Audiologic Natural History of Small Volume Cochleovestibular Schwannomas in Neurofibromatosis Type 2.
ABSTRACT: OBJECTIVE:To characterize the audiometric natural progression in patient-ears with small volume (<1,000?mm), treatment-naïve cochleovestibular schwannomas (CVSs) in Neurofibromatosis Type 2 (NF2). STUDY DESIGN:Prospective, longitudinal cohort study. SETTING:Quaternary medical research institute. PATIENTS:One hundred eleven ears in 71 NF2 patients with small, treatment-naïve CVSs observed from July 2006 to July 2016. INTERVENTION:Serial audiometric testing, including pure tone audiometry, speech audiometry, and magnetic resonance imaging (MRI). OUTCOME MEASURES:Four-frequency pure tone average (4f-PTA) of 0.5, 1, 2, and 4?kHz and word recognition score (WRS) were recorded. Their changes were compared with MRI changes in CVS volume over time. Times to significant hearing loss (10?dB loss in 4f-PTA) and WRS based on 95% critical difference were measured. RESULTS:Linear regression analysis showed a significant correlation with baseline hearing level (4f-PTA) and internal auditory canal (IAC) tumor volume to annual hearing decrease rate (AHDR) (p?=?0.003, p?=?0.0004). Hearing level at baseline and tumor volume correlate with AHDR while tumor volume growth rate does not. Two-way analysis of variance found significant differences in AHDR, risk of significant hearing loss, and risk of critical difference in WRS based on baseline hearing level (abnormal or normal) and IAC tumor volume (greater or less than 200 mm). CONCLUSION:Subjects with normal baseline hearing and small IAC tumor component had a low AHDR and low risk of significant hearing loss and may warrant conservative management while the presence of baseline hearing loss and large IAC volume resulted in higher ADHR and greater risk for further hearing loss and may benefit from early treatment interventions.
Project description:The purpose of this study was to examine the difference between self-reported hearing status and hearing impairment assessed using conventional audiometry. The associated factors were examined when a concordance between self-reported hearing and audiometric measures was lacking.In total, 19,642 individuals ?20 years of age who participated in the Korea National Health and Nutrition Examination Surveys conducted from 2009 through 2012 were enrolled. Pure-tone hearing threshold audiometry (PTA) was measured and classified into three levels: <25 dB (normal hearing); ?25 dB <40 dB (mild hearing impairment); and ?40 dB (moderate-to-severe hearing impairment). The self-reported hearing loss was categorized into 3 categories. The participants were categorized into three groups: the concordance (matched between self-reported hearing loss and audiometric PTA), overestimation (higher self-reported hearing loss compared to audiometric PTA), and underestimation groups (lower self-reported hearing loss compared to audiometric PTA). The associations of age, sex, education level, stress level, anxiety/depression, tympanic membrane (TM) status, hearing aid use, and tinnitus with the discrepancy between the hearing self-reported hearing loss and audiometric pure tone threshold results were analyzed using multinomial logistic regression analysis with complex sampling.Overall, 80.1%, 7.1%, and 12.8% of the participants were assigned to the concordance, overestimation, and underestimation groups, respectively. Older age (adjusted odds ratios [AORs] = 1.28 [95% confidence interval = 1.19-1.37] and 2.80 [2.62-2.99] for the overestimation and the underestimation groups, respectively), abnormal TM (2.17 [1.46-3.23] and 1.59 [1.17-2.15]), and tinnitus (2.44 [2.10-2.83] and 1.61 [1.38-1.87]) were positively correlated with both the overestimation and underestimation groups. Compared with specialized workers, service workers, manual workers, and the unemployed were more likely to be in the overestimation group (1.48 [1.11-1.98], 1.39 [1.04-1.86], and 1.50 [1.18-1.90], respectively), and service workers were more likely to be in the underestimation group (AOR = 1.42 [1.01-1.99]). Higher education level (0.77 [0.59-1.01] and 0.43 [0.33-0.57]) and hearing aid use (0.36 [0.17-0.77] and 0.23 [0.13-0.43]) were negatively associated with being in the underestimation group (0.43 [0.37-0.50]). Compared with males, females were less likely to be assigned to the underestimation group (0.43 [0.37-0.50]). Stress (1.98 [1.32-2.98]) and anxiety/depression (1.30 [1.06-1.59]) were associated with overestimation group.Older age, lower education level, occupation, abnormal TM, non-hearing aid use, and tinnitus were related to both overestimation and underestimation groups. Male gender was related to underestimation, and stress and anxiety/depression were correlated with overestimation group. An understanding of these factors associated with the self-reported hearing loss will be instrumental to identifying and managing hearing-impaired individuals.
Project description:Different guidelines are adopted in clinics and countries to assess pure tone hearing sensitivity in children with otitis media with effusion (OME). Some guidelines specify a broad range of audiometric frequencies that must be tested and from which average thresholds determined, while others leave test frequencies unspecified. For guidelines that suggest specific frequencies there are various pure tone frequencies and frequency ranges given. The present study investigated whether (1) a full range of audiometric frequencies is required to evaluate hearing loss caused by OME in children, or if neighboring frequencies provide essentially the same threshold information, and (2) if different combinations of test frequency pure tone averaging calculations may affect decision criteria for surgical treatment. In a retrospective cohort study, right and left ear air conduction pure tone threshold data were obtained, from 125 Hz to 8 kHz, for 96 children with OME aged 4 to 12 years. Paired t-tests, correlation tests (Pearson's r, Cronbach's alpha, intraclass correlation) and absolute differences were used to examine the relationships among pure tone audiometric (PTA) frequencies for all ears with hearing loss. 168 ears were found to have OME-related hearing loss. Only the 125 Hz-250 Hz comparison showed no statistically significant difference between neighboring thresholds. However, only the 4 kHz and 8 kHz comparison showed a clinically significant mean difference of ≥ 10 dB. When viewing individual differences, comparison between 250 Hz and 500 Hz, 125 Hz and 500 Hz, and 4 kHz and 8 kHz, showed a large number of ears with clinically significant differences between test frequencies. Comparisons among low frequency 3 PTA average (500 Hz, 1 kHz, 2 kHz), high frequency 3 PTA average (1 kHz, 2 kHz, 4 kHz), and 4 frequency PTA average (500 Hz, 1 kHz, 2 kHz, 4 kHz) showed no statistically significant differences, with very strong correlations for all comparisons. In addition, for all the combinations of PTA averages, no clinically significant differences were found for the various comparisons or among individual results. Clinically, testing hearing sensitivity in the 125 Hz to 8 kHz range is worthwhile in evaluating hearing sensitivity in children with OME due to large individual variability across audiometric frequencies. However, frequencies tested for criterion averages for surgical treatments of children with OME may be restricted to 3 frequency PTA averages, either an average of 500 Hz, 1 kHz, 2 kHz or an average of 1 kHz, 2 kHz, 4 kHz, as no clinically significant differences were found using these or a 4 frequency averaging technique. For research purposes, 250 Hz can proxy for hearing thresholds at 125 Hz; and the low frequency 3 PTA average, high frequency 3 PTA average and 4 frequency PTA average may be used interchangeably, as no statistically significant differences were found among these measures.
Project description:To assess long-term hearing outcomes in patients treated with third-generation bisphosphonates for otosclerosis-related progressive sensorineural hearing loss (SNHL).Retrospective case series review.We performed a retrospective case series review of patients with otosclerosis and progressive SNHL. Patients were treated with either risedronate or zoledronate after a diagnosis of otosclerosis with a significant SNHL component. Bone conduction pure tone threshold averages (BC-PTAs) and word recognition scores (WRS) before and after bisphosphonate administration in long-term follow-up was analyzed. Significant change in BC-PTA was defined as greater than 10dB or between 4% and 18% in WRS based on binomial variance.Seven patients were identified and 14 ears met inclusion criteria. Three patients were female and the mean age was 48.3?±?10.3 years. The mean duration between treatment with bisphosphonate administration and long-term post-treatment follow-up audiometry was 87.6?±?18.3 months, with a range of 61.6 to 109.1 months and median of 89.2 months. Analysis using BC-PTA and WRS demonstrated that 11 ears remained stable while 2 improved and 1 worsened. No patient experienced any major complication as the result of bisphosphonate therapy.Treatment with third-generation bisphosphonates is associated with stability in otosclerosis-related sensorineural hearing over 5- to 9-year period. These results suggest that such medications may prevent the progression of SNHL in patients with otosclerosis.4 (Case series).
Project description:Autoimmune inner ear disease (AIED) is a rare disease that results in progressive sensorineural hearing loss. Patients with AIED initially respond to corticosteroids; however, many patients become unresponsive to this treatment over time, and there is no effective alternative therapy for these individuals.We performed a phase I/II open-label, single-arm clinical trial of the IL-1 receptor antagonist anakinra in corticosteroid-resistant AIED patients. Given that the etiology of corticosteroid resistance is likely heterogeneous, we used a Simon 2-stage design to distinguish between an unacceptable (?10%) and an acceptable (?30%) response rate to anakinra therapy. Subjects received 100 mg anakinra by subcutaneous injection for 84 days, followed by a 180-day observational period.Based on patient responses, the Simon 2-stage rule permitted premature termination of the trial after 10 subjects completed the 84-day drug period, as the target efficacy for the entire trial had been achieved. Of these 10 patients, 7 demonstrated audiometric improvement, as assessed by pure tone average (PTA) and word recognition score (WRS). In these 7 responders, reduced IL-1? plasma levels correlated with clinical response. Upon discontinuation of treatment, 3 subjects relapsed, which correlated with increased IL-1? plasma levels.We demonstrated that IL-1? inhibition in corticosteroid-resistant AIED patients was effective in a small cohort of patients and that IL-1? plasma levels associated with both clinical hearing response and disease relapse. These results suggest that a larger phase II randomized clinical trial of IL-1? inhibition is warranted.ClinicalTrials.gov NCT01267994.NIH, Merrill & Phoebe Goodman Otology Research Center, and Long Island Hearing & Speech Society.
Project description:BACKGROUND:An understanding of the hearing outcomes is needed for treatment counseling for patients with vestibular schwannomas (VS). OBJECTIVE:To determine long-term hearing results following stereotactic radiosurgery (SRS) for VS and identify any influential variables. METHODS:Tertiary hospital retrospective cohort. RESULTS:There were 579 tumors (576 patients) treated with SRS. Eighty-two percent (473) of tumors had ?1 yr and 59% (344 ?3 yr follow-up. In the 244 tumor ears, with measurable hearing before SRS who were followed ?1 yr, 14% (31) had improved hearing, 13% (29) unchanged hearing, and 74% (158) had worsened hearing. In 175 patients with ?3 yr follow-up and who had measurable hearing pretreatment, 6% (11 ears) improved hearing, 31% (54 ears) unchanged hearing, and 63% (110 ears) had worsened hearing. Patients with tumors with larger target volumes (P = .040) and with neurofibromatosis type 2 (NF2; P = .017) were associated with poorer hearing (P = .040). Patients with word recognition scores (WRS) of 50% or poorer had tumors with a larger volume (P = .0002), larger linear size (P = .032), and NF2 (P = .045). Traditionally reported hearing outcomes using the Gardner Robertson maintenance of PTA ?50 db or WRS ?50% were 48% at 3 yr, which overestimates hearing outcomes compared to the above reporting standards. CONCLUSION:Hearing declines over time in VS treated with SRS in a high proportion of cases. The frequency and magnitude of long-term hearing decline following SRS argues against prophylactic radiation for small tumors in hearing ears with undetermined growth behavior.
Project description:OBJECTIVES:To investigate the correlation of objective audiometry with user satisfaction as measured with the questionnaire scores. METHODS:Twenty patients with hearing loss, who agreed to wear a hearing aid and were referred for hearing aid fitting, were included in this prospective clinical study. All patients used the in-the-canal type of Wide7 hearing aid provided by BSL Co., Ltd. We performed the Korean version of the Hearing Handicap Inventory for the Elderly (K-HHIE) and the International Outcome Inventory for Hearing Aids (K-IOI-HA) before and 1, 3, and 6 months after wearing the hearing aid. We also performed pure tone audiometry (PTA), speech audiometry (SA), functional gain (FG), hearing in noise test (HINT), and central auditory processing disorder tests, such as frequency pattern test (CA-f), duration pattern test (CA-d), and dichotic test (CA-Di). Patients were divided into two groups (group A-HHIE, improved; group B-HHIE, same or worse) by comparing the score of K-HHIE before and 6 months after wearing the hearing aid. In the 6-month K-IOI-HA questionnaire, 21 points were considered as the average score. Based on this, we further divided patients into two groups (group A-IOI, >21 points; group B-IOI, ?21 points). RESULTS:Group A-HHIE included six patients and group B-HHIE included 14 patients. In PTA, SA, HINT, CA-d, and CA-Di, group A-HHIE showed higher improvements than group B-HHIE, which were not statistically significant. Group A-IOI included 12 patients and group B-IOI included eight patients. No statistically significant difference was noted in the improvement of audiometric results over a period of 6 months after wearing the hearing aid between groups A-IOI and B-IOI. CONCLUSION:There were no significant and consistent audiometric results to reflect patient's satisfaction with the hearing aid. Therefore, when analyzing the hearing aid-fitting outcome, both the objective audiometric tests and subjective questionnaire should be performed together for validating hearing aid performance.
Project description:The decline of speech intelligibility in presbycusis can be regarded as resulting from the combined contribution of two main groups of factors: (1) audibility-related factors and (2) age-related factors. In particular, there is now an abundant scientific literature on the crucial role of suprathreshold auditory abilities and cognitive functions, which have been found to decline with age even in the absence of audiometric hearing loss. However, researchers investigating the direct effect of aging in presbycusis have to deal with the methodological issue that age and peripheral hearing loss covary to a large extent. In the present study, we analyzed a dataset of consonant-identification scores measured in quiet and in noise for a large cohort (n = 459, age = 42–92) of hearing-impaired (HI) and normal-hearing (NH) listeners. HI listeners were provided with a frequency-dependent amplification adjusted to their audiometric profile. Their scores in the two conditions were predicted from their pure-tone average (PTA) and age, as well as from their Extended Speech Intelligibility Index (ESII), a measure of the impact of audibility loss on speech intelligibility. We relied on a causal-inference approach combined with Bayesian modeling to disentangle the direct causal effects of age and audibility on intelligibility from the indirect effect of age on hearing loss. The analysis revealed that the direct effect of PTA on HI intelligibility scores was 5 times higher than the effect of age. This overwhelming effect of PTA was not due to a residual audibility loss despite amplification, as confirmed by a ESII-based model. More plausibly, the marginal role of age could be a consequence of the relatively little cognitively-demanding task used in this study. Furthermore, the amount of variance in intelligibility scores was smaller for NH than HI listeners, even after accounting for age and audibility, reflecting the presence of additional suprathreshold deficits in the latter group. Although the non-sense-syllable materials and the particular amplification settings used in this study potentially restrict the generalization of the findings, we think that these promising results call for a wider use of causal-inference analysis in audiology, e.g., as a way to disentangle the influence of the various cognitive factors and suprathreshold deficits associated to presbycusis.
Project description:Objectives To describe a unique case report of a patient who had partial hearing preservation after translabyrinthine (TL) removal of a vestibular schwannoma (VS). Study Design Case report. Methods The patient's chart was reviewed for hearing levels before and after surgery. Preoperative magnetic resonance imaging (MRI) was compared with postoperative MRI for determination of completeness of tumor removal. The literature on hearing preservation after TL resection is reviewed. Results A 42-year-old woman underwent a TL removal of a VS. The patient's preoperative pure tone average (PTA) was 70 dB and word recognition score (WRS) was 40%. Postoperatively, the patient was able to hear ambient noise in the surgical ear. Her bone conduction PTA was 70 dB, but the WRS score dropped to 2%. One year later, she continues to hear ambient noise and sound in the operative ear. Discussion This is the fifth reported case of partial hearing preservation after TL VS resection. It suggests that by preserving the vestibule and the fluids within the vestibule when possible, there maybe enough residual auditory neural structures for a traditional cochlear implant to benefit such a patient. In addition, preserving the incus when possible may help maintain air conduction to help patients with sound localization.
Project description:BACKGROUND: Noise exposure is a well-known contributor to work-related hearing loss. Recent biological evidence suggests that exposure to ototoxic chemicals such as organic solvents and heavy metals may be additional contributors to hearing loss. However, in industrial settings, it is difficult to determine the risks of hearing loss due to these chemicals in workplaces accompanied by excessive noise exposure. A few studies suggest that the effect of noise may be enhanced by ototoxic chemicals. Therefore, this study investigated whether co-exposure to organic solvents and/or heavy metals in the workplace modifies the risk of noise exposure on hearing loss in a background of excessive noise. METHODS: We examined 30,072 workers nationwide in a wide range of industries from the Korea National Occupational Health Surveillance 2009. Data on industry-based exposure (e.g., occupational noise, heavy metals, and organic solvents) and subject-specific health outcomes (e.g., audiometric examination) were collected. Noise was measured as the daily 8-h time-weighted average level. Air conduction hearing thresholds were measured from 0.5 to 6 kHz, and pure-tone averages (PTA) (i.e., means of 2, 3, and 4 kHz) were computed. RESULTS: In the multivariate linear model, PTA increment with occupational noise were 1.64-fold and 2.15-fold higher in individuals exposed to heavy metals and organic solvents than in unexposed individuals, respectively. CONCLUSION: This study provides nationwide evidence that co-exposure to heavy metals and/or organic solvents may exacerbate the effect of noise exposure on hearing loss in workplaces. These findings suggest that workers in industries dealing with heavy metals or organic solvents are susceptible to such risks.
Project description:A multicenter study was set up to elucidate the environmental and medical risk factors contributing to age-related hearing impairment (ARHI). Nine subsamples, collected by nine audiological centers across Europe, added up to a total of 4,083 subjects between 53 and 67 years. Audiometric data (pure-tone average [PTA]) were collected and the participants filled out a questionnaire on environmental risk factors and medical history. People with a history of disease that could affect hearing were excluded. PTAs were adjusted for age and sex and tested for association with exposure to risk factors. Noise exposure was associated with a significant loss of hearing at high sound frequencies (>1 kHz). Smoking significantly increased high-frequency hearing loss, and the effect was dose-dependent. The effect of smoking remained significant when accounting for cardiovascular disease events. Taller people had better hearing on average with a more pronounced effect at low sound frequencies (<2 kHz). A high body mass index (BMI) correlated with hearing loss across the frequency range tested. Moderate alcohol consumption was inversely correlated with hearing loss. Significant associations were found in the high as well as in the low frequencies. The results suggest that a healthy lifestyle can protect against age-related hearing impairment.