Search

Objective: Although screening protocols for patients who present with asymmetric sensorineural hearing loss (ASNHL) exist, there are no clear guidelines to direct practitioners. In particular, various thresholds have been proposed for the degree of hearing loss that should prompt MRI studies, but the topic remains understudied. This project aims to compare protocols followed by practitioners to guide their imaging practices., Study Design: Web-based survey., Setting: Otolaryngology faculty at academic medical centers., Methods: A list of 530 otolaryngologists (276 otology/neurotology specialists, 254 general otolaryngologists) was compiled. A survey consisting of three parts: demographics, general practice patterns, and simulated patient cases was distributed., Results: A total of 468 surveys were successfully distributed, resulting in 88 (18.8%) responses. The majority of respondents (63.8%) self-reported their definition of ASNHL as ">30 dB hearing asymmetry at one frequency OR >20 dB hearing asymmetry at two continuous frequencies OR >10 dB hearing asymmetry at three contiguous frequencies." Overall, general otolaryngologists were more likely to observe asymmetric findings with serial audiogram alone, whereas otology/neurotology specialists were more likely to obtain imaging., Conclusion: There is significant variability between providers with regard to managing patients with ASNHL and evidence-based guidelines would be useful in guiding imaging practices., Level of Evidence: NA Laryngoscope, 134:4745-4753, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: To comprehensively examine the characteristics and prognosis of bilateral sudden sensorineural hearing loss (BSSHL) and its subtypes compared to unilateral sudden sensorineural hearing loss (USSHL)., Data Sources: PubMed, Scopus, and CINAHL., Review Methods: Databases were searched from inception to December 5, 2023, for studies reporting patient characteristics and audiometric outcomes for BSSHL and its simultaneous (Si-BSSHL) and sequential (Se-BSSHL) subtypes. Meta-analysis of continuous measures, proportions (%), mean differences (Δ), and odds ratio (OR) were performed., Results: Eleven studies were included, consisting of 368 patients with BSSHL and 2,705 patients with USSHL. The pooled prevalence among all SSHL cases was 88.1% (95% CI: 81.2%-93.6%) for USSHL and 11.9% (95% CI: 6.4% to 18.8%) for BSSHL. PTA improvement following treatment with steroids was significantly worse in patients with BSSHL (Δ15.3 dB; 95% CI: 14.6 to 15.9; p < 0.0001) compared to patients with USSHL. There was no significant difference in post-treatment PTA improvement between the BSSHL subtypes. Patients with Si-BSSHL were significantly less likely to have an idiopathic etiology (OR: 0.4; 95% CI: 0.2 to 0.8; p = 0.01) and significantly more likely to have an autoimmune disease etiology (OR: 27.4; 95% CI: 2.2 to 336.1; p = 0.01), comorbid cardiovascular disease (OR: 2.3; 95% CI: 1.1 to 5.1; p = 0.03), and comorbid hypertension (OR: 2.5; 95% CI: 1.6 to 3.8; p < 0.0001) compared to patients with USSHL., Conclusions: BSSHL is a considerably rarer form of SSHL with worse prognosis compared to USSHL. BSSHL, and Si-BSSHL in particular, has significantly greater associations with systemic pathologies compared to USSHL. Laryngoscope, 134:3883-3891, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: Although corticosteroids and other treatments for idiopathic sudden sensorineural hearing loss (ISSNHL) have been described, understanding its prognosis without intervention provides valuable information for patient management. The objective of this study is to provide a comprehensive, quantitative statistical analysis of the natural history of untreated idiopathic sudden sensorineural hearing loss (ISSNHL)., Study Type and Design: A systematic review and meta-analyses., Methods: Two independent searches of PubMed, Scopus, Web of Science, and Cochrane Library databases up to June 30, 2022, were performed. Adults with idiopathic sudden sensorineural hearing loss who received placebo or were untreated and had audiometric outcome measures in all study types were reviewed. These data indicative of the natural history of ISSNHL were analyzed, as were study characteristics related to risk of bias. Heterogeneity as assessed via I 2 and random effects analyses were performed., Results: Six studies meeting the inclusion criteria yielded 319 untreated patients whose natural history could be assessed. Heterogeneity among studies was moderate, with a variety of reported outcomes. A hearing improvement of at least 30 dB HL was observed in 36% (95% CI 0.28-0.44) of untreated patients, and of at least 10 dB HL was observed in 70% (95% CI 0.57-0.82) of untreated patients at 3 months. The mean hearing gain among untreated patients was 24.0 dB HL (95% CI 2.65-45.37) at 2-3 months., Conclusions: The observed natural history of ISSNHL suggests that patients can regain some hearing without active treatment. In the absence of future studies collecting prospective natural history data from untreated or placebo-treated ISSNHL patients, the data presented here provide the best available historical control data for reconsideration of results in past ISSNHL studies, as well as a roadmap for design and interpretation of future ISSNHL treatment clinical trials. Furthermore, knowing there is a statistically significant mean hearing gain of 24.0 dB HL in the untreated/placebo group provides an ethical basis for future placebo study of ISSNHL. The current status on ISSNHL management calls for a multi-institutional, randomized, double-blind placebo-controlled trial with validated outcome measures to provide science-based treatment guidance. Laryngoscope, 134:S1-S15, 2024., (© 2024 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

We report two cases of carotid-cochlea dehiscence involving the cochlear apex, with, to our knowledge, the first description of this anomaly in two members of a family (mother-daughter). Pure tone audiometry revealed a bilateral mild sensorineural hearing loss predominantly on the left in the daughter, and a left mild mixed hearing loss with a predominance of sensorineural loss in the medium frequencies in the mother Carotid-cochlear dehiscence is a rare anomaly with a multiform expression, which should be investigated in cases of third mobile window symptoms, but also atypical sensorineural hearing loss, or before any cochlear implantation. Laryngoscope, 134:2912-2914, 2024., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: Despite its relatively high prevalence, our understanding of the natural clinical course of acute low-tone hearing loss (ALHL) without vertigo remains incomplete. The purpose of this study is to summarize the findings of studies that evaluated recovery from hearing loss (HL), recurrence and/or fluctuation of HL, and progression to Meniere's Disease (MD) of patients presenting with ALHL without vertigo., Methods: A scoping review of the English literature was performed. On May 14, 2020 and July 6, 2022, MEDLINE, Embase, and Scopus were searched to identify articles related to the prognosis of ALHL. To be included, articles had to present outcomes that were clearly distinguishable for patients with ALHL without vertigo. Two reviewers evaluated articles for inclusion and extracted data. Disagreements were adjudicated by a third reviewer., Results: Forty-one studies were included. There was extensive heterogeneity between studies in regard to defining ALHL, treatment methods, and time of follow-up. Most of the cohorts (39 out of 40) reported partial or complete recovery of hearing in the majority (>50%) of patients, although reports of recurrence were relatively common. Progression to MD was infrequently reported. Shorter time from onset of symptoms to treatment predicted better hearing outcomes in 6 of 8 studies., Conclusion: The literature suggests that although the majority of patients with ALHL experience hearing improvement, recurrence and/or fluctuation are common, and progression to MD occurs in a minority of patients. Additional trials utilizing standardized inclusion and outcome criteria are needed to determine the ideal treatment for ALHL., Level of Evidence: NA Laryngoscope, 133:2457-2469, 2023., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: Genetic testing is the standard-of-care for diagnostic evaluation of bilateral, symmetric, sensorineural hearing loss (HL). We sought to determine the efficacy of a comprehensive genetic testing method, exome sequencing (ES), in a heterogeneous pediatric patient population with bilateral symmetric, bilateral asymmetric, and unilateral HL., Methods: Trio-based ES was performed for pediatric patients with confirmed HL including those with symmetric, asymmetric, and unilateral HL., Results: ES was completed for 218 probands. A genetic cause was identified for 31.2% of probands (n = 68). The diagnostic rate was 40.7% for bilateral HL, 23.1% for asymmetric HL, and 18.3% for unilateral HL, with syndromic diagnoses made in 20.8%, 33.3%, and 54.5% of cases in each group, respectively. Secondary or incidental findings were identified in 10 families (5.52%)., Conclusion: ES is an effective method for genetic diagnosis for HL including phenotypically diverse patients and allows the identification of secondary findings, discovery of deafness-causing genes, and the potential for efficient data re-analysis., Level of Evidence: 4 Laryngoscope, 133:2417-2424, 2023., (© 2022 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: Clinical guidelines recommend genetic testing when evaluating congenital and late-onset sensorineural hearing loss (SNHL). Genetic diagnoses can provide parents additional information regarding anticipated hearing loss progression, comorbid conditions, and family planning. Additionally, obtaining a genetic diagnosis may increase parental acceptance of hearing loss and subsequent pursuit of intervention. This study evaluates the association between genetic diagnoses and hearing loss intervention., Methods: We included children ages 0-18 years with SNHL who were hearing aid or cochlear implant candidates but non-users and underwent hearing-loss gene panel testing prior to initiating intervention. Univariate analyses were performed to identify predictors of hearing aid fitting or cochlear implantation. Multivariate logistic regression evaluated the impact of demographic and clinical factors on subsequent intervention., Results: Of the 385 children with SNHL who underwent hearing loss gene panel testing, 111 were included. Median age was 7.5 years. 56% were underrepresented minorities, 71% were non-White, and 71% were publicly insured. Those found to have a genetic diagnosis were 4.6 times as likely to subsequently undergo intervention (p = 0.035). Additionally, bilateral hearing loss and earlier age of genetic testing were associated with increased likelihood of intervention., Conclusion: Up to half of children with SNHL are suspected to have an underlying genetic etiology. Children diagnosed with a genetic diagnosis are significantly more likely to subsequently utilize hearing aids or cochlear implantation. This provides additional support for clinical guidelines recommending genetic testing not only due to the impact of prognostication but also on treatment decision-making., Level of Evidence: 4 Laryngoscope, 133:1982-1986, 2023., (© 2022 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: Here, we aimed to (a) determine whether a clinically significant sensorineural hearing loss (SNHL) change could be detected in post-coronavirus disease (COVID-19) hearing levels on comparing them with pre-infection hearing levels after controlling for the effect of age and (b) to identify risk factors, such as hypertension, diabetes, and smoking, which increase the likelihood of hearing loss in COVID-19 patients., Methods: We retrospectively analyzed hearing thresholds in unvaccinated patient's pre- and post-COVID-19 infection. Thresholds were controlled for age and the duration between the pre- and post-COVID-19 hearing evaluations. Correlations between additional COVID-19-related symptoms, hypertension, diabetes, and smoking and hearing threshold changes were analyzed., Results: A significant (but not clinical) threshold elevation was found post-COVID-19 infection. However, on controlling for age and the duration between the pre- and post-COVID-19 hearing evaluations, no significant threshold elevation was found. No significant correlation was found between hearing threshold changes and additional COVID-19-related symptoms, hypertension, diabetes, or smoking., Conclusion: COVID-19 did not lead to a significant hearing threshold elevation in our cohort, even among patients with additional COVID-19 symptoms, hypertension, or diabetes mellitus or among those who smoked., Level of Evidence: 3: nonrandomized controlled cohort, follow-up study Laryngoscope, 133:1976-1981, 2023., (© 2022 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To determine if exfoliation syndrome (XFS) is associated with hearing loss (HL) or vestibular dysfunction., Data Sources: PubMed, Scopus, CINAHL, and Cochrane Library through April 1, 2022., Review Methods: Two reviewers independently screened abstracts, selected articles for inclusion, and extracted data. Studies included for qualitative analysis conducted audiometric, tympanometric, or vestibular evaluations on all subjects., Results: Twenty-one publications (1148 patients with XFS and 1212 controls) were included in the systematic review, and 16 publications (968 patients with XFS and 1147 controls) in the meta-analysis. Greater severity of HL was seen for patients with XFS compared to controls across all frequencies (odds ratio [OR] 8.8 [7.3-10.2]). Patients with XFS were more likely to have moderate to profound sensorineural HL (OR 1.8 [1.3-2.5]), and less likely to have none to mild HL (OR 0.34 [0.17-0.67]) or no HL (OR 0.37 [0.28-0.50]). Three studies found patients with XFS had lower tympanometric peaks. Two studies found that abnormal vestibular testing results could be more common for patients with XFS., Conclusions: HL is associated with XFS. A sensorineural component to HL is confirmed, and mixed HL is possible. Given the high prevalence and infrequent diagnosis of XFS, the authors hypothesize that the current understandings of presbycusis might be improved by further investigation in XFS. Laryngoscope, 133:1025-1035, 2023., (© 2022 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective/hypothesis: Sensorineural hearing loss (SNHL) is a common sequela of congenital cytomegalovirus (cCMV), potentially exacerbating neurocognitive delay. The objectives of this study were to assess: (1) age at which SNHL in children with cCMV; (2) stimulability of the auditory system in children with cCMV following cochlear implantation (CI); and (3) whether features of magnetic resonance imaging (MRI) potentially are predictive of hearing outcomes., Methods: In this retrospective study of a prospectively acquired cohort, 123 children with cCMV who were referred for hearing loss at a single tertiary referral hospital over 20 years were compared with an unmatched comparative group of 90 children with GJB2-related deafness. Outcome measures were results of newborn hearing screening (NHS), behavioral audiograms, and, in a subgroup of cochlear implant (CI) users, responses from the auditory nerve and brainstem evoked by CI at initial activation, as well as lesional volume of FLAIR-hyperintense signal alterations on MRI., Results: All but 3 of 123 children with cCMV had confirmed and persistent SNHL. At birth, 113 children with cCMV underwent NHS, 31 (27%) passed in both ears and 23 (20%) passed in one ear (no NHS data in 10 children). At the first audiologic assessment, 32 of 123 (26%) had normal hearing bilaterally; 35 of 123 (28%) had unilateral SNHL; and 57 of 123 (46%) had bilateral SNHL. More than half (67 of 123, 54%) experienced hearing deterioration in at least one ear. Survival analyses suggested that 60% of children who developed SNHL did so by 2.5 years and 80% by 5 years. In the children who passed NHS in one or both ears, 50% developed hearing loss by 3.5 years in the ear, which passed unilaterally (n = 23 ears), and 50% by 5 years in bilateral passes (n = 62 ears). Hearing loss was significant enough in all but one child with isolated high-frequency loss for rehabilitation to be indicated. Hearing thresholds in individual ears were in the CI range in 83% (102 of 123), although duration of deafness was sufficient to preclude implantation at our center in 13 children with unilateral SNHL. Hearing aids were indicated in 16% (20 of 123). Responses from the auditory nerve and brainstem to initial CI stimulation were similar in children with cCMV-related SNHL compared with GJB2-related SNHL. Characteristic white matter changes on MRI were seen in all children with cCMV-related SNHL (n = 91), but the lesion volume in each cortical hemisphere did not predict degree of SNHL., Conclusions: cCMV-related SNHL is often not detected by NHS but occurs with high prevalence in early childhood. Electrophysiological measures suggest equivalent stimulability of the auditory nerve and brainstem with CI in children with cCMV and GJB2-related SNHL. Hyperintense white matter lesions on FLAIR MRI are consistently present in children with cCMV-related SNHL but cannot be used to predict its time course or degree. Combined, the data show early and rapid deterioration of hearing in children with cCMV-related SNHL with potential for good CI outcomes if SNHL is identified and managed without delay. Findings support universal newborn screening for cCMV followed by careful audiological monitoring., Level of Evidence: 3 Laryngoscope, 132:S1-S24, 2022., (© 2022 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: Sensorineural hearing loss (SNHL) is a common sensory deficit affecting pediatric populations. The majority of pediatric SNHL is genetic in etiology, with over 123 identified nonsyndromic causative genes. One such gene is STRC, which has been identified as the second most frequent autosomal recessive nonsyndromic gene associated with SNHL in multiple populations. The objective of this study was to investigate the phenotypic presentation and incidence of audiologic progression in pediatric patients with STRC-related hearing loss (HL)., Methods: Thirty-nine pediatric patients with confirmed HL and biallelic pathogenic STRC mutations were identified at two pediatric hospitals. A retrospective chart review was completed including demographics, medical history, genetic testing results, and audiologic data. HL progression was assessed using air conduction thresholds from pure-tone audiograms and auditory brain stem responses, and masked bone conduction thresholds from pure-tone audiograms., Results: Thirty-six patients had homozygous STRC deletions. Three were compound heterozygotes. All patients had bilateral, symmetric SNHL. Baseline HL was mild in 39% of ears, moderate in 52%, and moderate-severe in 3%. Of the 31 patients for which sufficient data were available to evaluate progression, 18 (58%) had some degree of progressive HL. Among these 31 patients assessed for progression, the mean hearing threshold declined by 0.6 dB per year (95% confidence interval: 0.5, 0.8; P < .001)., Conclusions: These biallelic STRC patients displayed HL ranging from mild to moderate-severe at baseline and progressing in 58%. The variability of the STRC phenotype and the possibility of audiologic progression should be considered in the clinical management of pediatric STRC-related SNHL., Level of Evidence: 3 Laryngoscope, 131:E2897-E2903, 2021., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc..)

Objective: To review our hearing preservation rates and speech recognition outcomes in patients undergoing cochlear implantation with a recently developed lateral wall electrode., Study Design: Retrospective cohort study., Methods: Retrospective case series of all patients, both pediatric and adult, undergoing cochlear implantation with the Advanced Bionics Hifocus™ SlimJ electrode between December 2017 and January 2020. Main outcomes included hearing preservation rates using several definitions, speech recognition testing primarily through Arizona Biosciences (AzBio) and Consonant-Nucleus-Consonant (CNC) testing, intra- and postoperative complications., Results: Sixty-one ears underwent implantation with the new electrode. Hearing preservation rates were 13.0% to 36.0% depending on the definition used. Speech recognition testing showed significant increases from pre- to postoperative condition (Implant-only AzBio: 24.1 to 48.3, P = .004, binaural AzBio: 46.1 to 65.9, P = .002, Implant-only CNC: 9.7 to 35.1, P < .001, binaural CNC: 29.8 to 59.40, P < .001) with last speech recognition testing occurring an average of 8.8 months postoperatively. The elderly population had the worst hearing preservation rates across all definitions. Five explantations were required due to two infections and three device failures., Conclusion: Hearing preservation rates varied significantly depending on the definition used, but users experienced a significant improvement in speech recognition testing after implantation. More work is needed in the community to standardize the definition of residual hearing and hearing preservation., Level of Evidence: 4 Laryngoscope, 131:2782-2788, 2021., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc.)

Studies have shown that hearing preservation is possible in the context of reimplantation, but residual hearing could not be predicted or expected in these cases. We describe a case in which a patient with mild to profound sensorineural hearing loss who underwent cochlear implantation with a lateral wall array and had hearing preserved postoperatively. She developed facial nerve stimulation which was unresponsive to reprogramming. Using electrocochleography to measure intracochlear trauma during the insertion process, the patient underwent reimplantation with a perimodiolar electrode and hearing was preserved postoperatively. This case demonstrates the potential to use electrocochleography for hearing preservation during reimplantation. Laryngoscope, 131:2348-2351, 2021., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: Speech recognition with a cochlear implant (CI) tends to be better for younger adults than older adults. However, older adults may take longer to reach asymptotic performance than younger adults. The present study aimed to characterize speech recognition as a function of age at implantation and listening experience for adult CI users., Study Design: Retrospective review., Methods: A retrospective review identified 352 adult CI recipients (387 ears) with at least 5 years of device listening experience. Speech recognition, as measured with consonant-nucleus-consonant (CNC) words in quiet and AzBio sentences in a 10-talker noise masker (10 dB signal-to-noise ratio), was reviewed at 1, 5, and 10 years postactivation., Results: Speech recognition was better in younger listeners, and performance was stable or continued to improve through 10 years of CI listening experience. There was no indication of differences in acclimatization as a function of age at implantation. For the better performing CI recipients, an effect of age at implantation was more apparent for sentence recognition in noise than for word recognition in quiet., Conclusions: Adult CI recipients across the age range examined here experience speech recognition benefit with a CI. However, older adults perform more poorly than young adults for speech recognition in quiet and noise, with similar age effects through 5 to 10 years of listening experience., Level of Evidence: 3 Laryngoscope, 131:2106-2111, 2021., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc..)

Objectives/hypothesis: To assess the relative cost-effectiveness of exome sequencing for isolated congenital deafness compared with standard care., Study Design: Incremental cost-effectiveness and cost-benefit analyses were undertaken from the perspective of the Australian healthcare system using an 18-year time horizon., Methods: A decision tree was used to model the costs and outcomes associated with exome sequencing and standard care for infants presenting with isolated congenital deafness., Results: Exome sequencing resulted in an incremental cost of AU$1,000 per child and an additional 30 diagnoses per 100 children tested. The incremental cost-effectiveness ratio was AU$3,333 per additional diagnosis. The mean societal willingness to pay for exome sequencing was estimated at AU$4,600 per child tested relative to standard care, resulting in a positive net benefit of AU$3,600. Deterministic and probabilistic sensitivity analyses confirmed the cost-effectiveness of exome sequencing., Conclusions: Our findings demonstrate the cost-effectiveness of exome sequencing in congenital hearing loss, through increased diagnostic rate and consequent improved process of care by reducing or ceasing diagnostic investigation or facilitating targeted further investigation. We recommend equitable funding for exome sequencing in infants presenting with isolated congenital hearing loss., Level of Evidence: N/A. Laryngoscope, 131:E2371-E2377, 2021., (© 2020 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: To determine the prevalence of Sensorineural Hearing Loss (SNHL) attributable to Sickle Cell Disease (SCD) in the global pediatric population and to identify factors contributing to its severity., Study Design: Meta-analysis., Methods: We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations under 18 years of age with excluding studies analyzing patients on iron chelation therapy, adults, or those without objective audiological analysis., Results: We identified 138 initial studies with 17 selected for analysis after applying the exclusion criteria. A total of 1,282 SCD patients and 553 controls were included in the meta-analysis. There was a statistically significant increase in the prevalence of SNHL in children with SCD compared to the general population with a cumulative risk ratio of 3.33., Conclusion: This is the first systematic investigation of the relationship between SCD and SNHL in pediatric patients across the globe. The increased prevalence of SNHL in the pediatric SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of SCD patients to reduce the social and developmental morbidity of hearing loss at a young age. PROSPERO Registration #: CRD42019132601. Laryngoscope, 131:1147-1156, 2021., (© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).)

Objectives/hypothesis: Antimalarial drugs (chloroquine and hydroxychloroquine) are widely used for the treatment of systemic lupus erythematosus (SLE). However, these drugs may have side effects such as hearing loss. This study aimed to describe the hearing function in SLE patients using antimalarials. Secondarily, this study aimed to investigate whether SLE causes hearing loss and if there are any serological or clinical aspects of this diseases associated with inner ear damage., Study Design: Cross-sectional study., Methods: This study included 84 individuals (43 SLE patients and 41 controls) with audiometry and tympanometry tests. Epidemiological, clinical, serological, and treatment profiles of SLE patients were extracted from the charts., Results: SLE patients had more sensorineural hearing loss than controls (23.2% vs. 0; P = .001). Pure-tone averages in SLE patients using antimalarials and not using antimalarials were similar (8.75 vs. 8.75; P = .63). At 8,000 Hz, antimalarial dug nonusers performed worse than users (10.00 vs. 22.50; P = .03). Tympanometry was normal in all participants. SLE serological and clinical profiles in patients with and without hearing loss were the same (all P = nonsignificant)., Conclusions: There is a high prevalence of hearing loss in SLE that is not affected by antimalarial drug use., Level of Evidence: 3b Laryngoscope, 131:E957-E960, 2021., (© 2020 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To determine the improvement in word recognition score (WRS 65 ) after cochlear implant (CI) surgery in hearing aid (HA) users with preoperative hearing threshold ≤80 dB HL and inadequate speech recognition scores with HA. Secondarily, to identify predictive factors for WRS 65 with a CI (WRS 65 [CI]) 6 months after surgery, derived from the standard German CI preoperative assessment., Study Design: Retrospective chart review., Methods: Retrospective review of all adult patients who received a Nucleus cochlear implant in the ear, nose, and throat department of the University Hospital of Erlangen between January 2010 and April 2019. The inclusion criteria were a preoperative hearing threshold ≤80 dB HL in the ear to receive the implantation, German as the native language, and at least 6 months postimplantation care at our center., Results: The inclusion criteria were met by 128 patients. All but two patients (98.4%) showed a significant improvement, WRS 65 (CI) versus WRS 65 with an (HA) (WRS 65 [HA]), of at least 15 percentage points (pp). The median improvement was 55 pp with a median WRS 65 (CI) of 70%. Three preoperative audiometric measures, the maximum word recognition score, age at implantation, and WRS 65 (HA) were identified as predictive factors for WRS 65 (CI). For three-quarters of the CI recipients, the score was not poorer than 12 pp below the predicted WRS 65 (CI)., Conclusions: For patients with a hearing loss ≤80 dB HL, cochlear implantation should be considered when speech perception with an HA is insufficient. The prediction model can support counseling in this patient group., Level of Evidence: 4 Laryngoscope, 131:E940-E945, 2021., (© 2020 The Authors. The Laryngoscope published by Wiley Periodicals, Inc. on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

Two hundred and fifty years have passed since the birth of Ludwig van Beethoven, and the enigma about his hearing loss and overall health status seems to be not completely solved. However, the admission to the hospital of a 64-year-old woman in 2018 with symptoms extremely similar to those experienced by the great composer may add further evidence to a theory previously underestimated. The health issues of the modern patient were found to be due to chronic lead intoxication. The lead was released during daily cooking using a ceramic-coated frying pan with worn surface that poisoned her breakfast most probably for years. Abdominal pain, asthenia, and hearing loss affecting the high frequencies with a many impact on speech intelligibility tormented the patient, as they had Beethoven. An extensive review of the music and medical literature was performed, as well as re-examination of manuscripts, correspondence, and autopsy reports of the famous composer; and great similarities have been found. The soundness of the most-cited classical theories about Beethoven's hearing loss will be discussed. After close scrutiny of the theories, our analysis points toward a progressive sensorineural hearing loss due to lead intoxication as the most probable cause of not only Beethoven's hypoacusis but his overall health status as well. Laryngoscope, 131:179-185, 2021., (© 2020 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: Sudden sensorineural hearing loss (SSHL) is an otologic emergency that affects five to 30 subjects per 100,000/year. The cause of SSHL remains unknown or uncertain in 70% to 90% of cases, and treatment decisions are usually made without knowing the etiology., Study Design: Prospective case-control study., Methods: One hundred thirty-one idiopathic SSHL patients were recruited from January 2014 to June 2015 in concordance with the Statements of Clinical Practice Guideline and divided into groups according to the disease severity. A clinical laboratory assessment was completed on blood samples collected from SSHL patients and control subjects. Multivariable regression analysis was performed to investigate the association between laboratory data and SSHL basis., Results: Only a few SSHL patients were positive for autoimmunity or viral infection. Statistically significant (P < .05) higher levels of blood glucose, glycated hemoglobin (HbA1C), lipoprotein (a), and factor VIII were found in SSHL patients compared to controls. Furthermore, blood glucose, HbA1C, uric acid, factor VIII, and homocysteine were significantly associated to disease severity., Conclusions: Gluco-metabolic, lipidic, and coagulative laboratory data support the vascular hypothesis for SSHL and its severity., Level of Evidence: 3b Laryngoscope, 127:2375-2381, 2017., (© 2017 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: Fanconi anemia is a hereditary chromosomal instability disorder. Hearing loss and ear abnormalities are among the many manifestations reported in this disorder. In addition, Fanconi anemia patients often complain about hearing difficulties in situations with background noise (speech perception in noise difficulties). Our study aimed to describe the prevalence of hearing loss and speech perception in noise difficulties in Dutch Fanconi anemia patients., Study Design: Retrospective chart review., Methods: A retrospective chart review was conducted at a Dutch tertiary care center. All patients with Fanconi anemia at clinical follow-up in our hospital were included. Medical files were reviewed to collect data on hearing loss and speech perception in noise difficulties., Results: In total, 49 Fanconi anemia patients were included. Audiograms were available in 29 patients and showed hearing loss in 16 patients (55%). Conductive hearing loss was present in 24.1%, sensorineural in 20.7%, and mixed in 10.3%. A speech in noise test was performed in 17 patients; speech perception in noise was subnormal in nine patients (52.9%) and abnormal in two patients (11.7%)., Conclusions: Hearing loss and speech perception in noise abnormalities are common in Fanconi anemia. Therefore, pure tone audiograms and speech in noise tests should be performed, preferably already at a young age, because hearing aids or assistive listening devices could be very valuable in developing language and communication skills., Level of Evidence: 4. Laryngoscope, 127:2358-2361, 2017., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: We compared the clinical characteristics between acute low- and high-frequency hearing loss (LF and HF, respectively) patients, and the efficacy of three different treatment protocols (systemic steroids, intratympanic steroid injection, and combination therapy)., Study Design: Prospective, randomized controlled study., Methods: A total of 111 patients diagnosed with LF or HF were treated on an outpatient basis. Each group was randomly divided into three equal subgroups based on therapy: oral steroid, intratympanic dexamethasone injection (IT), and combination therapy. Hearing gain was estimated by comparing pre- and post-treatment pure-tone averages. Recovery rate was assessed by Clinical Practice Guideline: Sudden Hearing Loss from the American Academy of Otolaryngology-Head and Neck Surgery., Results: In comparison of chief complaints, ear fullness and hearing loss were more common in the LF and HF group, respectively (P = .033 and P = .001, respectively). Hearing recovery rates were significantly different between the two groups (i.e., 74.1% [40/54] in the LF group and 45.6% [26/57] in the HF group; P < .001). Oral steroid therapy was most effective in the LF group (P = .017). In the HF group, all three modalities showed similar results, although IT tended to be the most effective (P = .390)., Conclusions: There were differences in chief complaints and treatment responses between LF and HF patients. Although they showed similar partial damage in the cochlea, the pathophysiology of LF and HF may be quite different., Level of Evidence: 1b. Laryngoscope, 127:1878-1884, 2017., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: Facial nerve schwannomas (FNSs) and vestibular nerve schwannomas (VNSs) may occupy similar locations in the internal auditory canal (IAC). Risk factors for hearing loss in patients with FNS are unknown. Similar to VNSs, we hypothesize that the size and location of FNSs do not correlate with hearing loss., Study Design: Retrospective cohort study and otopathology case review., Methods: A single institution retrospective review of all patients diagnosed with FNS was performed. Demographic data, tumor size and location, severity and type of hearing loss, and facial nerve function were analyzed. Otopathologic review of a case of FNS was also performed., Results: Thirty-three patients with FNS were identified. Mean age was 44 years, and 61% (n = 21) were female. Nine patients had tumors involving the IAC facial nerve, whereas 24 had distal tumors without IAC involvement. Eleven patients (33%) had hearing loss, and 55% of patients with sensorineural hearing loss (SNHL) had tympanic segment tumors without IAC involvement. The presence of SNHL was not associated with IAC tumor location (P = 0.21). Maximal dimensions of FNSs were 3 mm to 42 mm, without correlation between size and SNHL severity (r = -0.017, P = 0.926). Otopathologic review of an untreated 5-mm IAC FNS demonstrated disproportionate spiral ganglion and hair cell loss within the ipsilateral cochlea compared to the contralateral ear., Conclusion: Tympanic segment FNS without IAC involvement may result in SNHL. Tumor size is not associated with the presence or severity of SNHL. Otopathologic review suggests degenerative cochlear changes in FNS. Mechanisms for SNHL in schwannomas are likely multifactorial and may be unrelated to cochlear nerve compression., Level of Evidence: 4. Laryngoscope, 127:1676-1682, 2017., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To develop a novel completely-in-the-canal device capable of directly driving the tympanic membrane (TM) and ossicular chain from the ear canal., Study Design: Development and feasibility study., Methods: A voice coil actuator design was developed to drive the TM. Bench testing of the device using laser Doppler vibrometry (LDV) and sound recording was performed. Temporal bone studies using LDV were performed using different designs of the contact tip-TM interface to find the most efficient method of sound transmission. Two short-term clinical performance studies were performed using the latest 3-mm-wide device. Comparison was made to natural sound and to the Vibrant SoundBridge floating mass transducer simulator., Results: On bench testing, the device was found to have a low (<0.5%) total harmonic distortion in all frequencies above 400 Hz. Temporal bone studies revealed the device was capable of producing vibrations equivalent to 104 to 120 dB sound across most frequencies. The most efficient method of stimulation was when the device was coupled to the malleus. Short-term clinical performance studies indicated that pure tones and complex sound can be presented with the device. The sound quality of the experimental device was rated as better than the SoundBridge simulator device., Conclusions: The direct-drive hearing device is capable of producing a wide range of sound frequencies and amplitudes. The device can transmit complex sound with low power requirements. Further work on the development of the device is needed for long-term and wider clinical use., Level of Evidence: NA Laryngoscope, 127:932-938, 2017., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To explore long-term hearing results, quality of life (QoL), quality of hearing (QoH), work-related stress, tinnitus, and balance problems after idiopathic sudden sensorineural hearing loss (ISSNHL)., Study Design: Cross-sectional study., Methods: We reviewed the audiograms of 680 patients with unilateral ISSNHL on average 8 years after the hearing impairment, and then divided the patients into two study groups based on whether their ISSNHL had recovered to normal (pure tone average [PTA] ≤ 30 dB) or not (PTA > 30 dB). The inclusion criteria were a hearing threshold decrease of 30 dB or more in at least three contiguous frequencies occurring within 72 hours in the affected ear and normal hearing in the contralateral ear. Audiograms of 217 patients fulfilled the criteria. We reviewed their medical records; measured present QoL, QoH, and work-related stress with specific questionnaires; and updated the hearing status., Results: Poor hearing outcome after ISSNHL was correlated with age, severity of hearing loss, and vertigo together with ISSNHL. Quality of life and QoH were statistically significantly better in patients with recovered hearing, and the patients had statistically significantly less tinnitus and balance problems. During the 8-year follow-up, the PTA of the affected ear deteriorated on average 7 dB, and healthy ear deteriorated 6 dB., Conclusion: Idiopathic sudden sensorineural hearing loss that failed to recover had a negative impact on long-term QoL and QoH. The hearing deteriorated as a function of age similarly both in the affected and the healthy ear, and there were no differences between the groups. The cumulative recurrence rate for ISSNHL was 3.5%., Level of Evidence: 4 Laryngoscope, 127:927-931, 2017., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: We performed the video head impulse test (vHIT) and caloric test in patients with enlarged vestibular aqueducts (EVA). We compared the results with those found in Ménière's disease (MD) and acute vestibular neuritis to clarify the characteristics of vestibular dysfunction in EVA and to suggest reasons for the discrepancy between the vHIT and caloric tests in endolymphatic hydrops., Study Design: Retrospective analysis., Methods: Ten patients with EVA and confirmed biallelic SLC26A4 genetic mutations were enrolled. The results of video head impulse and caloric tests were analyzed and compared with those of patients diagnosed with MD (n = 76) or acute vestibular neuritis (n = 19)., Results: Among patients with EVA, 40% showed unilateral caloric weakness and 30% had recurrent episodes of vertigo. All patients with recurrent vertigo had caloric weakness. However, only 25% of patients with abnormal caloric test results had abnormal vHIT results. The discrepancy between the caloric test and vHIT results was also found in patients with MD, but not in patients with acute vestibular neuritis (abnormal vHIT results in 47.3% and 94.7% of patients with unilateral caloric weakness, respectively)., Conclusion: Unilateral vestibular dysfunction was identified in 40% of patients with EVA by the caloric test, but caloric test results were not correlated with vHIT results. This finding was similar in patients with MD. Hence, it is possible that endolymphatic hydrops, a common pathologic feature of both EVA and MD, could cause the discrepancy, and that vestibulopathy in endolymphatic hydrops might be restricted to the low-frequency range., Level of Evidence: 4. Laryngoscope, 127:921-926, 2017., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To identify risk factors for perioperative morbidity among a large national cohort of pediatric patients undergoing cochlear implantation., Study Design: Retrospective study utilizing the American College of Surgeons National Surgical Quality Improvement Program Pediatric database (2012-2013)., Methods: Pediatric cochlear implantation cases were identified using current procedural terminology 69930. Patients were categorized by age, and operative characteristics along with 30-day perioperative outcomes were analyzed., Results: We identified 1,351 cases of pediatric cochlear implantation. The median age was 3.6 years, and 73 patients were less than 1 year of age. Of 21 complication occurrences (1.55%), superficial incisional surgical site infection (SSI) was the most common (n = 13, 61.9%). Thirty-nine patients (2.9%) required readmission. The median operative time was 142 minutes, and the mean postoperative length of stay was 0.58 days. When comparing patients younger than 1 year old to those 1 year or older, no significant differences were noted in complication rate, postoperative length of stay, or reoperation rate. Patients less than 1 year of age were more likely to be readmitted (6.9% vs. 2.7%, P = 0.04) and had longer mean operative times (191 minutes vs. 160 minutes, P = 0.0015). Steroid use was a risk factor for unplanned reoperation, SSI, and readmission., Conclusion: Despite a slight increase in readmission rates and operative times among patients less than 1 year of age, cochlear implantation appears to be safe in this population, with complication rates, reoperation rates, and postoperative lengths of stay similar to children undergoing the procedure at the current U.S. Food and Drug Administration-approved age of 1 year and older., Level of Evidence: 4. Laryngoscope, 127:720-724, 2017., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: The present study aimed to examine whether the response patterns to the chimeric lexical tone tokens, combined with their pure tone audiometry (PTA) results, could separate listeners with sensorineural hearing loss (SNHL) from listeners with auditory neuropathy spectrum disorder (ANSD)., Study Design: Case-control study., Methods: Forty-three SNHL subjects and 46 ANSD subjects participated in a Mandarin lexical tone perception test using original and chimeric tone tokens. Ten sets of monosyllables, with four tone patterns for each, were processed through a 16-channel chimeric synthesizer in which a temporal envelope (E) from a monosyllabic word of one tone was paired with a fine structure (FS) from the same monosyllable of other tones., Results: Significantly negative correlations were present between tone perception scores and PTA0.5-4 kHz for both SNHL (P < 0.001) and ANSD (P < 0.001) subjects. Overall, 72.4%, 66.4%, and 46.3% of the tone responses were consistent with FS for the SNHL subjects with mild, moderate, and severe degree of hearing loss, respectively; and 28.4%, 23.1%, and 22.7% were consistent with FS for the ANSD subjects, with the equivalent degree of hearing loss. Similarly, 17.6%, 24.2%, and 37.7% of the tone responses were consistent with E for the SNHL subjects with mild, moderate, and severe degree of hearing loss, respectively; and 45.5%, 44.3%, and 36.5% were consistent with E for the ANSD subjects., Conclusion: Subjects with SNHL and ANSD may be separated by representing their FS- and E-consistent tone responses as a function of their pure-tone hearing thresholds., Level of Evidence: 3b. Laryngoscope, 126:1420-1425, 2016., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: Determine the prevalence of high-frequency sensorineural hearing loss (HFSNHL) in our hearing loss population and a diagnostic algorithm for these patients., Study Design: Retrospective case series., Methods: We identified patients diagnosed with sensorineural hearing loss (SNHL) at our pediatric tertiary care institution from 1981 to 2010. Based on audiometric profiles, these patients were subdivided into those with a flat SNHL configuration and those with HFSNHL. Imaging and genetic testing data and data regarding age at diagnosis, laterality, and risk factors were obtained for both groups. Comparisons were then made between the two groups., Results: Of 2,867 patients included in the study, 7.6% had HFSNHL. Age at diagnosis was significantly higher in HFSNHL patients (8.3 years vs. 6.1 years; P < .0001). These patients also had a significantly higher proportion of unilateral versus bilateral loss (49.1% vs. 26.1%; P < .0001); unilateral losses were also less severe. Genetic testing showed no significant difference between groups in the proportion of patients tested or in those who tested positive. Similarly, imaging data revealed no difference in the proportion of patients tested in the two groups; however, overall diagnostic yield was significantly higher in flat SNHL patients (29.5% vs.17.3; P = .02)., Conclusions: The positive predictive value of simple genetic testing is similar to that of imaging studies. However, given cost differences between genetic testing and imaging, it is prudent to perform genetic testing as the initial diagnostic test. Determination of whether high-throughput, multigene diagnostic platforms offer an added benefit in the evaluation of children requires further study., Level of Evidence: 4. Laryngoscope, 126:1236-1240, 2016., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To evaluate the effect of crimping techniques in stapes surgery for otosclerosis patients measured by hearing outcomes on pure-tone audiometry., Data Sources: PubMed, EMBASE, and the Cochrane Library., Methods: A systematic search was conducted. Studies comparing the effect of different crimping methods on pure-tone audiometric results in patients undergoing stapes surgery for otosclerosis were included. Relevance and risk of bias were assessed. Absolute risks and risk differences, means and mean differences, and 95% confidence intervals were extracted or calculated for the primary and secondary outcomes, which were air-bone gap closure to 10 dB or less, mean postoperative air-bone gap, and postoperative sensorineural hearing loss., Results: Twenty-two studies with moderate or high risk of bias were included for data extraction. Air-bone gap closure to 10 dB or less was assessed in 17 studies and mean postoperative air-bone gap in 20 studies. The hearing outcomes did not consistently favor one crimping method. However, the differences that were statistically significant were consistently in favor of heat crimping over manual and no crimping (difference in air-bone gap closure to 10 dB or less ranged between 22% and 42% in these studies and difference in mean postoperative air-bone gap between 2.8 dB and 7.4 dB) and in favor of manual crimping over no crimping (30% difference in air-bone gap closure to 10 dB or less and difference in mean postoperative air-bone gap between 2.6 dB and 6.0 dB)., Conclusion: Moderate to high risk of bias and inconsistent results characterize the current evidence. Laryngoscope, 126:1207-1217, 2016., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To assess whether transcanal endoscopic access to the basal turn of the cochlea for cochlear implantation is appropriate., Study Design: Case series study., Methods: We reviewed 100 consecutive computed tomographic studies of the sinuses and temporal bone in our institution. We excluded studies that demonstrated evidence of congenital anomalies, chronic ear disease, or inadequate visualization of the cochlea. On axial sections, the angles of the basal turn of the cochlea and of the ear canal in reference to the sagittal plane were recorded as a function of age., Results: Eighty-four studies were included and 16 were excluded. There was significant variability in the relationship between the ear canal and the basal turn of the cochlea in reference to the sagittal plane. A clear majority of images demonstrated the basal turn of the cochlea to align with a more posterior angle than that of the ear canal., Conclusion: The trajectory provided by posterior tympanotomy aligns more favorably with the basal turn of the cochlea than transcanal access. Endoscopic technique, primarily an ear canal intervention, may not be useful in cochlear implant surgery., Level of Evidence: 4. Laryngoscope, 126:689-692, 2016., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/Hypothesis: The human cochlea is most commonly considered to have two and a half turns. Although the causes of cochlear hypoplasia are well described, cochlear hyperplasia is a rarer entity that is poorly understood. We describe rare anatomic cochlear malformations identified in a 4-month-old male originally referred for evaluation after a failed newborn hearing screening. The full diagnostic evaluation, imaging findings, treatment, and follow-up are described in detail. Cochleae with three turns are an uncommon malformation that is not included in current classifications schemes and may represent a distinct type of anomaly not caused by developmental arrest., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To evaluate in patients with idiopathic sensorineural hearing loss whether pulse therapy with methylprednisolone leads to better recovery of hearing than traditional oral prednisolone therapy., Study Design: Randomized controlled trial., Methods: Sixty-seven patients diagnosed with idiopathic sensorineural hearing loss were randomly divided into two groups based on therapy. The study group received 500-mg daily intravenous methylprednisolone for 3 consecutive days, followed by 1 mg/kg (maximum 60 mg) oral prednisolone for 11 days (total treatment: 14 days). The control group received 1 mg/kg (maximum 60 mg) oral prednisolone for 14 days. Hearing change was evaluated by comparing initial hearing tests and the third-month hearing tests in three ways: 1) pure tone improvement in each individual tone (0.5, 1, 2, 3, and 4 kHz); 2) word-recognition score improvement; and 3) complete, partial, and no recovery of hearing calculated (as defined by American Academy of Otolaryngology-Head and Neck Surgery Clinical Practice Guidelines)., Results: Sixty of 67 patients, 29 of 34 patients in the study group and 31 of 33 patients in the control group, completed the study. Frequency-specific hearing improvement did not differ significantly among the groups. Word-recognition score improvement was 20.34% ± 27.35% for the study group and 13.41% ± 23.48% for the control group, which had no statistically significant difference. There was also no significant difference in hearing recovery rates for the two groups., Conclusion: Pulse therapy with methylprednisolone and traditional oral prednisolone therapy resulted in similar hearing improvement., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To evaluate clinical characteristics and possible associated factors of idiopathic sudden sensorineural hearing loss (ISSNHL) in children using univariate and multivariate analyses., Study Design: A retrospective case series with comparisons., Methods: From January 2007 to December 2013, medical records of 37 pediatric ISSNHL patients were reviewed to assess hearing recovery rate and examine factors associated with prognosis (gender; side of hearing loss; opposite side hearing loss; treatment onset; presence of vertigo, tinnitus, and ear fullness; initial hearing threshold), using univariate and multivariate analysis, and compare them with 276 adult ISSNHL patients., Results: Pediatric patients comprised only 6.6% of pediatric/adult cases of ISSNHL, and those below 10 years old were only 0.7%. The overall recovery rates (complete and partial) of the pediatric and adult patients were 57.4% and 47.2%, respectively. The complete recovery rate of the pediatric group (46.6%) was higher than that of the adult group (30.8%, P = .040). According to multivariate analysis, absence of tinnitus, later onset of treatment, and higher hearing threshold at initial presentation were associated with a poor prognosis in pediatric ISSNHL., Conclusions: The recovery rate of ISSNHL in pediatric patients is higher than in adults, and the presence of tinnitus and earlier treatment onset is associated with favorable outcomes., Level of Evidence: 4., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To examine the relationship between bony cochlear nerve canal (BCNC) width, degree of hearing loss, and speech discrimination in children with unilateral sensorineural hearing loss (USNHL)., Study Design: Retrospective chart review (case-control study)., Methods: Audiometric database was cross-referenced with radiologic database at pediatric tertiary care facility to identify children with USNHL and temporal bone computed tomography. BCNC widths were measured independently by two radiologists blinded to affected ear. Regression analyses investigated associations among variables., Results: One hundred and sixty children with USNHL had temporal bone imaging. Mean BCNC width was significantly smaller in affected ears, P = 0.0001. Narrower width was associated with more severe hearing loss, P = 0.01. Among children who had narrower cochlear nerve canals in affected ears compared to unaffected ears, smaller width was associated with lower speech discrimination score, P = 0.03. Increasing asymmetry in BCNC width between affected and unaffected ears was associated with poorer discrimination scores, P = 0.02. Among ears with asymmetrically smaller cochlear nerve canals, a 1-mm reduction in cochlear canal width between the normal and affected ear was associated with 30.4% lower word recognition score percentage in the affected ear, P = < 0.001., Conclusion: There is a significant association between BCNC stenosis and impaired speech discrimination, independent of degree of hearing loss. Further investigation is needed to determine whether BCNC stenosis is a poor prognostic factor for auditory rehabilitation., (© 2014 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To evaluate the influence of the introduction of newborn hearing screening programs on the age at cochlear implantation in children., Study Design: Retrospective, multicenter cohort study., Methods: All 1,299 pediatric cochlear implant users who received their implants before the age of 5 years between 1995 and 2011 in the Medical University Hannover, Germany and University Medical Center Utrecht, the Netherlands were enrolled in this study. Age at implantation and the number of children implanted within the first year of life was assessed for each center., Results: Age at cochlear implantation gradually declined over the years in both centers. The introduction of the screening resulted in significant decline in the age at implantation in the Netherlands; simultaneously, the number of children implanted within their first year of life increased significantly. Comparing 4-year epochs immediately before and after introduction of the screening, the mean age decreased from 2.4 to 1.2 years, and the percentage of early implanted children increased from 9% to 37%. In the German population, a similar effect of the introduction of the hearing screening program was absent., Conclusions: The introduction of the national newborn hearing screening program has reduced the age at cochlear implantation in young children in the Netherlands but not in Germany. Correspondingly, it resulted in an increase in the number of children implanted early in life. The difference between the Dutch and German population might be due to differences in the follow-up and referral after the hearing screening., (© 2014 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: In the United States, falls are the leading cause of accidental deaths in adults aged over 65 years. Epidemiologic studies indicate that there is a correlation between hearing loss and the risk of falling among older people. The vestibular, proprioceptive, and visual systems are known to contribute to postural stability, but the contribution of audition to maintaining balance has not yet been determined., Study Design: Cross-sectional study to measure postural stability in bilateral hearing-aid users aged over 65 years in aided and unaided conditions., Methods: Balance was assessed using the Romberg on foam test and the tandem stance test. Tests were administered in the presence of a point-source broadband white-noise sound (0-4 kHz) source in both unaided and aided conditions in the dark. Subjective measures of balance were made using the Activities-specific Balance Confidence Scale., Results: Performance was significantly better in the aided than the unaided condition (P = 0.005 for both tests). No statistically significant relationship between improvement in balance, and hearing was identified. Participants did not report that they perceived a difference in balance between the two conditions., Conclusion: These results indicate that hearing aids are a novel treatment modality for imbalance in older adults with hearing loss and suggest that wearing hearing aids may offer a significant public-health benefit for avoiding falls in this population., (© 2014 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To determine the feasibility and cost effectiveness of incorporating cytomegalovirus (CMV) testing to determine the etiology of pediatric hearing loss., Study Design: Retrospective study of children presenting with sensorineural hearing loss (SNHL) at one institution from 2008 to 2013., Methods: Children aged 3 years or younger who presented to the senior author (A.P.) between May 2008 and September 2013 with confirmed SNHL were evaluated. These children underwent a sequential diagnostic paradigm that incorporated CMV testing if no obvious etiology could be determined from the history or physical examination., Results: One hundred eleven children with SNHL were evaluated between 2008 and 2013. Eighty-three children underwent CMV testing, imaging, and a genetic evaluation. Those with confirmed or probable CMV-induced SNHL made up 30% of all children tested (n = 25), the largest group identified. CMV screening had the lowest cost compared to genetic testing or imaging for all types of hearing loss, except for those with auditory neuropathy spectrum disorder., Conclusion: We present the first sequential diagnostic paradigm utilizing CMV testing for children presenting with SNHL. The relatively high incidence of CMV-induced SNHL, the low cost for this assay, and the indirect benefits from early diagnosis support the role of early CMV testing for these patients., Level of Evidence: 4., (© 2014 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To fully describe the otologic features seen in individuals with deletions of the distal long arm of chromosome 18 (distal 18q-)., Study Design: Cross-sectional/observational., Methods: More than 200 individuals with deletions of the long arm of chromosome 18 underwent a complete otologic and audiologic examination. In addition, chromosome microarray analysis to determine the chromosome copy number was completed for all participants. Overall, 113 subjects had clinical and audiological data to be reported here., Results: Sixty-six percent of this population had aural stenosis or atresia. No subject had microtia. In the 53 individuals for whom serial data was available, enlargement of ear canal diameter was seen in 48% of ears with stenosis, examined over time. Abnormalities of the palate were seen in nearly 18% of patients and included complete or incomplete clefts of the palate, submucous clefts, and velopharyngeal insufficiency. A conductive hearing impairment was identified in 112 ears (49.5%), and sensorineural hearing loss was identified in 28%. Eustachian tube dysfunction was common and found in 78% of ears examined., Conclusions: The otologic phenotype varies widely among individuals with distal 18q-. External auditory canal stenosis without microtia is a hallmark of the disease. Hearing impairment is also very common, with both sensorineural losses and conductive losses contributing to morbidity. Moreover, the critical region for sensorineural hearing loss will aid in the identification of the gene responsible for this aspect of the distal 18q- phenotype., Level of Evidence: 4., (© 2014 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To assess the hearing status of trisomy 21 patients by analyzing electrophysiological and radiological findings of any correlation between hearing impairment and major or minor inner ear malformations., Study Design: Prospective radiological and electrophysiological study., Methods: A group of 34 ears of Down syndrome subjects and 20 ears of a volunteer age- and sex-matched control group of 10 normal subjects were studied electrophysiologically by means of otoacoustic emissions and auditory brainstem response. Temporal bone computed tomography (CT) scans were carried out in both groups; radiological findings were compared. Inner ear structure measurements were applied attempting to disclose subtle bony labyrinthine anomalies. The findings from both groups were statistically analyzed employing the t test., Results: The rate of sensorineural hearing loss (SNHL) in Down syndrome group was 41%. Temporal bone CT scans showed no ossicular malformation in all Down syndrome cases. Major inner ear abnormalities were disclosed in 5.8%; they corresponded to two cases of lateral semicircular canal dysplasia detected on CT images by visual inspection. The application of various inner ear structure measurements increased the overall detection rate of common inner ear malformations to 47%. A statistically significant correlation was found between hearing level and vestibule length (P = .009) and internal auditory canal length (P = .028). Vestibular aqueduct width was correlated to different otic abnormalities., Conclusions: SNHL is mainly secondary to the underestimated subtle inner ear malformations that are adequately demonstrated by adopting standardized inner ear structure measurements on petrous bone imaging. Vestibular height and internal auditory canal length were correlated to SNHL., Level of Evidence: 3b., (© 2013 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective/hypothesis: Magnetic resonance imaging of the temporal bone has an important role in decision making with regard to cochlea implantation, especially in children with cochlear nerve deficiency. The purpose of this study was to evaluate the usefulness of the combination of an advanced high-resolution T2-weighted sequence with a surface coil in a 3-Tesla magnetic resonance imaging scanner in cases of suspected cochlear nerve aplasia., Study Design: Prospective study., Methods: Seven patients with cochlear nerve hypoplasia or aplasia were prospectively examined using a high-resolution three-dimensional variable flip-angle turbo spin-echo sequence using a surface coil, and the images were compared with the same sequence in standard resolution using a standard head coil. Three neuroradiologists evaluated the magnetic resonance images independently, rating the visibility of the nerves in diagnosing hypoplasia or aplasia., Results: Eight ears in seven patients with hypoplasia or aplasia of the cochlear nerve were examined. The average age was 2.7 years (range, 9 months-5 years). Seven ears had accompanying malformations. The inter-rater reliability in diagnosing hypoplasia or aplasia was greater using the high-resolution three-dimensional variable flip-angle turbo spin-echo sequence (fixed-marginal kappa: 0.64) than with the same sequence in lower resolution (fixed-marginal kappa: 0.06)., Conclusions: Examining cases of suspected cochlear nerve aplasia using the high-resolution three-dimensional variable flip-angle turbo spin-echo sequence in combination with a surface coil shows significant improvement over standard methods., (© 2013 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: Compare the epidemiology of pediatric unilateral sensorineural hearing loss before and after implementation of universal newborn hearing screening in Missouri., Study Design: Inception cohort., Methods: Charts of 134 children born between January 1, 1990 and December 31, 2007, diagnosed with unilateral sensorineural hearing loss at a single institution in Missouri were reviewed to determine the effects of universal newborn hearing screening on age of detection and etiology of hearing loss., Results: Mean age of detection declined from 4.4 (standard deviation [SD] 1.8) to 2.6 (SD 2.6) years of age, whereas the rate of detection by 6 months of age increased from 3% to 42%. The majority (58%) of cases had normal hearing at birth. The most common etiological category was unknown (41%) before screening and congenital (45%) after screening. The use of magnetic resonance imaging has increased by 21% (2-fold), whereas use of computed tomography has declined by 8% since 2002. Yields of connexin, Pendred, electrocardiogram, and syphilis testing were 0/48 and 2/31 before and after screening, respectively., Conclusions: Implementation of universal newborn hearing screening in Missouri is associated with a decrease in age of detection of unilateral sensorineural hearing loss. The majority of cases are either not present or not detectable at birth. The combination of hearing status at birth and imaging findings suggests that the majority of cases are congenital rather than of unknown etiology., (© 2013 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: The purpose of this study was to investigate whether near-continual transtympanic steroid perfusion is more effective than intermittent intratympanic steroid injection as a salvage therapy for idiopathic sudden sensorineural hearing loss., Study Design: Case control study., Methods: We designed a case-control study consisting of 60 patients with sudden sensorineural hearing loss who did not respond well to systemic steroid therapy. From November 2008 to October 2010, we prospectively enrolled subjects for the transtympanic steroid perfusion therapy. We retrospectively collected data from age- and sex-matched patients who had undergone intratympanic steroid injection between January 2003 and October 2008. The audiological results of the two groups were compared., Results: The presalvage pure tone threshold was 65.4 ± 13.5 dB in the transtympanic steroid perfusion group. After the therapy, the hearing threshold was improved by an average of 15.0 ± 9.7 dB, and 53.3% of subjects had improved by 10 dB or more. The speech discrimination score was improved from 12.6% ± 7.0% to 54.4 ± 6.4%. In the intratympanic steroid injection group, the presalvage pure tone threshold was 68.8 ± 16.0 dB. After the therapy, the hearing threshold was improved by an average of 10.7 ± 9.8 dB, and 43.3% of subjects had improved by 10 dB or more. The speech discrimination score was improved from 13.3 ± 6.0% to 46.4 ± 12%. The degree of hearing improvement was significantly greater in the transtympanic group., Conclusions: Both transtympanic steroid perfusion and intratympanic steroid injection can be used as salvage therapies for idiopathic sudden sensorineural hearing loss. Near-continual transtympanic steroid perfusions may provide better audiological results., (Copyright © 2013 The American Laryngological, Rhinological and Otological Society, Inc.)