Auditory Brainstem Implant Outcomes in Tumor and Nontumor Patients: A Systematic Review.

OBJECTIVE: To elucidate the differences in auditory performance between auditory brainstem implant (ABI) patients with tumor or nontumor etiologies. DATA SOURCES: PubMed, Embase, and Web of Science Core Collection from 1990 to 2021. REVIEW METHODS: We included published studies with 5 or more pediatric or adult ABI users. Auditory outcomes and side effects were analyzed with weighted means for closed-set, open-set speech, and categories of auditory performance (CAP) scores. Overall performance was compared using an Adult Pediatric Ranked Order Speech Perception (APROSPER) scale created for this study. RESULTS: Thirty-six studies were included and underwent full-text review. Data were extracted for 662 tumor and 267 nontumor patients. 83% were postlingually deafened and 17% were prelingually deafened. Studies that included tumor ABI patients had a weighted mean speech recognition of 39.2% (range: 19.6%-83.3%) for closed-set words, 23.4% (range: 17.2%-37.5%) for open-set words, 21.5% (range: 2.7%-48.4%) for open-set sentences, and 3.1 (range: 1.0-3.2) for CAP scores. Studies including nontumor ABI patients had a weighted mean speech recognition of 79.8% (range: 31.7%-84.4%) for closed-set words, 53.0% (range: 14.6%-72.5%) for open-set sentences, and 2.30 (range: 2.0-4.7) for CAP scores. Mean APROSPER results indicate better auditory performance among nontumor versus tumor patients (3.5 vs 3.0, P = .04). Differences in most common side effects were also observed between tumor and nontumor ABI patients. CONCLUSION: Auditory performance is similar for tumor and nontumor patients for standardized auditory test scores. However, the APROSPER scale demonstrates better ABI performance for nontumor compared to tumor patients.

Characterization and Clinical Use of Bone Conduction Transducers at Extended High Frequencies.

Measurement of bone conduction (BC) hearing thresholds at extended high frequencies (EHF; above 8 kHz) is of clinical interest but is technically complicated by limitations in standard BC transducer output, a lack of calibration standards and sparse clinical data from human subjects. A recently described calibration scheme using an artificial mastoid and interposed accelerometer is applied in this study to characterize and compare acceleration and computed force outputs over the 4-20 kHz range of two standard BC transducers: the RadioEar® B71 and B81, as well as two non-standard, commercially available BC transducers: the Tascam® HP-F200 and the Aftershokz® AS400. Measures of linear output growth, harmonic distortion and acoustic radiation are assessed and compared across devices. A maximum linear input voltage is established for each BC transducer using measurements of linear output growth and total harmonic distortion. At maximum linear input level, the Tascam shows superior force output by 25 to 40 dB above 8 kHz and the widest dynamic EHF range. Acoustic radiation per output force was lowest for the Tascam, whereas the AS400 behaved more like an air conduction earphone than a force generator. In a cohort of 15 normal hearing volunteers, BC thresholds, measured with the Tascam and reported in dB re 1 rms µN, were consistent with historical measures of EHF BC thresholds in similar subjects using an alternative BC transducer.

A Modified Pediatric Ranked Order Speech Perception Score to Assess Speech Recognition Development in Children With Cochlear Implants.

PURPOSE: Characterizing and comparing speech recognition development in children with cochlear implants (CIs) is challenging because of variations in test type. This retrospective cohort study modified the Pediatric Ranked Order Speech Perception (PROSPER) scoring system to (a) longitudinally analyze the speech perception of children with CIs and (b) examine the role of age at CI activation, listening mode (i.e., unilateral or bilateral implantation), and interimplant interval. METHOD: Postimplantation speech recognition scores from 31 children with prelingual, severe-to-profound hearing loss who received CIs were analyzed (12 with unilateral CI [UniCI], 13 with sequential bilateral CIs [SEQ BiCIs], and six with simultaneous BiCIs). Data were extracted from the Massachusetts Eye and Ear Audiology database. A version of the PROSPER score was modified to integrate the varying test types by mapping raw scores from different tests into a single score. The PROSPER scores were used to construct speech recognition growth curves of the implanted ears, which were characterized by the slope of the growth phase, the time from activation to the plateau onset, and the score at the plateau. RESULTS: While speech recognition improved considerably for children following implantation, the growth rates and scores at the plateau were highly variable. In first implanted ears, later implantation was associated with poorer scores at the plateau (ß = -0.15, p = .01), but not growth rate. The first implanted ears of children with BiCIs had better scores at the plateau than those with UniCI (ß = 0.59, p = .02). Shorter interimplant intervals in children with SEQ BiCIs promoted faster speech recognition growth of the first implanted ears. CONCLUSION: The modified PROSPER score could be used clinically to track speech recognition development in children with CIs, to assess influencing factors, and to assist in developing and evaluating patient-specific intervention strategies. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.20113538.

The Relationship Between Impedance, Programming and Word Recognition in a Large Clinical Dataset of Cochlear Implant Recipients.

Cochlear implant programming typically involves measuring electrode impedance, selecting a speech processing strategy and fitting the dynamic range of electrical stimulation. This study retrospectively analyzed a clinical dataset of adult cochlear implant recipients to understand how these variables relate to speech recognition. Data from 425 implanted post-lingually deafened ears with Advanced Bionics devices were analyzed. A linear mixed-effects model was used to infer how impedance, programming and patient factors were associated with monosyllabic word recognition scores measured in quiet. Additional analyses were conducted on subsets of data to examine the role of speech processing strategy on scores, and the time taken for the scores of unilaterally implanted patients to plateau. Variation in basal impedance was negatively associated with word score, suggesting importance in evaluating the profile of impedance. While there were small, negative bivariate correlations between programming level metrics and word scores, these relationships were not clearly supported by the model that accounted for other factors. Age at implantation was negatively associated with word score, and duration of implant experience was positively associated with word score, which could help to inform candidature and guide expectations. Electrode array type was also associated with word score. Word scores measured with traditional continuous interleaved sampling and current steering speech processing strategies were similar. The word scores of unilaterally implanted patients largely plateaued within 6-months of activation. However, there was individual variation which was not related to initially measured impedance and programming levels.

Cochlear Implantation Hearing Outcome in Ménière's Disease.

OBJECTIVE: The aim of this study was to evaluate the hearing outcome of cochlear implantation in patients deafened by Ménière's disease. STUDY DESIGN: Retrospective single-institution study. SETTING: Tertiary medical center. METHODS: Our institutional database of 1400 patients with cochlear implants was reviewed to identify cases with deafness due to Ménière's disease. Twenty-nine patients were identified: 24 with unilateral and 5 with sequential bilateral cochlear implants. Pre- and postoperative speech recognition scores and medical data were extracted from the medical record and analyzed. RESULTS: Overall the mean difference between pre- and postoperative speech recognition after >1 year was 56% (95% CI, 47.08%-64.92%). The mean preoperative monosyllabic word score was 9.5%, and the mean postoperative scores at 1 month, 3 months, 6 months, 1 year, and >1 year were 37.1%, 46.1%, 54.1%, 59.1%, and 66.8%, respectively. Cochlear implantation resulted in improved word scores in all patients regardless of prior medical or surgical treatment (endolymphatic sac, labyrinthectomy). The mean postoperative hearing improvement in patients aged <70 and ≥70 years was 65.26% (95% CI, 54.79%-75.73%) and 40.00% (95% CI, 27.22%-52.77%). Postoperative word scores in patients with bilateral cochlear implants were not significantly different between the first and second implanted ears or between the monoaural and binaural testing conditions. CONCLUSION: Cochlear implant in patients deafened by Ménière's disease significantly improves word recognition scores regardless of whether medical or surgical treatment is used prior to implantation. The potential improvement in word recognition scores decreases after age 70 years.

Congenital Cytomegalovirus Targeted Screening Implementation and Outcomes: A Retrospective Chart Review.

OBJECTIVE: To assess the effectiveness and outcomes of a targeted cytomegalovirus (CMV) testing protocol. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care institution. METHODS: Targeted screening for CMV in children who did not pass the newborn hearing screening (NHS) was introduced in July 2015 for the level 2 and 3 nurseries at our hospital. In January 2016, this policy was extended to include all nurseries. Retrospective chart review was performed for all newborns who did not pass their NHS between 2013 and 2020. CMV testing rates and related outcomes were compared before and after rollout. RESULTS: A total of 891 charts were reviewed for infants who did not pass their NHS: 530 (60%) had CMV testing, of which 8 (1.5%) tested positive. Three cases were detected prior to and 5 occurred after initiation of targeted screening. Six CMV+ infants demonstrated hearing loss on confirmatory auditory brainstem response, all of whom began treatment with oral valganciclovir. Hearing remained stable in 3 patients, progressed in 2, and improved in 1. The rate of CMV testing in children who did not pass their NHS increased from 14% to 88% after full implementation of targeted screening (P < .001). The average age at initial infectious disease consultation was significantly younger for infants born after targeted screening (P < .001). CONCLUSION: Targeted screening is a feasible and effective method to identify CMV+ infants early in life. Implementation of a targeted screening program for CMV in children who do not pass the NHS resulted in significantly higher rates of CMV testing and earlier referral to infectious disease.

Adult Auditory Brainstem Implant Outcomes and Three-Dimensional Electrode Array Position on Computed Tomography.

OBJECTIVES: Factors contributing to auditory brainstem implant (ABI) outcomes are poorly understood. The aims of this study are to (1) characterize ABI electrode array position on postoperative imaging and (2) determine if variability in position is related to perceptual outcomes. DESIGN: Retrospective cohort study. Subjects were selected from the adult ABI recipient population at Massachusetts Eye and Ear. Postoperative three-dimensional (3D) computed tomography (CT) reconstruction of the head was used to measure ABI array position in 20 adult ABI recipients (17 with Neurofibromatosis Type 2 (NF2) and three non-NF2 recipients). Three-dimensional electrode array position was determined based on angles from the horizontal using posterior and lateral views and on distances between the proximal array tip superiorly from the basion (D1), laterally (D2P) and posteriorly (D2L) from the midline. Array position was correlated with perceptual data (in 15 of the 20 recipients who used their ABI). Perceptual data included the number of electrodes that provided auditory sensation, location and type of side effects, level of speech perception (from no sound to open-set word recognition of monosyllables) and the amount of charge required for auditory perception. RESULTS: Although the 3D orientation of the ABI array exhibited a variety of angles, all arrays were posteriorly tilted from the lateral view and most were medially tilted from the posterior view. ABI position relative to the basion from posterior showed mean distances of 1.71 ± 0.42 and 1.1 ± 0.29 cm for D1 and D2, respectively, and a mean D2 of 1.30 ± 0.45 cm from the lateral view. A strong linear negative correlation was found between the number of active electrodes and the distance of the proximal array tip laterally from the basion (D2P; rs = -0.73, p = 0.006) when measured in the posterior view. Although side effects were experienced in all recipients and varied in type and location across the array, electrodes in the middle part of the array tended to elicit auditory sensations while the proximal and distal tips of the array tended to elicit nonauditory side effects. Arrays with and without low charge thresholds appeared to generally overlap in position. However, the two recipients with the best (open-set) speech perception had low charge thresholds and had arrays that were tilted superiorly in the posterior view. CONCLUSION: ABI recipients with better speech perception appear to share a profile of arrays that are tilted superiorly as compared to recipients with lower speech perception levels. These ABI recipients have a high number of active electrodes (10 or more) and require less electrical charge on individual electrodes to achieve optimal stimulation.

Optimized Diagnostic Approach to Patients Suspected of Superior Semicircular Canal Dehiscence.

OBJECTIVES: Current methods of diagnosing superior semicircular canal dehiscence syndrome (SCDS) include a clinical exam, audiometric testing, temporal bone computer tomography (CT) imaging, and vestibular evoked myogenic potential (VEMP) testing. The main objective of this study was to develop an improved diagnostic approach to SCDS optimized for accuracy, efficiency, and safety that utilizes clinical presentation, audiometric testing, CT imaging, high-frequency cervical VEMP (cVEMP) testing, and patient treatment preference. A secondary aim was to investigate the cost associated with the current versus proposed diagnostic paradigms. DESIGN: All patients who underwent cVEMP testing since introduction of the 2 kHz cVEMP in our clinical protocol in July 2018 were screened. Patients suspected of SCDS based upon symptoms who also had available audiogram, CT scan, and 2 kHz cVEMP were included (58 ears). Patients were categorized as dehiscent, thin, or not dehiscent based on their CT scan. Symptom prevalence and cVEMP outcomes were analyzed and compared for all groups. The accuracy of the 2 kHz cVEMP was calculated using CT imaging as the standard. Using a combination of patient symptomatology, audiometric, CT and 2 kHz cVEMP data, as well as patient preference, a best clinical practice approach was developed. The cost associated with this approach was calculated and compared with cost of the current SCDS diagnostic workup using Medicare reimbursement rates. RESULTS: In the overall patient population suspected of SCDS based on clinical presentation, the sensitivity and specificity of 2 kHz cVEMP were 76% and 100%, respectively, while the positive and negative predictive values were 100% and 84.6%, assuming that the CT scan finding was correct. Autophony was the most common symptom in patients who had both superior semicircular canal dehiscence on CT imaging plus abnormal 2 kHz cVEMP (p < 0.001). Combining patient symptomatology, 2 kHz normalized peak to peak cVEMP amplitude, and patient treatment preference to determine, which patients should undergo CT scanning resulted in a potential cost reduction between 45% and 61%. CONCLUSION: In patients suspected of SCDS based on their clinical presentation, the combination of symptomatology, 2 kHz cVEMP data, and patient preference can be used to determine which patients should undergo CT scanning, resulting in a diagnostic cost reduction and reduced patient radiation exposure.

Effect of anesthesia on evoked auditory responses in pediatric auditory brainstem implant surgery.

OBJECTIVE: Electrically evoked auditory brainstem responses (EABR) guide placement of the multichannel auditory brainstem implant (ABI) array during surgery. EABRs are also recorded under anesthesia in nontumor pediatric ABI recipients prior to device activation to confirm placement and guide device programming. We examine the influence of anesthesia on evoked response morphology in pediatric ABI users by comparing intraoperative with postoperative EABR recordings. STUDY DESIGN: Retrospective review. METHODS: Seven children underwent ABI surgery by way of retrosigmoid craniotomy. General anesthesia included inhaled sevoflurane induction and propofol maintenance during which EABRs were recorded to confirm accurate positioning of the ABI. A mean of 7.7 ± 2.8 weeks following surgery, the ABI was activated under general anesthesia or sedation (dexmedetomidine) and EABR recordings were made. A qualitative analysis of intraoperative and postoperative waveform morphology was performed. RESULTS: Seven subjects (mean age 20.6 months) underwent nine ABI surgeries (seven primary, two revisions) and nine activations. EABRs were observed in eight of nine postoperative recordings. In three cases, intraoperative EABRs during general anesthesia were similar to postoperative EABRs with sedation. In one case, sevoflurane and propofol were used for intra- and postoperative recordings, and waveforms were also similar. In four cases, amplitude and latency changes were observed for intraoperative versus postoperative EABRs. CONCLUSION: Similarity of EABR morphology in the anesthetized versus sedated condition suggests that anesthesia does not have a large effect on far-field evoked potentials. Changes in EABR waveform morphology observed postoperatively may be influenced by other factors such as movements of the surface array. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:507-513, 2020.

Predicting Development of Bilateral Menière's Disease Based on cVEMP Threshold and Tuning.

OBJECTIVE: To investigate if the cervical vestibular evoked myogenic potential (cVEMP) is predictive for developing bilateral Menière's disease (MD). STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary care center. PATIENTS: Records of 71 patients previously diagnosed with unilateral MD at our institution who underwent cVEMP testing between 2002 and 2011 were screened. INTERVENTION: Patients were contacted to answer a questionnaire to identify which patients had developed bilateral disease. Based on questionnaires and medical charts, 49 patients with a follow-up time of at least 5 years were included. The 49 originally asymptomatic ears are referred to as "study ears." Previously reported cVEMP criteria (original criteria) applied to study-ear cVEMPs separated them into Menière-like and normal-like groups. MAIN OUTCOME MEASURE: The main purpose was to determine if previously obtained cVEMP thresholds and tuning ratios of unilateral MD patients could predict who develops bilateral disease. RESULTS: From the 49 included patients, 12 developed bilateral disease (24.5%). The study ears characterized by original cVEMP criteria as Menière-like were significantly more likely to develop bilateral disease compared with the normal-like study ears. The original criteria predicted development of bilateral disease with a positive predictive value (PPV) and negative predictive value (NPV) of 58.3% and 86.5% respectively. ROC curves were used to revise cVEMP criteria for predicting the progression to bilateral disease. A revised criterion combining three cVEMP metrics, reached a PPV and NPV of 85.7% and 93.7%. CONCLUSION: cVEMP threshold and tuning in unilateral MD patients are predictive of which patients will develop bilateral disease.

Cervical Vestibular Evoked Myogenic Potentials in Menière's Disease: A Comparison of Response Metrics.

OBJECTIVE: The cervical vestibular evoked myogenic potential (cVEMP) has been used to evaluate patients with Menière's disease (MD). Studied cVEMP metrics include: amplitude, threshold, frequency tuning, and interaural asymmetry ratio (IAR). However, few studies compared these metrics in the same set of MD patients, and methodological differences prevent such a comparison across studies. This study investigates the value of different cVEMP metrics in distinguishing one set of MD patients from age-matched controls. STUDY DESIGN: Prospective study. SETTING: Tertiary care center. PATIENTS: Thirty patients with definite unilateral MD and 23 age-matched controls were prospectively included. All underwent cVEMP testing at 500, 750, 1000, and 2000 Hz on each side. Ears were separated into three groups: affected MD, unaffected MD, and control. MAIN OUTCOME MEASURES: Sound level functions were obtained at each frequency, and normalized peak-to-peak amplitude (VEMPn), VEMP inhibition depth (VEMPid), threshold, frequency-tuning ratio, and IAR were calculated. For all metrics, the differentiation between MD and control ears was compared using receiver operating characteristic (ROC) curves. RESULTS: 500 Hz cVEMP threshold, VEMPn, and VEMPid were similarly good at distinguishing affected MD ears from healthy ears, with ROC area under the curves (AUCs) of more than 0.828 and optimal sensitivities and specificities of at least 80 and 70%. Combinations of these three metrics yielded slightly larger AUCs (>0.880). Tuning ratios and IAR were less effective in separating healthy from affected ears with AUCs ranging from 0.529 to 0.720. CONCLUSION: The cVEMP metrics most useful in distinguishing MD patients from healthy controls are threshold, VEMPn, and VEMPid, using 500 Hz stimuli.

Normalizing cVEMPs: Which Method Is the Most Effective?

OBJECTIVES: To determine the most effective method for normalizing cervical vestibular evoked myogenic potentials (cVEMPs). DESIGN: cVEMP data from 20 subjects with normal hearing and vestibular function were normalized using 16 combinations of methods, each using one of the 4 modes of electromyogram (EMG) quantification described below. All methods used the peak to peak value of an averaged cVEMP waveform (VEMPpp) and obtained a normalized cVEMP by dividing VEMPpp by a measure of the EMG amplitude. EMG metrics were obtained from the EMG within short- and long-duration time windows. EMG amplitude was quantified by its root-mean-square (RMS) or average full-wave-rectified (RECT) value. The EMG amplitude was used by (a) dividing each individual trace by the EMG of this specific trace, (b) dividing VEMPpp by the average RMS or RECT of the individual trace EMG, (c) dividing the VEMPpp by an EMG metric obtained from the average cVEMP waveform, or (d) dividing the VEMPpp by an EMG metric obtained from an average cVEMP "noise" waveform. Normalization methods were compared by the normalized cVEMP coefficient of variation across subjects and by the area under the curve from a receiver-operating-characteristic analysis. A separate analysis of the effect of EMG-window duration was done. RESULTS: There were large disparities in the results from different normalization methods. The best methods used EMG metrics from individual-trace EMG measurements, not from part of the average cVEMP waveform. EMG quantification by RMS or RECT produced similar results. For most EMG quantifications, longer window durations were better in producing receiver-operating-characteristic with high areas under the curve. However, even short window durations worked well when the EMG metric was calculated from the average RMS or RECT of the individual-trace EMGs. Calculating the EMG from a long-duration window of a cVEMP "noise" average waveform was almost as good as the individual-trace-EMG methods. CONCLUSIONS: The best cVEMP normalizations use EMG quantification from individual-trace EMGs. To have the normalized cVEMPs accurately reflect the vestibular activation, a good normalization method needs to be used.

Audiometric and cVEMP Thresholds Show Little Correlation With Symptoms in Superior Semicircular Canal Dehiscence Syndrome.

OBJECTIVE: Evaluate the relationship between objective audiometric and vestibular tests and patient symptoms in superior canal dehiscence (SCD) syndrome. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care center. PATIENTS: Ninety-eight patients with SCD, preoperative threshold audiograms, cervical vestibular evoked myogenic potential (cVEMP) thresholds, and computed tomography (CT) imaging were included. Clinical reports were reviewed for self-reported SCD symptoms. Twenty-five patients completed the Hearing Handicap Inventory (HHI), Dizziness Handicap Inventory (DHI), Autophony Index (AI), and the 36-item Short Form Survey (SF-36). MAIN OUTCOME MEASURES: Correlations between preoperative low-frequency air-bone gap (ABG), cVEMP thresholds, and symptoms (including HHI, DHI, AI, and SF-36). Symptoms included hearing loss, aural fullness, autophony, hyperacusis, tinnitus, vertigo, imbalance and sound-, pressure and exercise provoked dizziness. Secondary outcome measure: Correlations between changes of objective and subjective measures before and after surgery. RESULTS: Patients who reported hearing loss had larger ABGs at 250 Hz than patients without subjective hearing loss (p = 0.001). ABGs and cVEMP thresholds did not correlate with any other symptom. No significant correlation was found between ABG or cVEMP threshold and the HHI, DHI, AI or Health Utility Value (derived from the SF-36 quality of life score). Following SCD surgery, ABG decreased (p < 0.001), cVEMP thresholds increased (p < 0.001) and overall symptoms, handicap scores and quality-of-life improved; however, there was no significant relationship between these measures. CONCLUSION: While threshold audiometry and cVEMP are important tools to diagnose SCD and monitor surgical outcomes, these measures showed no significant correlation with vestibular and most auditory symptoms or their severity.

Toward Optimizing VEMP: Calculating VEMP Inhibition Depth With a Generic Template.

OBJECTIVES: Cervical vestibular evoked myogenic potentials (cVEMP) indirectly reveal the response of the saccule to acoustic stimuli through the inhibition of sternocleidomastoid muscle electromyographic response. VEMP inhibition depth (VEMPid) is a recently developed metric that estimates the percentage of saccular inhibition. VEMPid provides both normalization and better accuracy at low response levels than amplitude-normalized cVEMPs. Hopefully, VEMPid will aid in the clinical assessment of patients with vestibulopatholgy. To calculate VEMPid a template is needed. In the original method, a subject's own cVEMP was used as the template, but this method can be problematic in patients who do not have robust cVEMP responses. We hypothesize that a "generic" template, created by assembling cVEMPs from healthy subjects, can be used to compute VEMPid, which would facilitate the use of VEMPid in subjects with pathological conditions. DESIGN: A generic template was created by averaging cVEMP responses from 6 normal subjects. To compare VEMPid calculations using a generic versus a subject-specific template, cVEMPs were obtained in 40 healthy subjects using 500, 750, and 1000 Hz tonebursts at sound levels ranging from 98 to 123 dB peSPL. VEMPids were calculated both with the generic template and with the subject's own template. The ability of both templates to determine whether a cVEMP was present or not was compared with receiver operating characteristic curves. RESULTS: No significant differences were found between VEMPid calculations using a generic template versus using a subject-specific template for all frequencies and sound levels. Based on the receiver operating characteristic curves, the subject-specific and generic template did an equally good job at determining threshold. Within limits, the shape of the generic template did not affect these results. CONCLUSIONS: A generic template can be used instead of a subject-specific template to calculate VEMPid. Compared with cVEMP normalized by electromyographic amplitudes, VEMPid is advantageous because it averages zero when there is no sound stimulus and it allows the accumulating VEMPid value to be shown during data acquisition as a guide to deciding when enough data has been collected.

Toward Optimizing Cervical Vestibular Evoked Myogenic Potentials (cVEMP): Combining Air-Bone Gap and cVEMP Thresholds to Improve Diagnosis of Superior Canal Dehiscence.

OBJECTIVE: To develop a novel approach combining low-frequency air-bone gap (ABG) and cervical vestibular evoked myogenic potential (cVEMP) thresholds to improve screening for superior canal dehiscence (SCD) syndrome. STUDY DESIGN: Retrospective study. SETTING: Tertiary care center. PATIENTS: One hundred forty patients with SCD and 21 healthy age-matched controls were included. Ears for each patient were divided into three groups based on computed tomography (CT) findings: 1) dehiscent, 2) thin, or 3) unaffected. MAIN OUTCOME MEASURES: cVEMP and audiometric thresholds were analyzed and differences among groups were evaluated. RESULTS: We define the third window indicator (TWI) as the cVEMP thresholds at 500, 750, and 1000 Hz adjusted for the ABG at 250 Hz (i.e., subtracting ABG from cVEMP threshold). The TWI differentiates between dehiscent and nondehiscent control ears with a sensitivity of 82% and specificity of 100%, corresponding to a positive predictive value of 100%. ABGs and cVEMP thresholds were similar for healthy controls and patients with thin bone over the superior canal. CONCLUSION: This is the largest study to date examining the usefulness of cVEMPs in the diagnosis of SCD. Our "third window indicator" (TWI) combines cVEMP thresholds with the ABG at 250 Hz to improve the ability to screen patients with SCD symptoms.

Toward Optimizing cVEMP: 2,000-Hz Tone Bursts Improve the Detection of Superior Canal Dehiscence.

BACKGROUND: The cervical vestibular evoked myogenic potential (cVEMP) test measures saccular and inferior vestibular nerve function. The cVEMP can be elicited with different frequency stimuli and interpreted using a variety of metrics. Patients with superior semicircular canal dehiscence (SCD) syndrome generally have lower cVEMP thresholds and larger amplitudes, although there is overlap with healthy subjects. The aim of this study was to evaluate which metric and frequency best differentiate healthy ears from SCD ears using cVEMP. METHODS: Twenty-one patients with SCD and 23 age-matched controls were prospectively included and underwent cVEMP testing at 500, 750, 1,000 and 2,000 Hz. Sound level functions were obtained at all frequencies to acquire threshold and to calculate normalized peak-to-peak amplitude (VEMPn) and VEMP inhibition depth (VEMPid). Third window indicator (TWI) metrics were calculated by subtracting the 250-Hz air-bone gap from the ipsilateral cVEMP threshold at each frequency. Ears of SCD patients were divided into three groups based on CT imaging: dehiscent, thin or unaffected. The ears of healthy age-matched control subjects constituted a fourth group. RESULTS: Comparing metrics at all frequencies revealed that 2,000-Hz stimuli were most effective in differentiating SCD from normal ears. ROC analysis indicated that for both 2,000-Hz cVEMP threshold and for 2,000-Hz TWI, 100% specificity could be achieved with a sensitivity of 92.0%. With 2,000-Hz VEMPn and VEMPid at the highest sound level, 100% specificity could be achieved with a sensitivity of 96.0%. CONCLUSION: The best diagnostic accuracy of cVEMP in SCD patients can be achieved with 2,000-Hz tone burst stimuli, regardless of which metric is used.

Histopathology of the Human Inner Ear in the Cogan Syndrome with Cochlear Implantation.

The Cogan syndrome is a rare disorder characterized by nonsyphilitic interstitial keratitis and audiovestibular symptoms. Profound sensorineural hearing loss has been reported in approximately half of the patients with the Cogan syndrome resulting in candidacy for cochlear implantation in some patients. The current study is the first histopathologic report on the temporal bones of a patient with the Cogan syndrome who during life underwent bilateral cochlear implantation. Preoperative MRI revealed tissue with high density in the basal turns of both cochleae and both vestibular systems consistent with fibrous tissue due to labyrinthitis. Histopathology demonstrated fibrous tissue and new bone formation within the cochlea and vestibular apparatus, worse on the right. Severe degeneration of the vestibular end organs and new bone formation in the labyrinth were seen more on the right than on the left. Although severe bilateral degeneration of the spiral ganglion neurons was seen, especially on the right, the postoperative word discrimination score was between 50 and 60% bilaterally. Impedance measures were generally higher in the right ear, possibly related to more fibrous tissue and new bone found in the scala tympani on the right side.

Auditory Brainstem Implant Array Position Varies Widely Among Adult and Pediatric Patients and Is Associated With Perception.

OBJECTIVES: The auditory brainstem implant (ABI) provides sound awareness to patients who are ineligible for cochlear implantation. Auditory performance varies widely among similar ABI cohorts. We hypothesize that differences in electrode array position contribute to this variance. Herein, we classify ABI array position based on postoperative imaging and investigate the relationship between position and perception. DESIGN: Retrospective review of pediatric and adult ABI users with postoperative computed tomography. To standardize views across subjects, true axial reformatted series of scans were created using the McRae line. Using multiplanar reconstructions, basion and electrode array tip coordinates and array angles from vertical were measured. From a lateral view, array angles (V) were classified into types I to IV, and from posterior view, array angles (T) were classified into types A to D. Array position was further categorized by measuring distance vertical from basion (D1) and lateral from midline (D2). Differences between array classifications were compared with audiometric thresholds, number of active electrodes, and pitch ranking. RESULTS: Pediatric (n = 4, 2 with revisions) and adult (n = 7) ABI subjects were included in this study. Subjects had a wide variety of ABI array angles, but most were aimed superiorly and posteriorly (type II, n = 7) from lateral view and upright or medially tilted from posterior view (type A, n = 6). Mean pediatric distances were 8 to 42% smaller than adults for D1 and D2. In subjects with perceptual data, electrical thresholds and the number of active electrodes differed among classification types. CONCLUSIONS: In this first study to classify ABI electrode array orientation, array position varied widely. This variability may explain differences in auditory performance.

The pattern and degree of capsular fibrous sheaths surrounding cochlear electrode arrays.

An inflammatory tissue reaction around the electrode array of a cochlear implant (CI) is common, in particular at the electrode insertion region (cochleostomy) where mechanical trauma often occurs. However, the factors determining the amount and causes of fibrous reaction surrounding the stimulating electrode, especially medially near the perimodiolar location, are unclear. Temporal bone (TB) specimens from patients who had undergone cochlear implantation during life with either Advanced Bionics (AB) Clarion ™ or HiRes90K™ (Sylmar, CA, USA) devices that have a half-band and a pre-curved electrode, or Cochlear ™ Nucleus (Sydney, Australia) device that have a full-band and a straight electrode were evaluated. The thickness of the fibrous tissue surrounding the electrode array of both types of CI devices at both the lower (LB) and upper (UB) basal turns of the cochlea was quantified at three locations: the medial, inferior, and superior aspects of the sheath. Fracture of the osseous spiral lamina and/or marked displacement of the basilar membrane were interpreted as evidence of intracochlear trauma. In addition, post-operative word recognition scores, duration of implantation, and post-operative programming data were evaluated. Seven TBs from six patients implanted with AB devices and five TBs from five patients implanted with Nucleus devices were included. A fibrous capsule around the stimulating electrode array was present in all twelve specimens. TBs implanted with AB device had a significantly thicker fibrous capsule at the medial aspect than at the inferior or superior aspects at both locations (LB and UB) of the cochlea (Wilcoxon signed-ranks test, p < 0.01). TBs implanted with a Nucleus device had no difference in the thickness of the fibrous capsule surrounding the track of the electrode array (Wilcoxon signed-ranks test, p > 0.05). Nine of fourteen (64%) basal turns of the cochlea (LB and UB of seven TBs) implanted with AB devices demonstrated intracochlear trauma compared to two of ten (20%) basal turns of the cochlea (LB and UB of five TBs) with Nucleus devices, (Fisher exact test, p < 0.05). There was no significant correlation between the thickness of the fibrous tissue and the duration of implantation or the word recognition scores (Spearman rho, p = 0.06, p = 0.4 respectively). Our outcomes demonstrated the development of a robust fibrous tissue sheath medially closest to the site of electric stimulation in cases implanted with the AB device electrode, but not in cases implanted with the Nucleus device. The cause of the asymmetric fibrous sheath may be multifactorial including insertional trauma, a foreign body response, and/or asymmetric current flow.

Toward Optimizing Vestibular Evoked Myogenic Potentials: Normalization Reduces the Need for Strong Neck Muscle Contraction.

BACKGROUND: The cervical vestibular evoked myogenic potential (cVEMP) represents an inhibitory reflex of the saccule measured in the ipsilateral sternocleidomastoid muscle (SCM) in response to acoustic or vibrational stimulation. Since the cVEMP is a modulation of SCM electromyographic (EMG) activity, cVEMP amplitude is proportional to muscle EMG amplitude. We sought to evaluate muscle contraction influences on cVEMP peak-to-peak amplitudes (VEMPpp), normalized cVEMP amplitudes (VEMPn), and inhibition depth (VEMPid). METHODS: cVEMPs at 500 Hz were measured in 25 healthy subjects for 3 SCM EMG contraction ranges: 45-65, 65-105, and 105-500 µV root mean square (r.m.s.). For each range, we measured cVEMP sound level functions (93-123 dB peSPL) and sound off, meaning that muscle contraction was measured without acoustic stimulation. The effect of muscle contraction amplitude on VEMPpp, VEMPn, and VEMPid and the ability to distinguish cVEMP presence/absence were evaluated. RESULTS: VEMPpp amplitudes were significantly greater at higher muscle contractions. In contrast, VEMPn and VEMPid showed no significant effect of muscle contraction. Cohen's d indicated that for all 3 cVEMP metrics contraction amplitude variations produced little change in the ability to distinguish cVEMP presence/absence. VEMPid more clearly indicated saccular output because when no acoustic stimulus was presented the saccular inhibition estimated by VEMPid was zero, unlike those by VEMPpp and VEMPn. CONCLUSION: Muscle contraction amplitude strongly affects VEMPpp amplitude, but contractions 45-300 µV r.m.s. produce stable VEMPn and VEMPid values. Clinically, there may be no need for subjects to exert high contraction effort. This is especially beneficial in patients for whom maintaining high SCM contraction amplitudes is challenging.