Congenital Deafness and Recent Advances Towards Restoring Hearing Loss

Renauld, Justine; Basch, Martín L.

doi:10.1002/cpz1.76

Cited by 22 publications

(20 citation statements)

References 383 publications

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“…The global threat to human health posed by pathogenic flaviviruses is likely to continue for the foreseeable future [46], necessitating further progress in understanding virusinduced pathologies. ZIKV now joins several pathogens whose exposure to the gestating fetus correlates with congenital hearing loss after birth [47,48]. Knowing whether infection of the peripheral auditory system in utero could underlie some aspects of the ensuing hearing loss is necessary for choosing appropriate therapeutic treatments.…”

Section: Discussionmentioning

confidence: 99%

Embryonic and Neonatal Mouse Cochleae Are Susceptible to Zika Virus Infection

Munnamalai

Sammudin

Young

et al. 2021

Viruses

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Congenital Zika Syndrome (CZS) is caused by vertical transmission of Zika virus (ZIKV) to the gestating human fetus. A subset of CZS microcephalic infants present with reduced otoacoustic emissions; this test screens for hearing loss originating in the cochlea. This observation leads to the question of whether mammalian cochlear tissues are susceptible to infection by ZIKV during development. To address this question using a mouse model, the sensory cochlea was explanted at proliferative, newly post-mitotic or maturing stages. ZIKV was added for the first 24 h and organs cultured for up to 6 days to allow for cell differentiation. Results showed that ZIKV can robustly infect proliferating sensory progenitors, as well as post-mitotic hair cells and supporting cells. Virus neutralization using ZIKV-117 antibody blocked cochlear infection. AXL is a cell surface molecule known to enhance the attachment of flavivirus to host cells. While Axl mRNA is widely expressed in embryonic cochlear tissues susceptible to ZIKV infection, it is selectively downregulated in the post-mitotic sensory organ by E15.5, even though these cells remain infectible. These findings may offer insights into which target cells could potentially contribute to hearing loss resulting from fetal exposure to ZIKV in humans.

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Section: Discussionmentioning

confidence: 99%

Embryonic and Neonatal Mouse Cochleae Are Susceptible to Zika Virus Infection

Munnamalai

Sammudin

Young

et al. 2021

Viruses

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“…Several protocols have been devised to direct hPSCs into inner ear HCs and neuron-like cells. The efficiency, reproducibility, and scalability of these protocols are enhanced by incorporating knowledge of inner ear development [13,17,18,[67][68][69][70]. Early studies on the transplantation of hPSCs-derived otic progenitors have been successful in certain animal models [103], but the hearing was transiently restored, and long-term cell survival continues to be a major challenge.…”

Section: Modeling Inner Ear Disorders: 2d and 3d Cell Culturementioning

confidence: 99%

“…The major achievements in disease modeling using a hiPSC-derived inner ear for genetic SNHL include the genes MYO7A, MYO15, and MERRF in HCs [70,89,90], and GJB2 and SLC26A4 (Pendred syndrome) in SCs [106,[123][124][125] (Table 2). These studies have defined the molecular mechanisms involving each gene and showed the cellular effects of each mutation.…”

Section: Clinical Trials In Inner Ear Disordersmentioning

confidence: 99%

Application of Human Stem Cells to Model Genetic Sensorineural Hearing Loss and Meniere Disease

Lamolda¹,

Frejo²,

Gallego‐Martinez³

et al. 2023

Preprint

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Genetic sensorineural hearing loss and Meniere disease have been associated with rare variations in the coding and non-coding region of the human genome. Most of these variants are classified as likely pathogenic or variants of unknown significance and require functional validation in cellular or animal models. Given the difficulties to obtain human samples and the raising concerns about animal experimentation, human induced pluripotent stem cells emerge as cellular models to investigate the interaction of genetic and environmental factors in the pathogenesis of inner ear disorders. The generation of human sensory epithelia and neuron-like cells carrying the variants of interest may facilitate a better understanding of their role during differentiation. These cellular models will allow us to explore new strategies for restoring hearing and vestibular sensory epithelia as well as neurons. This review summarizes the use of human induced pluripotent stem cells in sensorineural hearing loss and Meniere disease and proposes some strategies for its application in clinical practice.

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“…3 In the United States, in the year 2021 the disorder was found in 2-3 cases per 1000 live births. 4 The proportion of congenital deafness in children aged 24-59 months in Indonesia based on a survey in the year 2019 was 0.11%. 5 Appropriate management of patients with congenital deafness by establishing an early diagnosis and early intervention is very important to reduce the medical and social burden on patients, families, and communities, and also to improve patients' quality of life.…”

Section: Introductionmentioning

confidence: 99%

Financing hearing aids for patients with congenital deafness in Indonesia

Zachreini

Bashiruddin

Zizlavsky

et al. 2022

Oto Rhino Laryn Indones

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Background: The appropriate management of patients with congenital deafness is installing hearing aids, either external hearing aids or implanted in the ear (cochlear implant), aiming to reduce the medical and social burden, besides improving the quality of life of the sufferers. Objective: To ascertain the cost of hearing aids in patients with congenital deafness, in the form of external hearing aids or cochlear implants. Method: A descriptive study with cross-sectional design using questionnaires through interviews. The sample size was 535 mothers whose children had congenital deafness at 24 hospitals with facilities for establishing a diagnosis of congenital deafness in 17 provinces in Indonesia. Result: Most respondents were aged 30-39 years (55%), occupations were housewives (71.8%), and education level was high school (52.5%). The type of hearing aid used mostly was external (92.7%), with 45.9% paid by personal expense. The surgically planted hearing aids in 22 children was mostly cochlear implants (95.5%), which were financed by the Indonesian Healthcare and Social Security Agency (BPJS) plus personal costs (50%). Discussion: This study found that the most common type of hearing aid used by children with hearing impairments was external hearing aids (92.7%) through independent financing (45.9%). Only 7.3% of patients chose surgery in hearing habilitation, and 95.5% were cochlear implants. The small percentage of surgery were due to the high-priced of cochlear implants, and the government did not cover all financial expenses. Conclusion: Most external hearing aids were paid independently-out-of-pocket, while cochlear implant surgeries were funded by BPJS, plus extra costs independently. ABSTRAKLatar belakang: Penatalaksanaan terbaik untuk penderita tuli kongenital adalah pemasangan alat bantu dengar (ABD), baik berupa ABD eksternal maupun ABD yang ditanam dalam telinga (implan koklea), dengan tujuan untuk mengurangi beban medis dan sosial, serta meningkatkan kualitas hidup penderita. Tujuan: Untuk mengetahui seberapa besar biaya pemasangan ABD pada penderita tuli kongenital, baik berupa ABD eksternal maupun implan koklea. Metode: Penelitian deskriptif dengan rancangan cross sectional study menggunakan kuesioner melalui wawancara. Besar sampel 535 ibu yang anaknya menderita tuli kongenital pada 24 rumah sakit yang memiliki fasilitas penegakkan diagnosis tuli kongenital di 17 provinsi di Indonesia. Hasil: Sebagian besar responden berusia 30-39 tahun (55%), pekerjaan terbanyak adalah ibu rumah tangga (71.8%), dan tingkat pendidikan SMA (52.5%). Jenis ABD yang terbanyak adalah ABD eksternal (92,7%) dengan pembiayaan secara mandiri 45,9%. Pemasangan ABD dengan tindakan operasi dilakukan pada 22 anak, yang terbanyak adalah implan koklea (95,5%) yang dibiayai oleh Badan Penyelenggara Jaminan Sosial (BPJS) ditambah dengan biaya sendiri (50%). Diskusi: Penelitian ini mendapati bahwa ABD yang terbanyak digunakan oleh anak dengan gangguan pendengaran adalah ABD eksternal (92,7%) dengan biaya mandiri (45,9%). Habilitasi pendengaran dengan tindakan operasi hanya dilakukan pada 7,3% pasien, berupa implantasi koklea 95,5%. Kecilnya persentase habilitasi bedah dikarenakan tingginya harga implant koklea, dan bantuan dari BPJS tidak meliputi keseluruhan biaya. Kesimpulan: Sebagian besar pembiayaan alat bantu dengar eksternal secara mandiri, sedangkan operasi implan koklea menggunakan biaya BPJS ditambah biaya sendiri.

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Congenital Deafness and Recent Advances Towards Restoring Hearing Loss

Cited by 22 publications

References 383 publications

Embryonic and Neonatal Mouse Cochleae Are Susceptible to Zika Virus Infection

Embryonic and Neonatal Mouse Cochleae Are Susceptible to Zika Virus Infection

Application of Human Stem Cells to Model Genetic Sensorineural Hearing Loss and Meniere Disease

Financing hearing aids for patients with congenital deafness in Indonesia

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