loudness between the two devices is appropriately balanced. Directional microphones and programming software are new technologies that succeed in reducing environmental noise and improving verbal perception outcomes, although their features in the literature are controversial. In addition, the individuals using BS may face temporal mismatches mainly due to differing device latencies, affecting sound localization. Compensating for these mismatches can enhance localization accuracy. However, modulated noise remains a significant obstacle to verbal perception in noise. Valuable assessment tools such as music tests provide further information on hearing performance and quality of life. More research is needed to define certain selection criteria.

Cochlear implant/MXene-based electroacoustic stimulation modulates the growth and maturation of spiral ganglion neurons

Cochlear implantation (CI) offers a dependable treatment for sensorineural hearing loss, with precision electroacoustic stimulation parameters showing great potential in improving auditory outcomes in CI patients. Here, we report the attachment of MXene into CI systems which effectively mimic the neural electrode interface due to MXene's excellent electrical conductivity and biocompatibility. Low-frequency short-term biphasic electrical pulses emitted by the MXenes-based CI promoted the outgrowth of spiral ganglion neuron (SGN) neurites and growth cones, substantially boosting the calcium activity in SGNs. This study lays a theoretical foundation for the precision medicine approaches in CI patient care, and informs the selection of materials for cochlear implant electrode materials in the future.

Research progress on GSDME-mediated cell pyroptosis resulting in GSDME type hearing loss

Pyroptosis is a novel mode for programmed cell death discovered and confirmed in recent years. The Gasdermins (GSDMs) family is a key effector molecule mediating pyroptosis. As an important cause of extensive inflammatory damage and side effects of conventional chemotherapy drugs, anomalous pyroptosis has also been associated with hearing loss, tumor, and disorders of the immune system. The GSDME protein, encoded by the GSDME (DFNA5) gene, belongs to the GSDMs family and is a key factor mediating pyroptosis. Gain-of-function variants of the GSDME gene can lead to GSDME-related hearing loss, which shows an autosomal dominant inheritance. This article has reviewed the role of GSDME-mediated pyroptosis in the pathogenesis of GSDME-related hearing loss, with an aim to provide insights into the treatment of GSDME-related hearing loss.

Antiviral Treatment and Risk of Hearing Loss in Asymptomatic and Mild Symptomatic Infants With Congenital Cytomegalovirus.

To assess hearing outcomes at 24 months of age in infants with mild congenital cytomegalovirus (cCMV) infection, depending on whether they have received antiviral treatment or not. A retrospective study within the European Registry of Children with cCMV was performed. Included children had cCMV diagnosed in utero/in the first 21 days of life, with normal physical examination, alanine aminotransferase <80 U/L and platelets >100,000 cs/mm3 and absence of hearing loss (HL) at birth. Cranial ultrasound (cUS) and/or cranial magnetic resonance imaging was normal or with minor findings (isolated lenticulostriate vasculopathy and/or germinolysis/caudothalamic or subependymal cysts, and/or focal/multifocal white matter involvement). The main outcome was the presence of HL at 24 months of age. One hundred ninety-six patients met inclusion criteria. A total of 34.7% received antiviral treatment with valganciclovir/ganciclovir. Children treated had lower gestational age, birth weight and head circumference, and maternal primary infection was less frequent. Among treated children, 21.3% presented minor findings in cUS versus 6.3% in nontreatment group (P = 0.003). Nine patients (4.6%) developed HL at 24 months. Among children with HL, 20% presented minor findings in cUS versus 11.3% in non-HL group (P = NS). HL rate was similar in treated and nontreated groups (4.6% vs. 6.3%; P = 0.6). One-third of the children were treated with antivirals and infants with minor neuroimaging findings at birth were more likely to receive antiviral. There were no differences in the prevalence of HL at 2 years of age between treated and not-treated children. Minor neuroimaging findings were not clearly associated with an increased risk of delayed onset HL.

Perfusion Steroid via Ventilation Tube as Salvage Treatments for Sudden Sensorineural Hearing Loss.

Objectives: Intratympanic steroid injection (ISI) for sudden sensorineural hearing loss (SSNHL) is a relatively popular and effective method, but there is no standardized method for intratympanic steroids for the treatment of SSNHL and no consensus on how to deliver steroids to the middle ear. The purpose of this study was to compare 2 means of intratympanic steroid delivery as therapy for SSNHL. Methods: A retrospective chart review was performed for the period from November 2018 to October 2022 at our Department of Otorhinolaryngology-Head and Neck Surgery. Sixty patients with profound SSNHL who have failed initial steroid therapy were divided into the continuously transtympanic steroid perfusion (TSP) therapy and the intermittent ISI therapy. Results: Posttreatment pure-tone average was 60.3 ± 18.2 dB in the TSP group and 67.5 ± 22.6 dB in the ISI group, 70.0% of subjects in the TSP group, and 46.7% of subjects in the ISI group had improved by 15 dB or more after the therapy. The increased hearing threshold of the TSP group (24.6 ± 14.1 dB) was better than the ISI group (16.6 ± 14.9 dB), and the hearing recovery was significantly different (P < .05) in the 2 groups. Besides the hearing improvement was most obvious in low-frequency areas in the TSP group, the most significant hearing improvement was at 250 Hz, reaching 30.8 ± 3.3 dB. Conclusions: In SSNHL patients who have failed initial steroid therapy, TSP therapy via a ventilation tube can achieve good hearing outcomes and serve as a salvage therapy for patients with SSNHL.

Increased listening effort and decreased speech discrimination at high presentation sound levels in acoustic hearing listeners and cochlear implant users.

The sounds we experience in our everyday communication can vary greatly in terms of level and background noise depending on the environment. Paradoxically, increasing the sound intensity may lead to worsened speech understanding, especially in noise. This is known as the "Rollover" phenomenon. There have been limited studies on rollover and how it is experienced differentially across aging groups, for those with and without hearing loss, as well as cochlear implant (CI) users. There is also mounting evidence that listening effort plays an important role in challenging listening conditions and can be directly quantified with objective measures such as pupil dilation. We found that listening effort was modulated by sound level and that rollover occurred primarily in the presence of background noise. The effect on listening effort was exacerbated by age and hearing loss in acoustic listeners, with greatest effect in older listeners with hearing loss, while there was no effect in CI users. The age- and hearing-dependent effects of rollover highlight the potential negative impact of amplification to high sound levels and therefore has implications for effective treatment of age-related hearing loss.

Development of Magnetic Nanosystems with Potential for the Treatment of Inner Ear Pathologies.

Hearing loss (HL) affects more than 5% of the global population, with projections indicating an impact of up to 50% on young individuals in the next years. HL treatments remain limited due to the inner ear's hermeticism. HL often involves inflammatory processes, underscoring the need for enhanced delivery of antiinflammatory agents to the inner ear. Our research focuses on the development of a directed therapy based on magnetic nanoparticles (MNPs). We previously synthesized biocompatible folic acid-coated iron oxide-core nanoparticles (MNPs@FA) as potential carriers for the anti-inflammatory Diclofenac (Dfc). This study aims to incorporate Dfc onto MNPs@FA to facilitate targeted drug delivery to the inner ear. Through optimizing the loading procedure, we achieved optimal loading capacity. Dfc release was studied in the simulated target fluid and the administration vehicle. Complete characterization is also shown. In vitro biocompatibility testing ensured the biosafety of the resulting formulation. Subsequent ex vivo targeting assays on murine cochleae validated the nanosystems' ability to penetrate the round window membrane, one of the main HL therapy barriers. These findings serve as validation before continuing to more complex in vivo studies. Together, the data here presented represent an advancement in addressing unmet medical needs in HL therapy.

Ototoxicity-Alleviating and Cytoprotective Allomelanin Nanomedicine for Efficient Sensorineural Hearing Loss Treatment.

Sensorineural hearing loss (SNHL) represents a significant clinical challenge, predominantly attributed to oxidative stress-related mechanisms. In this work, we report an innovative antioxidant strategy for mitigating SNHL, utilizing synthetically engineered allomelanin nanoparticles (AMNPs). Empirical evidence elucidates AMNPs' profound capability in free radical neutralization, substantiated by a significant decrement in reactive oxygen species (ROS) levels within HEI-OC1 auditory cells exposure to cisplatin or hydrogen peroxide (H2O2). Comparative analyses reveal that AMNPs afford protection against cisplatin-induced and noise-induced auditory impairments, mirroring the effect of dexamethasone (DEX), a standard pharmacological treatment for acute SNHL. AMNPs exhibit notable cytoprotective properties for auditory hair cells (HCs), effectively preventing ototoxicity from cisplatin or H2O2 exposure, as confirmed by both in vitro assays and cultured organ of Corti studies. Further in vivo research corroborates AMNPs' ability to reverse auditory brainstem response (ABR) threshold shifts resulting from acoustic injury, concurrently reducing HCs loss, ribbon synapse depletion, and spiral ganglion neuron degeneration. The therapeutic benefits of AMNPs are attributed to mitigating oxidative stress and inflammation within the cochlea, with transcriptome analysis indicating downregulated gene expression related to these processes post-AMNPs treatment. The pronounced antioxidative and anti-inflammatory effects of AMNPs position them as a promising alternative to DEX for SNHL treatment.

Hearing restoration by gene replacement therapy for a multisite-expressed gene in a mouse model of human DFNB111 deafness

Gene therapy has made significant progress in the treatment of hereditary hearing loss. However, most research has focused on deafness-related genes that are primarily expressed in hair cells with less attention given to multisite-expressed deafness genes. MPZL2, the second leading cause of mild-to-moderate hereditary deafness, is widely expressed in different inner ear cells. We generated a mouse model with a deletion in the Mpzl2 gene, which displayed moderate and slowly progressive hearing loss, mimicking the phenotype of individuals with DFNB111. We developed a gene replacement therapy system mediated by AAV-ie for efficient transduction in various types of cochlear cells. AAV-ie-Mpzl2 administration significantly lowered the auditory brainstem response and distortion product otoacoustic emission thresholds of Mpzl2-/- mice for at least seven months. AAV-ie-Mpzl2 delivery restored the structural integrity in both outer hair cells and Deiters cells. This study suggests the potential of gene therapy for MPZL2-related deafness and provides a proof of concept for gene therapy targeting other deafness-related genes that are expressed in different cell populations in the cochlea.

Pathophysiological processes underlying hidden hearing loss revealed in Kcnt1/2 double knockout mice.

Presbycusis is a prevalent condition in older adults characterized by the progressive loss of hearing due to age-related changes in the cochlea, the auditory portion of the inner ear. Many adults also struggle with understanding speech in noise despite having normal auditory thresholds, a condition termed "hidden" hearing loss because it evades standard audiological assessments. Examination of animal models and postmortem human tissue suggests that hidden hearing loss is also associated with age-related changes in the cochlea and may, therefore, precede overt age-related hearing loss. Nevertheless, the pathological mechanisms underlying hidden hearing loss are not understood, which hinders the development of diagnostic biomarkers and effective treatments for age-related hearing loss. To fill these gaps in knowledge, we leveraged a combination of tools, including transcriptomic profiling and morphological and functional assessments, to identify these processes and examine the transition from hidden to overt hearing loss. As a novel approach, we took advantage of a recently characterized model of hidden hearing loss: Kcnt1/2 double knockout mice. Using this model, we find that even before observable morphological pathology, hidden hearing loss is associated with significant alteration in several processes, notably proteostasis, in the cochlear sensorineural structures, and increased susceptibility to overt hearing loss in response to noise exposure and aging. Our findings provide the first insight into the pathophysiology associated with the earliest and, therefore, most treatable stages of hearing loss and provide critical insight directing future investigation of pharmaceutical strategies to slow and possibly prevent overt age-related hearing loss.

Speech intelligibility prediction based on a physiological model of the human ear and a hierarchical spiking neural network.

A speech intelligibility (SI) prediction model is proposed that includes an auditory preprocessing component based on the physiological anatomy and activity of the human ear, a hierarchical spiking neural network, and a decision back-end processing based on correlation analysis. The auditory preprocessing component effectively captures advanced physiological details of the auditory system, such as retrograde traveling waves, longitudinal coupling, and cochlear nonlinearity. The ability of the model to predict data from normal-hearing listeners under various additive noise conditions was considered. The predictions closely matched the experimental test data under all conditions. Furthermore, we developed a lumped mass model of a McGee stainless-steel piston with the middle-ear to study the recovery of individuals with otosclerosis. We show that the proposed SI model accurately simulates the effect of middle-ear intervention on SI. Consequently, the model establishes a model-based relationship between objective measures of human ear damage, like distortion product otoacoustic emissions, and speech perception. Moreover, the SI model can serve as a robust tool for optimizing parameters and for preoperative assessment of artificial stimuli, providing a valuable reference for clinical treatments of conductive hearing loss.

Investigating the Effect of Coenzyme Q10 in the Treatment of Sudden Sensorineural Hearing Loss

Background and Objective: Sudden sensorineural hearing loss (SSNHL) is a complication that is usually unilateral and can be accompanied by tinnitus and vertigo. Although various infectious, vascular, and immune etiologies have been proposed for SSNHL, the cause is unknown in most cases. Therefore, this study aimed to determine the effect of coenzyme Q10 in the treatment of SSNHL. Materials and Methods: This study was a double-blind, randomized, controlled clinical trial, which was conducted using the available sampling method. A total of 60 patients with SSNHL (18-70 years old) lasting less than one month were divided into two groups: intervention and control. The routine treatment of both groups included weekly injections of dexamethasone in the middle ear for 3 weeks. The intervention group also received 100 mg of coenzyme Q10 per day for 4 weeks, while the control group received a placebo. Patients were evaluated by basic audiological tests at the baseline and third and sixth months after the treatment. Statistical analysis of findings was done in SPSS26 software. Results: The difference in the mean pure tone hearing thresholds of the intervention and control groups was significant in the third month (29.33±15.96 dB and 35.34±12.24 dB; P=0.02) and sixth month (dB 28.00±16.27 and dB 34.33±11.87; P=0.01). Conclusion: In patients with SSNHL, combined treatment with oral coenzyme Q10 at the rate of 100 mg per day, along with intratympanic injection of dexamethasone for four weeks, can improve pure tone hearing thresholds and facilitate the treatment

Sudden sensorineural hearing loss during the Covid-19 pandemic; experience of a tertiary referral center

ObjectiveThis study aimed to highlight the differences in the clinical management and treatment of sudden sensorineural hearing loss (SSNHL) due to the impact of the Coronavirus Disease 2019 (COVID-19) pandemic. MethodsThis study compared patients diagnosed with SSNHL between March 2020 and March 2022, following the first reported case of COVID-19 in our country, with patients diagnosed between March 2018 and March 2020. The evaluation encompassed demographic characteristics, comorbidities, other ear-related complaints, hearing loss thresholds at each frequency, medical treatment administered, treatment duration, and post-treatment follow-up audiograms. ResultsThe demographic characteristics and comorbidities of patients before and during the pandemic showed similar distribution. There was no significant difference in the duration from the onset of symptoms to hospital admission during the pandemic compared to the previous period. When evaluating the treatment responses of hospitalized and treated patients according to Siegel's criteria during the pandemic, a similar trend to the pre-pandemic period was observed. It was noted that the use of systemic steroids and hyperbaric oxygen therapy decreased during the pandemic period, while the use of antivirals increased. ConclusionsIn our study conducted as a reference center, we want to emphasize that no clear data indicating a relationship between COVID-19 infection and NHL. Also, we believe that COVID-19 infection does not affect the course and prognosis of SSNHL.

Artificial intelligence for hearing loss prevention, diagnosis, and management

This paper explores the transformative impact of artificial intelligence (AI), particularly machine learning (ML), on diagnosing and treating hearing loss, which affects over 5% of the global population across all ages and demographics. AI encompasses various applications, from natural language processing models like ChatGPT to image recognition systems; however, this paper focuses on ML, a subfield of AI that can revolutionize audiology by enhancing early detection, formulating personalized rehabilitation plans, and integrating electronic health records for streamlined patient care. The integration of ML into audiometry, termed "computational audiology," allows for automated, accurate hearing tests. AI algorithms can process vast data sets, provide detailed audiograms, and facilitate early detection of hearing impairments. Research shows ML's effectiveness in classifying audiograms, conducting automated audiometry, and predicting hearing loss based on noise exposure and genetics. These advancements suggest that AI can make audiological diagnostics and treatment more accessible and efficient. The future of audiology lies in the seamless integration of AI technologies. Collaborative efforts between audiologists, AI experts, and individuals with hearing loss are essential to overcome challenges and leverage AI's full potential. Continued research and development will enhance AI applications in audiology, improving patient outcomes and quality of life worldwide.

Dexamethasone nanocrystals-embedded hydroxypropyl methylcellulose hydrogel increases cochlear delivery and attenuates hearing loss following intratympanic injection

Herein, dexamethasone (DEX) nanocrystalline suspension (NS)-embedded hydrogel (NS-G) was constructed using a hydroxypropyl methylcellulose (HPMC) polymer to enhance cochlear delivery and attenuate hearing loss following intratympanic (IT) injection. Hydrophobic steroidal nanocrystals were prepared using a bead milling technique and incorporated into a polysaccharide hydrogel. The NS-G system with HPMC (average molecular weight, 86,000 g/mol; 15 mg/mL) was characterized as follows: rod-shaped drug crystalline; particle size <300 nm; and constant complex viscosity ≤1.17 Pa·s. Pulverization of the drug particles into submicron diameters enhanced drug dissolution, while the HPMC matrix increased the residence time in the middle ear cavity, exhibiting a controlled release profile. The IT NS-G system elicited markedly enhanced and prolonged drug delivery (> 9 h) to the cochlear tissue compared with that of DEX sodium phosphate (DEX-SP), a water-soluble prodrug. In mice with kanamycin- and furosemide-induced ototoxicity, NS-G markedly enhanced hearing preservation across all frequencies (8–32 kHz), as revealed by an auditory brainstem response test, compared with both saline and DEX-SP. Moreover, treatment with NS-G showed enhanced anti-inflammatory effects, as evidenced by decreased levels of inflammation-related cytokines. Therefore, the IT administration of DEX NS-loaded HPMC hydrogels is a promising strategy for treating hearing loss.

Age-based disparities in hearing loss diagnosis and treatment in the United States population

ObjectiveTo investigate age-related disparities in the diagnosis and treatment of borderline/mild hearing loss (HL) in the United States. Study designA cross-sectional epidemiologic study. SettingThe National Health and Nutrition Examination Survey (NHANES). MethodsMultivariable logistic regressions controlling for hearing level were performed to investigate the association between: (1) age and recent hearing test; (2) age and hearing aid use. Age was grouped into quartiles (<25, 25–49, 50–74, ≥75 years). The first quartile of life was used as a reference group in all odds ratios, controlling for hearing level. ResultsOf 2115 participants with borderline/mild HL, 3 % (n = 53) were in age quartile Q1; 7 % (n = 147) were in Q2, 56 % (n = 1190) were in Q3, and 34 % (n = 725) were in Q4. Compared to Q1, those in Q2, Q3, and Q4 had 4.06 times (95 % CI = 2.11–8.02, p < 0.001), 4.51 times (2.56–8.19, p < 0.001), and 4.56 times (2.55–8.39, p < 0.001) lower odds of a hearing test within the past 4 years. Similar, although slightly larger, odds ratios were obtained when the outcome was hearing test within 1 year. Compared to Q1, those in Q2, Q3, and Q4 respectively had 4.38 times (1.47–13.5, p < 0.05), 5.41 times (2.27–11.8, p < 0.001), and 3.95 times (1.65–8.72, p < 0.05) lower odds of using a hearing aid. ConclusionWe have characterized a large, unaddressed, and modifiable disparity in the treatment of borderline/mild HL as individuals age out of the first quartile of life. Future studies are needed to explore factors, such as ageism, that may underlie these findings.

Polymer-Based Nanoparticles for Inner Ear Targeted Trans Differentiation Gene Therapy.

Hearing loss is a significant disability that often goes under recognised, largely due to poor identification, prevention, and treatment. Steps are being made to amend these pitfalls in the investigation of hearing loss, however, the development of a cure to reverse advanced forms remains distant. This review details some current advances in the treatment of hearing loss, with a particular focus on genetic-based nanotechnology and how it may provide a useful avenue for further research. This review presents a broad background on the pathophysiology of hearing loss and some current interventions. We also highlight some potential genes that may be useful in the amelioration of hearing loss. Pathways of cellular differentiation from stem or supporting cell to functional hair cell are covered in detail, as this mechanism represents a key means of regenerating these cell types. Overall, we believe that polymer-based nanotechnology coupled with novel excipients represents a useful area of further research in the treatment of hearing loss, although further studies in this area are required.

In Utero Gene Therapy and its Application in Genetic Hearing Loss.

For monogenic genetic diseases, in utero gene therapy (IUGT) shows the potential for early prevention against irreversible and lethal pathological changes. Moreover, animal models have also demonstrated the effectiveness of IUGT in the treatment of coagulation disorders, hemoglobinopathies, neurogenetic disorders, and metabolic and pulmonary diseases. For major alpha thalassemia and severe osteogenesis imperfecta, in utero stem cell transplantation has entered the phase I clinical trial stage. Within the realm of the inner ear, genetic hearing loss significantly hampers speech, cognitive, and intellectual development in children. Nowadays, gene therapies offer substantial promise for deafness, with the success of clinical trials in autosomal recessive deafness 9 using AAV-OTOF gene therapy. However, the majority of genetic mutations that cause deafness affect the development of cochlear structures before the birth of fetuses. Thus, gene therapy before alterations in cochlear structure leading to hearing loss has promising applications. In this review, addressing advances in various fields of IUGT, the progress, and application of IUGT in the treatment of genetic hearing loss are focused, in particular its implementation methods and unique advantages.