The human OPA1 mutation induces adult onset and progressive auditory neuropathy in mice.

Dominant optic atrophy (DOA) is one of the most prevalent forms of hereditary optic neuropathies and is mainly caused by heterozygous variants in OPA1, encoding a mitochondrial dynamin-related large GTPase. The clinical spectrum of DOA has been extended to a wide variety of syndromic presentations, called DOAplus, including deafness as the main secondary symptom associated to vision impairment. To date, the pathophysiological mechanisms underlying the deafness in DOA remain unknown.

Validation of a tablet-based application for hearing self-screening in an adult population.

Objective: This study evaluated the diagnostic performances of a tablet-based hearing screening test by assisted-test and self-test modes.

Design/method: Measurements were performed with the SoTone tests in normal hearing and hearing-impaired adult participants using an Android tablet and calibrated Bluetooth headphones. The duration of assisted- and self-test modes were compared.

Early management of presbycusis: recommendations from the French Society of Otorhinolaryngology and Head and Neck Surgery, the French Society of Audiology, and the French Society of Geriatrics and Gerontology.

Introduction: Presbycusis is the physiological decrease in hearing due to advancing age and begins well before the sixth decade. These recommendations recall the principles of early diagnosis of presbycusis and the means of optimal rehabilitation as soon as the first symptoms appear.

Material And Methods: The recommendations are based on a systematic analysis of the literature carried out by a multidisciplinary group of ENT physicians, audiologists, geriatricians and hearing specialists from all over France.

[Early management of presbycusis: recommendations from the French Society of Otorhinolaryngology and Head and Neck Surgery, the French Society of Audiology, and the French Society of Geriatrics and Gerontology].

Introduction: Presbycusis is the physiological decrease in hearing due to advancing age and begins well before the sixth decade. These recommendations recall the principles of early diagnosis of presbycusis and the means of optimal rehabilitation as soon as the first symptoms appear.

Material And Methods: The recommendations are based on a systematic analysis of the literature carried out by a multidisciplinary group of doctors and audioprosthetists from all over France.

A disease-associated mutation in thyroid hormone receptor α1 causes hearing loss and sensory hair cell patterning defects in mice.

Resistance to thyroid hormone due to mutations in , which encodes the thyroid hormone receptor α (TRα1), shows variable clinical presentation. Mutations affecting TRβ1 and TRβ2 cause deafness in mice and have been associated with deafness in humans. To test whether TRα1 also affects hearing function, we used mice heterozygous for a frameshift mutation in that is similar to human mutations ( mice) and reduces tissue sensitivity to thyroid hormone.

Impulse Noise Induced Hidden Hearing Loss, Hair Cell Ciliary Changes and Oxidative Stress in Mice.

Recent studies demonstrated that reversible continuous noise exposure may induce a temporary threshold shift (TTS) with a permanent degeneration of auditory nerve fibers, although hair cells remain intact. To probe the impact of TTS-inducing impulse noise exposure on hearing, CBA/J Mice were exposed to noise impulses with peak pressures of 145 dB SPL. We found that 30 min after exposure, the noise caused a mean elevation of ABR thresholds of ~30 dB and a reduction in DPOAE amplitude.

French Version of the Antiphasic Digits-in-Noise Test for Smartphone Hearing Screening.

In France 58% of persons with hearing loss still do not wear hearing aids. Pure-tone audiometry is the traditional gold standard in assessment and screening of hearing impairment, but it requires the use of calibrated devices and soundproof booth. The antiphasic digits-in-noise (DIN) test does not require calibrated material and can run on a standard headset or earbuds connected to a smartphone or a computer.

Exacerbated age-related hearing loss in mice lacking the p43 mitochondrial T3 receptor.

Background: Age-related hearing loss (ARHL), also known as presbycusis, is the most common sensory impairment seen in elderly people. However, the cochlear aging process does not affect people uniformly, suggesting that both genetic and environmental (e.g.

Speech perception, real-ear measurements and self-perceived hearing impairment after remote and face-to-face programming of hearing aids: A randomized single-blind agreement study.

Introduction: Current literature does not provide strong evidence that remote programming of hearing aids is effective, despite its increasing use by audiologists. We tested speech perception outcomes, real-ear insertion gain, and changes in self-perceived hearing impairment after face-to-face and remote programming of hearing aids in a randomized multicentre, single-blind crossover study.

Methods: Adult experienced hearing aid users were enrolled during routine follow-up visits to audiology clinics.

ROS-Induced Activation of DNA Damage Responses Drives Senescence-Like State in Postmitotic Cochlear Cells: Implication for Hearing Preservation.

In our aging society, age-related hearing loss (ARHL) has become a major socioeconomic issue. Reactive oxygen species (ROS) may be one of the main causal factors of age-related cochlear cell degeneration. We examined whether ROS-induced DNA damage response drives cochlear cell senescence and contributes to ARHL from the cellular up to the system level.

Spiking Pattern of the Mouse Developing Inner Hair Cells Is Mostly Invariant Along the Tonotopic Axis.

During development, the sensory cells of the cochlea, the inner hair cells (IHCs), fire spontaneous calcium action potentials. This activity at the pre-hearing stage allows the IHCs to autonomously excite the auditory nerve fibers and hence, represents an efficient mechanism to shape the tonotopic organization along the ascending auditory pathway. Using calcium imaging, we show that the activity in the developing cochlea consists of calcium waves that propagate across the supporting and sensory cells.

Metachronal propagation of motor activity.

A diverse array of biomechanical systems has evolved to satisfy locomotor requirements (reptation, swimming, walking, etc.) and in all cases, successful behabior achievement requires the integrated functioning of various segments, to ensure the appropriate positioning of the different body regions. From comparative studies on a variety of invertebrate and vertebrate organisms, it is now established that the basic motor patterns underlying limb and/or trunk movements during locomotion are driven by central networks of neurons, so-called central pattern generators (CPGs).

Comparison of trunk activity during gait initiation and walking in humans.

To understand the role of trunk muscles in maintenance of dynamic postural equilibrium we investigate trunk movements during gait initiation and walking, performing trunk kinematics analysis, Erector spinae muscle (ES) recordings and dynamic analysis. ES muscle expressed a metachronal descending pattern of activity during walking and gait initiation. In the frontal and horizontal planes, lateroflexion and rotation occur before in the upper trunk and after in the lower trunk.

Real time control of a CPG-based model of the human trunk in different walking conditions.

Artificial central pattern generators (CPGs) framework is well adapted to the control of bio-mimetic systems during rhythmic tasks like locomotion. They have the ability to reproduce biological behavior as well as to be used as feedforward controllers for multi-articulated systems. In this paper we present a model of human gait activity based on an oscillator network.