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Objective: We used simple variables to construct prognostic prediction ensemble learning models for patients with sudden sensorineural hearing loss (SSNHL)., Study Design: Retrospectively study., Setting: Tertiary medical center., Patients: 1,572 patients with SSNHL., Intervention: Prognostic., Main Outcome Measures: We selected four variables, namely, age, days after onset of hearing loss, vertigo, and type of hearing loss. We also compared the accuracy between different ensemble learning models based on the boosting, bagging, AdaBoost, and stacking algorithms., Results: We enrolled 1,572 patients with SSNHL; 73.5% of them showed improving and 26.5% did not. Significant between-group differences were noted in terms of age ( p = 0.011), days after onset of hearing loss ( p < 0.001), and concurrent vertigo ( p < 0.001), indicating that the patients who showed improving to treatment were younger and had fewer days after onset and fewer vertigo symptoms. Among ensemble learning models, the AdaBoost algorithm, compared with the other algorithms, achieved higher accuracy (82.89%), higher precision (86.66%), a higher F1 score (89.20), and a larger area under the receiver operating characteristics curve (0.79), as indicated by test results of a dataset with 10 independent runs. Furthermore, Gini scores indicated that age and days after onset are two key parameters of the predictive model., Conclusions: The AdaBoost model is an effective model for predicting SSNHL. The use of simple parameters can increase its practicality and applicability in remote medical care. Moreover, age may be a key factor influencing prognosis., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2024, Otology & Neurotology, Inc.)

Objective: The purpose of this study was to evaluate the value of asymmetry values, gain, and pathological saccades of the video head impulse test (vHIT) in sudden sensorineural hearing loss (SSNHL)., Study Design: Retrospective study., Setting: Tertiary referral center., Patients: A total of 226 individuals diagnosed with unilateral definite SSNHL were hospitalized. The assessment included a comprehensive evaluation of medical history, pure-tone test, acoustic impedance, positional test, video nystagmography (VNG), vHIT, vestibular evoked myogenic potentials (VEMPs) and magnetic resonance., Interventions: vHIT, VNG, cVEMP, oVEMP. Statistical analysis was performed with SPSS version 22.0 for Windows., Main Outcome Measures: The asymmetry values, gain, and pathological saccades of the vHIT., Results: The abnormal gain of vHIT in anterior, horizontal, and posterior canal in SSNHL patients with vertigo were revealed in 20 of 112 (17.9%), 24 of 112 (21.4%), and 60 of 112 (53.6%), respectively. The vHIT pathological saccades (overt + covert) of anterior, horizontal, and posterior canal in SSNHL patients with vertigo were observed in 5 of 112 (4.6%), 52 of 112 (46.4%), and 58 of 112 (51.8%), respectively. Multivariate analysis indicated that the prognosis of patients with vertigo was correlated with vHIT gain of posterior canal, pathological saccade in horizontal canal, asymmetric ratio of horizontal canal gain, asymmetric ratio of posterior canal gain, Canal paresis (%) on caloric test and spontaneous nystagmus., Conclusion: In the vHIT of patients with SSNHL with vertigo, the posterior canal is most easily affected. Reduced gain of posterior canal, pathological saccade of horizontal canal, and larger asymmetric gain of posterior canal and horizontal canal may be negative prognostic factors., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Otology & Neurotology, Inc.)

Objective: To evaluate semicircular canal function using video head impulse test (vHIT) in relapsing polychondritis (RP) patients presenting with dizziness and sensorineural hearing loss., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Three patients with RP underwent vHIT and hearing tests., Intervention: Diagnostic., Main Outcome Measures: The gain in vestibulo-ocular reflex (VOR) and the presence of catch-up saccade were examined, and the correlation between semicircular canal dysfunction and hearing loss was investigated., Results: Of the six ears, five exhibited semicircular canal dysfunction. Among these, one ear showed dysfunction in two semicircular canals, while the remaining four ears demonstrated dysfunction in all three semicircular canals. Sensorineural hearing loss, ranging from moderate to profound, was detected by pure-tone audiometry in all six ears. Furthermore, a significant correlation was observed between VOR gain in the horizontal semicircular canal (HSC) and hearing level., Conclusions: This study demonstrated semicircular canal dysfunction in RP patients presenting with dizziness and hearing loss using vHIT. Moreover, a significant correlation was found between HSC dysfunction and the severity of hearing loss. While inner ear involvement is a key clinical symptom included in the diagnostic criteria for RP, there have been few reports evaluating vestibular dysfunction, and this is the first report on the evaluation of several cases using vHIT. Accurate assessment of vestibular function by vHIT may facilitate early diagnosis and intervention in RP, potentially improving patient outcomes., Competing Interests: Conflicts of interest and source of funding: The authors disclose no conflicts of interest and sources of funding., (Copyright © 2024, Otology & Neurotology, Inc.)

Objectives: The objectives of this study are to (i) estimate the incidence of vestibular schwannoma (VS) among patients in an integrated healthcare system who present for evaluation of sudden sensorineural hearing loss (SSNHL) and (ii) evaluate the efficacy of empiric steroid therapy on audiologic recovery among SSNHL patients ultimately diagnosed with VS., Methods: A retrospective chart review was performed on patients presenting with SSNHL in 2021 at a multicenter integrated healthcare system serving over 4 million members. Patient demographics, audiometric data, VS diagnosis, therapeutic steroid intervention, and data regarding treatment response were recorded. A clinically significant audiometric improvement was defined as (i) an increase of 15% in word recognition score, (ii) a decrease of 15 dB in four-frequency pure-tone average (PTA) using frequencies of 500, 1000, 2000, and 4000 Hz, or (iii) a PTA of <20 dB on follow-up audiogram., Results: Six hundred fifty-eight patients were reviewed, of which 309 (56.0% male; mean, 57.5 years) met the inclusion criteria with audiometric data and magnetic resonance imaging data. Ten patients (70.0% male; mean, 51.3 years) were found to have VS. Of these, five patients received oral steroid therapy alone, and five had combination therapy (oral + intratympanic steroid injections). No patients received intratympanic steroid therapy alone. Median PTA improvement with steroid therapy was 3.1-dB hearing loss, and median word recognition score improvement was 16.5%. Six of 10 patients demonstrated clinically significant audiometric improvement with steroid therapy., Conclusion: This study represents the largest US-based study showcasing the prevalence of VS in patients originally presenting with SSNHL. It also reinforces previous findings that VS does not preclude trials of steroid therapy., Competing Interests: Funding and conflicts of interest: None to disclose. No sources of support or funding., (Copyright © 2023, Otology & Neurotology, Inc.)

Objective: To investigate the diagnostic yield of targeted next-generation sequencing using hearing loss panels and to identify patient-related factors that are associated with a definite genetic cause., Study Design: Retrospective chart review., Setting: Tertiary referral center., Patients: Children with congenital or late-onset, bilateral sensorineural hearing loss., Interventions: Diagnostic., Main Outcome Measures: The number of patients with a definite genetic diagnosis., Results: We report on 238 patients with hearing loss: 130 were male and 108 were female. About 55% had congenital hearing loss. A genetic cause was identified in 94 of the patients (39.5%), with 72.3% of these showing nonsyndromic and 27.6% showing syndromic hearing loss. The diagnostic yield was highest among North African patients (66.7%). A multiple linear regression model shows that profound hearing loss, family history of hearing loss, congenital hearing loss, and North African ethnicity are significantly related to identifying a genetic cause., Conclusions: Targeted next-generation sequencing using a panel of hearing loss genes identified a genetic diagnosis in almost 40% of children with bilateral sensorineural hearing loss. We describe the predictors of a genetic diagnosis, and this information may be used during genetic counseling., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Objectives: To study the need for defining unilateral idiopathic sudden sensorineural hearing loss (ISSNHL) as an otologic emergency and establish an evidence-based cutoff for treatment initiation for optimal outcome., Methods: A systematic literature search of "MEDLINE" via "PubMed," "Embase," and "Web of Science" and original case series comparing the outcome of steroidal treatment for ISSNHL as a function of delays of patient presentation, of diagnosis, and of treatment initiation. Total delay was defined as days from ISSNHL onset to first steroidal dose and divided into up to 3 days, up to 7 days, up to 14 days, and >14 days., Results: The literature search identified 1,469 ears and our original case series contributed 154 ears suitable for study inclusion, resulting in 1,623 ears for statistical analysis. An odds ratio (OR) of 0.42 (95% confidence interval [CI], 0.25-0.71) was calculated for recovery if treatment had been initiated within the third day since the sudden occurrence of a unilateral hearing loss compared with treatment initiation on or after the fourth day (I 2 = 40.1%). The calculated OR for recovery was 0.35 (95% CI, 0.26-0.47) when treatment was initiated during the first 7 days after the sudden hearing loss onset compared with a delay of 8 days or more (I 2 = 52.1%). The OR was 0.31 (95% CI, 0.21-0.46) when treatment was initiated during the first 14 days after the event compared with a longer delay (I 2 = 0.0%)., Conclusion: Unilateral ISSNHL should be considered a medical emergency. Initiating treatment before 3 days have elapsed since the event portends the best outcome., Level of Evidence: Level I., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Background: There have been reports and studies indicating audiovestibular disturbances in COVID-19 patients with variations in the percentage of sensorineural hearing loss (SNHL). The purpose of this study is to compare the incidence of newly diagnosed SNHL, sudden idiopathic hearing loss (SIHL), tinnitus, and vestibular disturbances between infected and uninfected patients, as well as to identify population groups at risk., Methods: This study used TriNetX to obtain statistics on COVID-19 (+) and COVID-19 (-) patients from 61 healthcare organizations. Propensity score with 1:1 matching was used to control confounding variables. This study evaluated the relative risk of developing audiovestibular disturbances up to 1 month after a COVID-19 test and further investigated the incidence in COVID-19 (+) subset groups., Results: Between COVID-19 (+) and COVID-19 (-) patients who had an audiogram, there was no statistically significant difference in SNHL or SIHL (SNHL: relative risk [RR] = 0.69, 95% confidence interval [CI] = 0.46-1.04; SIHL: RR = 1.00, 95% CI = 0.42-2.40). Race/ethnicity or specified comorbidity did not affect the incidence of SNHL or SIHL. There was a statistically significant difference in tinnitus and vestibular disturbances between the COVID-19 (+) and the COVID-19 (-) groups (RR = 1.29, 95% CI = 1.01-1.66; RR = 2.33, 95% CI = 2.19-2.48)., Conclusion: New onset hearing loss is not more common in patients with a positive COVID-19 test than those with a negative COVID-19 test. Audiologic evaluation is needed to verify reported hearing disturbances. Although statistically significant in specific population groups, tinnitus and vestibular disturbances may not be clinically significant due to the low incidence., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: Idiopathic sudden sensorineural hearing loss (ISSNHL) affects 66,000 patients per year in the United States. Genetic mutations have been associated with progressive hearing loss; however, genetic mutations associated with ISSNHL have not been identified., Methods: A prospective cohort study of adults older than 18 years presenting with ISSNHL at a tertiary academic medical center. Whole exome sequencing (WES) was conducted using Genome Analysis Toolkit best practices. An automated diagnostic screen employing a variety of models for pathogenicity was conducted across all genes with no specific targets. Candidate pathogenic variants were reviewed by a team of geneticists and clinicians. Variants were crossed-referenced with 92 known hearing loss associated genes., Results: Twenty-nine patients with SSNHL were screened using WES. The average age of patients was 53 ± 17.1 years, and most patients were White (62%) and men (55%). The mean pure tone average was 64.8 ± 31.3 dB for the affected ear. Using a 0.1% allele frequency screen, 12 (41%) cases had a mutation in any of the nine selected myosin genes. When we restrict to singletons (allele frequency = 0%), 21% (n = 6) of cases have qualifying variants, whereas only 3.8% (n = 481) of 12,577 healthy controls carry qualifying variants (p < 0.01). Most mutations (80%) were missense mutations. Of the novel mutations, one was a frameshift mutation, and two were a stop-gained function. Three were missense mutations., Conclusion: Myosin mutations may be associated with ISSNHL. However, larger population screening is needed to confirm the association of myosin mutation with ISSNHL and steroid responsiveness., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: Acute spontaneous vertigo with idiopathic sudden sensorineural hearing loss (ISSNHL) can be diagnosed as labyrinthitis, which has a poorer prognosis than ISSNHL without vertigo. Thus, we aimed to identify the effect of the baseline vestibular function on the prognosis of labyrinthitis., Methods: A total of 23 patients with labyrinthitis was retrospectively divided into the recovered group (complete recovery, partial recovery) and the nonrecovered group (slight improvement, no improvement). Differences in caloric weakness and gain in the video head impulse test (vHIT) between the two groups were compared. In addition, the prognostic value of the vHIT on each of the three semicircular canals in predicting hearing recovery was analyzed using a linear regression model., Results: In final pure-tone audiometry, 2 patients (8.70%) exhibited complete recovery, 4 patients (17.39%) had partial recovery, and 17 patients (73.91%) had slight or no improvement. The initial ipsilesional posterior canal (iPC) gain and the contralesional anterior canal (cAC) gain were significantly decreased in the nonrecovered group (p < 0.013 for iPC and p < 0.007 for cAC, Mann-Whitney U test). The mean hearing gain was positively correlated with the iPC gain (R2 = 0.36, p = 0.003, Spearman correlation analysis)., Conclusion: An abnormal iPC gain may be a poor prognostic factor for hearing recovery. Additionally, the vHIT on the three semicircular canals can provide prognosis and insights into the pathophysiological mechanisms in patients with labyrinthitis., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: To investigate the utility and effectiveness of a noise-attenuating, tablet-based mobile health system combined with asynchronous telehealth evaluations for screening rural Nicaraguan schoolchildren for hearing loss., Study Design: Prospective population-based survey., Setting: Rural Nicaraguan communities., Patients: There were 3,398 school children 7 to 9 years of age., Interventions: Diagnostic automated and manual audiometry, detailed asynchronous telehealth evaluations., Main Outcome Measures: Referral rates, ambient noise levels, and audiometric results as well as hearing loss prevalence, types, and risk factors., Results: Despite high ambient noise levels during screening (46.7 dBA), no effect of noise on referral rates on automated audiometry or confirmatory manual audiometry in those who failed automated testing was seen. The overall audiometric referral rate was 2.6%. Idiopathic sensorineural hearing loss (SNHL) and cerumen impaction were the most common types of hearing loss in this population with an estimated prevalence of hearing loss (all types) of 18.3 per 1,000 children. SNHL was associated with both drug exposure during pregnancy (p = 0.04) and pesticide exposure in the home (p = 0.03)., Conclusion: Hearing screening using a tablet-based, noise-attenuating wireless headset audiometer is feasible and effective in rural low-resource environments with moderately elevated ambient noise levels. The referral rate with noise-attenuating headsets was much lower than that previous reports on this population. In addition, manual audiometry resulted in much lower referral rates than automated audiometry. The confirmed hearing loss rate in this study is comparable to reports from other low-income countries that use some form of noise attenuation during screening. Pesticide exposure and drug exposure during pregnancy are potential causes of SNHL in this population., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Hypothesis: Adult genetic sensorineural hearing loss (SNHL) may be underestimated., Background: The diagnosis of genetic hearing loss is challenging, given its extreme genetic and phenotypic heterogeneity, particularly in adulthood. This study evaluated the utility of next-generation sequencing (NGS) in the etiological diagnosis of adult-onset SNHL., Materials and Methods: Adults (>16 yr old) with SNHL were recruited at the Otolaryngology Department at Marqués de Valdecilla University Hospital (Spain). Environmental factors, acoustic trauma, endolymphatic hydrops, and age-related hearing loss were excluding criteria. An NGS gene panel was used, including 196 genes (OTOgenics v3) or 229 genes (OTOgenics v4) related to syndromic and nonsyndromic hearing loss., Results: Sixty-five patients were included in the study (average age at the onset of SNHL, 41 yr). Fifteen pathogenic/likely pathogenic variants considered to be causative were found in 15 patients (23% diagnostic yield) in TECTA (4), KCNQ4 (3), GJB2 (2), ACTG1 (1), COL2A1 (1), COCH (1), COCH/COL2A1 (1), STRC (1), and ABHD12 (1). Three patients had syndromic associations (20% of patients with genetic diagnosis) that had not been previously diagnosed (two Stickler type I and one polyneuropathy, hearing loss, ataxia, retinitis pigmentosa, cataract syndrome). Seven variants of unknown significance were found in COL11A1 (1), GSMDE (2), DNTM1 (1), SOX10 (1), EYA4 (1), and TECTA (1)., Conclusion: NGS gene panels can provide diagnostic yields greater than 20% for adult SNHL, with a significant proportion of variant of unknown significance that could potentially contribute to increasing diagnostic output. Identifying a genetic cause enables genetic counseling, provides prognostic information and can reveal unrecognized syndromes contributing to an accurate management of their associated manifestations., Competing Interests: The authors have no conflicts of interest to disclose or external funding to declare., (Copyright © 2022, Otology & Neurotology, Inc.)

Abstract: Sensorineural hearing loss (SNHL) is the most common sensory deficit, disabling nearly half a billion people worldwide. The cochlear implant (CI) has transformed the treatment of patients with SNHL, having restored hearing to more than 800,000 people. The success of CIs has inspired multidisciplinary efforts to address the unmet need for personalized, cellular-level diagnosis, and treatment of patients with SNHL. Current limitations include an inability to safely and accurately image at high resolution and biopsy the inner ear, precluding the use of key structural and molecular information during diagnostic and treatment decisions. Furthermore, there remains a lack of pharmacological therapies for hearing loss, which can partially be attributed to challenges associated with new drug development. We highlight advances in diagnostic and therapeutic strategies for SNHL that will help accelerate the push toward precision medicine. In addition, we discuss technological improvements for the CI that will further enhance its functionality for future patients. This report highlights work that was originally presented by Dr. Stankovic as part of the Dr. John Niparko Memorial Lecture during the 2021 American Cochlear Implant Alliance annual meeting., Competing Interests: The author discloses no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objectives: There is an unmet need to match the anticipated natural history of hearing loss (HL) in enlarged vestibular aqueduct (EVA) with clinical management strategies. The objectives of this study are therefore to provide a detailed case characterization of an EVA cohort and explore the relationship between candidate prognostic factors and timing of cochlear implant (CI) surgery., Study Design: A multicenter retrospective review of patients diagnosed with EVA., Setting: Patient data recruitment across three CI centers in the UK., Patients: One hundred fifty patients with a radiological diagnosis of EVA from January 1995 to January 2021., Main Outcome Measures: Age at audiological candidacy for CI and age at first implant surgery., Results: EVA was predominately a bilateral condition (144/ 150) with increased prevalence in women (M:F, 64:86). 51.7% of patients failed new-born hearing screening, with 65.7% having HL diagnosed by 1 year. Initial moderate to severe and severe to profound HL were reported most frequently. In 123 patients, median age that audiological candidacy for CI was met for at least one ear was 2.75 years. Median age at first CI was 5 years (140/150).Pendred syndrome (confirmed in 73 patients) and ethnicity, were not significantly associated with earlier CI surgery. Multivariate linear regression demonstrated that male patients have first CI surgery significantly earlier than females (coefficient -0.43, 95% CI [-0.82, -0.05), p-value = 0.028)., Conclusions: This large UK EVA cohort provides evidence that patients should be closely monitored for CI candidacy within the first 3 years of life. Significantly, male gender is emerging as an independent prognostic factor for earlier assessment and first CI surgery., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: Sudden sensorineural hearing loss (SSNHL) is an ideal entity for quality indicator (QI) development, providing treatment challenges resulting in variable or substandard care. The American Academy of Otolaryngology-Head and Neck Surgery recently updated their SSNHL guidelines. With SSNHL demonstrating a large burden of illness, this study sought to leverage the updated guidelines and develop QIs that support quality improvement initiatives at an individual, institutional, and systems level., Methods: Candidate indicators (CIs) were extracted from high-quality SSNHL guidelines that were evaluated using the Appraisal of Guidelines for Research and Evaluation II tool. Each CI and its supporting evidence were summarized and reviewed by a nine-member expert panel based on validity, reliability, and feasibility of measurement. Final QIs were selected from CIs using the modified RAND Corporation-University of California, Los Angeles appropriateness methodology., Results: Fifteen CIs were identified after literature review. After the first round of evaluations, the panel agreed on 11 candidate indicators as appropriate QIs with 2 additional CIs suggested for consideration. An expert panel meeting provided a platform to discuss areas of disagreement before final evaluations. The expert panel subsequently agreed upon 11 final QIs as appropriate measures of high-quality care for SSNHL., Conclusion: The 11 proposed QIs from this study are supported by evidence and expert consensus, facilitating measurement across a wide breadth of quality domains. With the recently updated SSNHL guidelines, and a greater focus on quality improvement opportunities, these QIs may be used by healthcare providers for targeted quality improvement initiatives., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2021, Otology & Neurotology, Inc.)

Objective: Idiopathic sudden sensorineural hearing loss (ISSNHL) may not only impair cochlear function but also damage vestibular structures, including the saccule, utricle, semicircular canals, and vestibular afferents. Poor recovery often occurs in patient with serious ISSNHL and accompanying vertigo. To define the severity of the disease and to predict prognosis, galvanic vestibular-evoked myogenic potentials (VEMPs) are added to the inner ear test battery., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Thirty patients with unilateral severe to profound ISSNHL and accompanying vertigo were enrolled in this retrospective study., Intervention: Diagnostic., Main Outcome Measures: All subjects underwent pure tone audiometry, cervical and ocular VEMPs, and caloric tests before the initiation of treatment. The treatment outcome, rates of abnormal responses in the tests, and the characteristic parameters of VEMPs such as latencies and amplitudes, were analyzed., Results: In affected ears, the rates of abnormal acoustic cVEMPs, vibratory oVEMPs, galvanic cVEMPs, and galvanic oVEMPs were 60, 47, 37, and 20%, respectively. The improvement in the hearing of the affected ear was specified as good recovery or poor recovery. The normal galvanic VEMP group had a significant higher rate of good recovery than abnormal galvanic VEMP group (87% versus 27%; p = 0.003)., Conclusions: Patients with unilateral severe to profound ISSNHL and accompanying vertigo who have normal galvanic VEMPs have a higher likelihood of hearing recovery than those who have abnormal galvanic VEMPs., Competing Interests: None of the authors has potential conflicts of interest to be disclosed., (Copyright © 2021, Otology & Neurotology, Inc.)

Objective: To evaluate auditory manifestations in children born to mothers who had exanthema during pregnancy, suspected to have been exposed to the Zika virus (ZIKV)., Study Design: Prospective observational., Setting: Tertiary referral center., Patients: Children born in Rio de Janeiro, Brazil, between April 2016 and September 2017, who were referred for newborn hearing screening (NHS)., Intervention: The NHS was performed by the automated brainstem auditory-evoked potential test at an intensity of 30 dBHL (decibels Hearing Level) with the result presented as "PASS/FAIL." A follow-up test was performed 6 months after the first examination., Main Outcome Measures: Hearing outcomes by audiological assessment., Results: Ninety-eight children were recruited and 78 underwent the NHS test. In the first evaluation, the FAIL NHS result was observed in 4 of the 78 children. Three were diagnosed with sensorineural hearing loss and one had conductive loss. Including the first and second evaluation, the frequency of audiological alterations was 5.1%. Of the four children diagnosed with hearing loss, two were carriers of ZIKV, one had suspected ZIKV infection, and one was asymptomatic with confirmed exposure to the virus. There was no progression of hearing loss or other hearing abnormality in the children by the time of the second evaluation. The group of nonexposed children (negative quantitative reverse transcription polymerase chain reaction for ZIKV) showed no hearing loss., Conclusion: Uni or bilateral sensorineural hearing loss was diagnosed in asymptomatic children at birth. These observations highlight the importance of periodic follow-up of patients with congenital Zika syndrome to better understand their long-term auditory clinical outcome.

Hypothesis: The phenotype of DFNA11 consists of specific features at diverse developmental and age stages., Background: Only eight mutations have been identified for autosomal dominant non-syndromic hearing loss related to MYO7A (DFNA11), and the onset and progression of DFNA11 are poorly understood., Methods: After linkage analysis and following Sanger sequencing in a family suspected to have autosomal dominant hereditary hearing loss, we analyzed the audiometric and vestibular functions and their long-term changes in the subjects carrying the variant., Results: A reported variant of uncertain significance, NP&#95;000251.3:p.Arg853His, in MYO7A was detected and cosegregation data of this large family provided evidence that the variant was likely pathogenic for DFNA11. Family members with the variant had no other symptoms associated with hearing loss and were confirmed to have autosomal dominant non-syndromic sensorineural hearing loss. Audiograms tended to show gently sloping configuration in childhood and flat configuration after the age of 30 years. Hearing loss at high frequencies progressed slowly, while hearing at low frequencies started to deteriorate later but progressed more rapidly. Some subjects showed partly abnormal results in the distortion products of otoacoustic emissions before the elevation of hearing thresholds. Vestibular function was within the normal range in all the subjects tested., Conclusion: We revealed that hearing loss at high frequencies was mainly noted in early developmental stages and that thresholds increased more rapidly in the low frequency range, resulting in changes in audiometric configuration. Deterioration of distortion product otoacoustic emissions (DPOAE) before the elevation of hearing thresholds was considered as a clinical feature of DFNA11.

Objective: To identify demographic and audiometric predictors of bothersome tinnitus within a large clinical cohort., Study Design: Retrospective chart review., Setting: Tertiary care hospital., Patients: 51,989 English-speaking patients between 18 and 80 years of age that received initial audiometric evaluations at the Massachusetts Eye and Ear Infirmary between the years 2000 and 2016., Main Outcome Measures: Patients were categorized according to whether or not tinnitus was the primary reason for their visit. The likelihood of tinnitus as a primary complaint (TPC) was evaluated as a function of age, sex, and audiometric configuration. Patient-reported tinnitus percepts were qualitatively assessed in relation to audiometric configuration., Results: Approximately 20% of adults who presented for an initial hearing evaluation reported TPC. The prevalence of TPC increased with advancing age until approximately 50 to 54 years, and then declined thereafter. In general, men were significantly more likely to report TPC than women. TPC was statistically associated with specific audiogram configurations. In particular, TPC was most prevalent for notched and steeply sloping hearing losses, but was relatively uncommon in adults with flat losses. Patients with frequency-restricted threshold shifts often reported tonal tinnitus percepts, while patients with asymmetric configurations tended to report broadband percepts., Conclusions: The probability of seeking audiological evaluation for bothersome tinnitus is highest for males, middle-aged patients, and those with notched or high-frequency hearing losses. These findings support the theory that tinnitus arises from sharp discontinuities in peripheral afferent innervation and cochlear amplification, which may induce topographically restricted changes in the central auditory pathway.

Objectives: The aims of the study are to identify the limitations of the current newborn hearing screening (NHS) programs and provide recommendations for better protocols., Study Design: Retrospective analysis., Setting: Tertiary referral center., Patients: The study participants were 185 children who received cochlear implants (CIs) at ≤5 years of age., Interventions: Therapeutic and rehabilitative., Main Outcome Measures: The results of NHS, screening tools used, age, and hearing thresholds at which hearing loss was confirmed, causes of the hearing loss, age of CI insertion, aided pure-tone audiogram findings, and language development level were analyzed., Results: NHS data was available for 109 children, and 24 patients (22.0%) had passed NHS for both ears. Hearing loss was confirmed considerably later in children who had passed NHS than in children who were referred for further evaluation (p < 0.01). The most common cause for hearing loss in the NHS-pass group was SLC26A4 mutations (41.7%). Patients in the NHS-pass group received CIs considerably later than those in the NHS-referred group (p < 0.01). Among patients with SLC26A4 mutations, the language development level was significantly lower in the NHS-pass group than in the NHS-referred group (p < 0.01)., Conclusions: Careful counseling regarding NHS results is necessary for parents to understand that the absence of hearing loss at birth does not mean that the child will not develop hearing loss later in life. Genetic testing for SLC26A4 mutations may be necessary in regions with a high incidence of these mutations, such as East Asia.

Objective: To investigate the association of serum bilirubin level with hearing outcomes in bilateral sudden sensorineural hearing loss (BSSHL) patients., Participants: One hundred thirteen in-patient BSSHL patients were consecutively enrolled between July 2008 and December 2015 in a tertiary center., Main Outcome Measures: Multivariable linear regression, generalized estimating equations (GEE), and stratified analyses were applied to examine the association between serum bilirubin level and hearing outcome measures such as final hearing threshold and absolute and relative hearing gains in BSSHL., Results: After full adjustment for potential confounders, total bilirubin levels (TBIL) were observed to be positively and independently associated with hearing outcomes as measured by final hearing (β [95% confidence interval {CI}]: -1.5 [-2.7, -0.2] dB HL per 1 μmol/L increase in TBIL) and absolute and relative hearing gains (β [95% CI]: 1.4 [0.2, 2.7] dB and 1.6 [0.2, 3.1] dB, respectively) in the severe to profound hearing loss subpopulation., Conclusions: Higher TBIL levels, within the normal or mildly elevated ranges, were independently and significantly associated with better hearing outcome in BSSHL patients with severe to profound hearing loss. Given bilirubin elevation treatments exist, our finding suggests a novel pharmacological strategy for this specific subpopulation.

Objective: There is a common misconception that improvement in sudden sensorineural hearing loss (SSNHL) after treatment with steroid therapy effectively excludes the diagnosis of a vestibular schwannoma (VS) and such cases do not warrant an MRI. Paralleling this, steroids are commonly withheld for SSNHL in patients with an existing diagnosis of VS, believing that this condition is not steroid-responsive. This study seeks to underscore that improvement or recovery of SSNHL with steroid therapy does not exclude the diagnosis of VS and does not preclude the need for magnetic resonance imaging., Methods: A retrospective chart review was performed (2002-2017) of patients with previously untreated sporadic VS who developed SSNHL that improved after steroid treatment. A clinically significant audiometric improvement was defined as an increase of more than or equal to 15% in word recognition score (WRS) and/or decrease of more than or equal to 15 dB in 4-frequency pure-tone average (PTA). To supplement these data, a separate population of patients with incomplete or missing audiometric data, who reported unequivocal subjective improvement in hearing after steroid treatment, were also described to reinforce the study objective. Patient demographics, tumor characteristics, steroid regimen, and data regarding treatment response were recorded., Results: A total of 29 patients (55% women; median age of 47 yr) met inclusion criteria. Fourteen (48%) cases had objective audiometric documentation of SSNHL, while 15 (52%) had either subjective report only or incomplete audiometric data available. Eighteen (62%) had a single event, while 11 (38%) had more than one episode of SSNHL that was treated with steroids. For all patients, the median time between SSNHL and diagnosis of VS was 1.3 months (range, 0.13-148.4 mo). At the time of diagnosis, 15 tumors were purely intracanalicular, while 15 tumors had cerebellopontine angle extension. Of the latter, the median cisternal tumor size was 15.9 mm (range, 5.3-33). Twenty-six (90%) cases received oral steroid therapy alone, two (9%) had intratympanic steroid therapy alone, and one (3%) required combination therapy. The median PTA improvement with steroid therapy was 21 dB HL (range, -10-101.2) and the median WRS improvement was 40% (range, 4-100%)., Conclusion: A therapeutic response to steroid therapy for SSNHL does not exclude the diagnosis of VS. All patients with SSNHL should undergo appropriate diagnostic imaging to prevent delays in diagnosis and potential treatment.

Hypothesis: We hypothesized that patients with DFNB16 caused hearing loss show characteristical audiological findings depending on genetic results., Background: Hearing loss belongs to the most frequent congenital diseases. In 50-70% of individuals, hearing loss is caused by genetic defects. DFNB1 (deafness, neurosensory, autosomal-recessive) is the most frequently affected locus. Despite its great genetic heterogeneity, comprehensive analysis of genes like STRC, encoding stereocilin (DFNB16) is possible. The genetic architecture of the DFNB16 locus is challenging and requires a unique molecular genetic testing assay. The aim of the study is a systematic characterization of the audiological phenotype in DFNB16-positive patients., Methods: Since 2011, 290 patients with suspicion of inherited hearing loss received a human genetic exploration. Eighty two DFNB1-negative patients advanced to further testing in the DFNB16 locus. STRC-positive patients obtained complete audiological diagnostic workup. Additionally, epidemiological data was collected., Results: Nine of 82 (11%) of the examined patients (mean age 5 yr) showed mutations in the STRC (3 homozygous, 6 compound heterozygous). Aside from a moderate hearing loss in the pure tone audiogram, auditory brainstem response thresholds were 40-50 dB nHL. Otoacoustic emissions were detectable in only one patient., Conclusions: Examination of the DFNB16-locus should be a standard diagnostic test after negative DFNB1-gene screening result. Notably, DFNB16-associated hearing loss can be audiologically characterized as moderate sensorineural hearing loss in the main speech field with absent otoacoustic emissions. Our study is the first to correlate audiological findings with genetic results in patients with hearing loss due to STRC.

Objective: High blood viscosity has been proposed as a mechanism for sudden sensorineural hearing loss (SSNHL); however, the relationship between blood markers of fibrinolysis or coagulation and severity or prognosis of SSNHL is still unclear. The aim of this study is to investigate the relationship between serum fibrinogen and SSNHL., Data Sources: PubMed and Scopus were searched for English language articles using the following keywords: SSNHL, sudden hearing loss, sudden deafness, idiopathic hearing loss or idiopathic sensorineural hearing loss, and fibrinogen., Study Selection: The articles in the study related to SSNHL and provided data about the serum fibrinogen level., Data Extraction: The data included patient profiles, fibrinogen level, recovery, and treatment modality., Data Synthesis: Nineteen articles were selected. The aggregated data were analyzed using the random effect model. Two articles that included the fibrinogen level with recovery rates were analyzed for the relationship between the fibrinogen level and recovery., Conclusions: The average fibrinogen level was 318 ± 8.5 mg/dl (mean ± standard error, within normal range). There was no difference in the fibrinogen level between SSNHL patients and the control group. The fibrinogen level of the recovery group was lower than that of the no recovery group. This showed that a high fibrinogen level was associated with poor prognosis, and it could be related to the severity of the pathological change rather than being the cause of the SSNHL. SSNHL includes various pathologies; therefore, the appropriate therapy should be selected based on each condition.

Objective: To investigate the characteristics, treatment, and prognostic factors of sudden sensorineural hearing loss (SSNHL) in children., Methods: Seventy-five cases (78 ears) of SSNHL in children from February 2011 to June 2016 were retrospectively analyzed. We scrutinized the clinical manifestations, audiological assessments, and serologic examinations of these pediatric cases by univariate and multivariate logistic analysis methods. The patients were divided into four groups according to their audiometric curve type: ascending, descending, flat, and profound., Results: Of the 75 patients (78 ears), 25 patients were in the ascending group (32.00%), 9 patients were in the descending group (12.00%), 17 patients were in the flat group (22.67%), and 24 patients were in the profound group (32.32%). The overall recovery rates (complete + partial + slight) of the different groups were as follows: ascending group, 96.00%; flat group, 76.47%; profound group, 50.00%; and descending group, 44.44%. The overall recovery rate of all patients was 70.67%. The multivariate logistic analysis showed that the type of audiometric curve and the interval from onset to intervention were two independent risk factors that correlated with the prognosis of SSNHL in children. Some children had positive cytomegaoviyns, rubella virus, and herpes simplex virus immunoglobulin G antibodies. Twenty-one children were treated with additional intratympanic methylprednisolone as salvage therapy and 13 of these children showed improved (complete + partial + slight) recoveries. Three children had postauricular compound betamethasone injections, but none of them showed improvement. One of three children recovered slightly after treatment with intratympanic methylprednisolone combined with postauricular betamethasone injection., Conclusions: The prognosis of SSNHL in children is closely related to the type of audiometric curve and the onset of treatment. Intratympanic methylprednisolone and compound betamethasone injected postauricularly could be effective for SSNHL in children.

Objective: Sensorineural hearing loss is a common sequela of bacterial meningitis. The objective of this study is to delineate the incidence and course of hearing loss after bacterial meningitis., Study Design: Retrospective cohort study., Setting: Tertiary referral center., Patients: Data of 655 patients who suffered from bacterial meningitis between 1985 and 2015 were analyzed., Interventions: None., Main Outcome Measurements: Availability of audiometric data, incidence of hearing loss, and onset and course of hearing loss., Results: In this cohort the incidence of hearing loss (>25 dB) was 28% (95% confidence interval 23-34%). The incidence of profound hearing loss (>80 dB) was 13% (95% confidence interval 10-18%). Normal hearing at the first assessment after treatment for meningitis remained stable over time in all these patients. In 19 of the 28 patients with diagnosed hearing loss, the hearing level remained stable over time. Hearing improved in six patients and deteriorated in two patients. One patient showed a fluctuating unilateral hearing loss., Conclusion: Audiological tests in patients with bacterial meningitis, especially children, should be started as soon as possible after the acute phase is over. As we found no deterioration of initial normal hearing after bacterial meningitis, repeated audiometry seems indicated only for those with diagnosed hearing loss at first assessment.

Objective: To examine the possible speech recognition and health related quality of life (HRQoL) benefits of cochlear implantation among adults with asymmetric sensorineural hearing loss., Study Design: Retrospective chart review, single-subject design., Methods: A total of 45 adult cochlear implant recipients with asymmetric sensorineural hearing loss where performance for the best-aided condition exceeded 60% correct open set sentence recognition in quiet, and the implanted ear met traditional candidacy criteria. End point testing of the implanted ear was evaluated with use of the Consonant-Vowel Nucleus-Consonant (CNC) word test and AzBio sentence test materials in quiet, and bimodally with the AzBio sentence test materials in noise at +5 dB signal-to-noise ratio (SNR). HRQoL was measured using the Nijmegen Cochlear Implant Questionnaire (NCIQ)., Results: Measured in quiet, with the non-implanted ear plugged, the average CNC word scores increased from 9.1% preoperatively to 55.7% (p < 0.01) at the 6-month post-activation test interval. Similarly, average AzBio sentence scores in quiet, with the non-implanted ear plugged, increased from 13.9% preoperatively to 73.4% (p < 0.01) at the 6-month post-activation test interval. Finally, in the bilateral/bimodal condition, the AzBio sentence score in +5 dB SNR improved from an average of 26.8% preoperatively to 52.4% (p < 0.01) at the 6-month test interval. Results of the NCIQ showed improved scores on all six subdomains., Conclusions: These data demonstrate significant benefit of cochlear implantation among a group of postlingually deafened adults whose preoperative hearing and aided speech recognition fell outside of the currently specified Food and Drug Administration candidacy guidelines. Results of this study support the evaluation of a candidate's speech recognition in noise in the best-aided condition to adequately assess candidacy for a cochlear implant.

Objective: To determine if discussing cochlear implantation (CI) with patients with enlarged vestibular aqueducts (EVA) and their families before reaching audiological criteria for CI candidacy effects the length of time between reaching audiological candidacy and CI surgery, and to describe the universal newborn hearing screening (UNHS) results and communication modality in this sample., Patients: Forty-two patients (25 females) with confirmed EVA and cochlear implants., Intervention(s): Diagnostic CI visit., Main Outcome Measures: The primary outcome measure is the difference in length of time between reaching audiological candidacy for CI and surgical implantation between those who had preliminary discussions regarding CI with their medical and healthcare providers before reaching audiological candidacy versus who had discussions after reaching candidacy. The secondary outcome measure is the result of the UNHS and primary mode of communication used by each patient., Results: Discussing CI before reaching audiological candidacy was associated with a significantly shorter duration between reaching audiological candidacy and receiving CI (median = 3.1 mo; interquartile range [IQR] = 1.7-5.4) as compared with discussing CI after reaching candidacy (median = 5.8 mo; IQR = 3.2-11.2; p = 0.012). Participants born after the implementation of the UNHS, 16 of 24 patients referred on one or both ears. Communication modalities were evenly divided between utilizing sign-support English and oral/aural communicators only., Conclusions: Discussion of CI in patients with EVA before reaching audiological candidacy reduces the amount of time the child is without adequate auditory access and contributes to a constructive and interactive preparatory experience.

Objective: To demonstrate the feasibility of a nerve integrity monitor as a tool for promontory stimulation testing in patients with profound sensorineural hearing loss considering cochlear implantation., Patients: Adult patients considered for cochlear implantation with no auditory response on audiometric testing INTERVENTION:: Promontory stimulation testing using the nerve integrity monitor., Main Outcome Measure: By using a facial nerve stimulator and the nerve integrity monitor, transtympanic promontory stimulation testing was performed to assess auditory nerve function and determine candidacy for cochlear implantation. Patients indicated if they heard the stimulus., Results: Of the four patients completing the promontory stimulation tests, three patients heard the stimulus and one patient did not hear the stimulus. Of the three patients with a positive stimulation test, two patients have a history of progressive profound sensorineural hearing loss and one patient had a history of severe blunt temporal bone trauma. Two of these patients proceeded with cochlear implantation. The patient who had a negative promontory stimulation test has a history of neurofibromatosis type 2., Conclusion: The nerve integrity monitor is a convenient tool that can be used in the clinic setting to perform promontory stimulation tests and aid in determining cochlear implant candidates, specifically in those patients who require verification of auditory nerve function. This tool is a feasible and reasonable method for promontory stimulation testing.

Objective: Mobile hearing applications (apps) are available for hearing testing, personal sound amplification, as well as hearing aid modulation. Hearing testing apps are gaining popularity, especially in resource-limited settings. The reliability of mobile hearing testing apps, however, is not well characterized., Patients/interventions: A case study of a single patient with a complaint of sudden hearing loss presenting to a tertiary-care hospital., Main Outcome Measure: Comparison of a mobile hearing testing app results with standard audiogram., Results: A commercially available mobile hearing testing app was used after hours to determine if a patient's hearing complaints were consistent with sudden sensorineural hearing loss. The hearing app produced a rudimentary audiogram that was consistent with unilateral SSNHL. Given contraindications to oral treatment, preparations for possible intratympanic dexamethasone after a full audiometric evaluation were completed. Confirmatory audiogram the following day demonstrated normal hearing without evidence of hearing loss. Steroid treatment was aborted and appropriate counseling provided., Conclusion: While mobile hearing testing apps offer improved access to hearing screening in resource-limited settings, caution must be exercised when interpreting data and making clinical decisions based upon results. The role of professional audiologists remains critical. Further testing and validation of specific apps is required.

Objective: To highlight Noonan syndrome as a clinically recognizable cause of severe to profound sensorineural hearing impairment., Study Design: New clinical cases and review., Setting: Patients evaluated for etiological diagnosis by a medical geneticist in a reference center for hearing impairment., Patients: Five patients presenting with confirmed Noonan syndrome and profound sensorineural hearing impairment., Interventions: Diagnostic and review of the literature., Results: Five patients presented with profound sensorineural hearing impairment and molecularly confirmed Noonan syndrome. Sensorineural hearing impairment has been progressive for three patients. Cardiac echography identified pulmonary stenosis in two patients and was normal for the three other patients. Short stature was found in two patients. Mild intellectual disability was found in one patient. Inconspicuous clinical features as facial dysmorphism, cryptorchidism, or easy bruising were of peculiar interest to reach the diagnosis of Noonan syndrome., Conclusion: Profound sensorineural hearing impairment can be the main feature of Noonan syndrome. Associated features are highly variable; thus, detailed medical history and careful physical examination are mandatory to consider the diagnosis in case of a sensorineural hearing impairment.

Objective: The aims of the present study were to investigate the prevalence of cochlear dead regions (DRs) in patients with sudden sensorineural hearing loss (SSNHL) and compare the hearing outcome according to the presence of DRs., Study Design: Retrospective chart review., Setting: Tertiary referral center., Patients and Methods: The threshold-equalizing noise (HL) test was performed on a total of 112 ears diagnosed with SSNHL. Ears were divided into two groups based on the presence of DRs. Eighty-two ears belonged to the group without DRs and 30 ears belonged to the group with DRs. There was no difference between the two groups with respect to age, sex, side of affected ear, presence of bilateral SSNHL, presence of vertigo, history of treatment, and initial pure-tone thresholds. Pure-tone audiograms were gathered at the time of initial presentation and at 1, 3, and 6 months after onset of symptoms., Results: The prevalence of DRs was observed to be 29% and was found to be prevalent at 1k and 1.5k Hz in patients with SSNHL. When the hearing thresholds over times were compared between the two groups, subjects with DRs showed significantly poorer hearing outcome compared with those without DRs. The improvements in word recognition scores over times were also less in subject with DRs than those without DRs., Conclusion: DRs are associated with worse hearing thresholds at follow-up audiogram and might be associated with unfavorable hearing outcome in patients with SSNHL.

Objectives: To evaluate the cost-effectiveness of obtaining a magnetic resonance imaging (MRI) in patients with abnormal electronystagmography (ENG) or videonystagmography (VNG) results., Study Design: Retrospective chart review., Settings: Academic specialty center., Patients: Patients presenting with vertigo between January 1, 2010, and August 30, 2013., Methods: Patients who fit the following abnormal criteria were included in the study: unilateral caloric weakness (≥20%), abnormal ocular motor testing, and nystagmus on positional testing. Patients with abnormal findings who then underwent MRI with gadolinium were evaluated., Results: Of the 1,996 charts reviewed, there were 1,358 patients who met the inclusion criteria. The average age of these patients was 62 years (12-94 yr). The male:female ratio was approximately 1:2. Of the 1,358 patients, 253 received an MRI with the following pathologies: four vestibular schwannomas, three subcortical/periventricular white matter changes suspicious for demyelinating disease, four acute cerebellar/posterior circulation infarct, two vertebral artery narrowing, one pseudomeningocele of internal auditory canal, and two white matter changes indicative of migraines. The positive detection rate on MRI was 5.5% based on MRI findings of treatable pathologies causing vertigo. Average cost of an MRI is $1,200, thereby making the average cost of identifying a patient with a positive MRI finding $15,180., Conclusion: In our study, those patients with a positive MRI had a constellation of symptoms and findings (asymmetric sensorineural hearing loss, tinnitus, vertigo, and abnormal ENG/VNG). Cost-effectiveness can be improved by ordering an MRI only when clinical examination and VNG point toward a central pathology. Clinical examination and appropriate testing should be factored when considering the cost-effectiveness of obtaining an MRI in patients with abnormal ENG/VNG findings.

Objectives: To measure autonomic nervous system function after idiopathic sudden sensorineural hearing loss (ISSHL)., Study Design: Diagnostic prospective cohort single-center study., Setting: Tertiary referral university hospital., Patients: Twenty-three adult patients with ISSHL and 10 normal-hearing control patients without ISSHL (CON) matched with respect to age, sex, hypertension, and medication., Main Outcome Measures: Bivariate analysis of autonomic regulation (ISSHL versus CON) using 30-minute heart rate (HR) and systolic and diastolic blood pressure (BP) time series at baseline, based on cardiovascular coupling, HR and BP regulatory patterns, high-resolution coupling analysis based on joint symbolic dynamics (High-Resolution Joint Symbolic Dynamics)., Intervention: No intervention., Results: Multivariate discrimination between ISSHL and CON achieved values of area under the receiver operator characteristic curve = 95.5, sensitivity = 90.9%, and specificity = 88.9%. Independent from medication and hypertension increased the complexity of nonlinear HR regulation and reduced cardiovascular coupling of ISSHL patients and independent from hypertension altered nonlinear systolic and diastolic BP regulation. Coupling patterns are characterized by a less pronounced strong and fast decrease of systolic BP when HR increases and rapidly changes in ISSHL patients. Disturbed BP modulation and complexity by impaired baroreflex activities resulting in short-term BP fluctuations, altered peripheral resistance, and reduced cochlear blood flow. Increased values for the pulse wave velocity in the aorta and carotid-femoral were early indicators that the elasticity of the arteries might be restricted in ISSHL patients., Conclusion: ISSHL patients show an altered autonomic regulation. At least a subgroup of ISSHL patients seems to exist where a vascular impairment might play an important role in the pathogenesis of this disease.

Objective: To explore the recurrence rate of idiopathic sudden sensorineural hearing loss (ISSNHL)., Design: Using the Taiwan Longitudinal Health Insurance Database, we compared in a retrospective cohort study 45,715 patients with ISSNHL between January 2001 and December 2006 with 45,715 age-, gender-, and comorbidity-matched controls without ISSNHL. We followed each patient from 180 days after the initial diagnosis until the end of 2009 and evaluated the incidence of ISSNHL for 3 years minimum., Results: The cumulative incidence of ISSNHL recurrence was 4.99%. Cox proportional hazard regressions showed that the 50- to 64-year-old age group had a higher adjusted hazard ratio (1.59; 95% confidence interval: 1.40-1.81) than did the 0- to 34-year-old age group. Different comorbidities did not significantly affect the incidence of ISSNHL relapse., Conclusions: The risk of an ISSNHL relapse in patients with a history of ISSNHL was higher than was the risk of a first occurrence of ISSNHL in the Controls. We suggest that physicians counsel patients with a history of ISSNHL to seek medical attention if they have hearing impairments because they may also have a high risk of an ISSNHL relapse., Evidence Level: 2B.

Introduction: We report the first findings of functional magnetic resonance imaging of the auditory cortex in a young woman with a bilateral cochleovestibular deficit as first manifestation of Brown-Vialetto-Van Leare syndrome. The patient had no open speech discrimination, even with hearing aids, and is depending on lip reading for communication., Methods: To evaluate the possible efficiency of a cochlear implantation, we investigated hemodynamic responses within the central auditory pathways using an auditory functional magnetic resonance imaging paradigm., Results: Blood oxygen level-dependent correlates were detected bilaterally along the auditory pathways after exposure to intermittent clicking tone stimulation at 2 kHz., Conclusion: These results suggest integrity of the central auditory pathways and represent a positive argument to propose a cochlear implantation with the aim to restore hearing.

Objective: To compare the degree of sensorineural hearing loss in patients with Ménière's disease (MD) with and without hyperinsulinism by different methods of assessment., Study Design: Historical cohort study., Setting: Ménière's Disease Care and Research Clinics of Hospital de Clinicas de Porto Alegre, a tertiary care university hospital in Southern Brazil., Patients: Patients with a definite diagnosis of MD based on the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) guidelines., Intervention: Patients were assessed by glucose overload tests (5-h glucose and insulin curves) and under baseline physiological conditions (Homeostasis Model Assessment/Insulin Resistance [HOMA-IR], Quantitative Insulin Sensitivity Check Index [QUICKI], and glucose/insulin ratio). These patients underwent annual pure-tone audiometry and were analyzed using 4-tone average (FTA), that is, arithmetic mean of 500, 1,000, 2,000, and 3,000 Hz, during the third, fourth, and fifth years of disease progression., Main Outcome Measure: Hearing loss assessed by FTA and classified in Stages I to IV (AAO-HNS)., Results: Forty-nine (76.6%) patients were defined as hyperinsulinemic and 15 (23.4%) as normoinsulinemic. Impairment on FTA was higher in the hyperinsulinemic group (52.04 ± 17.5 versus 39.75 ± 9.20, p = 0.027) when assessed by the 5-hour insulin curve. Hyperinsulinemic subjects were 3.5 times more likely to develop hearing damage greater than 40 dB (i.e., Stages III and IV) than normoinsulinemic subjects (OR = 3.52; 95% CI, 1.05-11.76). A moderate correlation between the insulin curve and the HOMA-IR was found (r = 0.524, p = 0.001)., Conclusion: Hyperinsulinism in MD is associated with greater clinical hearing damage.

Objectives: Kallmann syndrome (KS) usually combines an anosmia and a hypogonadotrophic hypogonadism. Hearing impairment was described in a few cases of KS. Our objective is to describe an unusual presentation of KS in 2 cases and to explore the pattern of inheritance in this family., Patients: Two brothers presented with a sensorineural hearing impairment associated with cryptorchidism and abnormal movements., Results: Genome-wide array analysis identified a large deletion of KAL1 in both patients confirming the diagnosis of Kallmann syndrome. The absence of familial history has been explained by a somatic mosaicism identified in their mother., Conclusion: The description of a hearing defect in 2 brothers with Kallmann syndrome allows asserting that deafness is part of the clinical features of this disease and must lead the physician to monitor the hearing function of Kallmann patients.

Objective: This study evaluated self-reported symptoms in patients with idiopathic sudden sensorineural hearing loss (ISSHL)., Study Design: Cross-sectional study., Setting: Multicenter clinical investigation in 9 university hospitals., Patients: In total, 140 patients with ISSHL and 24 patients with unilateral sensorineural hearing loss (USHL; control) were included., Main Outcome Measures: A questionnaire on symptoms of ISSHL was distributed and the Short-Form Health Survey (Version 2) was used for assessing the quality of life., Results: In response to questions on hearing difficulty, many of patients in both groups experienced symptoms. In response to questions on hearing-related discomfort, a significantly higher number of patients with ISSHL experienced symptoms compared with those with USHL. Compared with a high incidence of tinnitus in patients with ISSHL, very low incidence of tinnitus was observed in those with USHL.In the multiple linear regression analysis, hearing-related discomfort was the sole significant factor on the Mental Component Summary scores of the Short-Form Health Survey (Version 2)., Conclusion: Many patients with ISSHL experience several symptoms such as hearing difficulty, hearing-related discomfort, tinnitus, and anxiety. Hearing-related discomfort strongly affected the quality of life in patients with ISSHL.

Introduction: Idiopathic Sudden sensorineural hearing loss (ISSNHL) is a common otologic emergency. Recently, the cause of ISSNHL, which is still unclear, has been focused on chronic inflammation. Neutrophil-to-lymphocyte ratio (NLR) is a new and quick inflammatory marker, which is being measured routinely in CBC tests without any cost. We aimed to investigate the relationship between ISSNHL and inflammation by using NLR., Materials and Method: Forty-seven patients diagnosed with ISSNHL and 45 age- and sex-matched healthy subjects were enrolled in the study. An automated blood cell counter was used for NLR measurements. The hearing assessments of the patients were carried out by an audiometer, and audiometric patterns were assessed initially and after 1 month of the treatment. All the patients were treated with prednisone in the dose of 1 mg/kg per day, with a progressive dose reduction maintained for at least 2 weeks. Then, the patients were divided into 2 groups as " recovered" and "unrecovered" according to their response to the treatment., Results: The mean NLR, neutrophil, and lymphocyte values in patients with ISSNHL were significantly higher than the control group (p < 0.001, p < 0.001, and p = 0.004, respectively). In addition, NLR levels were higher in unrecovered patients compared with the recovered ones (p < 0.001)., Conclusion: This is the first study investigating the relationship between NLR levels and ISSNHL and its prognosis. While evaluating ISSNHL, determining NLR should not be overlooked as a quick and reliable indicator for predicting the diagnosis and the prognosis of the disease.

Objective: To review the literature regarding cortical hearing loss and document a case of cortical hearing loss including its presentation, diagnosis, and evolution over 32 months of follow-up., Patient: A 56-year-old woman with profound bilateral sensorineural hearing loss secondary to sequential hemorrhagic, temporal lobe infarctions separated in time by 8 months., Intervention: Diagnostic., Results: Sequential infarctions affecting the patient's auditory radiations and primary auditory cortices bilaterally combined to cause cortical hearing loss. At presentation, audiogram revealed a bilateral profound sensorineural hearing loss with no reliable responses to pure-tone or speech audiometry. She has subsequently recovered the ability to distinguish environmental sounds. At her 32-month follow-up, she had a pure-tone average (PTA) of 62 dB on the right and 70 dB on the left but continued to display a poor word recognition score (0%). A literature review was performed from the year 1891 until the present., Conclusion: Cortical deafness is an exceedingly rare entity. Presentation and recovery of hearing are dependent on the extent of the initial lesions. The majority of patients can expect improvements in pure-tone auditory thresholds over time; however patients should be counseled that recovery of the ability to understand speech is unlikely.

Objectives: To determine the role of MRI in the evaluation of patients with sensorineural hearing loss (SNHL) caused by meningitis. Gadolinium-enhanced T1-weighted MRI (GdMRI) and 3D heavily weighted T2-weighted MRI (T2MRI) were associated with the occurrence of SNHL and the peroperative surgical findings during cochlear implantation, respectively., Study Design: Retrospective cohort study., Setting: Tertiary referral otology and cochlear implant center., Patients: Seventeen patients who developed SNHL after bacterial meningitis were evaluated with MRI. Twenty-one cochlear implantations were performed in 11 patients with severe bilateral SNHL. Six patients developed unilateral SNHL and did not receive a CI., Interventions: MRI scans were independently scored by 3 observers. Sensitivity, specificity, positive predictive value, negative predictive value, and interobserver reproducibility were calculated., Results: Cochlea enhancement on GdMRI was present in 87% of the ears affected by SNHL. In patients with unilateral SNHL, a nonenhancing cochlea predicted the preservation of hearing on the ipsilateral side. In all cases with an incomplete electrode insertion (6/21), loss of cochlear patency was already seen on T2MRI. However, loss of fluid was also found in 29% of the cases in which full electrode insertion was achieved., Conclusion: MRI is crucial for decision making in patients with SNHL after meningitis. Diminished cochlear patency, as seen on T2MRI, is related to electrode insertion difficulty but does not always preclude full electrode insertion in cochlear implantation. Cochlear enhancement on GdMRI is associated with the occurrence of SNHL.

Objective: To assess the safety, stability, and performance of the broad-spectrum, light-based contact hearing device (CHD) on listeners with hearing impairment., Study Design: Feasibility study., Setting: Single-site research and development facility., Participants: Thirteen participants with symmetric mild-to-severe sensorineural hearing impairment had the CHD placed bilaterally., Intervention: A custom-molded light-activated tympanic contact actuator (TCA) was placed into each ear by a physician, where it stayed in contact with the umbo and a portion of the medial wall of the ear canal for 4 months. Each CHD was calibrated and programmed to provide appropriate broad-spectrum amplification., Main Outcome Measures: Safety was determined through routine otologic examinations. Aided and pre-TCA-insertion unaided audiometric thresholds (functional gain), maximum gain before feedback, tympanic membrane damping, Reception Threshold for Sentences (RTS), and Abbreviated Profile of Hearing Aid Benefit (APHAB) measurements were made to characterize system performance as well as the benefits of amplification via the CHD., Results: The TCAs remained on participants' ears for an average total of 122 days, without causing signs of inflammation or infection, and there were no serious device-related adverse events. Measured average maximum output of 90 to 110 dB SPL in the range of 0.25 to 10 kHz, average maximum gain before feedback of 40 dB, and functional gain through 10 kHz show extended-bandwidth broad-spectrum output and gain. RTS results showed significant aided improvements of up to 2.8 dB, and APHAB results showed clinically significant aided benefits in 92% of participants (11/12)., Conclusion: The safety, stability, and performance demonstrated in this initial 4-month study suggest that the CHD may offer a feasible way of providing broad-spectrum amplification appropriate to treat listeners with mild-to-severe hearing impairment.

Hypothesis: We investigated whether inflammatory biomarkers and stress are involved in the pathophysiology of idiopathic sensorineural hearing loss (ISHL)., Study Design: Individual cohort study., Setting: Two tertiary centers., Patients: Forty-three ISHL and 10 non-ISHL patients seen in our ENT departments from 2004 to 2010 within a week from the onset of new symptoms and without steroid administration before visiting our departments., Intervention: Multiple audiologic evaluations, blood tests including leukocyte counts, natural killer cell activity (NKCA), interleukin 6 (IL-6), tumor necrosis factor, high-sensitivity CRP (hCRP), and the General Health Questionnaire were used to evaluate the systemic stress and inflammatory response., Main Outcome Measures: Correlations between biomarkers and ISHL severity and prognosis were evaluated by statistical analysis., Results: In the ISHL patients, a neutrophil count above the reference range was associated with severe hearing loss and poor prognosis, and was accompanied by low NKCA and high IL-6. In the non-ISHL patients, these associations were not present. The abnormal neutrophil count was independent of preexisting vascular diseases. The abnormal counts responded to treatment and decreased into the reference range., Conclusion: Neutrophil counts above the reference range of a facility will be a useful indicator of poor prognosis of ISHL. Synchronism of different types of NF-κB activation pathways could be required to cause severe ISHL. An NKCA decrease, an acute neutrophil count increase, and an IL-6 increase can induce NF-κB activation in the cochlea and cause severe ISHL. Further epidemiologic surveys should be conducted to evaluate whether stressful life events increase the risk of severe ISHL onset.

Objective: To describe changes in the inner ear function and the vestibular neural pathway according to the progression of a labyrinthine infarction in a patient with an anterior inferior cerebellar artery (AICA) infarction., Patient: A 58-year-old woman with hypertension who presented with isolated inner ear symptoms similar to those of endolymphatic hydrops but finally progressed to an AICA infarction., Intervention: Serial neurotologic testing according to progression to an AICA infarction and antiplatelet therapy., Main Outcome Measures: Radiologic findings and neurotologic parameters, including pure tone audiogram thresholds, spontaneous nystagmus, summating potentials/action potentials on electrocochleography, interaural amplitude difference on the vestibular-evoked myogenic potential test, canal paresis and fixation index on the bithermal caloric test, and gain on oculomotor tests., Results: Our patient initially presented with sudden hearing loss and was diagnosed with an acute AICA infarction on a follow up MRI. Dynamic change in neurotologic testing was observed during disease progression. The vertigo and motion intolerance improved gradually after antiplatelet therapy., Conclusion: Changes in the clinical profile, which were documented during a transition from isolated labyrinthine ischemia to an AICA infarction, suggest that sensitivity to an ischemic injury is variable in different components of the labyrinthine organs in addition to providing a new insight into the response of vestibular neural pathway to ischemic injury.

Objective: Three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) magnetic resonance imaging (MRI) has recently been applied to the inner ear, but the relationship between 3D-FLAIR findings and audiovestibular function has been unclear in patients with sudden sensorineural hearing loss (SSNHL). We therefore used 3D-FLAIR MRI to investigate vestibular lesions in patients with SSNHL and determined the correlation between these radiologic findings and results of audiovestibular function tests., Study Design: Prospective study., Methods: We enrolled 35 patients who presented with SSNHL from 2008 to 2009. Before treatment, all patients underwent audiovestibular evaluation including pure tone audiometry, electronystagmography, subjective visual vertical, subjective visual horizontal, vestibular evoked myogenic potential, and caloric tests. Pathologic conditions of the inner ears were evaluated by 3D-FLAIR at 3 Tesla, with and without gadolinium enhancement., Results: Of the 35 patients with SSNHL, 12 (34.3%) showed high signals in the affected inner ear on precontrast 3D-FLAIR MRI. Rates of abnormal results on all vestibular function tests were significantly higher for patients with vestibular lesions than those without vestibular lesions on FLAIR (p < 0.05). Vestibular lesions significantly correlated with the presence of vertigo (relative risk, 3.857; 95% confidence interval, 2.039-7.297) and occurrence of vestibular dysfunction (p < 0.05). There was a significant negative correlation between hearing recovery and positive findings on FLAIR (relative risk, -0.475; 95% confidence interval, -0.698 to -0.175; p = 0.004)., Conclusion: High signals in the affected inner ear on 3D-FLAIR MRI closely correlate with vestibular dysfunction and poor hearing recovery in patients with SSNHL, especially when the vestibular apparatus is involved.

Objective: To describe a case of cochlear nerve deficiency (CND) with unique otologic findings., Patient: A 6-year-old girl., Main Outcome Measures: Magnetic resonance imaging, pure tone audiometry, distortion product otoacoustic emissions, and auditory brainstem response., Results: The patient showed moderate sensorineural hearing impairment limited to high frequency in the right ear on pure tone audiometry. The auditory brainstem response was absent on the right; however, distortion product otoacoustic emissions were detected on both sides. The right cochlear nerve at the level of the fundus was absent on 3-dimensional constructive interference in steady state magnetic resonance imaging., Conclusion: Most cases of CND show deafness or profound hearing loss, but the patient in this case had only moderate hearing loss. This finding provides evidence that auditory thresholds are variable in patients with CND. Therefore, careful evaluation is needed in diagnosis of patients with hearing loss.

Objective: Identify social and health care system factors that prevent congenitally deaf children from receiving cochlear implants (CIs) in a timely fashion., Study Design: Retrospective chart review and parental interviews., Setting: University medical center hospital in a state with mandatory newborn hearing screening (NBHS)., Patients: Fifty-nine congenitally deaf children who received CIs between January 1, 2002, and May 1, 2009., Interventions: Demographic and health care details were collected from the 59 patients., Main Outcome Measure: Age at implantation., Results: Thirty-four patients received implants at or before age 2 years (average age at implant surgery, 14 mo), and 25 patients received implants after age 2 years (average age, 65 mo). The presence of NBHS (p<0.001) and type of health insurance (p=0.05) the child had at the time of CI surgery were significant predictors of age at implantation. The following factors were associated with increased risk of delayed implantation: no NBHS (risk ratio [RR]=2.63), NBHS not identifying hearing loss (RR=1.63), Medicaid insurance alone (RR=1.21) or in combination with private insurance (RR=1.79), family physician as primary care provider (RR=1.50), and audiologist (RR = 1.30) or otolaryngologist (RR=1.31) as secondary care providers (versus implant center, RR=0.23). The main reasons for delay in CI surgery after age 2 years also were identified and include slow referrals for care (n=8) and parental delays (n=5)., Conclusion: The data suggest placing special focus on children with associated risk factors, ensuring NBHS, and parent and primary care provider education on the importance of early intervention and referral to an implant center would likely limit delays in children receiving CIs., (© 2011, Otology & Neurotology, Inc.)