Erdheim-Chester Disease Revealed by Central Positional Nystagmus: A Case Report.

Erdheim-Chester disease (ECD) is a rare histiocytic disorder, recently recognized to be neoplastic. The clinical phenotype of the disease is extremely heterogeneous, and depends on the affected organs, with the most frequently reported manifestations being bone pain, diabetes insipidus and neurological disorders including ataxia. In this article, we report on a case of a 48-year-old woman, whose initial symptom of gait instability was isolated.

Objective, audiological and quality of life measures with the CI532 slim modiolar electrode.

Objectives: To report on electrode array measurements for the Nucleus CI532 Slim Modiolar Electrode device including: ECAP thresholds, electrode impedances, and psychophysical comfort levels, as well as speech perception results pre- and post-operatively and standardized evaluations of quality of life.

Methods: Forty-four subjects were implanted with the CI532. Electrically evoked compound action potential (ECAP) thresholds and impedances were measured using automatic Neural Response Telemetry intra-operatively, at activation and at six months post implant.

Early Sentence Recognition in Adult Cochlear Implant Users.

Objective: Normal-hearing subjects listening to acoustic simulations of cochlear implants (CI) can obtain sentence recognition scores near 100% in quiet and in 10 dB signal-to-noise ratio (SNR) noise with acute exposure. However, average sentence recognition scores for real CI listeners are generally lower, even after months of experience, and there is a high degree of heterogeneity. Our aim was to identify the relative importance and strength of factors that prevent CI listeners from achieving early, 1-mo scores as high as those for normal-hearing-listener acoustic simulations.

Clinical investigation of the Nucleus Slim Modiolar Electrode.

Aims: The Nucleus CI532 cochlear implant incorporates a new precurved electrode array, i.e., the Slim Modiolar electrode (SME), which is designed to bring electrode contacts close to the medial wall of the cochlea while avoiding trauma due to scalar dislocation or contact with the lateral wall during insertion.