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.

Objective To assess the outcome of cochlear implantation in children with Autism Spectrum Disorder Study Design Retrospective case review and survey Setting Tertiary referral center Patients Children who meet criteria for cochlear implantation and diagnosis of Autism Spectrum Disorder Main Outcome Measures Receptive and expressive language scores and parental survey data. Results 15 patients with history of ASD and cochlear implantation were analyzed and compared to 15 patients who received cochlear implant and have no other disability. Post-operatively, more than 67% of children with ASD significantly improved their speech perception skills and 60% significantly improved their speech expression skills while all patients in the control group showed significant improvement in both aspects. The top three reported improvements after cochlear implantation were name recognition, response to verbal requests, and enjoyment of music. Of all behavioral aspects, the use of eye contact was the least improved. Survey results in regards to improvements in patient interaction were more subtle when compared to those related to sound and speech perception. The most improved aspects in the ASD patients’ lives after cochlear implantation appeared to be attending to other people’s requests and conforming to family routines. Of note, awareness of the child’s environment is the most highly ranked improvement attributed to the cochlear implant. Conclusions Cochlear implants are effective and beneficial for hearing impaired members of the ASD population even though development of language may lag behind that of implanted children with no additional disabilities. Significant speech perception and overall behavior improvement are noted.

The overall hearing preservation rate for all implanted patients was 64%. Loss of residual hearing was significantly more observed in cases of cochleostomy and/or non-soft surgical techniques. No correlation was observed with age at implantation, gender, side of implant, device manufacturer, and presence of pre-lingual deafness vs post-lingual, full or partial electrode insertion. In addition, there was a small but significant decrease in hearing between pre-stimulation and post-stimulation audiograms at 6000 Hz.

Asperger disorder (ASP) is one of the autism spectrum disorders (ASD) and is differentiated from autism largely on the absence of clinically significant cognitive and language delays. Analysis of a homogenous subset of families with ASP may help to address the corresponding effect of genetic heterogeneity on identifying ASD genetic risk factors. To examine the hypothesis that common variation is important in ASD, we performed a genome-wide association study (GWAS) in 124 ASP families in a discovery data set and 110 ASP families in a validation data set. We prioritized the top 100 association results from both cohorts by employing a ranking strategy. Novel regions on 5q21.1 (P = 9.7 × 10−7) and 15q22.1–q22.2 (P = 7.3 × 10−6) were our most significant findings in the combined data set. Three chromosomal regions showing association, 3p14.2 (P = 3.6 × 10−6), 3q25–26 (P = 6.0 × 10−5) and 3p23 (P = 3.3 × 10−4) overlapped linkage regions reported in Finnish ASP families, and eight association regions overlapped ASD linkage areas. Our findings suggest that ASP shares both ASD-related genetic risk factors, as well as has genetic risk factors unique to the ASP phenotype.

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Objectives:Determine whether asymmetric hearing loss (AHL) affects postoperative speech outcomes in cochlear implant (CI) patients.Study Design:Retrospective cohort study.Setting:Tertiary care hospital.Patients:Adult English-speaking patients with unilateral CIs implanted between 2014 and 2018 were stratified into NonAHL and AHL groups based on preoperative AzBio scores in quiet from the nonimplanted ear (0–50% vs. 51–100%, respectively).Interventions:CI surgery in the poorer performing ear.Main Outcome Measures:Postoperative consonant-nucleusconsonant (CNC) word and AzBio sentence test scores in quiet and/or noise at +5 dB signal-to-noise ratio (SNR).Results:Of 512 patients, 33 non-AHL and 27 AHL patients were included. Average ages were 65.6 and 63.6 years, respectively. As expected, preoperative AzBio scores in quiet from the nonimplanted ear were higher in the AHL group (95% confidence interval [95%CI]: 66.4–76.4%) than the non-AHL group at baseline (95%CI: 12.3–23.6%). In both cohorts, AzBio scores in quiet from the implanted ear improved from baseline, with 24-month scores (95%CI: 73.8 - 84.9%) being higher than preoperative scores (95%CI: 13.2–23.1%). There were also significant differences in AzBio scores in quiet between cohorts overall (p = 0.0120) on mixed model analysis, with the AHL group performing ∼6.4% better than the non-AHL group; however, differences were not significant when scores were stratified by time. In addition, there were no significant differences in CNC in quiet and AzBio scores in noise at +5 dB SNR between cohorts (p = 0.1786 and p = 0.6215, respectively).Conclusions:After CI, patients with AHL can achieve scores on word and sentence tests at least comparable to traditional CI candidates, supporting the expansion of CI candidacy to include patients with AHL.

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