International Consensus Statements on Intraoperative Testing for Cochlear Implantation Surgery.

OBJECTIVES: A wide variety of intraoperative tests are available in cochlear implantation. However, no consensus exists on which tests constitute the minimum necessary battery. We assembled an international panel of clinical experts to develop, refine, and vote upon a set of core consensus statements. DESIGN: A literature review was used to identify intraoperative tests currently used in the field and draft a set of provisional statements. For statement evaluation and refinement, we used a modified Delphi consensus panel structure. Multiple interactive rounds of voting, evaluation, and feedback were conducted to achieve convergence. RESULTS: Twenty-nine provisional statements were included in the original draft. In the first voting round, consensus was reached on 15 statements. Of the 14 statements that did not reach consensus, 12 were revised based on feedback provided by the expert practitioners, and 2 were eliminated. In the second voting round, 10 of the 12 revised statements reached a consensus. The two statements which did not achieve consensus were further revised and subjected to a third voting round. However, both statements failed to achieve consensus in the third round. In addition, during the final revision, one more statement was decided to be deleted due to overlap with another modified statement. CONCLUSIONS: A final core set of 24 consensus statements was generated, covering wide areas of intraoperative testing during CI surgery. These statements may provide utility as evidence-based guidelines to improve quality and achieve uniformity of surgical practice.

Functional Hearing Preservation in Cochlear Implantation: The Miami Cocktail Effect.

OBJECTIVE: To investigate if pharmacological treatment with prednisone and L-N-acetylcysteine (STE + NAC) influence functional hearing preservation in cochlear implant (CI) surgery. STUDY DESIGNS: Preimplantation and postimplantation longitudinal case-control study. SETTING: Tertiary referral center. PATIENTS: Pediatric and adult recipients of CI with preimplantation functional hearing defined as an average of air-conducted thresholds at 125, 250, and 500 Hz (low-frequency pure-tone average [LFPTA]) <80 dB. INTERVENTIONS: Preimplantation and postimplantation audiometry. Weight-adjusted oral prednisone and L-N-acetylcysteine starting 2 days before surgery (Miami cocktail). Prednisone was continued for 3 days and L-N-acetylcysteine for 12 days after surgery, respectively. Cochlear implantation with conventional length electrodes. MAIN OUTCOME MEASURES: Proportion of patients with LFPTA <80 dB, and LFPTA change at 1-year postimplantation. RESULTS: All 61 patients received intratympanic and intravenous dexamethasone intraoperatively, with 41 patients receiving STE + NAC and 20 patients not receiving STE + NAC. At 1-year postimplantation, the proportion of functional hearing preservation was 83% in the STE + NAC group compared with 55% of subjects who did not receive STE + NAC ( p = 0.0302). The median LFPTA change for STE + NAC-treated and not treated subjects was 8.33 dB (mean, 13.82 ± 17.4 dB) and 18.34 dB (mean, 26.5 ± 23.4 dB), respectively ( p = 0.0401, Wilcoxon rank test). Perioperative STE + NAC treatment resulted in 10 dB of LFPTA better hearing than when not receiving this treatment. Better low-frequency preimplantation hearing thresholds were predictive of postimplantation functional hearing. No serious side effects were reported. CONCLUSION: Perioperative STE + NAC, "The Miami Cocktail," was safe and superior to intraoperative steroids alone in functional hearing preservation 1-year after cochlear implantation.

Catchment Profile of Large Cochlear Implant Centers in the United States.

OBJECTIVE: To characterize the catchment area and patient profile of large cochlear implant (CI) centers in the United States. STUDY DESIGN: Multi-institutional retrospective case series. SETTING: Tertiary referral CI centers. METHODS: Patients who underwent CI surgery at 7 participating CI centers between 2015 and 2020 were identified. Patients' residential zip codes were used to approximate travel distances and urban vs rural residential areas. RESULTS: Over the 6-year study period (2015-2020), 6313 unique CI surgical procedures occurred (4529 adult, 1784 pediatric). Between 2015 and 2019, CI procedures increased by 43%. Patients traveled a median 52 miles (interquartile range, 21-110) each way; patients treated at rural CI centers traveled greater distances vs those treated at urban centers (72 vs 46 miles, P < .001). Rural residents represented 61% of the patient population and traveled farther than urban residents (73 vs 24 miles, P < .001). Overall, 91% of patients lived within a 200-mile radius of the institution, while 71% lived within a 100-mile radius. In adults, multiple regression analysis redemonstrated an association between greater travel distances and (1) older age at the time of CI and (2) residential rural setting (both P < .001, r2 = 0.2). CONCLUSIONS: While large CI centers serve geographically dispersed populations, most patients reside within a 200-mile radius. Strategies to expand CI utilization may leverage remote programming, telemedicine, and strategic placement of new centers and satellite clinics to ameliorate travel burden.

When to replace legacy cochlear implants for technological upgrades: Indications and outcomes.

OBJECTIVE: To determine indications, surgical efficacy, and audiologic outcomes of replacing Advanced Bionics Clarion C1.2 internal devices (Advanced Bionics, LLC, Valencia, CA) as a means of technology upgrade. STUDY DESIGN: Retrospective review, case series. METHODS: Ten patients were initially implanted as a child (mean age = 3.87 years) and underwent cochlear implant reimplantation (CIR) with current Advanced Bionics internal device as a young adult (mean duration of implant use = 15.66 years). Demographic data and pre- and post-CIR speech perception scores were collected. RESULTS: Technology upgrade was the primary (9) or secondary (1) motivation for CIR. No surgical complications were noted, and full insertion was obtained in nine cases. Intraoperative impedance levels and neural response imaging measures were within normal limits for eight patients. At most recent post-CIR follow-up evaluation, all patients (100%) performed within or better than the 95% confidence interval of their pre-CIR word and sentence recognition scores; and 55.6%, 50.0%, and 50.0% of patients performed above the 95% confidence interval of their pre-CIR scores for the CNC words, sentences in quiet, and sentences in noise, respectively. CONCLUSION: Post-CIR audiological benefit was stable or improved compared to pre-CIR results in all categories by 3 months after reactivation. Given these results, patients who are unable to use the most current external processors due to incompatibility with a legacy internal device could consider reimplanation to optimize their overall performance with a cochlear implant. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:748-753, 2019.

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.

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.

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.

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.

A Revision Surgery for Cochlear Implantation in a Case of Incomplete Partition Type I.

BACKGROUND: Patients with cochlear malformations were long considered poor candidates for cochlear implantation (CI), and surgical approaches different than the standard facial recess approach were used to access the inner ear. There is no previous long-term follow-up of a patient with significantly malformed inner ear operated through an untraditional route and requiring a revision surgery. PURPOSE: This case provides a long-term follow-up from the initial surgery, a short-term follow-up from the revision surgery, and it illustrates the evolving classification of inner ear malformations as well as the potential problems associated with nonstandard approaches to the cochlea. RESEARCH DESIGN: A case report. INTERVENTION: Herein, we report a case of revision CI in a patient with incomplete partition type I, through the round window via a facial recess approach, 18 yr after an initial implantation via a transmastoid labyrinthotomy approach. RESULTS: The patient had an uncomplicated surgery, and after activation, she noted auditory perception on all electrodes without facial stimulation. A sound field sound awareness threshold was obtained at 15 dB HL. CONCLUSIONS: As the prior generation of cochlear implant recipients ages, the probability of a revision surgery for various causes increases. Cochlear implant surgeons should be aware of the potential pitfalls associated in these often challenging cases.

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.