The Prognostic Value of Olfactory Dysfunction in Patients with COVID-19: The COVIDORA Study.

Background: Among all studies describing COVID-19 clinical features during the first wave of the pandemic, only a few retrospective studies have assessed the correlation between olfac-tory dysfunction (OD) and the evolution of disease severity. The main aim was to assess whether OD is a predictive factor of COVID-19 severity based on the patient's medical management (outpa-tient care, standard hospital admission, and ICU admission).

Methods: A national, prospective, mul-ticenter cohort study was conducted in 20 public hospitals and a public center for COVID-19 screen-ing.

Successful Burosumab Treatment in an Adult Patient with X-Linked Hypophosphatemia and Chronic Kidney Disease Stage 3b.

X-linked hypophosphatemic rickets (XLH) is a genetic cause of renal hypophosphatemia due to inactivation of the PHEX gene, with an inappropriate concentration of fibroblast growth factor 23 (FGF23). Burosumab, an anti-FGF23 monoclonal antibody, is a validated treatment for XLH, but its use in patients with chronic kidney disease (CKD) has not been validated. A 61-year-old man with XLH developed CKD during follow-up.

Performance of an active transcutaneous bone conduction implant, the Osia system, in high frequencies and hearing in noise in users converted from a Baha attract system: a prospective study.

Purpose: Hearing rehabilitation through middle ear surgery can be challenging, particularly for patients with chronic otitis media, but new hearing devices offer opportunity to reach this goal. The aim was to compare hearing outcomes and quality of life in patients who were converted from a Baha Attract system to the Osia system.

Methods: Prospective observational study, in which each subject acted as their own control.

Ear and upper airway clinical outcome measures for use in primary ciliary dyskinesia research: a scoping review.

Background: Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterised by pulmonary, otological and sino-nasal manifestations. Well-defined clinical outcome measures are needed in such rare diseases research to improve follow-up and treatments. Pulmonary outcome measures have recently been described.

Otological Manifestations in Adults with Primary Ciliary Dyskinesia: A Controlled Radio-Clinical Study.

Primary ciliary dyskinesia (PCD) is a rare genetical disease characterized by an abnormal structure or function of the cilia, causing sinusitis, otitis, and bronchiectasis. Hearing loss affects 60% of PCD patients, but data are lacking concerning hearing and temporal bone imaging in adults. Our aim was to describe clinical and radiological ear disease in adults with genetically confirmed PCD.

Olfactory function in congenital cytomegalovirus infection: a prospective study.

Congenital cytomegalovirus (CMV) infection leads to olfactory bulb lesions in the fetus, yet little is known about its impact on olfaction after birth. Here, we have assessed in a prospective study conducted on children in two French hospitals from 2016 to 2019, infection severity and olfactory performance after congenital CMV infection. Children with congenital CMV infection aged 3 to 10 years and healthy controls (CTL) matched for age and sex to CMV children symptomatic at birth (sCMV) were enrolled.

Magnetic resonance imaging is a valuable tool to evaluate the therapeutic efficacy of burosumab in children with X-linked hypophosphatemia.

Purpose: To examine the MRI diagnostic performance in the assessment of therapeutic response to burosumab in children with X-linked hypophosphatemia (XLH).

Design: Prospective longitudinal open cohort.

Patients: Seventeen children with XLH, average age of 10.

An antibody to RGMa promotes regeneration of cochlear synapses after noise exposure.

Auditory neuropathy is caused by the loss of afferent input to the brainstem via the components of the neural pathway comprising inner hair cells and the first order neurons of the spiral ganglion. Recent work has identified the synapse between cochlear primary afferent neurons and sensory hair cells as a particularly vulnerable component of this pathway. Loss of these synapses due to noise exposure or aging results in the pathology identified as hidden hearing loss, an initial stage of cochlear dysfunction that goes undetected in standard hearing tests.

Stability of the standard incus coupling of the Carina middle ear actuator after 1.5T MRI.

Limited data is available concerning the safety of active middle ear implants (AMEI) during Magnetic Resonance Imaging (MRI). Measurements in temporal bones are the gold standard for preclinical assessment of device safety. In this study the coupling stability of an actuator as used in a fully implantable AMEI was determined in temporal bones.

Transcutaneous Baha Attract system: Long-term outcomes of the French multicenter study.

Background: Bone conduction implants based on abutment-driven acoustic transmission result in good hearing outcomes; however, skin complications impact the quality of life (QOL) and possibly the viability of the device for many patients. The transcutaneous magnetic Baha Attract technology was developed with the goal of minimising skin complications.

Objectives: To analyse surgical, auditory and QOL outcomes for patients implanted with the Baha Attract.

[Adult deafness: Towards new paradigm].

Screening and early treatment of deafness regardless of age is essential. Deafness leads to social isolation, depression, and decreased cognitive function. The diagnosis requires an otoscopy and a confirmation of the type and degree of deafness by audiometry.

[Good practices with tinnitus in adult].

The interrogation is essential to trace the genesis of the tinnitus and to appreciate its repercussion. Clinical examination should look for a local, vascular or cervical cause. The ENT consultation with audiogram and tinnitus evaluation is essential to know the characteristics of the tinnitus and to consider the treatment.

[Medical devices correcting the deafness: Hearing aids and auditory implants].

The management of deafness has become a major public health issue as their lack of detection has a deleterious effect in children and increases the risk factors for aggravation of other pathologies in adults. The detection of deafness remains a real challenge: in the newborn, systematic screening at birth is a good strategy, in adults, much remains to be done. The functional rehabilitation of deafness is based on the use of hearing aids by aerial or bone conduction or of auditory implants.

[The infections of the ear].

In front of external otitis in spite of a well-conducted treatment, especially in immunodeficient patient, it is always necessary to look for an osteomyelitis of the skull base that requires an urgent parenteral antibiotic treatment of several weeks. Acute otitis media (AOM) is the most common bacterial infection of the child. In children under 2 years with purulent AOM, antibiotic therapy with amoxicilline is systematic for a period of 8-10 days.

[Genetic hearing loss].

Deafness is the most common sensory disability in developed countries affecting more than 2 births in 1000. Eighty percent of congenital deafness is genetic. Universal newborn hearing screening has been in place since 2012 in France.

Prenatal Diagnosis of a Melanotic Neuroectodermal Tumor of Infancy (MNTI): A Case Report With a Favorable Outcome After Chemotherapy Failure and Incomplete Resection.

We present the case of a woman referred to our department at 34 weeks of pregnancy with a fetal ultrasonographic scan showing a mass that had developed within the right maxilla with invasion of the orbit. A retrospective examination showed that this tumor had been present since the 12th week of pregnancy. At 39+4 weeks of gestation, a boy was born.