Meredith A. Holcomb

Cochlear nerve deficiency in children with CHARGE syndrome

To describe cochlear nerve deficiency and abnormalities of the internal auditory canal (IAC) associated with CHARGE (coloboma, heart defects, atresia choanae, retardation of growth and/or development, genital hypoplasia, and ear anomalies and/or deafness) syndrome.

Outcomes of cochlear implantation in adults with asymmetric hearing loss.

Objective To analyze speech recognition outcomes in adult cochlear implant recipients who have asymmetric sensorineural hearing loss between the two ears. Study Design Retrospective cohort study of 35 adults with asymmetric hearing loss. Preoperative unaided pure-tone averages (PTA), and pre- and post-op (6 and 12 mo) aided Consonant Nucleus Consonant (CNC) words and sentence recognition scores were obtained for ears in isolation (opposite ear plugged). Patients were categorized according to the PTA of the implanted and contralateral ears as (a) moderate-severe, (b) moderate-profound, and (c) severe-profound. Setting A single tertiary care center. Patients Adults with asymmetric PTA implanted unilaterally at our institution. Intervention Cochlear implantation with devices approved by the U.S. Food and Drug Administration. Main Outcome Measure(s) Post-implantation word, phoneme, and sentence recognition in the cochlear implant alone testing condition. Results Patients who were implanted in the worse-hearing ear (n = 21) performed significantly better on CNC words and phonemes at 6 and 12 months than patients who were implanted in the better-hearing ear (n = 14). Patients with the worse ear implanted also obtained 6 to 12% higher sentence recognition scores than the group received an implant in their better ear, although this difference is not statistically significant (p = 0.06 at 6 mo; p = 0.1 at 12 mo). Conclusion Patients with asymmetrical hearing loss who were implanted in the worse-hearing ear achieved 15% greater CNC word scores and 6 to 12% greater sentence scores than patients implanted in the better-hearing ear in the first year after surgery. Our results provide further support for clinicians to use caution if considering implanting the better-hearing ear in patients with asymmetric hearing loss.

Safety of cochlear implantation before 12 months of age: Medical University of South Carolina and Pediatric American College of Surgeons–National Surgical Quality improvement program outcomes

The primary objective of this study was to determine the safety profile of cochlear implantation (CI) in infants <12 months old.

Cochlear Implantation in Inner Ear Malformations: Systematic Review of Speech Perception Outcomes and Intraoperative Findings:

Objective (1) To analyze reported speech perception outcomes in patients with inner ear malformations who undergo cochlear implantation, (2) to review the surgical complications and findings, and (3) to compare the 2 classification systems of Jackler and Sennaroglu. Data Sources PubMed, Scopus (including Embase), Medline, and CINAHL Plus. Review Methods Fifty-nine articles were included that contained speech perception and/or intraoperative data. Cases were differentiated depending on whether the Jackler or Sennaroglu malformation classification was used. A meta-analysis of proportions examined incidences of complete insertion, gusher, and facial nerve aberrancy. For speech perception data, weighted means and standard deviations were calculated for all malformations for short-, medium-, and long-term follow-up. Speech tests were grouped into 3 categories—closed-set words, open-set words, and open-set sentences—and then compared through a comparison-of-means t test. Results Complete insertion was seen in 81.8% of all inner ear malformations (95% CI: 72.6-89.5); gusher was reported in 39.1% of cases (95% CI: 30.3-48.2); and facial nerve anomalies were encountered in 34.4% (95% CI: 20.1-50.3). Significant improvements in average performance were seen for closed- and open-set tests across all malformation types at 12 months postoperatively. Conclusions Cochlear implantation outcomes are favorable for those with inner ear malformations from a surgical and speech outcome standpoint. Accurate classification of anatomic malformations, as well as standardization of postimplantation speech outcomes, is necessary to improve understanding of the impact of implantation in this difficult patient population.

Effects of Age and Implanted Ear on Speech Recognition in Adults with Unilateral Cochlear Implants

The objective of this study was to examine how age and implanted ear contribute to functional outcomes with cochlear implantation (CI). A retrospective review was performed on 96 adults who underwent unilateral CI. Older adults with right-ear implants had higher Hearing in Noise Test (HINT) scores at 1 year by 10.3% (p = 0.06). When adjusted to rationalized arcsine units (rau), right-ear HINT scores in older adults were higher by 12.1 rau (p = 0.04). Older adults had an 8.9% advantage on the right side compared to the left in post- versus preimplant scores for consonant-vowel nucleus-consonant words (p = 0.05). No significant differences were observed for younger adults. In conclusion, although adults of all ages experience improvements in speech perception following CI, there might be a subtle but consistent right-ear advantage in older adults.

Does pediatric cochlear implant insertion technique affect intraoperative neural response telemetry thresholds

OBJECTIVE Recent reports of mostly adult patients suggest round window insertion is less traumatic than cochleostomy for cochlear implantation (CI), while other reports have indicated that curved electrode arrays lower the neural response telemetry (NRT) threshold and consume less power. We aimed to compare the intraoperative neural response telemetry (NRT) thresholds in children receiving cochlear implants through a cochleostomy (COCH) vs. a round window (RW) approach, as well as patients receiving a curved array vs. a straight one. DESIGN A direct case-cohort comparison of NRT in pediatric CI recipients at two large tertiary pediatric hospitals from 2008 through 2014 was done. Univariate Mann-Whitney analyses and one-wayANOVA were performed to compare average NRT in RW vs. COCH insertion, and curved vs. straight electrodes. Multivariate regression was performed to control for age and pre- vs. postlingual patient status. RESULTS Overall, 236 children were included, between January 2008 and October 2014 at two large tertiary referral medical centers. A total of 52 patients received a RW insertion and 184 received a cochleostomy. There was no statistically significant difference between RW insertion (187.9±18.7) and COCH (183.4±17.1) (p=0.125). The patients were divided into four categories: RW insertion with curved electrode (175.0±11.2), RW with straight electrode (192.1±18.8), COCH with curved electrode (182.2±16.7), and COCH with straight electrode (193.0±20.8). The lowest NRT current thresholds were achieved with curved electrode array insertions through the RW (p=0.001). Multivariate regression analysis revealed the following parameters were not independently associated with differences in NRT responses: RWI vs. COCH (p=0.12) and pre- vs. postlingual (p=0.18). The difference in NRT levels between curved electrode arrays and straight was shown to be statistically significant (p=0.00075). CONCLUSIONS When controlling for insertion technique or pre- vs. postlingual hearing loss, the curved electrode array correlates with lower NRT thresholds. Although studies to examine functional language performance of these recipients are pending, initial results of this multi-institutional trial suggest that curved electrodes indeed result in lower NRT levels, particularly when inserted through the RW.

The influence of obesity on operating room time and perioperative complications in cochlear implantation

Objective The rising incidence of obesity in the United States is associated with increased healthcare expenditures and resource allocation. Obesity has been associated with prolonged operating times during surgical procedures. The primary objective of this study is to compare body mass index (BMI) to length of surgery during cochlear implantation. Methods A retrospective case control study from a tertiary academic referral center was performed. Patients included were adults who underwent primary, single-sided cochlear implantation with documented BMI and operating room (OR) times from January 2009 to July 2015. The following data were collected: BMI, total operating room time (TORT), surgical operating room time (SORT), ASA status, perioperative and postoperative complications, age, and gender. Results Two hundreds and thirty-four patients were included and stratified into obese (BMI >30) and non-obese (BMI < 30) categories. Statistical analysis was performed comparing TORT against the obesity category along with other variables. Independent sample t-test demonstrated that obesity increases TORT and SORT by 16.8 min (P = 0.0002) and 9.3 min (P = 0.03), respectively, compared to the non-obese group. Multivariate linear regression analysis demonstrated no statistically significant impact of gender, or ASA status on total operating or surgical time. Obesity was associated with increased perioperative complications (odds ratio [OR], 6.21; 95% CI, 1.18–32.80; P = 0.03) and postoperative complications (OR, 3.97; 95% CI, 1.29–12.26; P = 0.02). Conclusions Obesity leads to longer TORT and SORT during primary cochlear implant surgery. Obesity is also associated with increased perioperative and postoperative complications compared to non-obese patients. These data have implications with utilization of operating room resources.

Bilateral cochlear implantation in a child with Johanson Blizzard Syndrome

Sensorineural hearing loss (SNHL) occurs in more than 80% of cases of Johanson Blizzard Syndrome (JBS). However, limited knowledge exists in medical literature of cochlear implantation (CI) outcomes in children with JBS. We report the case of a 5 year-old male with JBS and bilateral CI. While minimal progress in spoken language scores was noted after 4 years of bilateral CI use, substantial improvements in discrimination of speech sounds and audibility of spoken language and environmental sounds were documented. Cochlear implantation is an available treatment option of profound SNHL in children with JBS even if spoken language outcomes are marginal.

Use of Adult Patient Focus Groups to Develop the Initial Item Bank for a Cochlear Implant Quality-of-Life Instrument

Importance No instrument exists to assess quality of life (QOL) in adult cochlear implant (CI) users that has been developed and validated using accepted scientific standards. Objective To develop a CI-specific QOL instrument for adults in accordance with the Patient Reported Outcomes Measurement Information System (PROMIS) guidelines. Design, Setting, and Participants As required in the PROMIS guidelines, patient focus groups participated in creation of the initial item bank. Twenty-three adult CI users were divided into 1 of 3 focus groups stratified by word recognition ability. Three moderator-led focus groups were conducted based on grounded theory on December 3, 2016. Two reviewers independently analyzed focus group recordings and transcripts, with a third reviewer available to resolve discrepancies. All data were reviewed and reported according to the Consolidated Criteria for Reporting Qualitative Research. The setting was a tertiary referral center. Main Outcomes and Measures Coded focus group data. Results The 23 focus group participants (10 [43%] female; mean [range] age, 68.1 [46.2-84.2] years) represented a wide range of income levels, education levels, listening modalities, CI device manufacturers, duration of CI use, and age at implantation. Data saturation was determined to be reached before the conclusion of each of the focus groups. After analysis of the transcripts, the central themes identified were communication, emotion, environmental sounds, independence and work function, listening effort, social isolation and ability to socialize, and sound clarity. Cognitive interviews were carried out on 20 adult CI patients who did not participate in the focus groups to ensure item clarity. Based on these results, the initial QOL item bank and prototype were developed. Conclusions and Relevance Patient focus groups drawn from the target population are the preferred method of identifying content areas and domains for developing the item bank for a CI-specific QOL instrument. Compared with previously used methods, the use of patient-centered item development for a CI-specific QOL instrument will more accurately reflect patient experience and increase our understanding of how CI use affects QOL.