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.

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.

Background: DFNA9 is a form of autosomal progressive sensorineural hearing loss, caused by more than 30 variants in the COCH gene. p.Pro51Ser (p.P51S) variant is characterized by late-onset functional deterioration toward bilateral severe hearing loss and vestibulopathy. Focal sclerosis on computed tomography (CT) and T2-weighted magnetic resonance imaging (MRI) signal loss of semicircular canals are presumably radiologic biomarkers of advanced otovestibular deterioration. Objectives: The aim of this study was to investigate whether these biomarkers are more frequent in cochlear implant candidates carrying the p.P51S mutation versus noncarriers. Second, the correlation between the hearing and vestibular function and carrier status was studied. Finally, the relationship between the presence of these radiologic features and the degree of hearing and vestibular deterioration was investigated. Methods: A prospective cohort study was performed on 38 candidates for cochlear implantation in a tertiary referral center. Patients underwent pure tone audiometry, videonystagmography, video head impulse tests and vestibular-evoked myogenic potentials. In addition, three dizziness questionnaires were used. All subjects were administered CT, MRI, and molecular genetic analysis. Results: Sixteen of 38 patients were carriers of the p.P51S COCH mutation. Radiologic lesions were almost exclusively observed in carriers. MRI was more sensitive in showing lesions than CT. Furthermore, p.P51S carriers showed significantly lower function on most vestibular tests, including questionnaires, than noncarriers. Patients with imaging abnormalities showed more pronounced vestibulopathy. Conclusion:The present study supplements previous data that endorse the hypothesis that focal sclerosis of semicircular canals are biomarkers of advanced vestibular deterioration, especially in DFNA9.