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Enlarged vestibular aqueduct (EVA) is the most frequent inner ear abnormality found on computed tomography in children with sensorineural hearing loss. The effects EVA abnormalities have on electrocochleography (ECochG) are unknown. Positive deflections in summation potential evoked by tone bursts were observed in 3/5 subjects, while a large negative deflection, similar to endolymphatic hydrops (EH), was observed for 2/5 subjects. The presence of an enlarged summation potential, with and without a compound action potential, was observed in response to a broadband click stimulus. Results suggest likely effects of a third window on ECochG responses and presence of EH in EVA., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Purpose The goal of this study was to evaluate differences in the electrode-neuron interface as a function of hearing loss etiology in pediatric cochlear implant (CI) listeners with enlarged vestibular aqueduct (EVA) syndrome and in those with autosomal recessive connexin-26 mutations (DFNB1). Method Fifteen implanted ears (9 participants, 5 ears with EVA, 10 ears with DFNB1) were assessed. Single-channel auditory detection thresholds were measured using broad and spatially focused electrode configurations (steered quadrupolar; focusing coefficients = 0 and 0.9). Cochlear resistivity estimates were obtained via electrode impedances and electrical field imaging. Between-group differences were evaluated using linear mixed-effects models. Results Children with EVA had significantly higher auditory detection thresholds than children with DFNB1, irrespective of electrode configuration. Between-group differences in thresholds were more pronounced on apical electrodes than on basal electrodes. In the apex, electrode impedances and electrical field imaging values were higher for children with EVA than for those with DFNB1. Conclusions The electrode-neuron interface differs between pediatric CI listeners with DFNB1 and those with EVA. It is possible that optimal clinical interventions may depend, in part, on hearing loss etiology. Future investigations with large samples should investigate individualized CI programming strategies for listeners with EVA and DFNB1.

Background and Objectives: Patients with SCL26A4 mutations presenting with Mondini deformity and enlarged vestibular aqueduct (EVA) tend to have comparable residual hearing. Although cochlear implantation (CI) produces good results in this group, deterioration of residual hearing can be an adverse event after surgery due to accompanying cochlear malformation and perilymph leakage during cochleostomy. The purpose of this study was to investigate if CI in patients with SCL26A4 mutations via the round window (RW) approach could achieve preservation of residual hearing, and to evaluate their speech reception with electroacoustic stimulation (EAS)., Subjects and Methods: This is a retrospective chart review of eight patients with bilateral EVA, who were bi-allelic patients with SCL26A4 mutations. CI was performed in all patients by a single surgeon using the RW approach. Audiological results were compared before and after implantation., Results: Additional hearing loss after CI was less than 10 dBHL in five out of eight patients. Average hearing deterioration after CI was 8.75 dB (range, 0-26). Six out of eight patients used EAS mode after CI. The acoustic stimulation frequency ranged from 271 to 438 Hz. Patients showed better speech recognition in quiet and in noise using EAS mode compared with electrical stimulation alone., Conclusions: Preservation of residual hearing could be achieved after CI in patients with the SLC26A4 mutation via the RW approach. For successful preservation of residual hearing, application of newly-developed soft electrode and meticulous surgical is necessary. Our study showed that patients with the SLC26A4 mutation can be good candidates for EAS surgery.

Inner ear bone malformations are one cause of profound sensorineural hearing loss. This investigation focused on those affecting the posterior labyrinth, especially enlarged vestibular aqueduct syndrome, which is associated with fluctuating and progressive hearing loss. The objectives of this study were to analyze the behavior of the electrical stimulation, auditory functionality and linguistic development in patients with inner ear malformations involving the posterior labyrinth. The study included ten patients undergoing cochlear implantation (cases: five with enlarged vestibular aqueduct, two with vestibular aqueduct stenosis/aplasia, and three with semicircular canal disorders). Post-implantation, data were gathered on the electrical stimulation threshold and maximum comfort levels and on the number of functioning electrodes. Evaluation of Auditory Responses to Speech (EARS) subtests were used to assess auditory functionality and language acquisition at 6, 12, and 24 months post-implantation. Results were compared with findings in a control group of 28 cochlear implantation patients without these malformations. No significant differences were found between case and control groups in electrical stimulation parameters; auditory functionality subtest scores were lower in cases than controls, although the difference was only statistically significant for some subtests. In conclusion, cochlear implantation patients with posterior labyrinth bone malformations and profound hearing loss, including those with enlarged vestibular aqueduct syndrome, showed no significant difference in electrical stimulation threshold with controls. Although some auditory functionality test results were lower in cases than in controls, cochlear implantation appears to be beneficial for all patients with these malformations.

Objectives: The aim of this study was to assess if large vestibular aqueduct syndrome (LVAS), with the increase in perilymphatic pressure, affects impedance changes over time with different types of Cochlear(®) implant electrode arrays Contour, Straight, and CI 422. To report speech perception outcomes for these cochlear implant recipients., Methods: Retrospective case review of impedance levels and categories of auditory performance. Impedance data were collected at switch on, 1 month, 3, 6, 12, and 24 months after cochlear implantation and compared with control (non-LVAS cochlear implant recipient) data for each array type. Forty-seven patients with exclusive LVAS and no other vestibulocochlear abnormalities or other identifiable cause of deafness were eligible for inclusion in the study., Results: In LVAS patients, there was a significant difference in impedance between the three types of device (P < 0.0001). Time since switch on was associated with a decrease in impedance for all three devices (P < 0.0001). The mean impedance reduced between switch on and 1 month and remained relatively constant thereafter. Sound variation with softening of sounds was seen in four CI 422 (Straight Research Array) recipients due to ongoing fluctuations in electrode compliance., Discussion: For all three array types, there was no significant difference in the mean impedance between the LVAS patients and controls over the first 12 months. In keeping with previous studies cochlear implant recipients with LVAS hear very well through the cochlear implant.

Objective: To evaluate the hearing and speech recognition in patients with large vestibular aqueduct syndrome undergoing multi-channel cochlear implantation., Methods: From 2005 to 2008, multi-channel cochlear implantation surgeries were performed in 22 patients with large vestibular aqueduct syndrome. All the patients received multi-channel cochlear implantation through transmastoid facial recess approach. The postoperative outcomes of the patients were compared with those of 22 patients with non-malformed cochlear., Results: Severe gusher occurred in 3 cases during the implantation, and perilymph fluctuation was found in another 15 cases, with a rate of anomalies of 81.8%. All the electrodes were totally inserted into the cochlear, and no facial paralysis or cerebrospinal fluid leakage occurred after the operation. The hearing threshold in these patients was similar to that in patients with normal cochlear receiving the implantation. After speech rehabilitation for over 6 months, all the patients showed improved hearing and verbal ability., Conclusion: Multi-channel cochlear implantation can be performed in patients with large vestibular aqueduct syndrome, but preoperative hearing assessment and radiographic examinations should be performed.

Objective: To investigate and compare the usefulness of preoperative magnetic resonance (MR) imaging and high-resolution temporal bone computed tomography (HRCT) in pediatric cochlear implant candidates., Study Design: Prospective., Setting: Tertiary referral center., Patients: A cohort of 92 pediatric patients with profound hearing. Inclusion criteria were MR, computed tomography, and cochlear implantation. INTERVENTION., Diagnostic: All patients had preoperative imaging of the petrous temporal bone (HRCT, T2-weighted fast spin echo, axial 3D Fast Imaging Employing Steady-state Acquisition [FIESTA] MR) and brain (Fast Fluid-attenuated Inversion-recovery [FLAIR] MR)., Main Outcome Measure(s): Overall prevalence of inner ear dysplasias in this population and comparison of detection rates between HRCT, T2 Fast Spin Echo (FSE), and FIESTA MR sequences., Results: Radiological abnormalities were observed in 32 and 59% of MR and HRCT temporal bone, respectively. Synchronous intracranial findings were noted in 40% on brain MR. Common vestibulocochlear nerve was observed in 3% ears and directed side of implantation. Consistent discrepancies noted on HRCT were inability to diagnose early obliterative labyrinthitis and presence of the cochlear nerve in the internal auditory canal. With respect to MR, enlarged vestibular aqueducts and narrow cochlear nerve canals were consistently under identified., Conclusion: Dual-modality imaging with HRCT and MR of petrous bone and MR brain in the precochlear implant pediatric population detects abnormalities related to deafness, which would not otherwise be found using either modality alone. There is overlap between the imaging modalities in the type of abnormalities detected, and we present a case for selective use of HRCT within a diagnostic algorithm, using the patient risk factors we have identified.

Objectives: In this investigation, we report the outcomes of 14 adults (age >18 y) and 9 children (age <18 y) with radiographically proven large vestibular aqueduct syndrome (LVAS) who received cochlear implants at Indiana University School of Medicine., Study Design: This is a retrospective case-control study detailing the outcomes of 23 patients with LVAS and 46 control patients implanted with Nucleus (Cochlear Corp., Englewood, CO), Clarion (Advanced Bionics Corp., Sylmar, CA), or Med-El (MED-EL Corp., Innsbruck, Austria) cochlear implants., Methods: Performance data on pure-tone averages, speech detection thresholds, and a variety of auditory and speech recognition tasks from these patients with LVAS were compared with performance data obtained from a matched group of 46 cochlear implant users who did not have LVAS. Specific patient characteristics used in matching included the age of the patient, the age at implant of the patient, and whether the patient was pre- or postlingually deafened. Data for the adult group was analyzed using the Student t test, and data for the pediatric patient group was compared using a chi2 test., Results: The results indicated positive outcomes for both pediatric and adult groups. With both adult and pediatric groups, auditory and speech recognition performance did not differ significantly between those patients with LVAS and control subjects., Conclusions: This study adds further support for the use of cochlear implantation in patients with LVAS. Cochlear implantation is beneficial and can be offered as an eventual treatment of LVAS if hearing loss progresses to profound levels in these patients.