Speech Intelligibility in Noise With a Pinna Effect Imitating Cochlear Implant Processor.

OBJECTIVE To evaluate the speech intelligibility in noise with a new cochlear implant (CI) processor that uses a pinna effect imitating directional microphone system. STUDY DESIGN Prospective experimental study. SETTING Tertiary referral center. PATIENTS Ten experienced, unilateral CI recipients with bilateral severe-to-profound hearing loss. INTERVENTION All participants performed speech in noise tests with the Opus 2 processor (omnidirectional microphone mode only) and the newer Sonnet processor (omnidirectional and directional microphone mode). MAIN OUTCOME MEASURE The speech reception threshold (SRT) in noise was measured in four spatial settings. The test sentences were always presented from the front. The noise was arriving either from the front (S0N0), the ipsilateral side of the CI (S0NIL), the contralateral side of the CI (S0NCL), or the back (S0N180). RESULTS The directional mode improved the SRTs by 3.6 dB (p < 0.01), 2.2 dB (p < 0.01), and 1.3 dB (p < 0.05) in the S0N180, S0NIL, and S0NCL situations, when compared with the Sonnet in the omnidirectional mode. There was no statistically significant difference in the S0N0 situation. No differences between the Opus 2 and the Sonnet in the omnidirectional mode were observed. CONCLUSION Speech intelligibility with the Sonnet system was statistically different to speech recognition with the Opus 2 system suggesting that CI users might profit from the pinna effect imitating directionality mode in noisy environments.

Clinical manifestation of Fuchs uveitis syndrome in childhood

PURPOSE The aim of this study was to describe clinical signs and complications of Fuchs uveitis syndrome (FUS) with onset in childhood. METHODS Ophthalmologic findings and complications in patients with FUS becoming manifest before the age of 16 years were analyzed in a retrospective study at a tertiary referral uveitis center. Inclusion criteria were the presence of pathognomonic FUS findings at any time point and exclusion of any systemic immune-mediated or infectious disease. RESULTS A total of 23 patients (male = 16, female = 7) with juvenile FUS (unilateral n = 20, bilateral n = 3 patients) were included in the study. Mean ages at uveitis and FUS diagnosis were 12.0 ± 4.2 and 22.7 ± 10.7 years, respectively. In six patients, inflammation was noted at age ≤ 7 years. The following inflammatory signs were observed in a total of 26 eyes: ≤ 1+ anterior chamber cell grade (n = 26), vitreous cells (n = 24), fine keratic precipitates (KPs; n = 23), stellate KPs (n = 11), mutton-fat KPs (n = 23), diffuse (n = 24) or inferior (n = 8) distribution of KPs, Koeppe nodules (n = 10), and iris heterochromia (n = 14). A representative subgroup of patients (n = 5) is shown who presented with non-specific clinical signs in the beginning and in whom typical FUS signs became manifest only at a later stage. Secondary complications such as cataract (n = 19), ocular hypertension (n = 3), or glaucomatous disc damage (n = 2) were found after a mean uveitis duration of 11.6, 19.5, and 20.3 years, respectively. CONCLUSION FUS may begin in early childhood, and the characteristic findings may not be present at onset of disease. The diagnosis is often delayed for years, occasionally with the consequence of overtreatment with anti-inflammatory drugs.

Venous pattern of polymicrogyria detected by susceptibility weighted imaging (SWI).

We report a case of a 9-year-old boy presenting with spastic-dystonic movement disorder of the right arm. MRI showed vast unilateral left-sided polymicrogyria (PMG) with perisylvian, temporal, frontal, and parietal location. Corresponding to the distinctly reduced gyration, the focal pattern of cortical veins in susceptibility weighted imaging (SWI) was absent due to missing sulcal depth. In contrast, adjacent regions with sufficient sulcal depth revealed a pattern with numerically increased and finer cortical veins. Therefore, with its atypical venous pattern SWI indicates an abnormal parenchymal anatomy and might be an additional helpful tool for diagnosing PMG.