Speech understanding in noise with the Roger Pen, Naida CI Q70 processor, and integrated Roger 17 receiver in a multi-talker network.

Roger is a digital adaptive multi-channel remote microphone technology that wirelessly transmits a speaker's voice directly to a hearing instrument or cochlear implant sound processor. Frequency hopping between channels, in combination with repeated broadcast, avoids interference issues that have limited earlier generation FM systems. This study evaluated the benefit of the Roger Pen transmitter microphone in a multiple talker network (MTN) for cochlear implant users in a simulated noisy conversation setting. Twelve post-lingually deafened adult Advanced Bionics CII/HiRes 90K recipients were recruited. Subjects used a Naida CI Q70 processor with integrated Roger 17 receiver. The test environment simulated four people having a meal in a noisy restaurant, one the CI user (listener), and three companions (talkers) talking non-simultaneously in a diffuse field of multi-talker babble. Speech reception thresholds (SRTs) were determined without the Roger Pen, with one Roger Pen, and with three Roger Pens in an MTN. Using three Roger Pens in an MTN improved the SRT by 14.8 dB over using no Roger Pen, and by 13.1 dB over using a single Roger Pen (p < 0.0001). The Roger Pen in an MTN provided statistically and clinically significant improvement in speech perception in noise for Advanced Bionics cochlear implant recipients. The integrated Roger 17 receiver made it easy for users of the Naida CI Q70 processor to take advantage of the Roger system. The listening advantage and ease of use should encourage more clinicians to recommend and fit Roger in adult cochlear implant patients.

Focal sclerosis of semicircular canals with severe DFNA9 hearing impairment caused by a P51S COCH-mutation: is there a link?

HYPOTHESIS: Focal sclerosis of one or more semicircular canals on computed tomographic (CT) scans and a corresponding signal loss on magnetic resonance (MR) imaging are radiologic lesions that are linked to patients who are suffering from advanced otovestibular impairment caused by hereditary DFNA9 hearing loss. BACKGROUND: DFNA9 is a hereditary hearing loss that is characterized by late-onset progressive imbalance and hearing deterioration, caused by mutations in the COCH gene. To date, no radiologic lesions have been associated with this condition. STUDY DESIGN: A retrospective chart review SETTING: Tertiary referral center SUBJECTS: The radiologic data of 9 patients who presented between 2007 and 2012 with otovestibular deterioration caused by a mutation in the COCH gene were reviewed. RESULTS: All 9 subjects were carriers of the same c.151C > T, p.Pro51Ser (P51S) - missense mutation in the COCH gene. In 8 of them similar sclerotic lesions and/or narrowing were demonstrated in one or more semicircular canals on computed tomography CT scan, with a signal loss at corresponding areas on T2-weighted magnetic resonance (MR) images. In 1 patient, the posterior part of the vestibule was also affected. The posterior canals were affected in most cases (58%), compared with the superior (21%) and lateral canals (16%) or the vestibule (5%). Only 68.4% of the lesions on MR images were also visible on CT scans, suggesting a fibrotic process without calcification. Ears presenting radiologic lesions showed significantly more severe hearing loss (median PTA 104 dB HL) compared with unaffected ears (58 dB HL). CONCLUSION: Eight of 9 subjects with the same P51S mutation in the COCH gene showed similar radiologic lesions, affecting the PSCC in the majority of the cases. These radiologic abnormalities occurred in more advanced stages of the otovestibular deterioration, supporting the hypothesis that these lesions might represent the end phase of a low-grade chronic inflammation or protein deposition. A new phenotypic and characteristic radiologic feature of DFNA9 has been discovered.