Speech perception measures used with cochlear implant candidates and recipients should reflect the listening challenges that individuals encounter in natural communication situations. These data provide the basis for recommending new candidacy criteria based on speech recognition tests presented at 60 and/or 50 dB SPL, intensity levels that reflect real-life listening, rather than 70 dB SPL.

We report that mutation of COL11A2 causes deafness previously mapped to the DFNA13 locus on chromosome 6p. We found two families (one American and one Dutch) with autosomal dominant, non-syndromic hearing loss to have mutations in COL11A2 that are predicted to affect the triple-helix domain of the collagen protein. In both families, deafness is non-progressive and predominantly affects middle frequencies. Mice with a targeted disruption of Col11a2 also were shown to have hearing loss. Electron microscopy of the tectorial membrane of these mice revealed loss of organization of the collagen fibrils. Our findings revealed a unique ultrastructural malformation of inner-ear architecture associated with non-syndromic hearing loss, and suggest that tectorial membrane abnormalities may be one aetiology of sensorineural hearing loss primarily affecting the mid-frequencies.

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Elderly patients benefit significantly from cochlear implantation. Compared with a younger cohort matched for preimplantation performance, however, their postimplantation scores are significantly lower on some measures. These results may provide guidelines for candidacy and counseling regarding elderly patients with cochlear implants.

Recent advances in cochlear implant technology have focused renewed attention on the preservation of residual hearing. The focus on preservation of residual hearing is driven by the concept of electroacoustic stimulation. This option depends on the insertion of a short cochlear implant electrode into the basal region of the cochlea while preserving native function in the apical region. The desire to preserve residual hearing has led to the development of the soft-surgery cochlear implantation technique. Here, the authors evaluate its various components. Avoiding entry of blood into the cochlea and the use of hyaluronate seem to be reasonably supported, whereas the use of topical steroids is unlikely to be beneficial. The site of entry into the cochlea, the use of contoured or straight devices, and the depth of insertion are also evaluated. The authors highlight the importance of systematic recording of outcomes and surgical events.

Objective:To demonstrate the safety and effectiveness of the MED-EL Electric-Acoustic Stimulation (EAS) System, for adults with residual low-frequency hearing and severe-to-profound hearing loss in the mid to high frequencies.Study Design:Prospective, repeated measures.Setting:Multicenter, hospital.Patients:Seventy-three subjects implanted with PULSAR or SONATA cochlear implants with FLEX24 electrode arrays.Intervention:Subjects were fit postoperatively with an audio processor, combining electric stimulation and acoustic amplification.Main Outcome Measures:Unaided thresholds were measured preoperatively and at 3, 6, and 12 months postactivation. Speech perception was assessed at these intervals using City University of New York sentences in noise and consonant–nucleus–consonant words in quiet. Subjective benefit was assessed at these intervals via the Abbreviated Profile of Hearing Aid Benefit and Hearing Device Satisfaction Scale questionnaires.Results:Sixty-seven of 73 subjects (92%) completed outcome measures for all study intervals. Of those 67 subjects, 79% experienced less than a 30 dB HL low-frequency pure-tone average (250–1000 Hz) shift, and 97% were able to use the acoustic unit at 12 months postactivation. In the EAS condition, 94% of subjects performed similarly to or better than their preoperative performance on City University of New York sentences in noise at 12 months postactivation, with 85% demonstrating improvement. Ninety-seven percent of subjects performed similarly or better on consonant–nucleus–consonant words in quiet, with 84% demonstrating improvement.Conclusion:The MED-EL EAS System is a safe and effective treatment option for adults with normal hearing to moderate sensorineural hearing loss in the low frequencies and severe-to-profound sensorineural hearing loss in the high frequencies who do not benefit from traditional amplification.

Both older and younger patients can receive significant improvement in speech recognition and health utility after receiving a CI.

Objectives:To compare the hearing performance of patients with conductive and mild mixed hearing loss and single-sided sensorineural deafness provided with a new transcutaneous bone conduction hearing implant (the Baha Attract System) with unaided hearing as well as aided with a sound processor on a softband. Furthermore, to evaluate safety and subjective benefit before and after implantation of the test device.Participants: Fifty-four adult patients in five participating centres were enrolled in this prospective study. Baseline data were collected during a pre-operative visit, and after a softband trial, all patients were implanted unilaterally. Follow-up visits were scheduled at 10 days, 4, 6, 12 weeks and 6 months. Main outcome measures.Free-field hearing thresholds pure-tone average (PTA4 in dB HL; mean threshold at 500, 1000, 2000, 4000 Hz; primary outcome measure). Individual free-field hearing thresholds, speech recognition in quiet and in noise, soft tissue status during followup and subjective benefit as measured with the Abbreviated Profile of Hearing Aid Benefit (APHAB), Speech, Spatial and Qualities of Hearing Scale (SSQ) and Health Utilities Index (HUI) questionnaires.Results: Implantation of the Baha Attract System resulted in favourable audiological outcomes compared to unaided conditions. On the primary outcome parameter, a statistically significant improvement was observed compared to unaided hearing for the patients with conductive/mixed hearing loss (mean PTA4 difference −20.8 dB HL, SD 9.8; P < 0.0001) and for the patients with single-sided sensorineural deafness (SSD) (mean PTA4 difference −21.6 dB HL, SD 12.2; P < 0.0001). During all audiology tests, the non-test ear was blocked. Statistically significant improvements were also recorded in speech tests in quiet and noise compared to unaided hearing for the conductive/mixed hearing loss group and for speech in quiet in the SSD group. Compared to the pre-operative measurement with softband, no significant differences were recorded in the PTA4 free-field hearing threshold or the other audiological outcomes in either of the groups (P > 0.05). Soft tissue-related issues observed during follow-up included numbness, pain/discomfort at the implant site and to a lesser extent pressure-related skin complications. A declining trend was