Three cases of adult-onset Brown-Vialetto-Van Laere syndrome: Novel variants in SLC52A3 gene and MRI abnormalities.

Brown-Vialetto-Van Laere syndrome is a rare, autosomal, recessive neurological condition caused by variants in the riboflavin transporter genes SLC52A2 and SLC52A3. Here, we report on three cases. Case 1 was a 35-year-old woman from a consanguineous family who presented with progressive deafness, subacute multiple cranial nerve impairments (III, VII, IX, XII), and MRI abnormalities (including as hypersignal from the cranial nerves).

Adult Users of the Oticon Medical Neuro Cochlear Implant System Benefit from Beamforming in the High Frequencies.

The Oticon Medical Neuro cochlear implant system includes the modes Opti Omni and Speech Omni, the latter providing beamforming (i.e., directional selectivity) in the high frequencies.

The French Cochlear Implant Registry (EPIIC): General indicators.

Cochlear and brainstem implants have been included on the list of reimbursable products (LPPR) in France since March of 2009. The implants were initially inscribed for 5 years, after which an application for renewal with the French National Commission for the Evaluation of Medical Devices and Health Technologies (Commission Nationale d'évaluation des dispositifs médicaux et des technologies de santé - CNEDiMTS) was required [Haute Autorité de santé, 2009]. Upon registration to the list of reimbursable products, the companies and the reference centers for cochlear and brainstem implants were asked to set up a post-registration registry called EPIIC.

Azimuthal sound source localization of various sound stimuli under different conditions.

Aim: To evaluate azimuthal sound-source localization performance under different conditions, with a view to optimizing a routine sound localization protocol.

Material And Method: Two groups of healthy, normal-hearing subjects were tested identically, except that one had to keep their head still while the other was allowed to turn it. Sound localization was tested without and then with a right ear plug (acute auditory asymmetry) for each of the following sound stimuli: pulsed narrow-band centered on 250Hz, continuous narrowband centered on 2000Hz, 4000Hz and 8000Hz, continuous 4000Hz warble, pulsed white noise, and word ("lac" (lake)).

Long-term evaluation of sound localisation in single-sided deaf adults fitted with a BAHA device.

Objective: To perform a long-term evaluation of the localisation capabilities in the horizontal plane of single-sided deaf patients fitted with a BAHA device.

Design: Single-centre retrospective study.

Participants: Twenty-one adults with single-sided deafness (SSD) with normal hearing in the contralateral ear (pure tone average <20 dB, SDS > 90%) rehabilitated with a Cochlear BAHA device from 2003 to 2012 on the deaf side over a median follow-up of 8 years.