Click-evoked auditory brainstem response (ABR) alterations are associated with autism spectrum disorder (ASD), but the specificity of these findings to the disorder is unclear. We therefore performed ...a meta-analysis on ABRs and attention-deficit/hyperactivity disorder (ADHD), a neurodevelopmental disorder that shares some etiologic and symptom overlap with ASD.
Seven papers compared ABR latency components (I, III, V, I-III, III-V, and I-V) between participants with and without ADHD. We used random-effects regression to generate component-specific estimates (Hedges's g) that adjusted for study sample sizes and the number of studies contributing to each estimate. We compared these estimates to our recently published meta-analysis of ABRs and ASD.
All ADHD studies employed cross-sectional designs. ADHD was associated with longer latencies for waves III and V (g = 0.6, 95% confidence interval (CI) 0.3, 1.0 and g = 0.6, 95% CI 0.3, 0.9) and waves I-III and I-V (g = 0.7, 95% CI 0.2, 1.3 and g = 0.6, 95% CI 0.3, 1.0). Effect sizes from the ASD and ADHD meta-analyses did not differ from each other.
Similar patterns of ABR alterations are observed in ADHD and ASD. However, studies rarely screen for middle ear dysfunction or hearing loss and rely upon cross-sectional designs. Addressing these issues will inform the viability of ABRs as a prognostic and/or etiologic biomarker for these disorders.
Click-evoked ABR alterations are associated with ASD, but the specificity of these findings to the disorder is unclear. We therefore performed a meta-analysis of the association between ABRs and ADHD, a disorder that shares some etiologic and symptom overlap with ASD. ADHD was associated with longer ABR latencies for several components. These components are identical to those implicated in ASD. Effect sizes were similar in magnitude across disorders. The viability of ABRs as prognostic and/or etiologic biomarkers for neurodevelopmental risk requires addressing limitations in the literature (e.g., cross-sectional data, non-standardized ABR protocols, minimal characterization of symptom heterogeneity).
Superior semicircular canal dehiscence (SSCD) is the best-known and most common presentation of so-called "third window conditions." There are a variety of diagnostic measures and tests for this ...condition in the current literature, including air-bone gap, vestibular-evoked myogenic potentials, and electrocochleography (EcochG). The purpose of this study was to investigate the diagnostic utility of EcochG and its relationship to air-bone gap in a cohort of patients with confirmed SSCD.
We reviewed data from 20 patients (11 female and 9 male subjects, age ranging 21-78 years), with confirmed unilateral or bilateral superior canal dehiscence. In total, 11 patients had unilateral SSCD and 9 patients had bilateral SSCD as determined by high-resolution CT scan. This resulted in the inclusion of twenty-nine ears with superior canal dehiscence and 11 normal ears.
Our results indicated that all confirmed SSCD ears presented with an abnormal EcochG SP/AP value and that there was a statistically significant difference between normal and dehiscent ears. There was no statistically significant relationship between air-bone gap and SP/AP ratio in the ears diagnosed with SSCD nor was there a significant difference between dehiscent and normal ears in terms of air-bone gap at three frequencies.
These results are consistent with previous studies showing the diagnostic utility of EcochG for this condition and the variability of air-bone gap. While an unexpected air-bone gap continues to be a red flag for SSCD, its absence along with the presence of subjective symptoms is a reasonable indicator for further clinical investigation to include EcochG.
To describe postoperative hearing outcomes following transmastoid (TM) and middle cranial fossa (MCF) approaches for semicircular canal dehiscence (SSCD) repair.
Retrospective review.
Academic, ...tertiary referral center.
Adults with SSCD who underwent repair between 2005 and 2019.
Pure tone audiometry pre- and postoperatively after SSCD repair.
Change in air-bone gap (ABG) at 250 and 500 Hz, pure tone average (PTA), bone conduction (BC), and air conduction (AC) thresholds at 500, 1000, 2000, and 4000 Hz for patients undergoing TM and MCF approaches for SSCD repair.
The average change in BC PTA for patients undergoing TM (n = 26) and MCF (n = 24) SSCD repair was not significantly different between the two groups. The first and final postoperative PTAs were recorded an average of 1.7 (range 0.30-3.0) and 29.1 (range 3.5-154) months postoperatively. For patients who underwent MCF repair, the average BC PTAs increased (+) by 2.2 dB HL (p 0.43) and 0.57 dB HL (p 0.88) at the first and final audiograms respectively compared to +1.27 dB HL (p 0.53) and a decrease (-) of 0.57 dB HL (p 0.63) for the TM group. The average changes in low frequency ABG for patients undergoing MCF repair were -4.7 dB (p 0.08) and -6.9 dB (p 0.15) at first and final audiograms respectively compared to -4.9 dB (p 0.06) and -4.1 dB (p 0.36) for patients who underwent TM repair. There was a high frequency hearing loss noted at 8000 Hz for the MCF (30.0 dB ± 18.7 preop; 41.7 dB ± 21.7 postop; p 0.01) and TM (32.1 dB ± 23.2 preop; 44.3 dB ± 29.6 postop; p 0.001) groups which persisted on long term follow up.
Both TM and MCF approaches to SSCD repair can be performed with long-term preservation of hearing. ABGs were reduced in each treatment group but did not reach significance. A high frequency hearing loss (8000 Hz) may be expected with either approach.
To determine the degree to which electrocochleography (ECoG) correlates with auditory and vestibular outcomes after repair of superior semicircular canal dehiscence (SSCD) via transmastoid (TM) and ...middle cranial fossa (MCF) approaches.
Retrospective review.
Academic tertiary referral center.
Adults with SSCD who underwent repair between 2005 and 2019.
Pre-, intra-, and postoperative ECoG.
Patient-reported vestibular and auditory symptoms; pre-, intra-, and postoperative ECoG measures, dizziness handicap inventory (DHI) scores.
Forty-six patients underwent SSCD repair (40 unilateral, six bilateral) between 2005 and 2019, including 24 MCF and 28 TM approaches. There were no differences in preoperative, intraoperative, or postrepair ECoG SP/AP values between the MCF and TM groups (p 0.12, 0.77, 0.58). Patients had subjective improvement in vestibular symptoms (or stable vestibular function in patients operated for predominantly auditory manifestations) with both approaches (MCF: 87.5%; TM: 92.3%; p 0.64). A successful outcome correlated with intraoperative SP/AP ratio normalization (p 0.0005). Similarly, DHI scores were similar in both groups preoperatively (p 0.66) and returned to their preoperative baseline postoperatively with both (p 0.52). Reported vestibular symptoms persisted or worsened more often in patients with migraine (66.6% vs. 28.9%, p 0.03), and with persistently abnormal ECoG measures, though the latter was not statistically significant in this population (38% vs. 15%, p 0.10). Patients had subjective improvement or stability in auditory symptoms using either approach (MCF: 96%; TM: 100%; p 0.62), also correlating with SP/AP ratio normalization (p 0.008).
Correction of abnormal preoperative ECoG reliably correlates to patient symptom improvement after SSCD repair. No significant differences in postoperative outcomes were noted between patients undergoing TM versus MCF repair. Circumspection regarding the likelihood of an ideal outcome after SSCD repair should be exercised when counseling patients with concomitant migraine.
It is not certain whether outcomes differ between the two dominant approaches for SSCD repair. Surgeons and patients would benefit from an intraoperative metric that reflects satisfactory plugging of SSCD.
To highlight the reliability and unique utility of intraoperative ECoG and demonstrate the correlation between ECoG correction and symptom improvement for SSCD repair.
To report subjective and objective outcomes following SSCD repair and encourage adoption of intraoperative ECoG monitoring.
Level V.
IRB review considers this study exempt (HUM00169949).
Hearing loss, the second most common disability in the United States, is under-diagnosed and under-treated. Identifying it in early stages could prevent its known substantial adverse outcomes.
A ...multiple baseline design was implemented to assess a screening paradigm for identifying and referring patients aged ≥55 years with hearing loss at 10 family medicine clinics in 2 health systems. Patients completed a consent form and the Hearing Handicap Inventory for the Elderly (HHI). An electronic alert prompted clinicians to screen for hearing loss during visits.
The 14,877 eligible patients during the study period had 36,701 encounters. Referral rates in the family medicine clinics increased from a baseline rate of 3.2% to 14.4% in 1 health system and from a baseline rate of 0.7% to 4.7% in the other. A general medicine comparison group showed referral rate increase from the 3.0% baseline rate to 3.3%. Of the 5,883 study patients who completed the HHI 25.2% (n=1,484) had HHI scores suggestive of hearing loss; those patients had higher referral rates, 28% vs 9.2% (
<.001). Of 1,660 patients referred for hearing testing, 717 had audiology data available for analysis: 669 (93.3%) were rated appropriately referred and 421 (58.7%) were considered hearing aid candidates. Overall, 71.5% of patients contacted felt their referral was appropriate.
An electronic alert used to remind clinicians to ask patients aged ≥55 years about hearing loss significantly increased audiology referrals for at-risk patients. Audiologic and audiogram data support the effectiveness of the prompt. Clinicians should consider adopting this method to identify patients with hearing loss to reduce its known and adverse sequelae.
Superior canal dehiscence syndrome (SCDS) is an increasingly recognized cause of hearing loss and vestibular symptoms, but the etiology of this condition remains unknown.
To describe 7 cases of SCDS ...across 3 families.
This retrospective case series included 7 patients from 3 different families treated at a neurotology clinic at a tertiary academic medical center from 2010 to 2014. Patients were referred by other otolaryngologists or were self-referred. Each patient demonstrated unilateral or bilateral SCDS or near dehiscence.
Clinical evaluation involved body mass index calculation, audiometry, cervical vestibular evoked myogenic potential testing, electrocochleography, and multiplanar computed tomographic (CT) scan of the temporal bones. Zygosity testing was performed on twin siblings.
The diagnosis of SCDS was made if bone was absent over the superior semicircular canal on 2 consecutive CT images, in addition to 1 physiologic sign consistent with labyrinthine dehiscence. Near dehiscence was defined as absent bone on only 1 CT image but with symptoms and at least 1 physiologic sign of labyrinthine dehiscence.
A total of 7 patients (5 female and 2 male; age range, 8-49 years) from 3 families underwent evaluation. Family A consisted of 3 adult first-degree relatives, of whom 2 were diagnosed with SCDS and 1 with near dehiscence. Family B included a mother and her child, both of whom were diagnosed with unilateral SCDS. Family C consisted of adult monozygotic twins, each of whom was diagnosed with unilateral SCDS. For all cases, dehiscence was located at the arcuate eminence. Obesity alone did not explain the occurrence of SCDS because 5 of the 7 cases had a body mass index (calculated as weight in kilograms divided by height in meters squared) less than 30.0.
Superior canal dehiscence syndrome is a rare, often unrecognized condition. This report of 3 multiplex families with SCDS provides evidence in support of a potential genetic contribution to the etiology. Symptomatic first-degree relatives of patients diagnosed with SCDS should be offered evaluation to improve detection of this disorder.
Purpose: The aim of this study was to examine how the brains of individuals with cochlear implants (CIs) respond to spoken language tasks that underlie successful language acquisition and processing. ...Method: During functional near-infrared spectroscopy imaging, CI recipients with hearing impairment (n = 10, mean age: 52.7 ± 17.3 years) and controls with normal hearing (n = 10, mean age: 50.6 ± 17.2 years) completed auditory tasks--phonological awareness and passage comprehension--commonly used to investigate neurodevelopmental disorders of language and literacy. Results: The 2 groups had similar reaction time and performance on experimental tasks, although participants with CIs had lower accuracy than controls. Overall, both CI recipients and controls exhibited similar patterns of brain activation during the tasks. Conclusions: The results demonstrate that CI recipients show an overall neurotypical pattern of activation during auditory language tasks on which individuals with neurodevelopmental language learning impairments (e.g., dyslexia) tend to show atypical brain activation. These findings suggest that advancements in functional near-infrared spectroscopy neuroimaging with CI recipients may help shed new light on how varying types of difficulties in language processing affect brain organization for language.
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