A5055649987- Hearing, Cochlea, Tinnitus, Genetics
- Ear Surgery and Otitis Media
- Meningioma and schwannoma management
- Facial Nerve Paralysis Treatment and Research
- Hearing Loss and Rehabilitation
- Neurofibromatosis and Schwannoma Cases
- Vestibular and auditory disorders
- RNA regulation and disease
- Head and Neck Surgical Oncology
- Vascular Malformations Diagnosis and Treatment
- Noise Effects and Management
- Ear and Head Tumors
- Reconstructive Facial Surgery Techniques
- Voice and Speech Disorders
- Virus-based gene therapy research
- Tumors and Oncological Cases
- Teratomas and Epidermoid Cysts
- Sinusitis and nasal conditions
- Hearing Impairment and Communication
- Connexins and lens biology
- Language Development and Disorders
- Data Analysis with R
- CRISPR and Genetic Engineering
- Trigeminal Neuralgia and Treatments
- Osteomyelitis and Bone Disorders Research
Sorbonne Université
2016-2025
Assistance Publique – Hôpitaux de Paris
2015-2025
Inserm
2016-2025
Pitié-Salpêtrière Hospital
2016-2025
Institut Pasteur
2017-2025
Université Paris Cité
2022-2025
Centre National de la Recherche Scientifique
2025
Sorbonne Paris Cité
2019-2024
Institut de Génomique Fonctionnelle
2023
Université Sorbonne Nouvelle
2020-2022
Significance In humans, inner ear development is completed in utero, with hearing onset at ∼20 weeks of gestation. However, genetic forms congenital deafness are typically diagnosed during the neonatal period. Gene therapy approaches animal models should therefore be tested after period onset, to determine whether they can reverse an existing phenotype. Here, we used a mouse model DFNB9, human form accounting for 2–8% all cases deafness. We show that local gene mutant mice not only prevents...
Significance Hearing and balance impairments are major concerns a serious burden for public health, but still lack an effective curative therapy. We assessed inner ear functions in mouse model of Usher syndrome type 1, developmental disorder characterized by profound congenital deafness deficit, after local gene Viral transfer the wild-type cDNA to mutant mice shortly birth resulted partial restoration hearing long-lasting, almost complete, removal defect. The present results provide basis...
Objective: To describe the first cochlear array insertions using a robot-assisted technique, with different types of straight or precurved electrode arrays, compared arrays manually inserted into cochlea. Study Design: Retrospective review. Setting: Tertiary otologic center. Patients: Twenty implantations in group and 40 group. Interventions: Cochlear technique (RobOtol) (eight CI522/622, eight Advanced Bionics Hifocus Slim J) (four Mid-Scala) matched to manual implantations....
<h3>Importance</h3> Although hearing loss is common in the population worldwide, prevalence of and aid use not known. <h3>Objective</h3> To estimate adult French population. <h3>Design, Setting, Participants</h3> This cohort study used data from CONSTANCES cohort, a representative sample Volunteers aged 18 to 75 years were recruited at 21 preventive health centers between January 1, 2012, December 31, 2019. The present included participants with audiometric data. <h3>Main Outcomes...
Hearing depends on fast and sustained calcium-dependent synaptic vesicle fusion at the ribbon synapses of cochlear inner hair cells (IHCs). The implication canonical neuronal SNARE complex in this exocytotic process has so far remained controversial. We investigated role SNAP-25, a key component complex, hearing, by generating analyzing conditional knockout mouse model allowing targeted postnatal deletion Snap-25 IHCs. Mice subjected to IHC inactivation after hearing onset developed severe...
Neonatal gene therapy has been shown to prevent inner ear dysfunction in mouse models of Usher syndrome type I (USH1), the most common genetic cause combined deafness-blindness and vestibular dysfunction. However, hearing onset occurs after birth mice utero humans, making it questionable how transpose murine outcomes clinical settings. Here, we sought extend therapeutic time window a model for USH1G periods corresponding human neonatal stages, more suitable intervention patients. Mice with...
To evaluate long-term hearing outcomes following cochlear implantation in patients with neurofibromatosis type 2 and ipsilateral vestibular schwannoma.
Cerebrospinal fluid leak can occur after retrosigmoid craniectomy and lead to substantial patient morbidity. The aim of this study was compare two closure techniques for vestibular schwannoma resection in terms cerebrospinal other procedure-related issues. This retrospective monocentric included patients who underwent surgery via a approach by the same oto-neurosurgical team. Before 2019, consisted involved autologous abdominal fat graft obliteration (previous procedure). After authors...
Abstract Riboflavin transporter deficiency (RTD) was recently characterized as a cause of genetic recessive childhood‐onset motor neuron disease (MND) with hearing loss, formerly described Brown‐Vialetto‐Van‐Lear syndrome. We describe 18‐year‐old woman probable RTD mimicking juvenile Amyotrophic Lateral Sclerosis (ALS) who presented an inaugural respiratory failure and moderate distal four limbs weakness. Only one heterozygous SLC52A3 mutation detected, but presence sub‐clinical auditory...
Cochlear implantation is usually not recommended for prelingual profoundly deaf adults, although some of these patients might benefit from it. This study aims to define the candidates cochlear in this population. retrospective reviewed 34 who had received a implant at 32 ± 1.7 years old (16−55), with least 1 year follow-up. Speech perception and quality life were assessed before 3, 6, 12 months after implantation, then every thereafter. According word speech intelligibility quiet (WSI) two...
In vivo gene delivery to tissues using adeno-associated vector (AAVs) has revolutionized the field of therapy. Yet, while sensorineural hearing loss is one most common sensory disorders worldwide, therapy applied human inner ear still in its infancy. Recent advances development recombinant AAVs have significantly improved their cell tropism and transduction efficiency across diverse types a level that renders this tool valuable for conditionally manipulating expression context developmental...
Titanium ossicular chain replacement prosthesis is often used for rehabilitation of the columellar effect in otologic surgeries. This retro- spective study aims to analyse anatomical and functional results surgeries which a titanium was used. Two hundred eighty procedures 256 patients operated on tertiary referral center were analysed. Aetiologies, preoperative audiograms, peropera- tive data postoperative outcomes at 2 12 months postoperatively reviewed. Chronic suppurative otitis media...
Facial nerve tumors are rare lesions mostly located in the geniculate ganglion. This study aims to compare those limited ganglion terms of clinical features and postoperative outcomes.Case series with chart review.University tertiary reference center.Medical charts were reviewed for 17 patients who had surgery tumor removal (10 hemangiomas, 6 schwannomas, 1 meningioma). Hemangiomas schwannomas compared preoperative facial function, hearing, size, outcomes.Facial palsy was observed all cases....
To evaluate the influence of diameter stapes prosthesis on functional outcomes in surgery.Prospective cohort study.Fifty consecutive small fenestra stapedotomies performed using a 0.4-mm-diameter were compared with 50 carried out 0.6-mm-diameter piston. Audiological assessment following recommendations Committee Hearing and Equilibrium was 1 month after surgery. Postoperative complications between two groups noted.There no statistically significant differences demographic data groups,...