Ruth Mtamo, Jenna Vallario, Ambuj Kumar, Jesse Casanova, Julia Toman
{"title":"Assessment of Outer and Middle Ear Pathologies in Lilongwe, Malawi.","authors":"Ruth Mtamo, Jenna Vallario, Ambuj Kumar, Jesse Casanova, Julia Toman","doi":"10.3390/audiolres14030041","DOIUrl":"10.3390/audiolres14030041","url":null,"abstract":"<p><p>Outer and middle ear pathologies are known to disproportionately affect low-income countries but data is limited. We aim to quantify the prevalence rate of patients presenting with middle/outer ear pathologies at ABC Hearing Clinic and Training Centre in Lilongwe, Malawi. Audiological consultations (adult and paediatric) from 2018-2020 were reviewed for outer and middle ear pathologies. Secondary outcomes included patient type (private vs. community) compared to otoscopy findings, tympanometry findings, need for follow up, and follow up compliance. Out of 1576 patients reviewed, the proportion of abnormal cases' was 98.2%, with 41.4% being unilateral and 57.4% bilateral. Eighty-three percent presented with outer/middle ear pathologies. 68% of those presented with a pathology often associated with some degree of conductive hearing loss (occluding wax, perforation, discharge, Type B/Type C tympanogram). Average age was 29 + 0.527 years; 41.6% private and 58.2% community patients. Cerumen impaction was most common finding (51%). Higher rates of otoscopic abnormalities and type B tympanograms were noted in community vs. private patient (~40% vs. ~30%; ~70% vs. ~30%). Adherence to follow up was higher for community vs. private patients (29% vs. 17%); ~70% reported subjective improvement upon follow up. The majority required multiple interventions on follow up. Secondary follow up was recommended in 64.8%. A significant disease burden of outer and middle ear pathologies was identified. Further research is required to understand the disease burden and promote health policy.</p>","PeriodicalId":44133,"journal":{"name":"Audiology Research","volume":"14 3","pages":"493-504"},"PeriodicalIF":2.1,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11200490/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141451783","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The Current State of Evidence Regarding Audiologist-Provided Cognitive Behavioural Therapy for the Management of Tinnitus: A Scoping Review.","authors":"Louise A Burke, Amr El Refaie","doi":"10.3390/audiolres14030035","DOIUrl":"10.3390/audiolres14030035","url":null,"abstract":"<p><strong>Background: </strong>Cognitive behavioural therapy (CBT) for tinnitus management is effective and widely recommended by national and international practice guidelines. However, all the evidence for CBT so far has come from Psychologist-led programs, and the potential role of Audiologists in providing CBT for tinnitus remains an important consideration.</p><p><strong>Objectives: </strong>This study sets out to systematically map the body of literature relating to Audiologist-provided CBT for tinnitus, in order to summarise the current state of evidence and determine directions for future research.</p><p><strong>Eligibility criteria: </strong>Sources were eligible for inclusion if they addressed the concept of Audiologist-provided CBT. No restrictions were imposed on the date of publication. Only sources published in English were included.</p><p><strong>Sources of evidence: </strong>A wide range of primary and secondary literature sources were sought.</p><p><strong>Charting methods: </strong>Data from included sources were charted systematically using a pre-designed data charting form.</p><p><strong>Results: </strong>Of the 267 identified sources, 30 were included in this review. This included both primary and secondary literature sources. Primary sources were compared and showed variation across Audiologist-provided CBT programs both in terms of procedural details and from a research standpoint.</p><p><strong>Conclusions: </strong>A growing body of evidence has addressed the concept of Audiologist-provided CBT. Directions for future research include further primary research with an increased focus on face-to-face Audiologist-provided CBT, and a comparison of the outcomes of Audiologist-provided vs. Psychologist-provided CBT.</p>","PeriodicalId":44133,"journal":{"name":"Audiology Research","volume":"14 3","pages":"412-431"},"PeriodicalIF":2.1,"publicationDate":"2024-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11130867/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141157321","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pietro Canzi, Elena Carlotto, Elisabetta Zanoletti, Johan H M Frijns, Daniele Borsetto, Antonio Caruso, Luisa Chiapparini, Andrea Ciorba, Giorgio Conte, Nathan Creber, Stefania Criscuolo, Filippo Di Lella, Sebastiano Franchella, Erik F Hensen, Lorenzo Lauda, Stefano Malpede, Marco Mandalà, Liselotte J C Rotteveel, Anna Simoncelli, Anna Chiara Stellato, Diego Zanetti, Marco Benazzo
{"title":"Multi-Magnet Cochlear Implant Technology and Magnetic Resonance Imaging: The Safety Issue.","authors":"Pietro Canzi, Elena Carlotto, Elisabetta Zanoletti, Johan H M Frijns, Daniele Borsetto, Antonio Caruso, Luisa Chiapparini, Andrea Ciorba, Giorgio Conte, Nathan Creber, Stefania Criscuolo, Filippo Di Lella, Sebastiano Franchella, Erik F Hensen, Lorenzo Lauda, Stefano Malpede, Marco Mandalà, Liselotte J C Rotteveel, Anna Simoncelli, Anna Chiara Stellato, Diego Zanetti, Marco Benazzo","doi":"10.3390/audiolres14030034","DOIUrl":"10.3390/audiolres14030034","url":null,"abstract":"<p><p>Despite the spread of novel-generation cochlear-implant (CI) magnetic systems, access to magnetic resonance imaging (MRI) for CI recipients is still limited due to safety concerns. The aim of this study is to assess and record the experiences of Hires Ultra 3D (Advanced Bionics) recipients who underwent an MRI examination. A multicentric European survey about this topic was conducted focusing on safety issues, and the results were compared with the current literature. We collected a total of 65 MRI scans performed in 9 otologic referral centers for a total of 47 Hires Ultra 3D recipients, including, for the first time, 2 children and 3 teenagers. Preventive measures were represented by scanning time and sedation for children. Head wrapping was used in eight cases, and six of the eight cases received local anesthesia, even if both measures were not needed. Only three patients complained of pain (3/65 examinations, 4.6%) due to the tight head bandage, and one of the three cases required MRI scan interruption. No other adverse events were reported. We believe that these results should encourage MRI execution in accordance with manufacturer recommendations for Ultra 3D recipients.</p>","PeriodicalId":44133,"journal":{"name":"Audiology Research","volume":"14 3","pages":"401-411"},"PeriodicalIF":1.7,"publicationDate":"2024-04-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11130805/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141157313","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Paweł Rozbicki, Jacek Usowski, Sandra Krzywdzińska, Dariusz Jurkiewicz, Jacek Siewiera
{"title":"Assessing the Effectiveness of Different Hyperbaric Oxygen Treatment Methods in Patients with Sudden Sensorineural Hearing Loss.","authors":"Paweł Rozbicki, Jacek Usowski, Sandra Krzywdzińska, Dariusz Jurkiewicz, Jacek Siewiera","doi":"10.3390/audiolres14020029","DOIUrl":"https://doi.org/10.3390/audiolres14020029","url":null,"abstract":"<p><strong>Introduction: </strong>Hyperbaric oxygen therapy (HBOT) is one of the treatment methods in patients with sudden sensorineural hearing loss (SSNHL). It is recommended as an elective treatment in patients undergoing steroid therapy. According to current scientific reports, HBOT should be implemented within two weeks after the first symptoms. However, as far as the profile of HBOT is concerned, there are no straightforward recommendations.</p><p><strong>Methods: </strong>The data obtained from the medical records of 218 patients undergoing HBOT for SSNHL at the Military Institute of Medicine-National Research Institute were analyzed statistically for the impact of the duration and the delay in implementing HBOT on the end results of pure-tone audiometry (PTA).</p><p><strong>Results: </strong>A statistically significant hearing improvement in patients undergoing more than 15 cycles of HBOT was detected at all frequencies except for 1500 Hz; in the group reporting for treatment with a delay of more than 10 days, hearing improvement was statistically unsignificant at frequencies of 1500, 3000, and 4000 Hz.</p><p><strong>Conclusions: </strong>The statistical analysis showed that the urgent onset of HBOT could be a significant factor in the therapy of SSNHL.</p>","PeriodicalId":44133,"journal":{"name":"Audiology Research","volume":"14 2","pages":"333-341"},"PeriodicalIF":1.7,"publicationDate":"2024-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11047301/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140860625","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Issam Saliba, Sarah Alshehri, Isabelle Fournier, Nasser Altamami
{"title":"Large Vestibular Aqueduct-Associated Symptoms: Endolymphatic Duct Blockage as a Surgical Treatment.","authors":"Issam Saliba, Sarah Alshehri, Isabelle Fournier, Nasser Altamami","doi":"10.3390/audiolres14020027","DOIUrl":"10.3390/audiolres14020027","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to evaluate the effectiveness of endolymphatic duct blockage (EDB) on dizziness control in patients with a large vestibular aqueduct (LVA) and to evaluate its effect on hearing.</p><p><strong>Study design: </strong>This is a prospective nonrandomized study.</p><p><strong>Setting: </strong>Five adults and one child with dizziness and five children with progressive hearing loss were referred to our tertiary centers.</p><p><strong>Methods: </strong>The dizziness handicap inventory (DHI) and DHI-PC (dizziness handicap inventory-patient caregiver) questionnaires were used before and after surgery. All patients underwent a preoperative temporal bone HRCT scan and pure tone audiometry one day before surgery, then four and twelve months after surgery and at the last follow-up. The mean follow-up time was 5.6 years. Student's <i>t</i>-test was used to compare DHI/-PC results.</p><p><strong>Results: </strong>The DHI scores were 44, 24, 84, 59 and 56 before surgery, respectively, for Patients 1 to 5. The DHI scores at four months was significantly different, i.e., 4, 6, 0, 7 and 18 (<i>p</i> = 0.001). No differences were found between 4 and 12 months. Patient 6 (child) had Trisomy 21; their DHI-PC score dropped from 38 (preoperative score) to 8 (postoperative score), showing no activity limitations; clinical evaluation showed the complete resolution of symptoms. We found no significant differences between hearing loss before the surgery and at 1 and 12 months post operation for four adult patients. Our fifth adult patient's hearing changed from severe to profound SNHL. For 5 out of 6 pediatric patients, preoperative PTA and mean ABG were 63 dB and 20 dB, respectively; postoperatively, they improved to 42 dB and 16 dB, respectively. The hearing loss level for the sixth pediatric patient dropped from moderate (PTA = 42 dB) to severe (PTA = 85 dB) due to an opening of the endolymphatic sac and a sudden leak of the endolymph.</p><p><strong>Conclusions: </strong>EDB, using two titanium clips, seems to be helpful for controlling vestibular symptoms and for stabilizing hearing or even to improve hearing in 82% of cases. Nevertheless, there is a risk of hearing worsening.</p>","PeriodicalId":44133,"journal":{"name":"Audiology Research","volume":"14 2","pages":"304-316"},"PeriodicalIF":1.7,"publicationDate":"2024-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10961693/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140207836","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Over-the-Counter (OTC) Hearing Aid Availability across the Spectrum of Human Skin Colors.","authors":"Shade Avery Kirjava, Sam Jones Faulkner","doi":"10.3390/audiolres14020026","DOIUrl":"10.3390/audiolres14020026","url":null,"abstract":"<p><strong>Background: </strong>Over-the-counter (OTC) hearing aids were recently approved for sale in the United States. Research has shown that consumers prefer hearing devices that match their skin color because these devices are less noticeable. Colorism is discrimination against individuals with relatively darker skin that manifests in \"skin-color\" product offerings as products being offered primarily in relatively lighter colors.</p><p><strong>Methods: </strong>This study compared images of U.S. Food and Drug Administration (FDA)-registered over-the-counter hearing aids to a range of human skin colors.</p><p><strong>Results: </strong>Most over-the-counter hearing aids are only offered in relatively lighter beige colors. Few over-the-counter hearing aids are available in darker skin colors.</p><p><strong>Conclusions: </strong>These findings may represent structural bias, preventing equitable access to darker skin-color OTC hearing aids for individuals with darker skin.</p>","PeriodicalId":44133,"journal":{"name":"Audiology Research","volume":"14 2","pages":"293-303"},"PeriodicalIF":1.7,"publicationDate":"2024-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10961692/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140207838","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mahmoud Keshavarzi, Marina Salorio-Corbetto, Tobias Reichenbach, Josephine Marriage, Brian C J Moore
{"title":"Development of New Open-Set Speech Material for Use in Clinical Audiology with Speakers of British English.","authors":"Mahmoud Keshavarzi, Marina Salorio-Corbetto, Tobias Reichenbach, Josephine Marriage, Brian C J Moore","doi":"10.3390/audiolres14020024","DOIUrl":"10.3390/audiolres14020024","url":null,"abstract":"<p><strong>Background: </strong>The Chear open-set performance test (COPT), which uses a carrier phrase followed by a monosyllabic test word, is intended for clinical assessment of speech recognition, evaluation of hearing-device performance, and the fine-tuning of hearing devices for speakers of British English. This paper assesses practice effects, test-retest reliability, and the variability across lists of the COPT.</p><p><strong>Method: </strong>In experiment 1, 16 normal-hearing participants were tested using an initial version of the COPT, at three speech-to-noise ratios (SNRs). Experiment 2 used revised COPT lists, with items swapped between lists to reduce differences in difficulty across lists. In experiment 3, test-retest repeatability was assessed for stimuli presented in quiet, using 15 participants with sensorineural hearing loss.</p><p><strong>Results: </strong>After administration of a single practice list, no practice effects were evident. The critical difference between scores for two lists was about 2 words (out of 15) or 5 phonemes (out of 50). The mean estimated SNR required for 74% words correct was -0.56 dB, with a standard deviation across lists of 0.16 dB. For the participants with hearing loss tested in quiet, the critical difference between scores for two lists was about 3 words (out of 15) or 6 phonemes (out of 50).</p>","PeriodicalId":44133,"journal":{"name":"Audiology Research","volume":"14 2","pages":"264-279"},"PeriodicalIF":1.7,"publicationDate":"2024-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10961685/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140207799","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Navigating the Usher Syndrome Genetic Landscape: An Evaluation of the Associations between Specific Genes and Quality Categories of Cochlear Implant Outcomes.","authors":"Micol Busi, Alessandro Castiglione","doi":"10.3390/audiolres14020023","DOIUrl":"10.3390/audiolres14020023","url":null,"abstract":"<p><p>Usher syndrome (US) is a clinically and genetically heterogeneous disorder that involves three main features: sensorineural hearing loss, retinitis pigmentosa (RP), and vestibular impairment. With a prevalence of 4-17/100,000, it is the most common cause of deaf-blindness worldwide. Genetic research has provided crucial insights into the complexity of US. Among nine confirmed causative genes, <i>MYO7A</i> and <i>USH2A</i> are major players in US types 1 and 2, respectively, whereas <i>CRLN1</i> is the sole confirmed gene associated with type 3. Variants in these genes also contribute to isolated forms of hearing loss and RP, indicating intersecting molecular pathways. While hearing loss can be adequately managed with hearing aids or cochlear implants (CIs), approved RP treatment modalities are lacking. Gene replacement and editing, antisense oligonucleotides, and small-molecule drugs hold promise for halting RP progression and restoring vision, enhancing patients' quality of life. Massively parallel sequencing has identified gene variants (e.g., in <i>PCDH15</i>) that influence CI results. Accordingly, preoperative genetic examination appears valuable for predicting CI success. To explore genetic mutations in CI recipients and establish correlations between implant outcomes and involved genes, we comprehensively reviewed the literature to gather data covering a broad spectrum of CI outcomes across all known US-causative genes. Implant outcomes were categorized as excellent or very good, good, poor or fair, and very poor. Our review of 95 cochlear-implant patients with US, along with their CI outcomes, revealed the importance of presurgical genetic testing to elucidate potential challenges and provide tailored counseling to improve auditory outcomes. The multifaceted nature of US demands a comprehensive understanding and innovative interventions. Genetic insights drive therapeutic advancements, offering potential remedies for the retinal component of US. The synergy between genetics and therapeutics holds promise for individuals with US and may enhance their sensory experiences through customized interventions.</p>","PeriodicalId":44133,"journal":{"name":"Audiology Research","volume":"14 2","pages":"254-263"},"PeriodicalIF":1.7,"publicationDate":"2024-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10961690/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140207837","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Stefano Concheri, Davide Brotto, Marzia Ariano, Antonio Daloiso, Valerio Maria Di Pasquale Fiasca, Flavia Sorrentino, Beatrice Coppadoro, Patrizia Trevisi, Elisabetta Zanoletti, Sebastiano Franchella
{"title":"Intraoperative Measurement of Insertion Speed in Cochlear Implant Surgery: A Preliminary Experience with Cochlear SmartNav.","authors":"Stefano Concheri, Davide Brotto, Marzia Ariano, Antonio Daloiso, Valerio Maria Di Pasquale Fiasca, Flavia Sorrentino, Beatrice Coppadoro, Patrizia Trevisi, Elisabetta Zanoletti, Sebastiano Franchella","doi":"10.3390/audiolres14020021","DOIUrl":"10.3390/audiolres14020021","url":null,"abstract":"<p><strong>Objectives: </strong>The objectives were to present the real-time estimated values of cochlear implant (CI) electrode insertion speed (IS) during intraoperative sessions using the Cochlear Nucleus SmartNav System to assess whether this measure affected CI outcomes and to determine whether real-time feedback assists expert surgeons in achieving slow insertion.</p><p><strong>Methods: </strong>The IS was measured in 52 consecutive patients (65 implanted ears) using the CI632 electrode. The IS values were analyzed in relation to procedure repetition over time, NRT ratio, and CI audiological outcomes.</p><p><strong>Results: </strong>The average IS was 0.64 mm/s (SD = 0.24); minimum and maximum values were 0.23 and 1.24 mm/s, respectively. The IS significantly decreased with each array insertion by the operator (<i>p</i> = 0.006), and the mean decreased by 24% between the first and last third of procedures; however, this reduction fell within the error range of SmartNav for IS (+/-0.48 mm/s). No correlation was found between IS and the NRT ratio (<i>p</i> = 0.51), pure-tone audiometry (PTA) at CI activation (<i>p</i> = 0.506), and PTA (<i>p</i> = 0.94) or word recognition score (<i>p</i> = 0.231) at last evaluation.</p><p><strong>Conclusions: </strong>The estimated IS reported by SmartNav did not result in a clinically significant reduction in insertion speed or an improvement in CI hearing outcomes. Real-time feedback of IS could potentially be used for training, but its effectiveness requires confirmation through additional studies and more accurate tools. Implementation of IS assessment in clinical practice will enable comparisons between measurement techniques and between manual and robot-assisted insertions. This will help define the optimal IS range to achieve better cochlear implant (CI) outcomes.</p>","PeriodicalId":44133,"journal":{"name":"Audiology Research","volume":"14 2","pages":"227-238"},"PeriodicalIF":1.7,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10961689/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140207801","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Davide Brotto, Marco Greggio, Cosimo De Filippis, Patrizia Trevisi
{"title":"Autosomal Recessive Non-Syndromic Deafness: Is AAV Gene Therapy a Real Chance?","authors":"Davide Brotto, Marco Greggio, Cosimo De Filippis, Patrizia Trevisi","doi":"10.3390/audiolres14020022","DOIUrl":"10.3390/audiolres14020022","url":null,"abstract":"<p><p>The etiology of sensorineural hearing loss is heavily influenced by genetic mutations, with approximately 80% of cases attributed to genetic causes and only 20% to environmental factors. Over 100 non-syndromic deafness genes have been identified in humans thus far. In non-syndromic sensorineural hearing impairment, around 75-85% of cases follow an autosomal recessive inheritance pattern. In recent years, groundbreaking advancements in molecular gene therapy for inner-ear disorders have shown promising results. Experimental studies have demonstrated improvements in hearing following a single local injection of adeno-associated virus-derived vectors carrying an additional normal gene or using ribozymes to modify the genome. These pioneering approaches have opened new possibilities for potential therapeutic interventions. Following the PRISMA criteria, we summarized the AAV gene therapy experiments showing hearing improvement in the preclinical phases of development in different animal models of DFNB deafness and the AAV gene therapy programs currently in clinical phases targeting autosomal recessive non syndromic hearing loss. A total of 17 preclinical studies and 3 clinical studies were found and listed. Despite the hurdles, there have been significant breakthroughs in the path of HL gene therapy, holding great potential for providing patients with novel and effective treatment.</p>","PeriodicalId":44133,"journal":{"name":"Audiology Research","volume":"14 2","pages":"239-253"},"PeriodicalIF":1.7,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10961695/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140207798","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}