The relationship of cognitive dysfunction and vestibular dysfunction has been established by various studies. However, the available Patient-Reported Outcome Measures questionnaires that address the main vestibular complaint fail to highlight this domain of dysfunction in this patient population. The objective of this study was to quantify and compare cognitive impairment using a validated cognitive questionnaire across several vestibular diagnoses. Cross-sectional study of 186 patients presenting to a tertiary care vestibular clinic with a diagnosis of vestibular migraine, Meniere's disease (MD), benign positional paroxysmal vertigo, or persistent postural-perceptual dizziness (PPPD). Patients completed the Cognitive Failures Questionnaire (CFQ) and the Dizziness Handicap Inventory (DHI). Mean CFQ scores for this cohort were significantly higher than similarly aged published controls (34.9/100 versus 31.3/100; p < 0.01; mean age, 45 years) as well as published controls between 65 and 74 years of age (34.9/100 versus 31.2/100; p < 0.05). Patients with PPPD or combined vestibular migraine and MD scored the highest on the CFQ and significantly higher than controls (45.1/100, p = 0.001; and 44.1/100, p = 0.006, respectively). Patients with benign positional paroxysmal vertigo had lower scores than normal controls. There is a weak but significant correlation between CFQ and DHI (r = 0.31; p < 0.001). Multivariate linear regression shows that CFQ scores were largely driven by the duration of symptoms (p < 0.001), type of diagnosis (notably PPPD; p = 0.026), and DHI (p < 0.001). Our results indicate that cognitive impairment is prevalent with chronic vestibular disorders, even in peripheral disorders such as MD. The duration of vestibular symptoms before diagnosis (and management), as well as certain etiologies, may play a bigger role in cognitive disability than age. This is not currently well-addressed in Patient-Reported Outcome Measures questionnaires and may be overlooked at the time of the diagnosis. Appropriate identification may help tailor treatment, namely rehabilitation programs, to individual patients.

The primary objective of this study was to quantify local (within ear) and global (between ear) variation in the cochlear implant (CI) electrode-neuron interface (ENI) using the electrically evoked compound action potential (ECAP). We tested the hypothesis that, within an ear, ECAP measures can be used to identify channels with presumed good and poor ENIs, which may be influenced by a combination of spiral ganglion neuron (SGN) density, electrode position, and cochlear resistivity. We also hypothesized that ECAP responses would reflect age-related differences in the global quality of the ENI between younger and older listeners who theoretically differ in SGN density. Data were obtained from 18 implanted ears (13 individuals) with Advanced Bionics HiRes 90K devices. Six participants (8 ears) were adolescents or young adults (age range: 14-32 years), and 7 participants (10 ears) were older adults (age range: 54-88 years). In each ear, single-channel auditory detection thresholds were measured on channels 2 through 15 in response to a spatially focused electrode configuration (steered quadrupolar; focusing coefficient = 0.9). ECAP amplitudes, amplitude growth function (AGF) slopes, and thresholds were assessed on a subset of channels in each ear in response to three interphase gaps (0, 7, and 30 µs). ECAP peak amplitudes were assessed on all channels between 2 and 15. AGFs and ECAP thresholds were measured on the two nonadjacent channels with the lowest and highest focused behavioral thresholds in each ear. ECAP responses were compared across low- and high-threshold channels and between younger and older CI listeners. Channels that were estimated to interface poorly with the auditory nerve (i.e., high-focused-threshold channels) had steeper ECAP AGF slopes, smaller dynamic ranges, and higher ECAP thresholds than channels with low focused thresholds. Younger listeners had steeper ECAP AGF slopes and larger ECAP peak amplitudes than older listeners. Moreover, younger listeners showed greater interphase gap sensitivity for ECAP amplitude than older listeners. ECAP responses may be used to quantify both local (within ear) and global (between ear) variation in the quality of the ENI. Results of this study support future investigation into the use of ECAP responses in site-selection CI programming strategies. The present results also support a growing body of evidence suggesting that adolescents and young adults with CIs may have denser populations of functional SGNs relative to older adults. Potential differences in global SGN integrity between younger and older listeners warrant investigation of optimal CI programming interventions based on their divergent hearing histories.

Previous research has investigated whether the apolipoprotein E (APOE) ε4 allele, which is associated with an increased risk of cognitive decline, is also associated with hearing loss in older people. Results of the very limited research to date are conflicting, and sample sizes for all but one study were small. The present study aimed to investigate whether there is an association between the APOE ε4 allele and hearing loss in a large, population-based sample of community-dwelling older adults. Cross-sectional audiometric data on hearing levels and APOE genotypes for 2006 participants (aged 55 to 85 years) of the Hunter Community Study were analyzed using multiple linear regression to examine the association between APOE ε4 carrier status and the 4-frequency pure-tone average (0.5 to 4 kHz) in the better hearing ear, and also across individual frequencies in the better ear. Observed and expected APOE allele frequency distributions did not differ significantly overall from established general population allele frequency distributions. Unadjusted modeling using better ear pure-tone average showed a statistically significant association between APOE ε4 allele status (0, 1, 2 copies) and reduced hearing loss, but when the model was adjusted for age, this was no longer statistically significant. Across individual hearing frequencies, unadjusted regression modeling showed APOE ε4 status was significantly associated with a reduction in mean hearing thresholds at 1 and 2 kHz, but again this effect was no longer statistically significant after adjusting for age. The results of this study did not provide any evidence of a statistically significant association between APOE ε4 allele status and hearing loss for older adults. Further investigation of the effect of homozygous carrier status on hearing thresholds is required.

The primary objective of the study was to examine the occurrence and temporal structure of vocal turn-taking during spontaneous interactions between mothers and their children with cochlear implants (CI) over the first year after cochlear implantation as compared with interactions between mothers and children with normal hearing (NH). Mothers' unstructured play sessions with children with CI (n = 12) were recorded at 2 time points, 3 months (mean age 18.3 months) and 9 months (mean age 27.5 months) post-CI. A separate control group of mothers with age-matched hearing children (n = 12) was recorded at the same 2 time points. Five types of events were coded: mother and child vocalizations, vocalizations including speech overlap, and between- and within-speaker pauses. We analyzed the proportion of child and mother vocalizations involved in turn-taking, the temporal structure of turn-taking, and the temporal reciprocity of turn-taking using proportions of simultaneous speech and the duration of between- and within-speaker pauses. The CI group produced a significantly smaller proportion of vocalizations in turn-taking than the NH group at the first session; however, CI children's proportion of vocalizations in turn-taking increased over time. There was a significantly larger proportion of simultaneous speech in the CI compared with the NH group at the first session. The CI group produced longer between-speaker pauses as compared with those in the NH group at the first session with mothers decreasing the duration of between-speaker pauses over time. NH infants and mothers in both groups produced longer within- than between-speaker pauses but CI infants demonstrated the opposite pattern. In addition, the duration of mothers' between-speaker pauses (CI and NH) was predicted by the duration of the infants' between-speaker pauses. Vocal turn-taking and timing in both members of the dyad, the mother and infant, were sensitive to the experiential effects of child hearing loss and remediation with CI. Child hearing status affected dyad-specific coordination in the timing of responses between mothers and their children.

Lexical tone information provides redundant cues for the recognition of Mandarin sentences in listeners with normal hearing in quiet conditions. The contribution of lexical tones to Mandarin sentence recognition in listeners with hearing aids (HAs) is unclear. This study aimed to remove lexical tone information and examine the effects on Mandarin sentence intelligibility in HA users. The second objective was to investigate the contribution of cognitive abilities (i.e., general cognitive ability, working memory, and attention) on Mandarin sentence perception when the presentation of lexical tone information was mismatched. A text-to-speech synthesis engine was used to manipulate Mandarin sentences into three test conditions: (1) a Normal Tone test condition, where no alterations were made to lexical tones within sentences; (2) a Flat Tone test condition, where lexical tones were all changed into tone 1 (i.e., the flat tone); and (3) a Random Tone test condition, where each word in test sentences was randomly assigned one of four Mandarin lexical tones. The manipulated sentence signals were presented to 32 listeners with HAs in both quiet and noisy environments at an 8 dB signal to noise ratio. Speech intelligibility was reduced significantly (by approximately 40 percentage points) in the presence of mismatched lexical tone information in both quiet and noise. The difficulty in correctly identifying sentences with mismatched lexical tones among adults with hearing loss was significantly greater than that of adults with normal hearing. Cognitive function was not significantly related to a decline in speech recognition scores. Contextual and other phonemic cues (i.e., consonants and vowels) are inadequate for HA users to perceive sentences with mismatched lexical tone contours in quiet or noise. Also, HA users with better cognitive function could not compensate for the loss of lexical tone information. These results highlight the importance of accurately representing lexical tone information for Mandarin speakers using HAs.

Recessive mutations in GJB2 are the most common genetic cause of sensorineural hearing impairment (SNHI) in humans. SNHI related to GJB2 mutations demonstrates a wide variation in audiological features, and there has been no reliable prediction model for hearing outcomes until now. The objectives of this study were to clarify the predominant factors determining hearing outcome and to establish a predictive model for SNHI in patients with GJB2 mutations. A total of 434 patients confirmed to have biallelic GJB2 mutations were enrolled and divided into three groups according to their GJB2 genotypes. Audiological data, including hearing levels and audiogram configurations, were compared between patients with different genotypes. Univariate and multivariate generalized estimating equation (GEE) analyses were performed to analyze longitudinal data of patients with multiple audiological records. Of the 434 patients, 346 (79.7%) were homozygous for the GJB2 p.V37I mutation, 55 (12.7%) were compound heterozygous for p.V37I and another GJB2 mutation, and 33 (7.6%) had biallelic GJB2 mutations other than p.V37I. There was a significant difference in hearing level and the distribution of audiogram configurations between the three groups. Multivariate GEE analyses on 707 audiological records of 227 patients revealed that the baseline hearing level and the duration of follow-up were the predominant predictors of hearing outcome, and that hearing levels in patients with GJB2 mutations could be estimated based on these two parameters: (Predicted Hearing Level [dBHL]) = 3.78 + 0.96 × (Baseline Hearing Level [dBHL]) + 0.55 × (Duration of Follow-Up [y]). The baseline hearing level and the duration of follow-up are the main prognostic factors for outcome of GJB2-related SNHI. These findings may have important clinical implications in guiding follow-up protocols and designing treatment plans in patients with GJB2 mutations.

Oscillopsia is a disabling condition for patients with bilateral vestibular hypofunction (BVH). When the vestibulo-ocular reflex is bilaterally impaired, its ability to compensate for rapid head movements must be supported by refixation saccades. The objective of this study is to assess the relationship between saccadic strategies and perceived oscillopsia. To avoid the possibility of bias due to remaining vestibular function, we classified patients into two groups according to their gain values in the video head impulse test. One group comprised patients with extremely low gain (0.2 or below) in both sides, and a control group contained BVH patients with gain between 0.2 and 0.6 bilaterally. Binary logistic regression (BLR) was used to determine the variables predicting oscillopsia. Twenty-nine patients were assigned to the extremely low gain group and 23 to the control group. The BLR model revealed the PR score (saccades synchrony measurement) to be the best predictor of oscillopsia. Receiver operating characteristic analysis determined that the most efficient cutoff point for the probabilities saved with the BLR was 0.518, yielding a sensitivity of 86.6% and specificity of 84.2%. BVH patients with higher PR values (nonsynchronized saccades) were more prone to oscillopsia independent of their gain values. We suggest that the PR score can be considered a useful measurement of compensation.

Hearing aids provide various signal processing techniques with a range of parameters to improve the listening experience for a hearing-impaired individual. In previous studies, we reported significant differences in signal modification for mild versus strong signal processing in commercially available hearing aids. In this study, the authors extend this work to clinically prescribed hearing aid fittings based on best-practice guidelines. The goals of this project are to determine the range of cumulative signal modification in clinically fit hearing aids across manufacturers and technology levels and the effects of listening conditions including signal to noise ratio (SNR) and presentation level on these signal modifications. We identified a subset of hearing aids that were representative of a typical clinical setting. Deidentified hearing aid fitting data were obtained from three audiology clinics for adult hearing aid users with sensorineural hearing loss for a range of hearing sensitivities. Matching laboratory hearing aids were programmed with the deidentified fitting data. Output from these hearing aids was recorded at four SNRs and three presentation levels. The resulting signal modification was quantified using the cepstral correlation component of the Hearing Aid Speech Quality Index which measures the speech envelope changes in the context of a model of the listener's hearing loss. These metric values represent the hearing aid processed signal as it is heard by the hearing aid user. Audiometric information was used to determine the nature of any possible association with the distribution of signal modification in these clinically fit hearing aids. In general, signal modification increased as SNR decreased and presentation level increased. Differences across manufacturers were significant such that the effect of presentation level varied differently at each SNR, for each manufacturer. This result suggests that there may be variations across manufacturers in processing various listening conditions. There was no significant effect of technology level. There was a small effect of pure-tone average on signal modification for one manufacturer, but no effect of audiogram slope. Finally, there was a broad range of measured signal modification for a given hearing loss, for the same manufacturer and listening condition. The signal modification values in this study are representative of commonly fit hearing aids in clinics today. The results of this study provide insights into how the range of signal modifications obtained in real clinical fittings compares with a previous study. Future studies will focus on the behavioral implications of signal modifications in clinically fit hearing aids.

Cochlear implants (CIs) restore speech perception in quiet but they also eliminate or distort many acoustic cues that are important for music enjoyment. Unfortunately, quantifying music enjoyment by CI users has been difficult because comparisons must rely on their recollection of music before they lost their hearing. Here, we aimed to assess music enjoyment in CI users using a readily interpretable reference based on acoustic hearing. The comparison was done by testing "single-sided deafness" (SSD) patients who have normal hearing (NH) in one ear and a CI in the other ear. The study also aimed to assess binaural musical enjoyment, with the reference being the experience of hearing with a single NH ear. Three experiments assessed the effect of adding different kinds of input to the second ear: electrical, vocoded, or unmodified. In experiment 1, music enjoyment in SSD-CI users was investigated using a modified version of the MUSHRA (MUltiple Stimuli with Hidden Reference and Anchor) method. Listeners rated their enjoyment of song segments on a scale of 0 to 200, where 100 represented the enjoyment obtained from a song segment presented to the NH ear, 0 represented a highly degraded version of the same song segment presented to the same ear, and 200 represented enjoyment subjectively rated as twice as good as the 100 reference. Stimuli consisted of acoustic only, electric only, acoustic and electric, as well as other conditions with low pass filtered acoustic stimuli. Acoustic stimulation was provided by headphone to the NH ear and electric stimulation was provided by direct audio input to the subject's speech processor. In experiment 2, the task was repeated using NH listeners who received vocoded stimuli instead of electric stimuli. Experiment 3 tested the effect of adding the same unmodified song segment to the second ear, also in NH listeners. Music presented through the CI only was very unpleasant, with an average rating of 20. Surprisingly, the combination of the unpleasant CI signal in one ear with acoustic stimulation in the other ear was rated more enjoyable (mean = 123) than acoustic processing alone. Presentation of the same monaural musical signal to both ears in NH listeners resulted with even greater enhancement of the experience compared with presentation to a single ear (mean = 159). Repeating the experiment using a vocoder to one ear of NH listeners resulted in interference rather than enhancement. Music enjoyment from electric stimulation is extremely poor relative to a readily interpretable NH baseline for CI-SSD listeners. However, the combination of this unenjoyable signal presented through a CI and an unmodified acoustic signal presented to a NH (or near-NH) contralateral ear results in enhanced music enjoyment with respect to the acoustic signal alone. Remarkably, this two-ear enhancement experienced by CI-SSD listeners represents a substantial fraction of the two-ear enhancement seen in NH listeners. This unexpected benefit of electroacoustic auditory stimulation will have to be considered in theoretical accounts of music enjoyment and may facilitate the quest to enhance music enjoyment in CI users.

Electrocochleography (ECochG) obtained through a cochlear implant (CI) is increasingly being tested as an intraoperative monitor during implantation with the goal of reducing surgical trauma. Reducing trauma should aid in preserving residual hearing and improve speech perception overall. The purpose of this study was to characterize intracochlear ECochG responses throughout insertion in a range of array types and, when applicable, relate these measures to hearing preservation. The ECochG signal in cochlear implant subjects is complex, consisting of hair cell and neural generators with differing distributions depending on the etiology and history of hearing loss. Consequently, a focus was to observe and characterize response changes as an electrode advances. In 36 human subjects, responses to 90 dB nHL tone bursts were recorded both at the round window (RW) and then through the apical contact of the CI as the array advanced into the cochlea. The specific setup used a sterile clip in the surgical field, attached to the ground of the implant with a software-controlled short to the apical contact. The end of the clip was then connected to standard audiometric recording equipment. The stimuli were 500 Hz tone bursts at 90 dB nHL. Audiometry for cases with intended hearing preservation (12/36 subjects) was correlated with intraoperative recordings. Successful intracochlear recordings were obtained in 28 subjects. For the eight unsuccessful cases, the clip introduced excessive line noise, which saturated the amplifier. Among the successful subjects, the initial intracochlear response was a median 5.8 dB larger than the response at the RW. Throughout insertion, modiolar arrays showed median response drops after stylet removal while in lateral wall arrays the maximal median response magnitude was typically at the deepest insertion depth. Four main patterns of response magnitude were seen: increases > 5 dB (12/28), steady responses within 5 dB (4/28), drops > 5 dB (from the initial response) at shallow insertion depths (< 15 mm deep, 7/28), or drops > 5 dB occurring at deeper depths (5/28). Hearing preservation, defined as < 80 dB threshold at 250 Hz, was successful in 9/12 subjects. In these subjects, an intracochlear loss of response magnitude afforded a prediction model with poor sensitivity and specificity, which improved when phase, latency, and proportion of neural components was considered. The change in hearing thresholds across cases was significantly correlated with various measures of the absolute magnitudes of response, including RW response, starting response, maximal response, and final responses (p's < 0.05, minimum of 0.0001 for the maximal response, r's > 0.57, maximum of 0.80 for the maximal response). Monitoring the cochlea with intracochlear ECochG during cochlear implantation is feasible, and patterns of response vary by device type. Changes in magnitude alone did not account for hearing preservation rates, but considerations of phase, latency, and neural contribution can help to interpret the changes seen and improve sensitivity and specificity. The correlation between the absolute magnitude obtained either before or during insertion of the ECochG and the hearing threshold changes suggest that cochlear health, which varies by subject, plays an important role.