The Association of Hearing Loss with Hospitalization.

BACKGROUND: Individuals with hearing loss (HL) are at higher risk for hospitalizations, and may be for readmissions, compared with their hearing peers. The objective of this prospective study was to confirm retrospective studies suggesting that HL increases hospital readmissions, and, if confirmed, possible causes for it. METHODS: A prospective cohort study of English-speaking patients >55 years old admitted to general medical and surgical floors at 2 large hospital systems in southeastern Michigan over a 2-year period was conducted. All patients underwent bedside audiometric testing. HL presence and severity were categorized using World Health Organization pure tone assessment parameters. Readmission rates, Charlson comorbidity index, socio-demographic and medical variables were obtained from Epic EMR databases. OUTCOMES: There were 1247 hospitalized patients enrolled. Of these, 76.8% had documented HL of which 50.5% (630) was mild HL and 26.3% (328) moderate or worse HL. Patients with any HL were older and more likely to be non-Hispanic, white, male, and had less education, lower health literacy, more comorbidities, and more difficulty communicating with their doctor. Readmission rates at 30 and 90-days were similar between HL and hearing groups, after adjusting for HL severity, Charlston index, and numerous potential confounders. CONCLUSION: Patients with HL do not seem to have higher rates of hospital readmissions. We did find high frequency of HL in hospitalized patients along with significant communication difficulties that patients had with their clinicians. These findings have implications for measures to improve patient-physician communication, potentially improving long-term health outcomes.

Electrocochleography in the diagnosis of third window conditions.

Introduction: 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. Methods: 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. Results: 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. Discussion: 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.

Intraoperative Electrocochleography Correlates to Outcomes in Transmastoid and Middle Cranial Fossa Superior Semicircular Canal Dehiscence Repair.

OBJECTIVE: 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. STUDY DESIGN: Retrospective review. SETTING: Academic tertiary referral center. PATIENTS: Adults with SSCD who underwent repair between 2005 and 2019. INTERVENTION: Pre-, intra-, and postoperative ECoG. MAIN OUTCOME MEASURES: Patient-reported vestibular and auditory symptoms; pre-, intra-, and postoperative ECoG measures, dizziness handicap inventory (DHI) scores. RESULTS: 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). CONCLUSIONS: 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. DEFINE PROFESSIONAL PRACTICE GAP AND EDUCATIONAL NEED: 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. LEARNING OBJECTIVE: To highlight the reliability and unique utility of intraoperative ECoG and demonstrate the correlation between ECoG correction and symptom improvement for SSCD repair. DESIRED RESULT: To report subjective and objective outcomes following SSCD repair and encourage adoption of intraoperative ECoG monitoring. LEVEL OF EVIDENCE: Level V. INDICATE IRB OR IACUC: IRB review considers this study exempt (HUM00169949).

Click-evoked auditory brainstem responses and autism spectrum disorder: a meta-analytic investigation of disorder specificity.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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. IMPACT: 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).

Auditory Outcomes Following Transmastoid and Middle Cranial Fossa Approaches for Superior Semicircular Canal Dehiscence Repair.

OBJECTIVE: To describe postoperative hearing outcomes following transmastoid (TM) and middle cranial fossa (MCF) approaches for semicircular canal dehiscence (SSCD) repair. STUDY DESIGN: Retrospective review. SETTING: Academic, tertiary referral center. PATIENTS: Adults with SSCD who underwent repair between 2005 and 2019. INTERVENTIONS: Pure tone audiometry pre- and postoperatively after SSCD repair. MAIN OUTCOME MEASURES: 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. RESULTS: 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 ±â€Š18.7 preop; 41.7 dB ±â€Š21.7 postop; p 0.01) and TM (32.1 dB ±â€Š23.2 preop; 44.3 dB ±â€Š29.6 postop; p 0.001) groups which persisted on long term follow up. CONCLUSIONS: 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.

Effective Hearing Loss Screening in Primary Care: The Early Auditory Referral-Primary Care Study.

PURPOSE: 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. METHODS: 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. RESULTS: 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% (P <.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. CONCLUSION: 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.

Electrocochleography: New Uses for an Old Test and Normative Values.

Purpose This article combines the results of 3 studies that were presented at the HeAL 2018 Conference in Lake Como, Italy, in June 2018. Each study involved electrocochleography (ECochG), a neurodiagnostic evaluation that has been used clinically for over 80 years but whose applications continue to expand. The 1st study describes recent research wherein ECochG was recorded from asymptomatic subjects who were siblings or offspring of patients with a confirmed diagnosis of Ménière's disease (MD). Our results provide evidence that ECochG may be helpful in not only diagnosing MD but also predicting it as well. Second, case studies are described where ECochG was important in both diagnosing superior semicircular canal dehiscence and monitoring the repair of this condition during surgery. Finally, although ECochG has been practiced clinically for over 8 decades, the protocols for recording, measuring, and interpreting the electrocochleogram continue to lack standardization among clinicians and scientists. We thus present normative data for some of these features based on noninvasive recordings made from the tympanic membrane from 100 normal hearing subjects. Conclusions Although the primary use of ECochG continues to be in the diagnosis of MD, we report on 2 additional clinical applications for this important test of inner ear/auditory nerve function. First, a preliminary study on a small sample of subjects indicates that ECochG may also be useful in predicting MD prior to the onset of symptoms in individuals who may be genetically predisposed to developing it. Second, through a series of case studies, we demonstrate how ECochG is used to help diagnose superior semicircular canal dehiscence and monitor the status of the inner ear during the surgical repair of this condition. Finally, normative values for clinically important components of the electrocochleogram based on tympanic membrane recordings have been established from a large sample of subjects.

Click-evoked auditory brainstem responses and autism spectrum disorder: A meta-analytic review.

Behavior does not differentiate ASD risk prior to 12 months of age, but biomarkers may inform risk before symptoms emerge. Click-evoked auditory brainstem responses (ABRs) may be worth consideration due to their measurement properties (noninvasiveness; reliability) and conceptual features (well-characterized neural generators), but participant characteristics and assessment protocols vary considerably across studies. Our goal is to perform a meta-analysis of the association between ABRs and ASD. Following an electronic database search (PubMed, Medline, PsycInfo, PsycArticles), we included papers that were written in English, included ASD and typically-developing (TD) groups, and reported the information needed to calculate standardized mean differences (Hedges's g) for at least one ABR latency component (I, III, V, I-III, III-V, I-V). We weighted and averaged effect sizes across conditions and subsets of participants to yield one estimate per component per study. We then performed random-effects regressions to generate component-specific estimates. ASD was associated with longer ABR latencies for Waves III (g = 0.5, 95% CI 0.1, 0.9), V (g = 0.7, 95% CI 0.3, 1.1), I-III (g = 0.7, 95% CI 0.2, 1.2), and I-V (g = 0.6, 95% CI 0.2, 1.0). All components showed significant heterogeneity. Associations were strongest among participants ≤8 years of age and those without middle ear abnormalities or elevated auditory thresholds. In sum, associations between ABRs and ASD are medium-to-large in size, but exhibit heterogeneity. Identifying sources of heterogeneity is challenging, however, due to power limitations and co-occurrence of sample/design characteristics across studies. Research addressing the above limitations is crucial to determining the etiologic and/or prognostic value of ABRs for ASD. Autism Res 2018, 11: 916-927. © 2018 International Society for Autism Research, Wiley Periodicals, Inc. LAY SUMMARY: Auditory brainstem responses (ABR) may be associated with ASD, but participant characteristics and assessment protocols vary considerably across individual studies. Our goal is to combine the results across these studies to facilitate clarity on the topic. Doing so represents a first step in evaluating whether ABRs yield potential for informing the etiology of ASD risk and/or ASD symptom profiles.

Electrocochleography Results in Patients With Bilateral Vestibular Paresis and Sound- or Pressure-Induced Horizontal Nystagmus.

OBJECTIVE: To describe the electrocochleography (ECochG) findings in patients with bilateral vestibular paresis and sound- and/or pressure-induced horizontal nystagmus. DESIGN: Retrospective case series. SETTING: Tertiary care center. PATIENTS: Three adult patients with bilateral vestibular paresis and sound- and/or pressure-induced horizontal nystagmus were evaluated from 2012 to 2016. MAIN OUTCOME MEASURE: All patients underwent ECochG, vestibular evoked myogenic potential (VEMP) testing, bithermal caloric testing, rotary chair testing, audiometric testing, and temporal bone computed tomography (CT). For ECochG, the summating potential (SP) to action potential (AP) ratio was determined. RESULTS: All patients had normal temporal bone CT, reduced caloric responses bilaterally, decreased gain on rotary chair, and abnormal ECochG. For two subjects, the SP/AP was elevated bilaterally. One subject had unilateral SP/AP elevation. Cervical VEMPs were present in all subjects, but at reduced thresholds in two subjects. CONCLUSION: SP/AP elevation was found in all three patients with the syndrome of bilateral vestibular paresis and/or sound- or pressure-induced horizontal nystagmus. As the etiology of this syndrome remains unclear, understanding the basis for abnormal ECochG may shed insight into the pathophysiology of this condition.

Initial Results of the Early Auditory Referral-Primary Care (EAR-PC) Study.

INTRODUCTION: Hearing loss (HL) is the second most common disability in the U.S., yet is clinically underdiagnosed. To manage its common adverse psychosocial and cognitive outcomes, early identification of HL must be improved. METHODS: A feasibility study conducted to increase screening for HL and referral of patients aged ≥55 years arriving at two family medicine clinics. Eligible patients were asked to complete a self-administered consent form and the Hearing Handicap Inventory (HHI). Independently, clinicians received a brief educational program after which an electronic clinical prompt (intervention) alerted them (blinded to HHI results) to screen for HL during applicable patient visits. Pre- and post-intervention differences were analyzed to assess the proportion of patients referred to audiology and those diagnosed with HL (primary outcomes) and the audiology referral appropriateness (secondary outcome). Referral rates for those who screened positive for HL on the HHI were compared with those who scored negatively. RESULTS: There were 5,520 eligible patients during the study period, of which 1,236 (22.4%) consented. After the intervention's implementation, audiology referral rates increased from 1.2% to 7.1% (p<0.001). Overall, 293 consented patients (24%) completed the HHI and scored >10, indicating probable HL. Of these 293 patients, 28.0% were referred to audiology versus only 7.4% with scores <10 (p<0.001). Forty-two of the 54 referred patients seen by audiology were diagnosed with HL (78%). Overall, the diagnosis of HL on problem lists increased from 90 of 4,815 patients (1.9%) at baseline to 163 of 5,520 patients (3.0%, p<0.001) over only 8 months. CONCLUSIONS: The electronic clinical prompt significantly increased audiology referrals for at-risk patients for HL in two family medicine clinics. Larger-scale studies are needed to address the U.S. Preventive Services Task Force call to assess the long-term impact of HL screening in community populations.

Superior Canal Dehiscence Syndrome Affecting 3 Families.

Importance: Superior canal dehiscence syndrome (SCDS) is an increasingly recognized cause of hearing loss and vestibular symptoms, but the etiology of this condition remains unknown. Objective: To describe 7 cases of SCDS across 3 families. Design, Setting, and Participants: 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. Interventions: 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. Main Outcomes and Measures: 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. Results: 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. Conclusions and Relevance: 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.

Functional Near-Infrared Spectroscopy Brain Imaging Investigation of Phonological Awareness and Passage Comprehension Abilities in Adult Recipients of Cochlear Implants.

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.

Temporal processing in the auditory brainstem response by full-term 6-week- and 9-month-old infants.

Early auditory temporal processing abilities are important for language acquisition and for later reading abilities. In the present study, auditory brainstem responses (ABRs) were recorded in a forward-masking paradigm in healthy, full-term infants aged 6 weeks (n = 111) and 9 months (n = 62). Our purpose was to establish normative values of forward-masking ABRs and investigate the development of auditory temporal processing in infants at these ages. Infants were presented with pairs of stimuli (an initial "masker" followed by a "probe") separated by different time intervals (8, 16, and 64 ms). Results showed that as masker-probe intervals became longer and as infants got older, Wave V latency to the probe shortened. The greatest improvements in Wave V latencies from 6 weeks to 9 months of age were observed in the 64-ms masker-probe interval, suggesting that central auditory nervous system related to the temporal processing at this interval might undergo rapid development during the first year of life.

Multiple electrostimulation treatments to the promontory for tinnitus.

OBJECTIVES: To assess the safety and efficacy of multiple sessions of electrostimulation by a transtympanic needle electrode on the promontory for tinnitus relief. STUDY DESIGN: Prospective open, unblinded, uncontrolled clinical trial. SETTING: Tertiary academic referral center. PATIENTS: Ten patients (8 male and 2 female subjects), mean age 50.1 ± 12 years (range, 34-67) with severe unilateral tinnitus completed all stages of the study. Patients with tinnitus duration between 6 months to 3 years were included. INTERVENTION: The patients underwent 3 consecutive 30-minute sessions, every other day, of biphasic, charge balanced electrostimulation pulses to the promontory delivered by a transtympanic needle electrode. MAIN OUTCOME MEASURES: 1) Tinnitus loudness reported by visual analog scale (VAS) between 1 and 10, at baseline, before and after each treatment, and 1, 2, 3, and 4 weeks after the last treatment. 2) Tinnitus Handicap Inventory (THI) questionnaire at baseline and 4 weeks after treatment. 3) Basic audiometry and tinnitus specific tests such as minimum masking level, tinnitus loudness, and pitch. RESULTS: No long-term adverse safety outcomes were noted in physical examination or audiologic evaluation. VAS levels decreased by ≥2 levels in 5 patients (50%) and returned to baseline 4 weeks after treatment. The VAS decrease was found significant (p = 0.048) in those patients. A statistically significant decrease in THI score was noted 4 weeks after treatment. Tinnitus specific tests at that time were unchanged from baseline. CONCLUSION: Multiple sessions of electrostimulation to the promontory seem to be safe and may be beneficial for some tinnitus patients. Further clinical trials are warranted.

Surgical treatment of hearing loss when otosclerosis coexists with superior semicircular canal dehiscence syndrome.

OBJECTIVE: Document a case of bilateral otosclerosis with coexisting bilateral superior semicircular canal dehiscence syndrome and the treatment of hearing loss in this setting. PATIENT: A 33-year-old woman presented with bilateral mixed hearing loss; worse in the left ear. This was gradual in onset, and she denied dizziness. Computerized tomographic scan revealed fenestral otosclerosis and a large dehiscence of the superior semicircular canal bilaterally. She declined amplification. INTERVENTION: Sequential laser-assisted stapedotomy with insertion of a Kurz titanium CliP Piston prosthesis. MAIN OUTCOME MEASURE: Comparison of audiovestibular symptoms, hearing thresholds, and neurodiagnostic testing results preoperatively and postoperatively. RESULTS: Hearing improved bilaterally with closure of the air-bone gaps at most frequencies, and she has not had permanent vestibular symptoms. Postoperative follow-up time is 37 months for the left ear and 13 months for the right ear. CONCLUSION: When otosclerosis and superior semicircular canal dehiscence syndrome coexist and hearing loss is the dominant symptom, stapes surgery can be effective for improving hearing without permanent vestibular symptoms.

Developmental trends in auditory processing can provide early predictions of language acquisition in young infants.

Auditory processing capabilities at the subcortical level have been hypothesized to impact an individual's development of both language and reading abilities. The present study examined whether auditory processing capabilities relate to language development in healthy 9-month-old infants. Participants were 71 infants (31 boys and 40 girls) with both Auditory Brainstem Response (ABR) and language assessments. At 6 weeks and/or 9 months of age, the infants underwent ABR testing using both a standard hearing screening protocol with 30 dB clicks and a second protocol using click pairs separated by 8, 16, and 64-ms intervals presented at 80 dB. We evaluated the effects of interval duration on ABR latency and amplitude elicited by the second click. At 9 months, language development was assessed via parent report on the Chinese Communicative Development Inventory - Putonghua version (CCDI-P). Wave V latency z-scores of the 64-ms condition at 6 weeks showed strong direct relationships with Wave V latency in the same condition at 9 months. More importantly, shorter Wave V latencies at 9 months showed strong relationships with the CCDI-P composite consisting of phrases understood, gestures, and words produced. Likewise, infants who had greater decreases in Wave V latencies from 6 weeks to 9 months had higher CCDI-P composite scores. Females had higher language development scores and shorter Wave V latencies at both ages than males. Interestingly, when the ABR Wave V latencies at both ages were taken into account, the direct effects of gender on language disappeared. In conclusion, these results support the importance of low-level auditory processing capabilities for early language acquisition in a population of typically developing young infants. Moreover, the auditory brainstem response in this paradigm shows promise as an electrophysiological marker to predict individual differences in language development in young children.

Electrocochleography as a diagnostic and intraoperative adjunct in superior semicircular canal dehiscence syndrome.

OBJECTIVE: To determine the electrocochleographic characteristics of ears with superior semicircular canal dehiscence (SSCD) and to examine its use for intraoperative monitoring in canal occlusion procedures. STUDY DESIGN: Case series. SETTING: Academic medical center. PATIENTS: Thirty-three patients (45 ears) had clinical and computed tomographic evidence of SSCD; 8 patients underwent intraoperative electrocochleography (ECoG) during superior canal occlusion; 9 patients underwent postoperative ECoG after SSCD occlusion. INTERVENTIONS: Diagnostic, intraoperative, and postoperative extratympanic ECoG; middle fossa or transmastoid occlusion of the superior semicircular canal. MAIN OUTCOME MEASURE: Summating potential (SP) to action potential (AP) ratio, as measured by ECoG, and alterations in SP/AP during canal exposure and occlusion. RESULTS: Using computed tomography as the standard, elevation of SP/AP on ECoG demonstrated 89% sensitivity and 70% specificity for SSCD. The mean SP/AP ratio among ears with SSCD was significantly higher than that among unaffected ears (0.62 versus 0.29, p < 0.0001). During occlusion procedures, SP/AP increased on exposure of the canal lumen (mean change ± standard deviation, 0.48 ± 0.30). After occlusion, SP/AP dropped below the intraoperative baseline in most cases (mean change, -0.23 ± 0.52). All patients experienced symptomatic improvement. All patients who underwent postoperative ECoG 1 to 3 months after SSCD repair maintained SP/AP of 0.4 or lesser. CONCLUSION: These findings expand the differential diagnosis of abnormal ECoG. In conjunction with clinical findings, ECoG may support a clinical diagnosis of SSCD. Intraoperative ECoG facilitates dehiscence documentation and allows the surgeon to confirm satisfactory canal occlusion.

The right not to hear: the ethics of parental refusal of hearing rehabilitation.

OBJECTIVE: To explore the ethics of parental refusal of auditory-oral hearing rehabilitation. STUDY DESIGN: Case study with medical ethical discussion and review. METHODS: Two young brothers present with severe-to-profound congenital sensorineural hearing loss. The parents, both of whom have normal hearing and work as sign language interpreters, have decided to raise their children with American Sign Language as their only form of communication. They have chosen not to pursue cochlear implantation nor support the use of hearing aids. DISCUSSION: This case raises significant questions concerning whether hearing rehabilitation should be mandated, and if there are circumstances in which parental preferences should be questioned or overridden with regard to this issue. In addition, legal concerns may be raised regarding the possible need to file a report with Child Protective Services. Although similar cases involving the Deaf community have historically favored parental rights to forego hearing rehabilitation with either cochlear implantation or hearing aids, we explore whether conclusions should be different because the parents in this case are not hearing impaired. CONCLUSIONS: The ethics of parental rights to refuse hearing rehabilitation are complex and strikingly context-dependent. A comprehensive appreciation of the medical, practical, and legal issues is crucial prior to intervening in such challenging situations.