Faculty Bibliography

OBJECTIVES/OBJECTIVE:To compare the diagnostic performance of the Hum Test against the Weber Test using pure tone audiometry (PTA) as the "gold standard" comparator. METHODS:29 participants with normal hearing of ages 18 to 35 without any history of hearing abnormalities or otologic conditions were enrolled. Subjects underwent three tests (Hum Test, Weber Test, and PTA) across two conditions: with an ear plug in one ear (side randomized) and without ear plugs. RESULTS:When examining the ability of the Hum Test to detect simulated conductive hearing loss (CHL), the test had a sensitivity of 89.7% and specificity of 100% with high pitched humming and 93.1% and 100%, respectively, with low pitched humming. The Weber Test had a sensitivity and specificity of 96.6% and 100%, respectively. McNemar's test demonstrated agreement between the Hum Test, performed with either high pitched ( P = .32) or low pitched ( P = .56) humming, and the Weber Test. Receiver operating characteristic (ROC) curves for the Hum Test (both high and low pitched) and Weber test were compared and demonstrated no statistically significant difference. CONCLUSION/CONCLUSIONS:The Hum Test is comparable to the Weber Test with regards to its sensitivity, specificity, and diagnostic accuracy in assessing new onset unilateral CHL in previously normal hearing subjects.

OBJECTIVE:To assess bimodal auditory performance in children with a cochlear implant (CI) and contralateral auditory brainstem implant (ABI). METHODS:This is a retrospective case review performed at a tertiary referral center. Four patients with cochlear nerve deficiency initially underwent cochlear implantation but were not benefiting from their devices and underwent ABI in the contralateral ear. The main outcome measures included age-appropriate speech perception and production assessments. RESULTS:Three subjects performed better on their auditory perception assessments using both of their devices than with either device alone. One subject had only preliminary outcomes, but subjectively performed best with both devices. CONCLUSIONS:We observed continued improvement in CI performance over time, even if no benefit was evident before the decision for ABI. This could suggest that ABI and CI have a synergistic effect, or it could simply be the adaptive ability of the developing brain to utilize the signals coming from these devices. There is preliminary evidence to support choosing the ear contralateral to the CI for an ABI in a pediatric patient with bilateral cochlear nerve deficiency.

OBJECTIVE: To compare the intraoperative electrically evoked auditory brainstem response (EABR) morphologies between neurofibromatosis II (NF2) adult auditory brainstem implant (ABI) recipients who had auditory percepts post-operatively and those who did not and between NF2 adult ABI recipients and non-NF2 pediatric ABI recipients. METHODS: This was a retrospective case series at a single tertiary academic referral center examining all ABI recipients from 1994 to 2016, which included 34 NF2 adults and 11 non-NF2 children. The morphologies of intraoperative EABRs were evaluated for the number of waveforms showing a response, the number of positive peaks in those responses, and the latencies of each of these peaks. RESULTS: 27/34 adult NF2 patients and 9/10 children had EABR waveforms. 20/27 (74.0%) of the adult patients and all of the children had ABI devices that stimulated post-operatively. When comparing the waveforms between adults who stimulated and those who did not stimulate, the proportion of total number of intraoperative EABR peaks to total possible peaks was significantly higher for the adults who stimulated than for those who did not (p < 0.05). Children had a significantly higher proportion of total number of peaks to total possible peaks when compared to adults who stimulated (p < 0.02). Additionally, there were more likely to be EABR responses at the initial stimulation than intraoperatively in the pediatric ABI population (p = 0.065). CONCLUSIONS: The value of intraoperative EABR tracing may lie in its ability to predict post-operative auditory percepts based on the placement of the array providing the highest number of total peaks.

Ninety-four unilateral CI patients with bimodal listening experience (CI plus HA in contralateral ear) completed a questionnaire that focused on attitudes toward hearing aid use postimplantation, patterns of usage, and perceived bimodal benefits in daily life. Eighty participants continued HA use and 14 discontinued HA use at the time of the questionnaire. Participant responses provided useful information for counseling patients both before and after implantation. The majority of continuing bimodal (CI plus HA) participants reported adapting to using both devices within 3 months and also reported that they heard better bimodally in quiet, noisy, and reverberant conditions. They also perceived benefits including improved sound quality, better music enjoyment, and sometimes a perceived sense of acoustic balance. Those who discontinued HA use found either that using the HA did not provide additional benefit over the CI alone or that using the HA degraded the signal from the CI. Because there was considerable overlap in the audiograms and in speech recognition performance in the unimplanted ear between the two groups, we recommend that unilateral CI recipients are counseled to continue to use the HA in the contralateral ear postimplantation in order to determine whether or not they receive functional or perceived benefit from using both devices together.

OBJECTIVES: Although there are various available treatment options for unilateral severe-to-profound hearing loss, these options do not provide the benefits of binaural hearing since sound is directed from the poorer ear to the better ear. The purpose of this investigation was to review our center's experience with cochlear implantation in such patients in providing improved auditory benefits and useful binaural hearing. STUDY DESIGN: Retrospective chart review. METHODS: Twelve adult patients and four pediatric patients with unilateral severe-to-profound hearing loss received an implant in the poorer ear. Outcome measures performed preoperatively on each ear and binaurally included consonant-nucleus-consonant (CNC) monosyllabic words and sentences in noise. The mean pure-tune average in the better ear was within normal range. RESULTS: Test scores revealed a significant improvement in CNC and sentence in noise test scores from the preoperative to most recent postoperative evaluation in the isolated implant ear. All adult subjects use the device full-time. CONCLUSIONS: The data reveal significant improvement in speech perception performance in quiet and in noise in patients with single-sided deafness after implantation. Performance might depend on factors including length of hearing loss, age at implantation, and device usage.

The programming of CIs is essential for good performance. However, no Good Clinical Practice guidelines exist. This paper reports on the results of an inventory of the current practice worldwide. A questionnaire was distributed to 47 CI centers. They follow 47600 recipients in 17 countries and 5 continents. The results were discussed during a debate. Sixty-two percent of the results were verified through individual interviews during the following months. Most centers (72%) participated in a cross-sectional study logging 5 consecutive fitting sessions in 5 different recipients. Data indicate that general practice starts with a single switch-on session, followed by three monthly sessions, three quarterly sessions, and then annual sessions, all containing one hour of programming and testing. The main focus lies on setting maximum and, to a lesser extent, minimum current levels per electrode. These levels are often determined on a few electrodes and then extrapolated. They are mainly based on subjective loudness perception by the CI user and, to a lesser extent, on pure tone and speech audiometry. Objective measures play a small role as indication of the global MAP profile. Other MAP parameters are rarely modified. Measurable targets are only defined for pure tone audiometry. Huge variation exists between centers on all aspects of the fitting practice.

OBJECTIVE: To analyze the effects of residual hearing on postoperative speech performance in traditional cochlear implant (CI) patients implanted with a conventional electrode. STUDY DESIGN: Retrospective review. SETTING: Academic tertiary referral center. PATIENTS: A total of 129 adults implanted by a single surgeon at a tertiary care facility between June 2005 and November 2010 with measurable preoperative pure tone thresholds at any frequency were included. INTERVENTION: Cochlear implantation with a conventional electrode via an anterior inferior cochleostomy. MAIN OUTCOME MEASURE: Speech perception using monosyllabic word scores in quiet and sentences in quiet and noise in the electric (CI-only) condition of the implanted ear. Preservation of hearing was defined as complete for postoperative thresholds within 10 dB of preimplant values and partial if greater than 11 dB. Pure tone audiometry and speech perception testing were performed preoperatively and at regular intervals postoperatively, with the 1-year evaluation being the final outcome period. RESULTS: Preservation at any frequency or level was not a factor in speech perception outcome, although preservation was more common in low frequencies. Hearing preservation was correlated with younger age at implantation, but was not related to length of hearing loss, cause of deafness, device type, sex, preoperative speech performance, or low-frequency pure-tone average. CONCLUSION: Hearing can be preserved in traditional CI patients implanted with a conventional electrode. Although preservation of hearing may have implications for future technology, it is not currently correlated with speech performance in the CI-only condition.

Cochlear implants have become a viable treatment option for individuals who present with severe to profound hearing loss. While there are several parameters that affect the successful use of this technology, quality programming of the cochlear implant system is crucial. This review chapter focuses on general device programming techniques, programming techniques specific to children, objective programming techniques, a brief overview of programming parameters of the currently commercially available multichannel systems, and managing patient complaints and device failures. The chapter also provides what the authors believe the future may hold for new programming techniques

OBJECTIVE: To generate an evidence-based algorithm for the use of intraoperative testing during cochlear implantation (CI). STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: A total of 277 children (aged 6 mo to 17 yr) and adults 18 years and older with normal cochlear anatomy who underwent primary and revision cochlear implantation at a single center between 2005 and 2010 were included. INTERVENTION: Intraoperative electrophysiologic monitoring and intraoperative Stenver's view plain film radiography. MAIN OUTCOME MEASURE: Intraoperative testing included the following: 1) individual electrode impedance measurements; 2) neural response telemetry (tNRT) levels for electrodes E20, E15, E10, and E5; and 3) plain film radiograph assessment of electrode position. RESULTS: No patient demonstrated abnormalities on all 3 modalities. Open or short electrodes on impedance testing were found in 6% of patients; half of these normalized when remeasured. Absent tNRT responses on 1 or more electrodes occurred in 14% of patients, although complete lack of response was rare (1.4%) and did not correlate with a dysfunctional device. Spread of excitation was performed in 1 patient and was consistent with a tip rollover. Intraoperative radiography identified tip-rollover and extracochlear electrode placement in all cases (n = 5, 1.8%) and prompted the use of the backup device. CONCLUSION: Immediate intraoperative determination of device functionality and optimal electrode placement is advantageous. Of the modalities tested, including electrode impedance, tNRT, and plain radiograph, only the radiographic results impacted intraoperative surgical decision making and led to the use of the backup device

OBJECT: The aim of this study was to determine whether patients with neurofibromatosis Type 2 (NF2) who have intact ipsilateral cochlear nerves can have open-set speech discrimination following cochlear implantation. METHODS: Records of 7 patients with documented NF2 were reviewed to determine speech discrimination outcomes following cochlear implantation. Outcomes were measured using consonant-nucleus-consonant words and phonemes; Hearing in Noise Test sentences in quiet; and City University of New York sentences in quiet and in noise. RESULTS: Preoperatively, none of the patients had open-set speech discrimination. Five of the 7 patients had previously undergone excision of ipsilateral vestibular schwannoma (VS). One of the patients who received a cochlear implant had received radiation therapy for ipsilateral VS, and another was undergoing observation for a small ipsilateral VS. Following cochlear implantation, 4 of 7 patients with NF2 had open-set speech discrimination following cochlear implantation during extended follow-up (15-120 months). Two of the 3 patients without open-set speech understanding had a prolonged period between ipsilateral VS resection and cochlear implantation (120 and 132 months), and had cochlear ossification at the time of implantation. The other patient without open-set speech understanding had good contralateral hearing at the time of cochlear implantation. Despite these findings, 6 of the 7 patients were daily users of their cochlear implants, and the seventh is an occasional user, indicating that all of the patients subjectively gained some benefit from their implants. CONCLUSIONS: Cochlear implantation can provide long-term auditory rehabilitation, with open-set speech discrimination for patients with NF2 who have intact ipsilateral cochlear nerves. Factors that can affect implant performance include the following: 1) a prolonged time between VS resection and implantation; and 2) cochlear ossification