Faculty Bibliography

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

OBJECTIVE:: To determine whether intraoperative neural response telemetry (tNRT) is predictive of postoperative speech perception. STUDY DESIGN:: Retrospective review. SETTING:: Tertiary referral center. PATIENTS:: Children (n = 24) aged between 5 and 17 years and adults 18 years and older (n = 73) with severe-to-profound hearing loss and implanted with the Nucleus Freedom device between 2005 and 2008 and observed at least 1 year were included. INTERVENTION:: Intraoperative neural response telemetry after insertion of the electrode array. MAIN OUTCOME MEASURE:: Measures included 1) intraoperative tNRT measurements and 2) preoperative and 1-year postoperative open-set word recognition scores using age-appropriate open-set tests for children and adults. Intraoperative neural response telemetry levels for electrodes E20, E15, E10, and E5 in each patient were correlated to performance at the 1-year evaluation interval. RESULTS:: No correlation existed between tNRT responses and open-set speech performance at the 1-year evaluation. Several patients had absent tNRT in the OR but developed speech recognition abilities, whereas the remaining patients had intraoperative responses with levels of postoperative performance ranging from 0% to 100%. CONCLUSION:: This study suggests that there is no significant correlation between intraoperative tNRT and speech perception performance at 1 year. At the time of surgery, tNRT provides valuable information regarding the electrical output of the implant and the response of the auditory system to electrical stimulation and preliminary device programming data; however, it is not a valuable predictor of postoperative performance. Furthermore, the absence of tNRT does not necessarily indicate a lack of stimulation

OBJECTIVES: Intraoperative testing of cochlear implant devices, establishment of electrical threshold for acoustic reflex, and recording neural responses to electrical stimulation have traditionally required the presence of a cochlear implant audiologist in the operating room. The goal of this study was to determine the feasibility of remote testing to improve time efficiency and reduce cost. STUDY DESIGN: Prospective. METHODS: A standard PC with Tridia VNC software and either Cochlear Corporation or Advanced Bionics Corporation mapping software was configured to perform remote testing. The time required to perform on-site or remote testing was measured. RESULTS: With the availability of the laptop and internet access, there were no geographic restrictions regarding the site of remote testing. Remote testing was time efficient, requiring 9 minutes of audiologist's time compared with 93 minutes when the audiologist had to travel to the operating room. CONCLUSION: Remote testing of the cochlear implant device and patient's response to electrical stimulation is technically feasible. It is timesaving, practical, and cost efficient

OBJECTIVE: To assess word recognition and pitch-scaling abilities of cochlear implant users first implanted with a Nucleus 10-mm Hybrid electrode array and then reimplanted with a full length Nucleus Freedom array after loss of residual hearing. BACKGROUND: Although electroacoustic stimulation is a promising treatment for patients with residual low-frequency hearing,a small subset of them lose that residual hearing. It is not clear whether these patients would be better served by leaving in the 10-mm array and providing electric stimulation through it, or by replacing it with a standard full-length array. METHODS: Word recognition and pitch-scaling abilities were measured in 2 users of hybrid cochlear implants who lost their residual hearing in the implanted ear after a few months. Tests were repeated over several months, first with a 10-mm array, and after, these patients were reimplanted with a full array. The word recognition task consisted of 2 50-word consonant nucleus consonant (CNC) lists. In the pitch-scaling task, 6 electrodes were stimulated in pseudorandom order, and patients assigned a pitch value to the sensation elicited by each electrode. RESULTS: Shortly after reimplantation with the full electrode array, speech understanding was much better than with the 10-mm array. Patients improved their ability to perform the pitch-scaling task over time with the full array, although their performance on that task was variable, and the improvements were often small. CONCLUSION: 1) Short electrode arrays may help preserve residual hearing but may also provide less benefit than traditional cochlear implants for some patients. 2) Pitch percepts in response to electric stimulation may be modified by experience

CONCLUSION: Neither speech understanding nor frequency discrimination ability was better in Nucleus Contour users than in Nucleus 24 straight electrode users. Furthermore, perimodiolar electrode placement does not result in better frequency discrimination. OBJECTIVES: We addressed three questions related to perimodiolar electrode placement. First, do patients implanted with the Contour electrode understand speech better than with an otherwise identical device that has a straight electrode? Second, do these groups have different frequency discrimination abilities? Third, is the distance of the electrode from the modiolus related to frequency discrimination ability? SUBJECTS AND METHODS: Contour and straight electrode users were matched on four important variables. We then tested these listeners on CNC word and HINT sentence identification tasks, and on a formant frequency discrimination task. We also examined X-rays and measured the distance of the electrodes from the modiolus to determine whether there is a relationship between this factor and frequency discrimination ability. RESULTS: Both speech understanding and frequency discrimination abilities were similar for listeners implanted with the Contour vs a straight electrode. Furthermore, there was no linear relationship between electrode-modiolus distance and frequency discrimination ability. However, we did note a second-order relationship between these variables, suggesting that frequency discrimination is worse when the electrodes are either too close or too far away from the modiolus

OBJECTIVES: This article presents the first report of cochlear implantation in a patient with congenital aural atresia, microtia, dysplastic cochlea and internal auditory canals, and bilateral profound sensorineural hearing loss (HL). This rare combination requires special management considerations. Preoperative issues include thorough evaluation of computed tomography and magnetic resonance imaging to determine favorable anatomy, cochlear implantation candidacy, and surgical planning. Intraoperative concerns include incision placement, surgical approach to the middle ear, and abnormal facial nerve anatomy. Postoperative use of a special headset combining a microphone and transmitter coil is required. STUDY DESIGN: Case report and literature review. METHODS: The patient's chart was reviewed for diagnostic studies, operative strategy, and postoperative auditory stimulation and testing. A literature review was performed. RESULTS: A 2-year-old male presented with bilateral aural atresia, microtia, and profound sensorineural HL. Imaging studies revealed multiple abnormalities of the cochlea, vestibule, and internal auditory canal, all of which were more favorable on the right side. An incision was designed to accommodate future microtia repair. The cochlear implant was placed without difficulty by way of a facial recess approach to the middle ear. Postoperative results include the detection of Ling sounds and voices in the environment as well as the ability to locate sounds. CONCLUSIONS: This is the first report of cochlear implantation in a patient with bilateral aural atresia, microtia, and profound sensorineural HL in conjunction with multiple inner ear abnormalities. Close collaboration among the otologist, neuroradiologist, and plastic surgeon is essential to coordinate surgical management and optimize cosmetic and functional outcomes in this unique population

OBJECTIVES: Multichannel auditory brainstem implants (ABI) are currently indicated for patients with neurofibromatosis type II (NF2) and schwannomas involving the internal auditory canal (IAC) or cerebellopontine angle (CPA), regardless of hearing loss (HL). The implant is usually placed in the lateral recess of the fourth ventricle at the time of tumor resection to stimulate the cochlear nucleus. This study aims to review the surgical and audiologic outcomes in 18 patients implanted by our Skull Base Surgery Team from 1994 through 2003. STUDY DESIGN: A retrospective chart review of 18 patients with ABIs. METHODS: We evaluated demographic data including age at implantation, number of tumor resections before implantation, tumor size, surgical approach, and postoperative surgical complications. The ABI auditory results at 1 year were then evaluated for number of functioning electrodes and channels, hours per day of use, nonauditory side effect profile and hearing results. Audiologic data including Monosyllable, Spondee, Trochee test (MTS) Word and Stress scores, Northwestern University Children's Perception of Speech (NU-CHIPS), and auditory sensitivity are reported. RESULTS: No surgical complications caused by ABI implantation were revealed. A probe for lateral recess and cochlear nucleus localization was helpful in several patients. A range of auditory performance is reported, and two patients had no auditory perceptions. Electrode paddle migration occurred in two patients. Patient education and encouragement is very important to obtain maximum benefit. CONCLUSIONS: ABIs are safe, do not increase surgical morbidity, and allow most patients to experience improved communication as well as access to environmental sounds. Nonauditory side effects can be minimized by selecting proper stimulation patterns. The ABI continues to be an emerging field for hearing rehabilitation in patients who are deafened by NF2

OBJECTIVES: Cochlear implant device failures and reimplantation have mainly been reported on in adults. The purpose of this study was to isolate precursors of device failures and assess the effects of reimplantation in children. METHODS: From 1997 to 2003, 27 children underwent cochlear reimplantation at our institution. The pre-failure complaints were categorized and correlated with actual failure modes and postimplantation results. Speech-recognition tests were used to evaluate pre- and post-reimplantation performance. RESULTS: Pre-failure length of usage ranged from 0 to 12 years. Symptoms including pain, intermittence, reduced performance, noise and the need for frequent device adjustments were associated with device failures, although not with a particular mode of failure. Post reimplantation performance was equal to but not immediately better than pre-failure results in children who received upgraded devices. CONCLUSIONS: Specific complaints, frequent need for device adjustments and reduced speech recognition can be precursors to device failures, underscoring the need for routine follow-up evaluations. Reimplantation with newer technology does not guarantee improved speech understanding and often requires an adjustment period to reach pre-reimplantation levels.