Frequency and Patterns of Hearing Dysfunction in Patients Treated with Teprotumumab.

PURPOSE: To better characterize the frequency and patterns of hearing dysfunction in patients who have received teprotumumab to treat thyroid eye disease. DESIGN: Noncomparative case series. PARTICIPANTS: Patients who underwent audiology testing before and after completion of teprotumumab infusions. METHODS: A review of patients who underwent audiology testing before and after completion of teprotumumab infusions was carried out. Additional audiogram testing during treatment was included when available. Hearing function was analyzed using audiogram data measuring threshold hearing levels at specific frequencies. Basic demographic data as well as information regarding otologic symptoms also were obtained and analyzed. MAIN OUTCOME MEASURES: Hearing loss demonstrated by a significant change in decibel hearing thresholds or that meets criteria for ototoxicity. RESULTS: Twenty-two patients (44 ears) were included in the study, with baseline and most recent audiology testing after treatment ranging from 84 days before to 496 days after treatment. Fifteen patients (30 ears) also underwent testing during treatment starting after the second infusion up until the day of, but before, the eighth infusion. Hearing loss after treatment met criteria for ototoxicity in 17 of the 44 ears (38.6%), with 11 of the 22 patients (50.0%) meeting criteria in at least 1 ear. The pure-tone average decibel hearing levels (HLs) across all 44 ears demonstrated hearing loss after treatment (P = 0.0029), specifically at high (P = 0.0008) and middle frequencies (P = 0.0042), but not at low frequencies (P = 0.8344). Patients who were older also were more likely to experience hearing loss after treatment (P = 0.0048). CONCLUSIONS: Audiometric data demonstrate that teprotumumab influences hearing function, most significantly at higher frequencies and in older patients. Audiometric testing is critical for counseling patients regarding teprotumumab treatment. A protocol for monitoring hearing during treatment is needed to detect and manage hearing changes associated with teprotumumab use. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Evaluation of Hearing Loss: Understanding Audiologic Testing to Refine Image Interpretation.

The standard of reference for diagnosing and characterizing hearing loss is audiologic testing. The results of audiologic testing inform the imaging algorithm and the differential diagnosis for the underlying cause. Pure-tone audiometry tests the ability to hear tones across different frequencies, and the results are displayed as an audiogram. Tympanometry measures tympanic membrane compliance as a function of pressure to generate a tympanogram. Acoustic reflex testing helps differentiate third window lesions from other causes of conductive hearing loss. Clinical and audiologic assessment of sensorineural hearing loss helps in differentiating cochlear from retrocochlear causes. Symmetrical sensorineural hearing loss is typical of cochlear disease. Asymmetry increases the likelihood of a retrocochlear lesion, the most common of which among adults is vestibular schwannoma. Unlike patients with sensorineural hearing loss, who commonly have normal imaging studies, patients with conductive hearing loss are expected to have abnormal temporal bone CT studies. By incorporating the results of audiologic testing into their evaluation, radiologists can perform a more informed and more intentional search for the structural cause of hearing loss. The authors describe several audiogram configurations that suggest specific underlying mechanisms of conductive hearing loss. By providing a practical and accessible summary of the basics of audiologic testing, the authors empower the radiologist to leverage relevant clinical information and audiologic test results to interpret temporal bone imaging more confidently and more accurately, particularly temporal bone CT in the setting of conductive hearing loss. ©RSNA, 2024.

Clinical Utility of the Standardized Word Recognition Score.

OBJECTIVES: An unexpectedly low word recognition (WR) score may be taken as evidence of increased risk for retrocochlear tumor. We sought to develop evidence for or against using a standardized WR (sWR) score in detecting retrocochlear tumors. The sWR is a z score expressing the difference between an observed WR score and a Speech Intelligibility Index-based predicted WR score. We retrospectively compared the sensitivity and specificity of pure-tone asymmetry-based logistic regression models that incorporated either the sWR or the raw WR scores in detecting tumor cases. Two pure-tone asymmetry calculations were used: the 4-frequency pure-tone asymmetry (AAO) calculation of the American Academy of Otolaryngology-Head and Neck Surgery and a 6-frequency pure-tone asymmetry (6-FPTA) calculation previously optimized to detect retrocochlear tumors. We hypothesized that a regression model incorporating the 6-FPTA calculation and the sWR would more accurately detect retrocochlear tumors. DESIGN: Retrospective data from all patients seen in the audiology clinic at Mayo Clinic in Florida in 2016 were reviewed. Cases with retrocochlear tumors were compared with a reference group with noise- or age-related hearing loss or idiopathic sensorineural hearing loss. Two pure-tone-based logistic regression models were created (6-FPTA and AAO). Into these base models, WR variables (WR, sWR, WR asymmetry [WRΔ], and sWR asymmetry [sWRΔ]) were added. Tumor detection performance for each regression model was compared twice: first, using all qualifying cases (61 tumor cases; 2332 reference group cases), and second, using a data set filtered to exclude hearing asymmetries greater than would be expected from noise-related or age-related hearing loss (25 tumor cases; 2208 reference group cases). The area under the curve and the DeLong test for significant receiver operating curve differences were used as outcome measures. RESULTS: The 6-FPTA model significantly outperformed the AAO model-with or without the addition of WR or WRΔ variables. Including sWR into the AAO base regression model significantly improved disease detection performance. Including sWR into the 6-FPTA model significantly improved disease detection performance when large hearing asymmetries were excluded. In the data set that included large pure-tone asymmetries, area under the curve values for the 6-FPTA + sWR and AAO + sWR models were not significantly better than the base 6-FPTA model. CONCLUSIONS: The results favor the superiority of the sWR computational method in identifying reduced WR scores in retrocochlear cases. The utility would be greatest where undetected tumor cases are embedded in a population heavily representing age- or noise-related hearing loss. The results also demonstrate the superiority of the 6-FPTA model in identifying tumor cases. The 2 computational methods may be combined (ie, the 6-FPTA + sWR model) into an automated tool for detecting retrocochlear disease in audiology and community otolaryngology clinics. The 4-frequency AAO-based regression model was the weakest detection method considered. Including raw WR scores into the model did not improve performance, whereas including sWR into the model did improve tumor detection performance. This further supports the contribution of the sWR computational method for recognizing low WR scores in retrocochlear disease cases.

The Chinese Zodiac-in-Noise Test: An Internet-Based Speech-in-Noise Test for Large-Scale Hearing Screening.

OBJECTIVES: Motivated by the growing need for hearing screening in China, the present study has two objectives. First, to develop and validate a new test, called the Chinese Zodiac-in-noise (ZIN) test, for large-scale hearing screening in China. Second, to conduct a large-scale remote hearing screening in China, using the ZIN test developed. DESIGN: The ZIN test was developed following a similar procedure as the digits-in-noise test but emphasizes the importance of consonant recognition by employing the 12 zodiac animals in traditional Chinese culture as speech materials. It measures the speech reception threshold (SRT) using triplets of Chinese zodiac animals in speech-shaped noise with an adaptive procedure. RESULTS: Normative data of the test were obtained in a group of 140 normal-hearing listeners, and the performance of the test was validated by comparisons with pure-tone audiometry in 116 listeners with various hearing abilities. The ZIN test has a reference SRT of -11.0 ± 1.6 dB in normal-hearing listeners with a test-retest variability of 1.7 dB and can be completed in 3 minutes. The ZIN SRT is highly correlated with the better-ear pure-tone threshold ( r = 0.82). With a cutoff value of -7.7 dB, the ZIN test has a sensitivity of 0.85 and a specificity of 0.94 for detecting a hearing loss of 25 dB HL or more at the better ear.A large-scale remote hearing screening involving 30,552 participants was performed using the ZIN test. The large-scale study found a hearing loss proportion of 21.0% across the study sample, with a high proportion of 57.1% in the elderly study sample aged over 60 years. Age and gender were also observed to have associations with hearing loss, with older individuals and males being more likely to have hearing loss. CONCLUSIONS: The Chinese ZIN test is a valid and efficient solution for large-scale hearing screening in China. Its remote applications may improve access to hearing screening and enhance public awareness of hearing health.

Occupational Lead Exposure Ototoxicity Evaluated With Distortion-Product Otoacoustic Emissions.

OBJECTIVES: To evaluate the extent of hearing loss among pottery workers in Mexico exposed to lead. DESIGN: The authors conducted a cross-sectional study including 315 adult pottery workers. Auditory function was evaluated by air conduction pure-tone audiometry (pure-tone average) and distortion-product otoacoustic emission (DPOAE) levels (amplitude and signal-to-noise ratio). Lead exposure was assessed with a single blood sample test and classified as low, medium, and high according to blood lead tertiles. Logistic regression models were calculated for the association between blood lead levels, pure-tone average, and DPOAE records. RESULTS: Median (25th-75th) blood lead levels were 14 µg/dL (7.5-22.6 µg/dL). The audiometric pattern and DPOAE records were similar across blood lead levels groups in all frequencies, and no statistically significant differences were found. Adjusted logistic regression models showed no increase in the odds for hearing thresholds >25 dB (HL) and DPOAE absence associated with blood lead levels, and no dose-response pattern was observed ( p > 0.05). CONCLUSIONS: Given the results from this cross-sectional study, no association was found between blood lead levels and hearing loss assessed with DPOAE. Future longitudinal work should consider chronic lead exposure estimates among underrepresented populations, which can potentially inform safer work practices to minimize the risk of ototoxicity.

Audiometry Interpretation for Hearing Loss in Adults.

Hearing loss is a prevalent, chronic condition in the United States; it is often gradual and progressive and is underreported by patients and undertreated by physicians. The impaired ability to effectively hear and communicate may result in negative emotional, cognitive, economic, and social consequences for individuals and may pose a safety risk. Questionnaires and smartphone apps are available to help identify and evaluate self-perceived hearing loss. Physicians should assess for objective hearing impairment when the patient or family member raises a concern or if cognitive or mood symptoms are present that could be influenced by hearing loss. Three types of hearing loss exist: conductive, sensorineural, and mixed. Pure-tone audiometry uses an audiometer and is reported on an audiogram; it is the most accurate method for hearing loss detection. It can be used for screening or comprehensive testing when combined with tympanometry, speech-reception thresholds, and word-recognition testing. Audiograms that show a unilateral or asymmetrical sensorineural hearing loss can be signs of retrocochlear pathology and warrant additional evaluation by an audiologist and otolaryngologist as well as imaging studies. Medicare Parts A and B do not pay for hearing aids, although some Medicare Advantage (Part C) or supplemental plans may provide insurance coverage for hearing aids. Less expensive, over-the-counter hearing aids may help mild to moderate hearing loss. Family physicians should counsel patients on the importance of protecting their hearing.

Comparative analysis of vestibular endolymphatic hydrops grading methods and hearing loss in Ménière's disease: a retrospective MRI study using 3D-real inversion recovery sequence.

PURPOSE: To compare the correlation between different grading methods of vestibular endolymphatic hydrops (EH) and the severity of hearing loss in Ménière's disease (MD), and evaluate the diagnostic value of these methods in diagnosing MD. METHODS: This retrospective study included 30 patients diagnosed with MD from June 2021 to August 2023. All patients underwent inner ear MR gadolinium-enhanced imaging using three-dimensional (3D)-real inversion recovery sequences and pure-tone audiometry. The EH levels were independently evaluated according to the classification methods outlined by Nakashima et al. (Acta Otolaryngol Suppl 5-8, 2009. https://doi.org/10.1080/00016480902729827 ) (M1), Fang et al. (J Laryngol Otol 126:454-459, 2012. https://doi.org/10.1017/S0022215112000060 ) (M2), Barath et al. (Am J Neuroradiol 35:1387-1392, 2014. https://doi.org/10.3174/ajnr.A3856 ), (M3), Liu et al. (Front Surg 9:874971, 2022. https://doi.org/10.3389/fsurg.2022.874971 ), (M4), and Bernaerts et al. (Neuroradiology 61:421-429, 2019. https://doi.org/10.1007/s00234-019-02155-7 ) (M5), with a subsequent comparison of interobserver agreement. After achieving a consensus, an analysis was performed to explore the correlations between vestibular EH grading using different methods, the average hearing thresholds at low-mid, high-, and full frequencies and clinical stages. The diagnostic capabilities of these methods for MD were then compared. RESULTS: The interobserver consistency of M2-M5 was superior to that of M1. The EH grading based on M4 showed a significant correlation with the average hearing thresholds at low-mid, high-, and full frequencies and clinical stages. M1, M2, M3, and M5 correlated with some parameters. A receiver operating characteristic curve analysis indicated that M5 significantly outperformed M1, M2, M3, and M4 in terms of diagnostic efficiency for MD. CONCLUSION: M4 showed the strongest correlation with the degree of hearing loss in patients with MD, whereas M5 showed the highest diagnostic performance.

Mobile audiometry for hearing threshold assessment: A systematic review and meta-analysis.

OBJECTIVES: Technological advancements in mobile audiometry (MA) have enabled hearing assessment using tablets and smartphones. This systematic review (PROSPERO ID: CRD42021274761) aimed to identify MA options available to health providers, assess their accuracy in measuring hearing thresholds, and explore factors that might influence their accuracy. DESIGN AND SETTING: A systematic search of online databases including PubMed, Embase, Cochrane, Evidence Search and Dynamed was conducted on 13th December 2021, and repeated on 30th October 2022, using appropriate Medical Subject Headings (MeSH) terms. Eligible studies reported the use of MA to determine hearing thresholds and compared results to conventional pure-tone audiometry (CA). Studies investigating MA for hearing screening (i.e. reporting just pass/fail) were ineligible for inclusion. Two authors independently reviewed studies, extracted data, and assessed methodological quality and risk of bias using the Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2) tool. PARTICIPANTS: Adults and children, with and without diagnosis of hearing impairment. MAIN OUTCOME MEASURES: A meta-analysis was performed to obtain the mean difference between thresholds measured using MA and CA in dB HL. RESULTS: Searches returned 858 articles. After systematic review, 17 articles including 1032 participants were analysed. The most used software application was ShoeboxTM (6/17) followed by Hearing TestTM (3/17), then HearTestTM (2/17). Tablet computers were used in ten studies, smartphones in six, and a computer in one. The mean difference between MA and CA thresholds was 1.36 dB (95% CI, 0.07-2.66, p = 0.04). Significant differences between mobile audiometry (MA) and conventional audiometry (CA) thresholds were observed in thresholds measured at 500Hz, in children, when MA was conducted in a sound booth, and when MA was self-administered. However, these differences did not exceed the clinically significant threshold of 10 decibels (dB). Included studies exhibited high levels of heterogeneity, high risk of bias and low concerns about applicability. CONCLUSIONS: MA compares favourably to CA in measuring hearing thresholds and has role in providing access to hearing assessment in situations where CA is not available or feasible. Future studies should prioritize the integration of pure-tone threshold assessment with additional tests, such as Speech Recognition and Digits-in-Noise, for a more rounded evaluation of hearing ability, assesses acceptability and feasibility, and the cost-effectiveness of MA in non-specialist settings.

Pure tone audiogram classification using deep learning techniques.

OBJECTIVE: Pure tone audiometry has played a critical role in audiology as the initial diagnostic tool, offering vital insights for subsequent analyses. This study aims to develop a robust deep learning framework capable of accurately classifying audiograms across various commonly encountered tasks. DESIGN, SETTING, AND PARTICIPANTS: This single-centre retrospective study was conducted in accordance with the STROBE guidelines. A total of 12 518 audiograms were collected from 6259 patients aged between 4 and 96 years, who underwent pure tone audiometry testing between February 2018 and April 2022 at Tongji Hospital, Tongji Medical College, Wuhan, China. Three experienced audiologists independently annotated the audiograms, labelling the hearing loss in degrees, types and configurations of each audiogram. MAIN OUTCOME MEASURES: A deep learning framework was developed and utilised to classify audiograms across three tasks: determining the degrees of hearing loss, identifying the types of hearing loss, and categorising the configurations of audiograms. The classification performance was evaluated using four commonly used metrics: accuracy, precision, recall and F1-score. RESULTS: The deep learning method consistently outperformed alternative methods, including K-Nearest Neighbors, ExtraTrees, Random Forest, XGBoost, LightGBM, CatBoost and FastAI Net, across all three tasks. It achieved the highest accuracy rates, ranging from 96.75% to 99.85%. Precision values fell within the range of 88.93% to 98.41%, while recall values spanned from 89.25% to 98.38%. The F1-score also exhibited strong performance, ranging from 88.99% to 98.39%. CONCLUSIONS: This study demonstrated that a deep learning approach could accurately classify audiograms into their respective categories and could contribute to assisting doctors, particularly those lacking audiology expertise or experience, in better interpreting pure tone audiograms, enhancing diagnostic accuracy in primary care settings, and reducing the misdiagnosis rate of hearing conditions. In scenarios involving large-scale audiological data, the automated classification system could be used as a research tool to efficiently provide a comprehensive overview and statistical analysis. In the era of mobile audiometry, our deep learning framework can also help patients quickly and reliably understand their self-tested audiograms, potentially encouraging timely consultations with audiologists for further evaluation and intervention.

Audiovestibular Involvement in Patients With Systemic Sclerosis.

INTRODUCTION: Audiovestibular dysfunction has been reported in many autoimmune connective tissue diseases, including systemic sclerosis (SSc). OBJECTIVE: To assess the prevalence and features of audiological and vestibular disturbances in SSc patients and evaluate their relationship with disease duration, clinical features, nailfold videocapillaroscopy pattern, and immunologic profiles. METHOD: A matched case-control study was conducted in a rheumatology clinic of a second-level hospital over 24 months. All patients underwent a detailed ear, nose, and throat examination, as well as audiometric and vestibular assessments, including pure tone audiometry, speech audiometry, immittance tests, and the Video Head Impulse Test. RESULTS: Thirty-five SSc patients and 24 healthy controls were included in the study. In the SSc group, subjective hearing loss was reported by 17.1% of patients, vertigo by 14.3%, tinnitus by 11.4%, and dizziness by 5.7%. Sensorineural hearing loss was identified in 42.9% of SSc patients, significantly higher than in the control group ( p = 0.013). There was no correlation between audiological manifestations and clinical symptoms, organ involvement, immunologic characteristics, and treatment. Vestibular dysfunction was detected in 60% of SSc patients, significantly higher than the control group ( p = 0.05). A significant correlation was found between abnormal Video Head Impulse Test and the presence of anti-RNA polymerase III and anti-Th/To antibodies ( p = 0.05 and p = 0.034, respectively). CONCLUSION: Our study revealed an increased prevalence of sensorineural hearing loss and vestibulopathy in SSc patients.

Audiological Manifestations in Patients with Granulomatosis with Polyangiitis.

Background and Objectives: Granulomatosis with Polyangiitis (GPA) is a rare, autoimmune, multisystemic disease characterized by vasculitis and necrotizing granuloma that commonly affects the upper and lower respiratory tract and kidneys. Audiovestibular dysfunction in GPA diseases may have different clinical presentations. The aim of the present study was to evaluate hearing function in patients with GPA and to compare the results with a healthy control group. Materials and Methods: A total of 34 individuals participated in the study. The GPA group consisted of 14 participants, and the control group was composed of 20 healthy participants with no signs or symptoms of ear disease. The ages ranged from 18 to 65 years old, with a mean age of 43.8 years. The participants underwent a complete audiological evaluation using otoscopy, impedance audiometry, pure tone audiometry, speech audiometry-evaluation of speech thresholds, and speech recognition in quiet. Both ears were tested. All of the participants of the study were native Lithuanian speakers. Data were statistically analyzed using the Statistical Analysis System software SAS® Studio 3.8. A p value < 0.05 was regarded as statistically significant. Results: 92.85% of patients from the GPA group reported hearing-related symptoms: hearing loss, tinnitus, and fullness in the ears. The arithmetic means of all hearing thresholds at frequencies from 125 Hz to 8000 Hz were significantly higher in the GPA group. The results revealed statistically significant differences between the two groups in the Speech Detection Threshold, Speech Recognition Threshold, Speech Discomfort level, and Word Recognition Scores. Conclusions: The frequency of hearing loss, the average hearing thresholds, and speech thresholds were higher in GPA patients than in healthy individuals. The most common type of hearing loss was sensorineural. Audiological assessments should be considered during the routine evaluation of patients with GPA disease to prevent hearing-related disabilities.

Audiometric and Vestibular Function after Classic and Reverse Stapedotomy.

Background and Objectives: Besides classical stapedotomy, reverse stapedotomy has been used for many years in the management of otosclerosis. Our study aims to investigate whether reversing the surgical steps in stapedotomy impacts vestibular function and hearing improvement. Materials and Methods: A cohort of 123 patients underwent either classic or reverse stapedotomy procedures utilizing a fiber-optic argon laser. Audiological assessments, following the guidelines of the Committee on Hearing and Equilibrium, were conducted, including pure tone average, air-bone (AB) gap, overclosure, and AB gap closure. Vestibular evaluation involved pre- and postoperative comparison of rotatory test parameters, including frequency, amplitude, and slow phase velocity of nystagmus. Results: The study demonstrated an overall median overclosure of 3.3 (3.3, 5.0) dB and a mean AB gap closure of 20.3 ± 8.8 dB. Postoperative median AB gap was 7.5 (7.5, 11.3) dB in the reverse stapedotomy group and 10.0 (10.0, 12.5) dB in the classic stapedotomy group. While overclosure and AB gap closure were marginally superior in the reverse stapedotomy group, these differences did not reach statistical significance. No significant disparities were observed in the frequency, slow phase velocity, or amplitude of nystagmus in the rotational test. Conclusions: Although not always possible, reverse stapedotomy proved to be a safe surgical technique regarding postoperative outcomes. Its adoption may mitigate risks associated with floating footplate, sensorineural hearing loss, and incus luxation/subluxation, while facilitating the learning curve for less experienced ear surgeons.

Correlation Analysis and Comparison of Adult CE-Chirp ABR Response Threshold and Pure Tone Hearing Threshold.

OBJECTIVES: To study the application of CE-Chirp in the evaluation of hearing impairment in forensic medicine by testing the auditory brainstem response (ABR) in adults using CE-Chirp to analyze the relationship between the V-wave response threshold of CE-Chirp ABR test and the pure tone hearing threshold. METHODS: Subjects (aged 20-77 with a total of 100 ears) who underwent CE-Chirp ABR test in Changzhou De'an Hospital from January 2018 to June 2019 were selected to obtain the V-wave response threshold, and pure tone air conduction hearing threshold tests were conducted at 0.5, 1.0, 2.0 and 4.0 kHz, respectively, to obtain pure tone listening threshold. The differences and statistical differences between the average pure tone hearing threshold and V-wave response threshold were compared in different hearing levels and different age groups. The correlation, differences and statistical differences between the two tests at each frequency were analyzed for all subjects. The linear regression equation for estimating pure tone hearing threshold for all subjects CE-Chirp ABR V-wave response threshold was established, and the feasibility of the equation was tested. RESULTS: There was no statistical significance in the CE-Chirp ABR response threshold and pure tone hearing threshold difference between different hearing level groups and different age groups (P>0.05). There was a good correlation between adult CE-Chirp ABR V-wave response threshold and pure tone hearing threshold with statistical significance (P<0.05), and linear regression analysis showed a significant linear correlation between the two (P<0.05). CONCLUSIONS: The use of CE-Chirp ABR V-wave response threshold can be used to evaluate subjects' pure tone hearing threshold under certain conditions, and can be used as an audiological test method for forensic hearing impairment assessment.

[Assessment of the risk of hearing loss in children with cystic fibrosis]. / Otsenka riska patologii slukha pri mukovistsidoze u detei.

BACKGROUND: Cystic fibrosis (CF) is a severe hereditary disease with a multisystem lesion. Manifestations of CF include severe infectious purulent lesions of all parts of the respiratory tract, including purulent rhinosinusitis with nasal polyps. The involvement of the sinonasal region and the need for systemic use of ototoxic drugs (primarily aminoglycosides to treat resistant bacterial infection) potentially create a risk of both conductive and sensorineural hearing loss (SNHL). The available data on the epidemiology of hearing disorders in CF is contradictory. Currently, genetic determinants of the development of aminoglycoside SNHL have been identified. MATERIAL AND METHODS: For 136 CF patients (75 girls, 61 boys) aged 3 to 17 (9.4±3.9) years were performed audiological examination: tympanometry, transient-evoked otoacoustic emission and the pure tone threshold audiometry (standard frequency range) (n=126). History of systemic therapy with aminoglycosides was evaluated for each patient. Sequencing of c.35delG mutations in the GJB2 gene (nuclear DNA) and A1555G in the 12S rRNA gene (mitochondrial DNA) was performed in 215 patients with cystic fibrosis (the group partially overlaps with the audiological group), and as a control - 106 children with bronchial asthma and 103 healthy children, their age ranged from 3 to 17 (8.8±3.8) years. RESULTS: Audiological examination of CF children reveled a prevalence of conductive hearing loss comparable to the general population (2.4%). The frequency of SNHL was 1.6%, wich exceeds that of non-CF children. A genetic study revealed one case of heterozygous carriage of the c.35delG mutation in the GJB2 gene in a patient with bronchial asthma. In the group of patients with CF (n=215), mutations in the connexin 26 gene were not detected. No A1555G mutation was detected either in the group of patients with CF or in the control groups. CONCLUSIONS: Children with CF are at risk for the development of sensorineural, but not conductive hearing loss. Routine total screening for A1555G and c.35delG mutations probably seems not to be recommended.

Diagnostic Value of the Air-Bone Threshold Gap in Stapes Fixation.

INTRODUCTION: The Carhart notch is a well-known sign of stapes fixation. However, previous studies have reported that the Carhart notch is not specific to stapes fixation and is also present in other middle ear diseases. Therefore, this study investigated the diagnostic value of threshold gap between air conduction and bone conduction (ABG) for stapes fixation, instead of the bone conduction dip representing the Carhart notch. METHODS: A total of 199 ears that underwent exploratory tympanotomy were enrolled in this retrospective study. They were categorized into three groups according to surgical findings: stapes fixation (SF), other ossicle fixation (OF), and chain disconnection (CD). Preoperative pure-tone audiograms and impedance audiograms were compared between the groups. RESULTS: The incidence of the Carhart notch did not differ between the groups. The ABG at 2,000 Hz showed a good diagnostic performance for distinguishing between the SF and CD groups (area under the curve, AUC = 0.816, p < 0.001), but poor performance for distinguishing between the SF and OF groups (AUC = 0.662, p = 0.003). Bone conduction at 2,000 Hz showed a moderate performance for distinguishing between the SF and CD groups (AUC = 0.707, p < 0.001) and did not show statistically significant results for distinguishing between the SF and OF groups (AUC = 0.594, p = 0.080). The tympanic membrane compliance was significantly higher in the CD group than in the SF group (p = 0.001). CONCLUSIONS: The Carhart notch was not a specific finding of SF. The sensitivity and specificity of ABG ≤15 dB at 2,000 Hz for distinguishing between SF and CD were 60.4% and 89.2%, respectively. To prepare for surgical interventions in patients with conductive hearing loss but a normal tympanic membrane, clinicians should comprehensively consider these results.

Probability Distributions for Associations Between Cognitive Screening and Pure-tone Thresholds in Older Adults.

OBJECTIVES: Lower general cognitive function is frequently reported in older adults with elevated pure-tone thresholds. Here, we examined reason(s) for this association, including whether this relationship is dependent on the frequency range or extent of hearing loss and cognitive screening performance. DESIGN: Linear regression was used to examine associations between better-ear pure-tone thresholds and Mini-Mental Status Exam (MMSE) performance in a cross-sectional sample of relatively healthy older adults (N = 508; 68% women, 60-89+ years; M age = 72). Quantile regression was also used to identify the ranges of 0.5 and 4.0 kHz thresholds and MMSE scores where these variables exhibited significant associations. RESULTS: MMSE scores and pure-tone thresholds exhibited small but significant associations, particularly for better-ear 0.5 kHz thresholds. This hearing threshold and cognitive screening association was present among participants with better hearing, including the oldest older adults. There was limited evidence for mediating health condition effects on this association. An item analysis of the MMSE revealed that the MMSE and pure-tone threshold associations were largely due to the delayed recall item of the MMSE. CONCLUSIONS: Together, the small effect results are consistent with the extant literature and suggest that there are multiple reasons for modest pure-tone threshold and cognitive screening performance associations.

Prevalence of Childhood Hearing Loss in Rural Alaska.

OBJECTIVES: Childhood hearing loss has well-known lifelong consequences. Certain rural populations are at higher risk for infection-related hearing loss. For Alaska Native children, historical data on hearing loss prevalence suggest a higher burden of infection-related hearing loss, but updated prevalence data are urgently needed in this high-risk population. DESIGN: Hearing data were collected as part of two school-based cluster-randomized trials in 15 communities in rural northwest Alaska over two academic years (2017-2019). All enrolled children from preschool to 12th grade were eligible. Pure-tone thresholds were obtained using standard audiometry and conditioned play when indicated. The analysis included the first available audiometric assessment for each child (n = 1634 participants, 3 to 21 years), except for the high-frequency analysis, which was limited to year 2 when higher frequencies were collected. Multiple imputation was used to quantify the prevalence of hearing loss in younger children, where missing data were more frequent due to the need for behavioral responses. Hearing loss in either ear was evaluated using both the former World Health Organization (WHO) definition (pure-tone average [PTA] > 25 dB) and the new WHO definition (PTA ≥ 20 dB), which was published after the study. Analyses with the new definition were limited to children 7 years and older due to incomplete data obtained on younger children at lower thresholds. RESULTS: The overall prevalence of hearing loss (PTA > 25 dB; 0.5, 1, 2, 4 kHz) was 10.5% (95% confidence interval [CI], 8.9 to 12.1). Hearing loss was predominately mild (PTA >25 to 40 dB; 8.9%, 95% CI, 7.4 to 10.5). The prevalence of unilateral hearing loss was 7.7% (95% CI, 6.3 to 9.0). Conductive hearing loss (air-bone gap of ≥ 10 dB) was the most common hearing loss type (9.1%, 95% CI, 7.6 to 10.7). Stratified by age, hearing loss (PTA >25 dB) was more common in children 3 to 6 years (14.9%, 95% CI, 11.4 to 18.5) compared to children 7 years and older (8.7%, 95% CI, 7.1 to 10.4). In children 7 years and older, the new WHO definition increased the prevalence of hearing loss to 23.4% (95% CI, 21.0 to 25.8) compared to the former definition (8.7%, 95% CI, 7.1 to 10.4). Middle ear disease prevalence was 17.6% (95% CI, 15.7 to 19.4) and was higher in younger children (23.6%, 95% CI, 19.7 to 27.6) compared to older children (15.2%, 95% CI, 13.2 to 17.3). High-frequency hearing loss (4, 6, 8kHz) was present in 20.5% (95% CI, 18.4 to 22.7 [PTA >25 dB]) of all children and 22.8% (95% CI, 20.3 to 25.3 [PTA >25 dB]) and 29.7% (95% CI, 27.0 to 32.4 [PTA ≥ 20 dB]) of children 7 years and older (limited to year 2). CONCLUSIONS: This analysis represents the first prevalence study on childhood hearing loss in Alaska in over 60 years and is the largest cohort with hearing data ever collected in rural Alaska. Our results highlight that hearing loss continues to be common in rural Alaska Native children, with middle ear disease more prevalent in younger children and high-frequency hearing loss more prevalent with increasing age. Prevention efforts may benefit from managing hearing loss type by age. Lastly, continued research is needed on the impact of the new WHO definition of hearing loss on field studies.

Comparison of Intratympanic Steroid and Hyperbaric Oxygen Salvage Therapy Hearing Outcomes in Idiopathic Sudden Sensorineural Hearing Loss: A Retrospective Study.

OBJECTIVES: Systemic steroids are the most common first-line therapy in sudden sensorineural hearing loss (SSNHL), with significant improvement in hearing outcomes in over 60% of patients. It is unknown why 40% of patients do not respond to systemic steroid therapy. Salvage treatment includes intratympanic steroids (ITS) and hyperbaric oxygenation (HBO) therapy, with inconsistent results reported. This study aimed to compare the results of ITS and HBO therapy in patients with SSNHL that previously failed systemic steroid therapy. DESIGN: This is a comparative retrospective nonrandomized interventional cohort study, enrolling 126 patients with SSNHL. Out of these, 35 patients received HBO therapy, 43 patients received ITS, and 48 patients did not receive any second-line therapy (control group). Pure-tone audiograms were performed before and after the salvage therapy in the IT and HBO groups and at the same time interval in the control group. Study variables included age, time until therapy initiation, tinnitus status, and hearing outcomes, with a cutoff criteria of cumulative >30 dB improvement on all frequencies indicating recovery. RESULTS: ITS and HBO therapy were associated with statistically significant hearing recovery at all frequencies compared to systemic steroids. The results show an average hearing improvement of 13.6 dB overall frequencies (250 to 8000 Hz) after ITS therapy and 7.4 dB in HBO therapy in comparison to the control group. Presence of significant hearing improvement positively correlated with age, ITS therapy, and HBO therapy. Presence of tinnitus before therapy was negatively correlated with hearing improvement. Patients with tinnitus present at the start of therapy improve 4.67 dB less on average compared to those without tinnitus. ITS therapy significantly reduced tinnitus compared to the other two treatment options. Patients with tinnitus present before therapy significantly improve hearing at low frequencies, compared to the control group. CONCLUSIONS: ITS and HBO therapy show superior hearing results compared to observation alone after failed oral steroid therapy for SSNHL. ITS shows an additional positive impact on tinnitus reduction and shows superior hearing outcomes after salvage therapy.

Sex differences in autoimmune inner ear disease treatment and audiological outcomes.

OBJECTIVE: The primary objective of this study was to assess possible sex-related differences in AIED treatment. The secondary aim was to assess the long-term outcomes of treatment using pre- and post-treatment audiometry and speech discrimination scores. METHODS: Adult patients with a diagnosis of AIED who were treated for their condition at the senior author's (RTS) practice from 2010 to 2022 were included in this study. Patients were grouped as males or females for further analysis and comparison. Data included past medical history, medication use, surgical history and social history. Air-conduction thresholds (500 to 8000 Hz) were collected and averaged into discrete variables for pre- and post-treatment. The change (∆) and percent change (%∆) of these variables following therapy were analyzed. Speech discrimination score (SDS) testing results were taken at the same time points as pure tone averages, and patients were sub-stratified based on evidence of improvement in SDS and compared accordingly. RESULTS: One hundred eighty-four patients (78 male/106 female) were included in this study. The mean age of the male participants was 57.18 ± 15.92 years, and the mean age of the female participants was 53.49 ± 16.04 years (p = 0.220). Comorbid autoimmune disease (AD) was more prevalent in females than in males (38.7 % vs. 16.7 %, p = 0.001). Of the patients treated with oral steroids, females received significantly more courses than males (2.554 ± 2.078 vs. 1.946 ± 1.301, p = 0.020). However, the average duration of oral steroids used per trial did not differ significantly between males and females (21.02 ± 18.05 vs. 20.62 ± 7.49, p = 0.135). Audiological results demonstrated that the change (∆) in pure tone average (PTA) at 0.5, 1, 2, and 3 kHz (-4.21 ± 6.394 vs. -3.91 ± 6.105) and high-frequency pure tone average (HFPTA) at 4, 6, and 8 kHz (-4.55 ± 6.544 vs. -2.19 ± 6.842) was not significantly different between the sexes following treatment (p = 0.376 and p = 0.101, respectively). Similarly, the percentage change (%∆) in PTA (-13.17 % vs. -15.01 %) and HFPTA (-8.50 % vs. -6.76 %) also did not differ significantly between males and females (p = 0.900 and p = 0.367, respectively). One hundred-five (57.1 %) patients qualified for the SDS improvement subgroup for analysis, with 50 (47.6 %) being male and 55 (51.9 %) being female (p = 0.159). The change (∆) in SDS (15.12 ± 21.159 vs. 10.62 ± 19.206) and the percentage change (%∆) in SDS (16.71 % vs. 12.40 %) also showed no significant difference between male and female patients (p = 0.312 and p = 0.313, respectively). CONCLUSION: AIED is not a uniform condition regarding clinical presentation, audiological findings or progression, nor is its treatment straightforward. The utilization and duration of cytotoxic medications, as well as PTA and SDS findings, did not differ between the sexes. However, significantly more courses of oral steroids were prescribed to females than males. Sex as a biological variable and its implications in the pathogenesis and treatment of AIED warrant further study.

A novel technique in treatment of advanced tympanosclerosis: results of malleus replacement and loop prostheses combination; pure endoscopic transcanal approach.

PURPOSE: The objectives of the present clinical trial are to describe our surgical technique for advanced tympanosclerosis (TS) and to present the comparison of pre-and postoperative audiometric results. METHODS: 21 patients suffering from advanced TS (Only type III and IV patients according to Wielinga-Kerr classification) were enrolled in this prospective study. A pure transcanal endoscopic approach was preferred for all patients included in the study. Titanium malleus replacement prosthesis (MRP) and polytetrafluoroethylene (PTFE) loop prosthesis combination were used to bypass fixed ossicles to achieve sound transmission to the inner ear. Pre-and postoperative pure tone audiometry (PTA) measurements and air-bone gap (ABG) results compared, and surgical technique was considered successful if postoperative ABG was less than 20 dB. RESULTS: Six of the 21 patients had been operated on before and the tympanic membrane (TM) was intact. The remaining 15 of the 21 patients were the primary cases, and TM perforation repair and ossiculoplasty using combination of the MRP and PTFE loop prosthesis were performed at single-staged operation. The average preoperative ABG values significantly improved from 37.1 ± 6.2 to 14.5 ± 1.2 dB postoperatively (p < 0.001). The mean preoperative air conduction threshold (ACT) significantly decreased from 51.7 ± 11.4 to 28.5 ± 9.1 dB (p < 0.001). CONCLUSION: TS surgery is still a controversial issue particularly in stapes footplate fixation. However, our surgical and audiological results in this study are quite promising. The combination of titanium MRP and PTFE loop prosthesis for restoring sound transmission in advanced TS cases is reliable, effective and novel treatment option via endoscopic transcanal approach.