David Shipp

A Comparison of Postcochlear Implantation Speech Scores in an Adult Population

Objectives: The vast majority of cochlear implant recipients realize significant improvement in speech perception. However, there continue to be a small group that does not realize such a benefit. In an effort to identify possible predictors for this, we have compared pre‐ and postimplant audiologic data using Hearing In Noise Test (HINT), City University of New York (CUNY), or Central Institute for the Deaf (CID) scores for 445 consecutive English‐speaking adult patients followed for a minimum of 1 year postimplantation in two distinct groups, poor versus excellent performers.

The effects of unilateral cochlear implantation on the tinnitus handicap inventory and the influence on quality of life.

Cochlear implantation is now the standard of care in patients with significant sensorineural hearing loss. It is well known that patients with severe hearing loss also experience disabling tinnitus. The purpose of this study was to assess the effects of cochlear implants on the perception of tinnitus using the Tinnitus Handicap Inventory (THI).

Hearing preservation with full insertion of the FLEXsoft electrode.

Objectives Hearing preservation (HP) in the context of cochlear implantation (CI) is indicative of an atraumatic insertion, which could potentially offer a clinical advantage to performance, whether such preservation was intended for the application of electoacoustic stimulation (EAS). Our goal was to determine the degree of HP after the implantation of a full-length electrode array (MedEl FLEXsoft). Study Design Prospective, within-subject repeated measure design. Methods Adult patients with residual low-frequency hearing who received a 31.5-mm FlexSoft electrode array were included. Implantation using soft surgery techniques occurred at a single tertiary referral center between 2008 and 2011. Preoperative and postoperative audiometric data were compared. Results In 36 consecutive patients at 1 year postimplantation, 6 (21%) maintained complete HP (an increase of low-frequency pure-tone average (PTA) ⩽10 dB from the preoperative value), and 19 (65%) maintained partial HP (an increase in low-frequency PTA ⩽40 dB from the preoperative values) throughout the follow-up period (average, 368 d). Higher preoperative hearing threshold levels (HTLs) at 1,000 Hz were associated with HP. Conclusion Low-frequency HP is possible in patients implanted with the full-length FlexSoft electrode. Longer follow-up is required to determine if results are maintained over time and if such preservation is in fact advantageous to clinical outcomes. Level of Evidence: 4

Cochlear implantation in patients with autoimmune inner ear disease including cogan syndrome: A comparison with age‐ and sex‐matched controls

Evaluate the characteristics and outcomes of patients with autoimmune inner ear disease (AIED) who have undergone cochlear implantation (CI) and compare post‐CI performance in AIED to matched controls.

Ten-year health-related quality of life in cochlear implant recipients: prospective SF-36 data with SF-6D conversion.

To evaluate the long‐term impact of cochlear implantation on quality of life measured by the Medical Outcomes Study 36‐Item Short‐Form Health Survey (SF‐36). Scores were also converted to the SF‐6D to derive health utility scores.

Prevalence of Connexin 26 (GJB2) and Pendred (SLC26A4) mutations in a population of adult cochlear implant candidates.

Objective: To assess the prevalence of Connexin 26 (GJB2), Connexin 30 (GJB6), and Pendred (SLC26A4) mutations in a population of adult cochlear implant patients with a history of either early idiopathic or hereditary progressive sensorineural deafness. Background: Significant efforts have been applied in defining the epidemiology of Connexin 26 (GJB2)-associated hearing impairment in the pediatric population, yet the issue remains ambiguous for adult patients. Causation is important in this population because there are implications to prognosis, risk of associated medical manifestations, and for genetic counseling purposes. Patients: Adult patients meeting criteria for cochlear implantation with early-onset hearing loss assessed at an adult cochlear implant center from November 2007 to April 2009. Intervention: Genomic DNA samples from whole blood were tested with bidirectional sequence analysis for mutations in the coding region of the GJB2 and SLC26A4 genes and tested for large deletions of the GJB6 gene. Results: Fifty-seven patients were analyzed for GJB2 mutations. Eight patients (14%) were found to have GJB2-related hearing impairment; 5 patients were homozygous for the c.35delG mutation (genotype c.35delG/c.35delG), and 3 additional patients were compound heterozygotes with 2 different GJB2 mutations. Of these 8 patients with GJB2-related hearing impairment, 3 had serviceable hearing into their teenage years. One additional patient had 1 GJB2 variant (p.Met195Ile, heterozygous). None had GJB6 mutations. Of the 57 patients, 30 were also analyzed for SLC26A4 mutations. Three of these patients were compound heterozygotes for disease-causing SLC26A4 mutations, confirming SLC26A4-related hearing impairment. Three additional patients were found to have a single variant in SLC26A4. Conclusion: The prevalence of GJB2- and SLC26A4-related hearing impairment in an adult population with early-onset severe sensorineural hearing loss is significant, suggesting the need for routine assessment for genetic etiologies in this group. We also note 3 individuals with causal connexin 26 mutations with subjective serviceable hearing into adolescence in our cohort.

Cochlear implantation in patients with advanced Ménière's disease.

Background/Objectives Ménière’s disease (MD) that results in bilateral severe to profound sensorineural hearing loss is a rare indication for cochlear implantation; only a few studies exist documenting performance in these patients. The primary objective was to compare the difference in preoperative to 12-month postoperative speech perception scores among subjects with MD and controls. Groupwise comparisons of secondary postoperative outcomes (Tinnitus Handicap Inventory [THI] scores, 36-Item Short Form [SF-36] scores, and postoperative dizziness) were also performed. Methods A retrospective cohort study was conducted. Subjects with MD and controls matched by age, device manufacturer and model, preoperative sentence score, and sentence test used for preimplantation and postimplantation performance assessments were identified from 1,130 patients in the prospectively maintained cochlear implant database at our center. Speech perception, THI, and SF-36 scores and demographic variables were obtained from the database. Vestibular outcomes were obtained by retrospective chart review. Statistical comparisons were performed to compare preoperative to postoperative change between groups. Results Twenty patients with MD were identified. At 1 year after CI, improvements in sentence and word understanding did not differ in magnitude from the controls. Tinnitus was reduced significantly in patients with MD, whereas there was a trend for improvement in the controls. Quality of life as measured by the SF-36 improved in both groups. Patients with MD had significant improvements in 1 domain compared with 5 domains for the controls. Subjects with MD had significantly more chronic dizziness in the postoperative period than did controls. Conclusions Patients with MD who have bilateral severe to profound sensorineural hearing loss benefit significantly from CI. Ongoing dizziness in some patients with MD may result in quality of life improvements that are slightly less than seen for the average adult patient with CI. Larger studies are needed to corroborate the results.

Cochlear implantation in elderly patients: stability of outcome over time.

BACKGROUND Cochlear implantation is the standard of care for treating severe to profound hearing loss in all age groups. There is limited data on long-term results in elderly implantees and the effect of ageing on outcomes. This study compared the stability of cochlear implantation outcome in elderly and younger patients. METHODS A retrospective chart review of cochlear implant patients with a minimum follow up of five years was conducted. RESULTS The study included 87 patients with a mean follow up of 6.8 years. Of these, 22 patients were older than 70 years at the time of implantation. Hearing in Noise Test scores at one year after implantation were worse in the elderly: 85.3 (aged under 61 years), 80.5 (61-70 years) and 73.6 (aged over 70 years; p = 0.039). The respective scores at the last follow up were 84.8, 85.1 and 76.5 (p = 0.054). Most patients had a stable outcome during follow up. Of the elderly patients, 13.6 per cent improved and none had a reduction in score of more than 20 per cent. Similar to younger patients, elderly patients had improved Short Form 36 Health Survey scores during follow up. CONCLUSION Cochlear implantation improves both audiometric outcome and quality of life in elderly patients. These benefits are stable over time.

Quality of life in cochlear implantees: Comparing utility values obtained through the Medical Outcome Study Short‐Form Survey‐6D and the Health Utility Index Mark 3

To evaluate the changes in health‐related quality of life in unilateral adult cochlear implant patients using the Medical Outcome Study Short‐Form Survey‐36 (SF‐36) and the Health Utility Index Mark 3 (HUI‐3). To do so, a health utility index was obtained by converting the SF‐36 to the Medical Outcome Study Short‐Form Survey‐6D (SF‐6D) to permit comparison with HUI‐3 scores in the context of health preference as measured by quality‐adjusted life years.

Assessment of the psychosocial impacts of cochlear implants on adult recipients and their partners

Objectives: In the present study we sought to evaluate the psychosocial and quality of life (QOL) impacts of cochlear implant (CI) treatment on both CI recipients and their partners in a Canadian population. Methods: A qualitative cross-sectional contemporary cohort evaluation was conducted by distributing specifically designed questionnaires to both CI recipients and their partners at their follow-up appointments over a month at Sunnybrook Health Sciences Centre. Results: It was found that both CI recipients and their partners demonstrated substantial benefit and improvement in multiple psychosocial domains. Discussion: Musical listening was found to show variable improvements between recipients. Further analysis found a simple correlation where the group of recipients who all had a hearing disability for a longer duration had demonstrated a higher proportion of improvement in musical listening. CI recipients endorsed having improved QOL which was in agreement with proxy observation by partners. Conclusion: Overall, CI use has been shown to have significant benefit to psychosocial well-being of CI recipients. This benefit is also conferred to their caregivers/partners; which in our society concerned about caregiver burden is quite important to recognize.