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Background: The outcome of clinical treatment for idiopathic sudden sensorineural hearing loss (ISSNHL) is frequently the primary concern., Aim: For the convenient prediction of reference prognosis outcomes in patients with ISSNHL., Materials and Methods: Patients diagnosed with ISSNHL who were admitted to the otorhinolaryngology ward of Zhejiang Provincial Hospital of Traditional Chinese Medicine from January 2020 to December 2023 were included. Univariate and multivariate logistic regression analyses were employed to identify independent prognostic factors for the treatment outcome of ISSNHL, which were subsequently used to develop nomograms. Discrimination, calibration, and clinical utility were assessed to evaluate the performance of the ISSNHL nomograms., Results: 371 ISSNHL patients were enrolled in this study. Multivariable logistic regression analysis showed that history of deafness, aural fullness, time of onset, and age were independent prognostic factors for ISSNHL patients, with statistically significant regression coefficients. Predictive nomograms were developed with excellent discrimination, calibration, and clinical value., Conclusions: Leveraging data from ISSNHL patients, we developed a predictive nomogram to assess prognostic factors upon admission. This nomogram facilitates clinicians in approximating the likelihood of favorable prognosis., Significance: By accumulating clinical data from ISSNHL patients, it's anticipated that the possibility of recovery following treatment can be determined.

Background: Sudden sensorineural hearing loss with vertigo (SHLV) and vestibular neuritis (VN) can result in prolonged dizziness., Objectives: This study aimed to compare the video head impulse test (vHIT) of patients with SHLV and VN., Methods: Fifteen patients with SHLV and 21 patients with VN who visited the Vertigo/Dizziness Center of our hospital between December 2016 and February 2023 were included. vHIT was performed at the time of admission, and the VOR gain and catch up saccade (CUS) in the three types of semicircular canals (SCCs) were analyzed., Results: Pathologic vHIT results were observed most frequently in the posterior SCC (73%), followed by lateral (53%) and anterior (13%) SCCs in the SHLV group. In contrast, pathologic vHIT results were observed most frequently in the lateral SCC (100%), followed by the anterior (43%) and posterior SCC (24%) SCCs in the VN group. Pathological vHIT results in the lateral and posterior SCC showed significant differences between the two groups, but for anterior SCC, no significant differences were found., Conclusions and Significance: Comparison of the two vHIT results revealed differences in the SCC dysfunction patterns. This may be due to the different pathophysiological mechanisms of the two vestibular disorders, which may result in prolonged vertigo.

Background: There is no comprehensive and up-to-date overview of audiovestibular approach to the posterior fossa tumors in the literature., Objective: This paper reviewed the literature relating to tumors at the posterior cranial fossa to find red flags alerting a posterior fossa lesion from audiovestibular perspectives., Methods: This review was developed from articles published in those journals listed on the journal citation reports. Through the PubMed database, Embase, Google Scholar, and Cochrane library, 60 articles were finally obtained based on the PRISMA guidelines for reporting reviews., Results: The presence of one red flag indicates a positive predictive value of 33% for detecting a posterior fossa lesion. Clinical features, namely, 1) mid-frequency sudden sensorineural hearing loss (SNHL), 2) bilateral sudden SNHL, and 3) rebound nystagmus may indicate a posterior fossa lesion, representing one, two, and three red flags, respectively., Conclusion: Those with 1) mid-frequency sudden SNHL, 2) bilateral sudden SNHL, and 3) rebound nystagmus trigger one, two, and three red flags, respectively, alerting clinicians the possibility of a posterior fossa lesion, which warrant MR imaging to exclude life-threatening or treatable conditions., Significance: Patients with posterior fossa tumors may have potential life-threatening outcome.

Background: Sudden sensorineural hearing loss (SSNHL), which is frequently observed in otolaryngology clinics, is characterized by sudden onset hearing loss that can develop within hours or days. Its etiology is still not fully understood., Aims/objectives: This research aims to identify prognostic biomarkers that can be utilized to assess the progress of SSNHL as well as circumstances that may predispose individuals to the disease., Materials and Methods: Between 1 January 2019 and 1 October 2020, patients diagnosed with SSNHL in our clinic and a control group consisting of healthy people were examined retrospectively. The files of the groups were examined and the levels of hemoglobin, Neutrophil-Lymphocyte Ratio (NLR), ferritin, iron, iron binding capacity (UIBC), vitamin D, vitamin B12, folate were statistically compared with the control group. In addition, the effect of the parameters studied in the patient group on the degree of recovery was examined., Results: There was no statistically significant difference between the groups in terms of mean age and gender distribution ( p  > .05). NLR level of the patient group was higher than the control group ( p  < .05). Vitamin D level of the patient group was lower than the control group ( p  < .05). NLR and vitamin D levels had no effect on the degree of recovery ( p  > .05). There was no significant difference between the groups in terms of other parameters ( p  > .05)., Conclusions and Significance: We think that our study can be a guide for adding vitamin D as a routine laboratory test in patients with SSNHL. In addition, we think that NLR value can be used as a marker in patients with SSNHL. It is recommended to investigate the role of vitamin D supplementation in better and faster response in these patients.

Background and Objectives: Tinnitus is one of the most common symptoms of sudden sensorineural hearing loss (SSNHL), with the incidence of tinnitus in patients with SSNHL ranging from 60% to 90%. Little is known, however, about the specific audiologic and hematologic factors that may be associated with the development of tinnitus. To better understand the relationship between tinnitus and SSNHL, the present study compared audiologic and hematologic factors in SSNHL patients with tinnitus and without tinnitus., Subjects and Method: The present study compared 120 patients with SSNHL with tinnitus and 59 patients with SSNHL without tinnitus at their initial examination. Their audiology and hematologic test results were analyzed, and hearing recovery was determined by comparing the hearing thresholds before and after treatment., Results: 120 patients with tinnitus showed longer III and V latency in auditory brainstem response (ABR) tests, lower signal-to-noise ratios (SNR) at 2 kHz in transient evoked otoacoustic emissions (TEOAE) tests, and lower response rates at 2 kHz in distortion product otoacoustic emissions (DPOAE) tests of the affected ear ( p  < 0.05 each) than the 59 patients without tinnitus. However, there were no significant between-group differences in the mean hearing threshold and hearing recovery rate of the affected ear. Patients with tinnitus had significantly worse mean hearing thresholds and hearing thresholds at 4 kHz in the nonaffected ear. The percentages of monocytes and large unstained cells (%LUCs) were higher in the group without tinnitus ( p  < 0.05), although there were no significant between-group differences in inflammatory markers, such as neutrophil-to-lymphocyte ratio (NLR), monocyte-to-lymphocyte ratio (MLR) and platelet-to-lymphocyte ratio (PLR)., Conclusion: Tinnitus accompanying SSNHL may be associated with baseline hearing level, as well as being an indicator of damage to outer hair cells and auditory nerves. Additional studies are needed to evaluate hematologic data in SSNHL patients with and without tinnitus.

Background: Sudden sensory-neural hearing loss ( SSNHL), with positive findings on magnetic resonance imaging (MRI), possibly related to labyrinthine hemorrhage, is a rare condition and difficult to be diagnosed., Objectives: We evaluated the role of MRI in detecting labyrinthine signal changes and the impact of these changes on the prognosis of SSNHL after the intratympanic corticosteroid injection., Methods: A prospective study was held between January and June 2022. We included patients who complained of SSNHL, either idiopathic (30 patients) or labyrinthine signal alterations (14 patients), as diagnosed by MRI performed 15 days after the SSNHL onset. In addition, all patients underwent a course of intratympanic prednisolone injections., Results: 83.3% of the idiopathic group showed a complete or marked improvement after the intratympanic injection. Conversely, most cases of positive MR signal alterations (92.8%) had slight or poor improvement after the therapeutic course., Conclusions: Our study revealed that MRI imaging is essential for assessing any SSNHL case. It can diagnose labyrinthine hemorrhage, whose course and prognosis differ significantly from idiopathic SSNHL., Significance: Intratympanic prednisolone injection was effective in managing idiopathic SSNHL. On the other hand, this therapeutic modality was ineffective in improving SSNHL associated with labyrinthine hemorrhage.

Background: There have been few studies to date exploring prognostic outcomes associated with idiopathic sudden sensorineural hearing loss (ISSNHL) in order adults., Objective: This investigation was designed to explore the relationship between atherosclerosis-related risk factors and ISSNHL outcomes among older individuals., Material and Methods: In total, 172 older adults diagnosed ISSNHL from 2016 to 2021 were retrospectively evaluated to compare demographic and clinical test results., Results: Relative to healthy controls, ISSNHL patients exhibited significant differences in hypertension incidence and factors related to coagulation. With respect to prognosis, age, onset days, hypertension, the degree of hearing loss, type of hearing curve, fibrinogen and D-dimer levels were significant univariate prognostic factors, whereas multivariate logistic analysis showed that hypertension ( p  = .005) and D-dimer concentration ( p  = .000) were related to the treatment outcome of older ISSNHL patients. The area under the curve (AUC) for D-dimer levels was 0.795 (95% CI: 0.724-0.866). When using a D-dimer cut-off threshold value of 107.5 ng/mL, the sensitivity and specificity values of 77.0% and 76.7%., Conclusions: The present results indicate that hypertension incidence and D-dimer levels may be presented as an important prognostic indicator in older affected ISSNHL individuals.

Background: Zika virus (ZIKV) infection can result in hearing loss in babies, consequently, audiological monitoring is necessary., Aims: This study aimed to evaluate the frequency of hearing impairment in neonates and children exposed to ZIKV during the intrauterine period., Materials and Methods: A cohort of 30 children born to mothers infected with ZIKV during pregnancy (March 2016-January 2017) underwent repeated hearing assessments performed 48 h after birth. Universal Newborn Hearing Screening revealed normal results in all children at 6, 13, 24, and 36 months. Children were divided into two subgroups based on real-time polymerase chain reaction: RT-PCR(+) and RT-PCR(-)., Results: At 24 months, the cumulative incidence of hearing alteration was 57.1%. There was no significant difference in the detection of hearing alteration between RT-PCR(+) and (-) groups. None of the children had sensorineural hearing loss., Conclusions and Significance: None of the children had sensorineural hearing loss. Total incidence conductive type (per 1000 live births), RT-PCR ZIKV (-) 2.2, prevalence 20% and RT-PCR ZIKV 3.1, prevalence 35.7%.The incidence of hearing alteration was highest at 24 months of age (57.1%, n  = 8; only conductive type).

Background: Presbycusis is bilateral sensorineural hearing loss associated with the progressive degeneration of cochlear and central auditory pathways with aging., Aims/objectives: We aimed to reveal age-related changes in middle ear function by using wideband tympanometry (WBT)., Materials and Methods: Fifty-eight patients diagnosed with presbycusis were compared to 52 audiologically healthy participants. WBT measurement was performed on both ears via wideband click stimulus with a tympanometer device using probe tone frequencies of 226-8000 Hz., Results: There were no statistically significant differences detected among the resonance frequencies or maximum absorbance ratios measured in both ears between groups ( p  > .05). The mean absorbance of the right and left ears at 4000 and 8000 Hz was statistically higher in the patient group than in the healthy controls ( r  = 0.038, 0.030; l  = 0.015, 0.012). Moreover, mean compliance values were found to be significantly lower in the patient group than in the control group ( r  = 0.030 and l  = 0.040)., Conclusion: The significant differences detected in compliance and absorbance values for high frequencies in presbycusis patients were remarkable. Thus, it has been shown that WBT yields an advantage compared to traditional tympanometry in the diagnosis and treatment of middle ear diseases.

Background: Sudden sensorineural hearing loss (SSNHL) has several aetiologies and may be a presenting symptom of vestibular schwannoma (VS)., Objectives: The aim of this study was to investigate the recovery rate after steroid treatment and the prognostic factors of SSNHL with VS., Materials and Methods: This was a retrospective observational study wherein 32 patients with VS who presented with SSNHL were analysed at a tertiary referral centre. Hearing gain and prognostic factors were the main outcome measures for steroid treatment intervention., Results: Among the 1698 patients presenting with SSNHL, VS was found in 43 (2.5%) patients. Eleven cases (34.3%) showed good recovery, with significant improvements in the pure-tone audiometry values. Even though age was a significant factor, there were no associations between steroid response and initial hearing level, presence of vertigo, tumour size, and tumour extension., Conclusions and Significance: Our study showed that hearing recovery of SSNHL does not exclude a VS diagnosis. We suggest that steroid treatment be considered in patients with VS presenting SSNHL.

Background: Cochlear ossification (CO) after meningitis can cause profound sensorineural hearing loss (SNHL). Cochlear implantation (CI) is the ideal treatment strategy for CO., Aims: To explore the strategy for CI in patients with CO after meningitis., Materials and Methods: In this retrospective study, the medical records of patients diagnosed with profound SNHL due to CO after meningitis and who underwent CI in our department between September 2010 and September 2021 were collected and reviewed. Their imaging and surgical findings were analyzed., Results: The data of 26 patients with unilateral CI were reviewed. All patients underwent preoperative temporal high-resolution computed tomography (HRCT) and 22 patients magnetic resonance imaging (MRI). The sensitivity of HRCT was 61.5% (10/26), whereas that of MRI was 81.8% (18/22). Combined HRCT and MRI achieved a detection rate of 92.3% (24/26). Twenty-two and four patients underwent complete and partial electrode implantations, respectively., Conclusions and Significance: Preoperative temporal bone HRCT and MRI are essential for determining whether a patient is suitable for CI and surgical planning. A false-negative diagnosis is possible when diagnosing CO, but combined HRCT and MRI can improve the positive rate of preoperative diagnosis of CO post meningitis. Early CI is recommended.

Background: Several studies have reported sudden sensorineural hearing loss (SHL) as an initial symptom of acoustic neuromas (ANs)., Aims/objectives: We aimed to retrospectively evaluate the incidence of SHL and the associated rates of post-treatment improvement in patients diagnosed with ANs., Materials and Methods: We analysed the clinical data of 686 patients with ANs and examined tumour size, correlation with hearing loss, number of episodes of SHL, extent of auditory recovery, and associated audiogram patterns., Results: Among 686 enrolled patients with ANs, 232 (86 with intracanalicular and 146 with extrameatal tumours) experienced SHL at some point in their clinical history. The incidence of SHL was not significantly associated with tumour size. Of the 172 patients analysed, 119, 44, and 9 patients experienced one, two, or three or more episodes of SHL, respectively. Confirmed auditory recovery occurred in 61%, 45.3%, and 33.3% of the patients after the first, second, or third or later episodes of SHL, respectively. The audiograms of 78/172 (45.3%) patients showed a typical trough-shaped pattern of hearing loss at medium-level frequencies., Conclusions and Significance: Among patients with ANs, 7.7% experienced two or more episodes of SHL. The recovery rate of SHL decreased with each successive occurrence.

Background: Understanding how sensorineural hearing loss (SNHL) impacts postural balance in patients is important, as postural balance predicts the risk of falls., Aims/objectives: We aimed to clarify the relationship between characteristics in the configuration of audiograms and static postural balance as measured by posturography., Materials and Methods: We evaluated 385 outpatients (mean [± standard deviation] age, 58.4 ± 18.4 years) with SNHL by audiometry and posturography. Data were analysed by multiple regression models with the outcome of postural sway area with eyes closed (PSA) and predictive variables of audiometric data, adjusted for sex, age and the presence of nystagmus., Results: The increased hearing threshold in the better hearing ear was associated with poorer or higher PSA (beta coefficient [ β ] = 0.39, 95% confidence interval [CI] = 0.03-0.75, per 10-dB increment). No difference in PSA was detected between patients with asymmetric or symmetric SNHL. None of the frequent diagnoses (presbyacusis, Meniere's disease, and idiopathic sudden SNHL) were associated with poorer PSA. Hearing thresholds at middle ( β  = 0.39, 95%CI = 0.10-0.67) and high frequencies ( β  = 0.31, 95%CI = 0.07-0.55) were associated with poorer PSA, whereas those at low frequencies was not., Conclusions and Significance: Postural balance in neurotologic patients may be associated with middle-high-frequency hearing levels during ageing.

Background: Sensorineural hearing loss in child has a profound impact on their neurodevelopment and on language acquisition. Thus, the diagnosis and early intervention are critical, namely in unilateral or bilateral asymmetric hearing losses, which are diagnosed later than symmetric bilateral hearing losses., Objectives: This study aims to examine the progression of asymmetric SNHL in children, and the link between hearing thresholds and the technological level of the hearing aid fitted., Material and Methods: Eighteen children with asymmetric SNHL were submitted to a pure tone audiogram at the age of 5 years (T0) and reassessed at the age of 10 years (T1). Children were divided according to the technological level of the hearing aid fitted: basic, medium, and advanced., Results: A positive correlation was established between the hearing thresholds at T0 and T1, revealing an accelerated progression of hearing thresholds in the better ear after a 5-year period., Conclusions: Hearing thresholds in the worse ear influence the progression of the SNHL. Moreover, technologically advanced hearing aids led to a slower progression of the hearing thresholds over time.

Background: Intratympanic dexamethasone is commonly conducted to treat refractory sudden sensorineural hearing loss (RSSNHL). However, no consensus has been reached on its effectiveness., Objectives: The study aimed to evaluate the effectiveness of otoendoscope-assisted salvage intratympanic dexamethasone treatment (IDT) on RSSNHL with different audiogram patterns after failure of initial therapy., Material and Methods: A total of 108 patients with unilateral RSSNHL were classified into 4 groups according to audiogram patterns. Hearing results were evaluated by pure-tone audiometry (PTA), which was performed at baseline and one month after otoendoscope-assisted salvage IDT. The effectiveness of otoendoscope-assisted salvage IDT was assessed in each group., Results: The efficiency in low-frequency, high-frequency, flat, and deaf group was 48%, 24.1%, 46.2%, 17.9%, respectively. The efficacy did not differ between the high-frequency and deaf group. Notably, the efficacy in the low-frequency and flat group was significantly higher than that in the deaf group., Conclusions: Otoendoscope-assisted salvage IDT is a safe and effective treatment for RSSNHL. This treatment provided better results for patients with low-frequency damaged and flat curve audiogram than patients with other audiogram patterns., Significance: Audiogram patterns should be considered in the clinical management of patients with RSSHNL prior to salvage IDT.

Background: Recent guidelines encourage the use of Webet test (WT) as a part of the physical examination of a newly suspected sudden sensorineural hearing loss (SSNHL) patient. However, the most sensitive tuning-fork frequency has never been identified., Objective: To identify the most sensitive frequency for initial WT of patients with suspected SSNHL., Methods: Medical records of patients with confirmed SSNHL, who underwent formal audiometry in which the WT was carried out with different frequencies were analyzed., Results: 319 medical records were identified. The most sensitive WT frequency was 500 Hz, with a sensitivity of 94.49% (223/236. confidence interval 90.76-97.03). There was a non-significant difference between 1000 Hz EBO and 500 Hz EBO ( p  = .1655), a tendency towards a significant difference between 1000 Hz EBO and 2000 Hz EBO ( p  = .0578), and a significantly better sensitivity than the 4000 Hz EBO ( p  = .0163)., Conclusion: The most sensitive WT frequency for SSNHL diagnosis is 500 Hz. However, the sensitivity of this frequency is 94.49%., Significance: 512 or 1024 Hz should be used to better identified SSNHL. Even WT lateralization to the affected ear, does not preclude the diagnosis of SSNHL. Formal audiometry should be used in any case of medical history suspected for sudden hearing loss with normal otoscopy.

Background: High signals in the inner ear of idiopathic sudden sensorineural hearing loss (ISSNHL) on magnetic resonance imaging (MRI) have been reported, but no quantitative evaluation has yet been done., Objectives: To evaluate hearing outcomes and cochlear signal intensities on 3-T heavily T 2 -weighted three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging (hT 2 W-3D-FLAIR) in patients with ISSNHL., Materials and Methods: Twenty-nine patients with ISSNHL were included. Patients underwent hT 2 W-3D-FLAIR with intravenous gadolinium injection and pure tone audiometry (PTA) at initial visits and 3 months later. Signal intensity ratios (SIRs) were measured in the basal or apical-middle turns of the affected cochlea. A statistical analysis of relationships between SIRs and the average hearing levels (HLs) at low (125, 250, and 500 Hz) and high (2, 4, and 8 kHz) tone frequencies was performed., Results: Hearing improvements at high-tone frequencies in ears with HLs ≥60 dB were significantly worse in those with high SIRs at the basal turns on pre-contrast images. Similarly, hearing improvements at low-tone frequencies in ears with HLs ≥60 dB were significantly worse in those with high SIRs at the apical-middle turns on post-contrast images., Conclusions and Significance: High SIRs on hT 2 W-3D-FLAIR indicate cochlear disturbances with severe ISSNHL and could provide lesion-specific prognostic information.

Background: Early detection of inner ear cell damage can reduce the chances of permanent damage to hearing ability. However, current inner ear cell damage detection methods can detect damage only after the patient has lost hearing ability. MicroRNA expression levels in circulating systems are affected in diseases or conditions arising from the distant lesions. Therefore, detection of circulating microRNA expression levels could be one of the best ways to obtain information on inaccessible lesion sites., Aims/objectives: This study aims to establish a method for monitoring idiopathic sudden sensorineural hearing loss (ISSNHL) by analyzing circulating microRNA expression levels. 21 ISSNHL patients and 24 healthy controls were enrolled., Material and Methods: Real-time quantitative polymerase chain reaction was performed for detecting expression levels of circulating microRNAs., Results: Among eight circulating microRNAs, expression levels of five circulating microRNAs significantly differed between ISSNHL patients and healthy controls. circulating microRNA expression levels correlates with treatment outcomes and hearing ability., Conclusions and Significance: Using methods combining the evaluation of miR-183, miR-210, miR-18b, and miR-23a cut-off values identified in ISSNHL patients and healthy controls during receiver operating characteristic curve analysis, sensitivity and specificity of 80.95% (17/21) and 87.50% (21/24) were obtained, respectively.

Background: To date, there have been few conventional algorithms for the treatment of idiopathic sudden sensorineural hearing loss (SSNHL), as there have been only limited reports with high evidence levels. Objectives: To propose an evidence- and trend-based treatment algorithm for SSNHL. Methods: We referred not only to the evidence for each treatment, but also to trends related to treatment selection in Japan based on epidemiologic surveys, and considered the balance of the advantages and disadvantages with regard to each patient's condition. Results: We propose an algorithm that begins with the grade of SSNHL severity as the prognosis of SSNHL is strongly related to the severity of hearing loss. We selected systemic corticosteroid therapy as the first-line therapy, and Intra-tympanic corticosteroid therapy as salvage therapy. We also proposed the use of prostaglandin E1 with corticosteroids for the treatment of SSNHL patients with severe hearing loss. According to the data obtained from an epidemiologic survey, we decided time limits for the application of each treatment. Conclusion: An algorithm for the treatment for SSNHL is presented according to the results of epidemiologic surveys in Japan. It is expected that this algorithm can provide a guide to choosing the suitable treatment for SSNHL patients.

Background: Serum thyroid hormone levels are closely related to the normal functioning of the cochlea. However, the relationship between initial auditory brainstem response (ABR) results and levels of thyroid hormone remained unclear until we adopted ABR as a prognostic factor in Idiopathic sudden sensorineural hearing loss (ISSNHL) patients. Objective: This investigation aimed to elucidate the association between ABR and outcomes in patients with ISSNHL. Material and methods: Thirty-three patients presenting with unilateral ISSNHL underwent blood sampling and ABR tests on the day of admission. The mean latencies of the ABR results were compared among the groups which were classified by ISSNHL outcome, based on Siegel's criteria. The association between the ABR results and the thyroid hormone serum levels (TSH, T3, and free T4) were assessed. Results: The overall successful recovery rate was 60.6% ( n  = 20). The mean latencies of all the ABR parameters were not significantly different between the different treatment outcome groups (Mann-Whitney U test). Wave V latency, III-V interval and I-V interval were negatively associated with T3 serum levels. Conclusion: The results indicate that clinical caution should be exercised when conducting ABR tests without assessing thyroid hormone levels.

Background: Few studies focused on the prognosis of sudden sensorineural hearing loss (ISSHL) of aged people. Objectives: The aim of this study is to analyze the characteristics, treatment, and prognostic factors of ISSHL in aged people. Material and methods: A total of 278 patients diagnosed of ISSHL in aged people from 2014 to 2019 were retrospectively analyzed. Univariates were analyzed by univariate and multivariate logistic analysis. Results: Among the 13 univariates, the patients' age was younger in the overall recovery group ORG ( p  = .018), while onset days was shorter in ORG ( p  = .000). The percentage of DM and HTN comorbidities were higher in ORG ( p  = .026 and .038). Meanwhile differences were significant in audiogram configurations ( p  = .037), the degree of hearing loss ( p  = .033), and types of lipid treatment ( p  = .020). Then these seven independent risk factors were included in the multivariate analysis, final results indicated that hypertension ( p  = .028), lipid control groups ( p  = .009), age ( p  = .000), and onset days ( p  = .001) were related to the treatment outcome of ISSHL. Conclusions: The prognosis of ISSHL in aged patients was closely related to age, the onset days of treatment, and good control of complications such as hypertension and hyperlipidemia, so vascular factors were considered as the main causes of morbidity.

Objective: This study aimed to explore clinical characteristics and prognosis of elderly cases with sudden sensorineural hearing loss. Methods: Fifty-five elderly cases with sudden sensorineural hearing loss and 55 younger cases with sudden sensorineural hearing loss were enrolled in the study. Recovery rates of hearing between elderly group and younger group were compared. In elderly group, the recovery rate of hearing in the patients with normal hearing of the contralateral ear was compared to those with hearing loss of the contralateral ear. Results: The overall recovery rate in the elderly group was 50.9%, significantly lower than 74.5% in the younger group ( p  < .01). Among the elderly group, the patients with normal hearing of the contralateral ear recovered better than those with hearing loss of the contralateral ear (recovery rates, 76.7% versus 20.0%). Among the elderly group, the recovery rate of cases with treatment onset ≤14 days was 64.7%, while the recovery rate of those with treatment onset longer than 14 days was only 28.6%, with significant difference ( p  < .01). Conclusion: Elderly cases with sudden sensorineural hearing loss had poor prognosis. Prognosis of elderly cases with sudden sensorineural hearing loss was associated with hearing of the contralateral ear and treatment onset.

Background: Idiopathic sudden sensorineural hearing loss (ISSNHL) requires early treatment. Objective: To describe our experience on intratympanic steroid treatment (ITS) of ISSNHL analyzing the delay to start therapy as prognostic factor. Material and methods: We perform a retrospective study on ISSNHL treated with systemic steroids without full recovery on PTA (pure tone average) according to Siegel criteria. They were divided into two different groups: one group that additionally received ITS as combined therapy (treatment group), and another without it (control group). We analyzed the hearing recovery at 6 months and 2 years, and the influence of the delay to start ITS in the recovery. Results: After ITS was added, further complete recovery was achieved in 10 patients of the treatment group. After 6 months, PTA improvement in the treatment group was 10.84 dB, compared to 1.13 dB in the control group ( p <.0001). Nevertheless, patients starting such combination of oral steroids and ITS within 8 days of diagnosis had an additional gain of 15 dB in the first 6 months, that increased to 19.17 dB after 24 months ( p <.022). Conclusions: When ITS was added within the first 8 days, a significantly better and more stable response was obtained.

Background: In Sweden, an estimated prevalence of adult patients with severe-to-profound hearing loss is 0.2%, which corresponds to roughly 20,000. We know little about the use of cochlear implants (CIs) in this population and why not most of them are not offered CI. Objectives: To investigate the reasons for no rehabilitation with CI among this patient group. Materials and methods: Data were collected from 1076 patients in the Swedish Quality Register of Otorhinolaryngology. A baseline questionnaire and the reason for no CI, was evaluated. Results: Only 14.5% of the patients started a CI investigation, and 8.5% were rehabilitated with CI. Significantly more women (56.5%) than men received CI. The most common reasons for not receiving CI, were hearing reason (30.5%), indicating satisfaction with technical equipment, and unknown reason (25%). The oldest patient group (81-100 years old) had the highest risk for unknown reasons . Patients receiving extended audiological rehabilitation (53.5%) had a significantly lower risk for unknown reasons. Conclusions: It is worrying that the oldest patient group (81-100 years old) seemed to have fewer chances to start a CI investigation. An extended audiological rehabilitation increased the chances that professionals would discuss CI. Significance: This study shows that surprisingly few patients are offered CI despite their severe-to-profound hearing loss.

Objectives: This study was designed to evaluate the relationship between the degree of round window membrane (RWM) exposure and hearing outcome. Materials and methods: Forty-six ears with cochlear implantation (CI) were enrolled. The degree of RWM exposure was divided into Grade I (<25%), Grade II (25-50%), and Grade III (>50%). The hearing outcomes were evaluated at 1.5 and 12 months postoperatively. Results: Twenty-seven ears were Grade I, 13 were Grade II, and 6 were Grade III. RW approach was used in all ears of Grades II and III and 20 ears of Grade I and cochleostomy was used in 7 ears of Grade I. The pattern of bony overhang was multidirectional in 41 ears. Threshold shift significantly decreased proportional to the increase of RWM exposure after CI. The mean RWM exposure was 32.1 ± 24.4% in ears with more than partial preservation ( n  = 17), and 13.3 ± 11.7% in the other ears ( n  = 6) at 12 months post-CI ( p  = .061). Age at CI differed significantly between ears that had more than partial preservation and the other ears at 1.5 months post-CI. Conclusions and significance: Degree of RWM exposure and age at CI might be factors predicting hearing outcome after CI using the RW approach.

Background: Intratympanic corticosteroid (IC), intravenous batroxobin (IB) as the treatment for sudden sensorineural hearing loss (SSNHL) has been reported. However, the data on combination therapy (CT) was scarce., Objective: The aim of this retrospective study was to compare the efficacy of IC, IB, and CT in the treatment of SSNHL with diabetes., Material and Methods: A total of 212 SSNHL patients with diabetes, who were initially treated within 14 days of onset of disease, were divided into three groups by treatment modality. The hearing recovery was evaluated by the results of pure-tone test after completion of treatment. The prognostic factors, including age, severity of initial hearing loss, duration to onset of treatment, and audiometric curve type, were further compared., Results: There was a significant difference in hearing recovery by the treatment (p < .05). Recovery rates in the CT group were significantly higher in patients with early treatment than with delayed treatment (p = .021). However, duration and recovery rate was not significantly correlated in IC and IB group (p > .05). In patients recieving early treatment, the recovery rate in CT group was significantly higher than that in IC (p = .013) and IB group (p = .029). Regardless of treatment, the recovery rates were higher in patients with flat and ascending audiograms (p < .05)., Conclusions and Significance: Patients receiving combined therapy, especially in the early stage of SSNHL, could achieve significantly superior recovery in the treatment of SSNHL with diabetes, compared with those using IC or IB alone.

Background: Recent studies of cochlear implants (CIs) in profound unilateral hearing loss (UHL) patients have demonstrated a restoration of some binaural hearing., Aims/objectives: The objective was to evaluate three possible advantages of binaural hearing in CIs adult users with UHL including single-side deafness (SSD) and asymmetric hearing loss (AHL) subgroups., Material and Methods: A prospective study was conducted that included 70 sequentially implanted patients. Subgroups of these subjects included 64 with a postlingual onset of a profound hearing loss on the implanted side and 6 with a prelingual onset of that loss. Three binaural effects - redundancy, head shadow, and squelch - were evaluated., Results: Significant differences between the 'CI on' and 'CI off' conditions were found for all three binaural effects for the study group as a whole and for the postlingual subgroup. However, results for the subjects in the prelingual subgroup did not demonstrate any of the binaural advantages., Conclusion and Significance: Patients with a postlingual onset of a profound hearing loss in one ear and normal hearing or only a moderate loss in the other ear are able to make the effective use of a CI in the profound-loss ear in conjunction with acoustic stimulation of the other ear.

Background: Sudden sensorineural hearing loss (SSNHL) is commonly encountered in clinical practice., Aim/objective: Determine if local administration of corticosteroids to the inner ear can improve hearing and speech intelligibility after the failure of conventional treatment for SSNHL loss when administered for 10 days after the onset of the hearing loss in a large cohort of 77 patients., Materials and Methods: A Silverstein MicroWick™ was placed under local anesthesia and endoscopic control in the round window niche, allowing self-administration of methylprednisolone twice daily for four weeks., Results: An improvement of the pure tone average was shown in 31% of patients. Speech intelligibility improved significantly in 55% of the total cohort and in 34% of the population with a stable pure tone average. Among the 77 patients, 22% used a hearing aid. Only 14% of the patients were hearing-aid users in the group with an improvement in speech intelligibility as opposed to 31% in the failure group., Conclusion and Significance: Local administration of steroids to the inner ear through the round window route improves hearing and speech intelligibility in patients after failure of conventional therapy. The use of a hearing aid was reduced by 50% when speech intelligibility was improved.

Background: Many adults with moderate-profound hearing loss whose speech recognition has deteriorated and are no longer benefitting from hearing aids (HAs) could benefit from cochlear implantation (CI). Of these, only <5% are implanted. In order to inform eligible patients about expected results and ease the route to implantation, better guidelines for candidate selection are needed., Objectives: To provide reliable guidelines by determining, in a well-characterized group of implantees, the minimal expected post-CI scores for monosyllabic (MS) word recognition., Patients and Methods: In total, 20 adults unilateral implantees considered (prior to implantation) straightforward CI candidates were assessed ≥20 months post-CI. Their post-CI CI-aided thresholds and MS word recognition were compared to their pre-CI scores. In addition, SSQ12 scores were evaluated post-CI., Results: Post-CI, thresholds were 40 dB or better in all participants, and 35 dB or better in 95% of them. 90% scored 60% or better on MS words in quiet. In speech noise all scored 15-60% and 50% scored 45-60%. Poorer unaided pre-CI thresholds yielded greater patient satisfaction., Conclusions: Adults with moderate-profound hearing loss, whose speech recognition has deteriorated, are no longer benefitting from HAs and fulfil defined criteria for straightforward CI, should be referred for formal CI candidacy evaluation.

Background: Efficacy of current treatment methods in idiopathic sudden sensorineural hearing loss (ISSNHL) is still unsatisfactory., Objective: This study aimed to discover in differences in effect between steroid applications responsible for promoting the prognosis in ISSNHL., Materials and Methods: A study was conducted to diagnose ISSNHL patients in our hospital from January 2014 to September 2016. All patients accepted treatments including intravenous injection (intravenous dexamethasone, [IV DXM]), intratympanic injection (intratympanic methylprednisolone [IT MP], intratympanic dexamethasone [IT DXM]) or combined injections with steroids (IV + IT DXM). Patients were divided into groups according to treatment outcomes and clinical characteristics of each group were compared for univariate comparison. Logistic regression was utilized to verify screening factors from univariate comparison for exclude biases., Results: There were 313 patients with ISSNHL enrolled in the study. Logistic regression verified that vertigo (p = .023), severity of hearing loss (p=.969), pattern of hearing loss (p = .03), and the treatment method (p < .001) were statistically related to the patients' prognosis based on the condition all biases had been excluded as possible. IT MP showed a better prognosis of hearing improvement compared to treatment with IT DXM (OR = 0.5), IV DXM (OR =0.226), and IV DXM + IV DXM (OR = 0.320)., Conclusions and Significance: IT MP treatment could be utilized as initial treatment in ISSNHL and might promote outcomes.

Background: Safe cochlear implantation (CI) is challenging in patients with a canal wall down (CWD) mastoidectomy cavity., Objectives: We reviewed the outcomes of CI and proposed surgical management principles according to the presentation status of CWD mastoidectomy cavity., Material and Methods: The cases of eight patients (nine ears) with CWD mastoidectomy cavity who underwent CI were retrospectively reviewed. The basis of the surgical decision, postoperative complications, and postimplant auditory performance were analysed., Results: In seven patients (eight ears), implantation was performed in a single stage; in six ears, the external auditory canal (EAC) was oversewn. In two patients with ossification, the electrode array was inserted into the scala tympani by drilling of the basal turn or in the second turn of the cochlea through a drill-out procedure. Seven patients had a follow-up of 12-50 months and one patient was lost to follow-up. None of the followed-up seven patients suffered complications., Conclusions: CI is safe and effective in patients with profound hearing loss after radical mastoidectomy. In patients with CWD mastoidectomy cavity, CI does not cause a higher rate of postoperative complications relative to standard CI procedures. The outcome is excellent and comparable to that in the general CI population.

Background: Idiopathic sudden sensorineural hearing loss (ISSNHL) is defined as a decline in hearing affecting three or more frequencies by 30 dB Objective: The aim of this study was to evaluate the results of intratympanic steroids as a salvage treatment for severe ISSNHL., Materials and Methods: A regimen of three IT steroid injections was offered to patients who failed a 7-days intravenous steroid treatment. Eighty-four patients underwent IT salvage treatment (IT group). Their outcomes were compared with those of 255 patients with severe ISSNHL who received the same intravenous steroid regimen without salvage IT steroid therapy (Control group)., Results: 56% of the patients in the IT group had a hearing improvement of >15 dB after one month. The average hearing improvements were 26.5 ± 28 dB and 27.9 ± 24 dB in the IT group and the Control group, respectively (p = .67). However, patients with a type E audiogram pattern (total deafness), displayed a substantial hearing gain., Conclusion: Intratympanic steroids failed to show a global auditory benefit as a salvage treatment in patients with severe ISSNHL., Significance: Our data suggest that a salvage treatment with intratympanic dexamethasone may be offered to patients with total deafness for whom the first systemic treatment has failed.

Conclusion: The auditory brainstem response (ABR) wave I threshold, latency and amplitude are insensitive to spiral ganglion neurons (SGNs) degeneration, but are sensitive to the degeneration of Schwann cells and can estimate the status of Schwann cells in a neural degeneration mouse model. The thorough pre-operative ABR assessment would be helpful in predicting cochlear implant performance., Objectives: This study aimed in finding a non-invasive electrophysiological method to evaluate the status of the auditory nerve and the Schwann cells in sensorineural hearing loss (SNHL) and auditory neuropathy (AN) ears, and providing useful information for candidates screening and outcome prediction in cochlear implantation., Methods: The frequency-specific acoustic ABR was recorded in mice. The immunohistochemical staining was performed to detect the SGNs and Schwann cells in mice cochlea. The correlations between ABR wave I metrics and SGNs, Schwann cells were investigated., Results: In SNHL and AN mice cochlea, statistically significant correlations between ABR wave I thresholds, latencies and amplitudes at 8, 16, and 32 kHz and their corresponding SGNs densities were found only in wave I amplitude at 8 kHz. While the ABR wave I metrics at all three frequencies showed strong significant correlations with their corresponding Schwann cells densities.

Objectives: The purpose of this study was to determine the prognostic potential of admission of a patient for idiopathic sudden sensorineural hearing loss (ISSNHL)., Study Design: Retrospective clinical study., Methods: We conducted a retrospective study of 301 patients with ISSNHL. The patients were divided into a study (57 patients at out-patients department (OPD) based treatment, using systemic steroids and intra-tympanic (IT) steroid injection) and a control groups (301 patients at admission, using systemic steroids and IT steroid injection). We compared the success rate (15 dB cut off), Siegel's criteria and hearing gain decibels between two groups., Results: The overall recovery rate of the OPD based treatment group (29.8%) was poor than that of the admission group (52.9%) (p = .002). The odd ratio of OPD based treatment factor was 2.035 when the OPD based treatment and poor prognostic factors were analyzed at logistic regression test (Duration of delayed treatment, 1.073; average hearing loss level, 0.972) (p = .041)., Conclusion: Admission must be strongly recommended when the patients with ISSNHL asked about the necessity of admission. We hypothesized that resting, getting out of their social stress, and relief of anxiety might be helpful to their hearing recoveries.

Objective: To clarify how the pure-tone threshold (PTT) on the PTA predicts speech perception (SP) in elderly Japanese persons., Methods: Data on PTT and SP were cross-sectionally analyzed in Japanese persons (656 ears in 353 patients, aged ≥65 years). Correlations of SP and average PTT in all tested frequencies were evaluated by Pearson's correlation coefficient and simple linear regression. After adjusting for sex, laterality of ears, and age, the relationship of average and frequency-specific PTT with impaired SP ≤50% was estimated by logistic regression models., Results: SP correlated well (r = -0.699) with the average PTT of all tested frequencies. On the other hand, the correlation between patient age and SP was weak, especially among ≤85-year-old persons (r = -0.092). Linear regression showed that the average PTT corresponding to SP of 50% was 76.4 dB nHL. Odds ratios for impaired SP were highest for PTT at 2000 Hz. Odds ratios were higher for middle (500, 1000, 2000 Hz) and high frequencies (4000, 8000 Hz) than low frequencies (125, 250 Hz)., Conclusion: The PTT on the pure-tone audiogram (PTA) is a good predictor of SP by speech audiometry among older persons, which could provide clinically important information for hearing aid fitting and cochlear implantation.

Conclusion: Alterations within a novel putative Exon 1a within the gap junction beta 2 (GJB2) gene may play a role in the development of genetic hearing impairment in Austria., Objectives: Mutations in the GJB2 gene are the most common cause of hereditary sensorineural deafness. Genome-wide screening for alternative transcriptional start sites in the human genome has revealed the presence of an additional GJB2 exon (E1a). This study tested the hypothesis of whether alternative GJB2 transcription involving E1a may play a role in the development of congenital sensorineural deafness in Austria., Methods: GJB2 E1a and flanking regions were sequenced in randomized normal hearing control subjects and three different patient groups with non-syndromic hearing impairment (NSHI), and bioinformatic analysis was performed. Statistical analysis of disease association was carried out using the Cochran-Armitage test for trend., Results: A single change 2410 bp proximal to the translational start site (c.-2410T > C, rs7994748, NM&#95;004004.5:c.-23 + 792T > C) was found to be significantly associated with the common c.35delG GJB2 mutation (p = .009). c.35delG in combination with c.-2410CC occurred at a 6.9-fold increased frequency compared to the control group. Additionally, one patient with idiopathic congenital hearing loss was found to be homozygous c.-2410CC.

Objective: To investigate whether genetic polymorphisms (single-nucleotide polymorphism [SNPs]) have a prognostic influence on hearing recovery after standardized corticosteroid therapy., Methods: A total of 192 gene samples from idiopathic sudden sensorineural hearing loss (SSNHL) patients registered in the Intractable Inner Ear Disease Gene Bank were enrolled and, as the candidate genes, 16 SNPs from 13 genes were selected for this study. Fischer's exact test was used to compare allele frequencies in each SNP between the patients with good hearing recovery and patients with poor hearing recovery., Results: The SNPs of the GSR gene (rs2251780 and rs3779647) and NOS3 gene (rs1799983) were associated with treatment outcome (p < .05). Furthermore, there was a marginal correlation between the SNP of the NR3C1 gene (rs4912910) and treatment outcome (p = .05)., Conclusions: The results of this study indicate that the analysis of genetic factors might make it possible to predict the treatment outcome, at least in part, in patients with idiopathic SSNHL.

Objectives: Using a large-scale nationwide survey database, we investigated the epidemiological characteristics for idiopathic SSNHL in Japan., Methods: The subjects for this analysis were patients registered in a Japanese multicentre database between April 2014 and March 2016. A total of 3419 idiopathic SSNHL patients were registered in the database, and the clinical characteristics of the idiopathic SSNHL patients were obtained. Several factors associated with the severity of hearing impairment and prognosis were then investigated. Statistical analysis was performed to clarify the factors associated with the severity of hearing impairment and prognosis., Results: There were significant correlations between the severity of hearing loss and diabetes mellitus, kidney disease, past history of brain infarction, heart disease, age (under 16 years/elderly), and symptoms of vertigo/dizziness. We also analyzed the prognostic factors for idiopathic SSNHL, and found that the severity of hearing loss (Grade 3 or 4), heart disease, aged 65 years or over, time from onset to treatment (over 7 days), and symptoms of vertigo/dizziness were all significantly related to poor prognosis., Conclusion: The present large-scale clinical survey revealed current epidemiological trends for idiopathic sudden sensorineural hearing loss (SSNHL) and various factors associated with the severity of hearing impairment and prognosis.

Objective: To investigate the hearing prognosis of idiopathic sudden sensorineural hearing loss (SSNHL) treated with different initial therapies., Methods: Subjects consisted of patients diagnosed with idiopathic SSNHL within 7 days from onset and showing severe hearing loss (≥60 dB), who were registered in a Japanese multicenter database between April 2014 and March 2016. Subjects were divided into four groups according to initial therapy: (1) steroids, (2) steroids + Prostaglandins (PGs), (3) intratympanic steroids (ITS), and (4) no steroids. Hearing outcomes were compared among the groups., Results: In total, 1305 patients were enrolled. The final hearing level and hearing gain of patients treated with steroids + PGs were significantly higher than those of patients treated with steroids alone or no steroids. The ratio of good prognosis (complete recovery or marked improvement) in patients treated with steroids + PGs was higher than that in patients treated with steroids alone or no steroids. There was no difference in the prognosis of patients treated with steroids alone or no steroids., Conclusion: A large number of patients with idiopathic SSNHL were registered in a multicenter database. PG use in combination with steroid administration was associated with a good hearing prognosis in patients with severe hearing loss.

Conclusion: Not all patients diagnosed with congenital infection using umbilical cord assay were found to be positive for CMV-DNA by perilymphatic fluid assay. In addition, a CMV-DNA-positive result was observed in one patient who had not been diagnosed with congenital infection. Sampling of perilymphatic fluid from a large population of patients with congenital SNHL caused by congenital CMV infection or of unknown etiology is required to determine the prevalence of CMV-related profound HL., Objectives: Sensorineural hearing loss (SNHL) is one of the most frequent manifestations in patients with congenital cytomegalovirus (CMV) infection. Using dried umbilical cord, a PCR-based assay was recently developed for the retrospective detection of congenital CMV infection. This study analyzed the presence of CMV in the perilymphatic fluid and evaluated differences in the effect of cochlear implantation between CMV-positive and -negative groups., Method: Perilymphatic fluid was collected from each patient at the time of cochlear implantation and analyzed for the presence of CMV using a PCR method., Results: The perilymphatic fluid in two of the five patients suffering from congenital CMV infection and in one of the 17 patients without congenital CMV infection was found to be positive for CMV.

Conclusion: Factors prognostic of recovery from low frequency sensorineural hearing loss without vertigo are co-occurrence of tinnitus, PTA determined hearing level on the affected side before treatment initiation, and time from onset to treatment initiation. The rate of hearing recovery was higher in the oral steroid than in the intra-tympanic steroid and combined groups., Objectives: Although many studies have assessed acute sudden hearing loss, few have analyzed low-frequency hearing loss. Clinical characteristics of patients with hearing loss may vary by type of hearing loss. This study, therefore, analyzed the clinical characteristics, recovery rates, and factors associated with hearing recovery in patients with low-frequency sensorineural hearing loss unaccompanied by vertigo., Method: This study included 50 patients with severe low frequency hearing loss unaccompanied by vertigo and with normal tympanic membrane status who visited hospitals from July 2005 to May 2014 due to sudden tinnitus, ear fullness, or hearing loss. Of these patients, 29 were treated with oral steroids, eight with tympanic steroid injections, and 13 with both. Clinical and auditory characteristics before and after treatment, as well as treatment outcomes, were compared in these three groups., Results: Age, sex, affected side, co-occurrence of ear fullness, and accompanying chronic diseases were similar in the three groups. All patients started on treatment within 10 days of hearing loss showed significant recovery, with complete recovery, unaccompanied by tinnitus, observed in 10 patients. Treatment outcomes were not affected by hearing thresholds on the unaffected side. Of the patients treated with both oral and intra-tympanic steroids, 39% showed complete recovery, and 77% showed audiometric improvement. The complete recovery rate was significantly higher in the oral steroid than in the other two groups (p = 0.029); and the audiometric improvement rate tended to be lower in the intra-tympanic steroid group than in the other groups. Cure rates, defined as complete disappearance of symptoms and normal findings on pure tone audiograms (PTA), in the oral, intra-tympanic, and combined steroid groups were 49%, 25%, and 23%, respectively. There were no significant differences among the three groups in cure, subjective improvement, and audiometric improvement rates. Factors prognostic of non-recovery of hearing included late treatment initiation (p = 0.044), accompanying tinnitus (p = 0.049), and higher hearing thresholds on the affected side before treatment initiation (p = 0.005).

Conclusions: Defibrinogenation therapy rather than corticosteroids therapy should be chosen for patients specifically with profound hearing loss and with initial high fibrinogen., Objectives: Corticosteroids therapy is the standard treatment for sudden sensorineural hearing loss (SSNHL) and prognostic factors by this therapy were reported. Defibrinogenation therapy is one of the treatment options for SSNHL. Aims of this study were to identify prognostic factors and correlative markers with hearing improvement in treating SSNHL by defibrinogenation therapy., Methods: During the early phase of the study, consecutive 61 patients were treated by defibrinogenation therapy with batroxobin (50 units), whereas corticosteroids (500 mg/day of hydrocortisone tapered by 9 days) were used for consecutive 64 patients during the late phase. Blood data that could predict a complete recovery were identified. Coagulation/fibrinolysis markers correlated with hearing improvement by defibrinogenation therapy were investigated., Results: Although there were no overall differences in hearing improvement between the two therapies, recovery rate in profound hearing loss patients was better in defibrinogenation therapy. In patients who showed complete recovery, serum fibrinogen level before treatment was significantly higher in the defibrinogenation group than the corticosteroid group. Responses of several fibrinolysis markers to defibrinogenation therapy evaluated by post-/pre-values were negatively correlated with hearing improvement.

Conclusion: The correlations between behavioral and auditory steady-state response (ASSR) thresholds were significant at 500, 1000, 2000, and 4000 Hz. ASSR presented high sensitivity and specificity in the detection of residual hearing in cochlear implant candidates when compared with warble-tone audiometry., Objectives: To assess residual hearing in cochlear implant candidates by comparing the electrophysiological thresholds obtained in dichotic single-frequency ASSR with behavioral thresholds at 500, 1000, 2000, and 4000 Hz., Methods: This was a comparative study between ASSR and warble-tone audiometry thresholds in 40 cochlear implant candidates (80 ears) before cochlear implantation with bilateral severe-to-profound sensorineural hearing loss., Results: Thresholds were obtained in 62.5% of all frequencies evaluated in warble-tone audiometry and in 63.1% in the ASSR. ASSR sensitivity was 96% and specificity was 91.6%. Mean differences between behavioral and ASSR thresholds did not reach significance at any frequencies. Strong correlations between behavioral and ASSR thresholds were observed in 500, 1000, and 2000 Hz and moderate in 4000 Hz, with correlation coefficients varying from 0.65 to 0.81. On 90% of occasions, ASSR thresholds were acquired within 10 dB of behavioral thresholds.

Conclusion: There is a correlation between the AN/AD disorder and the saccular dysfunction in children with severe sensorineural hearing loss, which means that saccular dysfunction can be a concomitant sign of AN/AD. In conclusion, the term of audio-vestibular dys-synchrony (AVS) is a more suitable description for this condition., Objectives: Patients with auditory neuropathy/auditory dys-synchrony (AN/AD) characteristically demonstrate poor neural responses from the vestibulocochlear nerve and brainstem while displaying evidence of intact outer hair cells function. Therefore, the objective of this study is studying of the relationship of the saccular dysfunction with AN/AD disorder in children with sensorineural hearing loss., Methods: In this cross-sectional study, 100 children with bilateral severe-to-profound sensorineural hearing losses underwent audiologic tests and cervical vestibular-evoked myogenic potentials (cVEMPs) at the Audiology Department of Hamadan University of Medical Sciences (Hamadan, Iran)., Results: Eleven children with bilateral severe sensorineural hearing loss were given to unilateral AN/AD disorder (11 ears), and two children (4 ears) had bilateral AN/AD (total = 13 children). The ears with AN/AD took the form of unrepeatable or absent waves of ABR and presence of OAEs. The statistical analysis of an independent t-test between AN/AD ears as compared to non-AN/AD ears of these 13 children showed that the mean latencies of p13 and the mean latencies of n23 and the mean peak-to-peak amplitude had significant differences.

Conclusion: As tools of confirmation of sensorineural hearing loss in neonates who are 'referred' from a newborn hearing screening program, both ASSR and DPOAE have high sensitivity and specificity. In addition, ASSR can be used as a substitute for ABR., Objectives: To analyze the confirmative audiological results of patients referred from a newborn hearing screening program., Patients and Method: From January 2007 to December 2013, hearing tests were performed on 474 babies (804 ears) who were 'referred' from the hospital or other maternity centers. Auditory brainstem response (ABR), auditory steady-state response (ASSR), and distortion product otoacoustic emissions (DPOAE) were used for hearing evaluation., Results: Of 474 babies (804 ears), 232 had normal hearing, while 242 babies (358 ears) had over 30 dB nHL threshold from ABR. Among the 156 babies (312 ears) who underwent both ABR and ASSR, the mean ASSR threshold had a strong correlation with ABR threshold (r = 0.942, p < 0.001). Assuming that ABR results were the yardstick of abnormal hearing, sensitivity and specificity of ASSR to ABR were 90.6% and 95%. DPOAE tests were performed on 180 babies (360 ears), with sensitivity of 85.9% and specificity of 84.4%.

Conclusion: Clinical features in the course of conversion differed between patients with SSNHL and cupulopathy, which indicates that the pathophysiology of persistent geotropic or apogeotropic DCPN and the mechanism of the change in nystagmus direction may differ between the two groups., Objective: The aim of this study is to investigate clinical characteristics of 10 patients with persistent DCPN who exhibited a conversion of nystagmus direction between geotropic and apogeotropic, and discuss possible mechanisms., Methods: Using video-oculography, serial examinations of nystagmus in a head-roll test were performed., Results: Of these 10 patients, five had sudden sensorineural hearing loss (SSNHL) and the remaining five had cupulopathy. In SSNHL, direction of nystagmus changed from geotropic to apogeotropic in three patients and from apogeotropic to geotropic in two patients. In cupulopathy, persistent apogeotropic DCPN always preceded persistent geotropic DCPN. The change in nystagmus direction occurred earlier in patients with cupulopathy (1 or 2 days after vertigo onset) than in patients with SSNHL (4-23 days after vertigo onset). While the null plane was consistently identified on one side, regardless of the nystagmus direction in cupulopathy, it was not always identified on the side of hearing loss in SSNHL.

Conclusion: Responses with greater amplitude were recorded when carrier frequencies were modulated at 37, 40, and 43 Hz. These responses can be recorded even in patients with significant sensorineural hearing loss, from the sternocleidomastoid (SCM) muscle for a 500 Hz tone, 100% modulated at 40 Hz., Objective: To determine the best carrier and modulating frequencies to evoke steady-state myogenic responses., Methods: The present study investigated 156 ears of 78 normal-hearing young adults, with carrier frequencies of 250, 500, and 1000 Hz, modulated at 20, 37, 40, 43, 70, 77, and 80 Hz, with an intensity of 95 dBA. Furthermore, we observed responses evoked by stimulus carrier frequency of 500 Hz, modulated at 40 Hz, with an intensity of 95 dBA in a group of five subjects with severe sensorineural loss., Results: Responses were found for all stimuli studied (p < 0.01). Modulated stimuli at frequencies of 37, 40, and 43 Hz evoked better steady-state vestibular evoked myogenic potential (S-VEMP) (p < 0.05). No statistically significant differences were found between the group of normal hearers and the group of subjects with hearing loss (p = 0.431), for the stimulus used.

Conclusion: The long-term follow-up study revealed a good audiovestibular prognosis in our patients with Vogt-Koyanagi-Harada disease (VKH) after adequate treatment., Objectives: To evaluate the audiovestibular findings and long-term prognosis of patients with VKH., Methods: A total of 82 ears in 41 patients (9 males and 32 females, aged 18-78 years) with VKH were examined in the audiovestibular study. Of the 41 patients, 16 patients with follow-up periods of more than 12 months (13-158 months) were included in the long-term follow-up study. Audiovestibular examinations included pure-tone audiometry; positional, positioning, and spontaneous nystagmus tests; and a caloric test., Results: Among the 41 patients, 28 (68%) complained of auditory symptoms at the initial visit and 28 (68%) showed sensorineural hearing loss (SNHL). Of the 24 patients who underwent nystagmus tests, only 3 (13%) presented with vestibular symptoms whereas 11 (46%) had nystagmus. Among 32 ears in 16 patients enrolled in the long-term follow-up study, 19 ears showed SNHL at the initial visit. The SNHL returned to normal in 14 ears (74%) and remained minimal in severity in the other 5 ears (26%) at the last visit. None of the patients showed persistent or repetitive vestibular symptoms.

Conclusions: An abnormally slower action potential (AP) recovery from adaptation (decreased recovery, dR) was characteristically detected in many ears with sensorineural hearing loss (SNHL) but not Meniere's disease and idiopathic sudden SNHL. We assumed that this abnormal AP recovery from adaptation was attributed to an imbalance in the distributions of auditory neurons with high and low spontaneous firing rates. The significant difference of initial hearing level between dR and normal AP recovery groups (nR) was assumed to partially result from AP recovery being determined by the inner hair cell synapse, and not from outer hair cells., Objective: This study aimed to detect the AP recovery pattern in SNHL., Methods: Electrocochleography (ECochG) was performed transtympanically in 30 patients with SNHL. AP recovery was measured by a paired click stimulation paradigm as a function of inter-click intervals from 5 to 100 ms., Results: The high prevalence of dR (9 of 30 ears) appears to be a characteristic ECochG finding in SNHL. Initial hearing level differed significantly between dR and nR groups.

Conclusion: Otologic and audiologic abnormalities are both highly prevalent in Chinese children with skeletal dysplasias. Audiologic and otologic evaluations with medical intervention are recommended for these children., Objective: Children with skeletal dysplasia have a number of otolaryngologic issues including hearing loss. This study aimed to investigate the otologic and audiologic features of Chinese children with skeletal dysplasia in central China., Methods: Otologic evaluations and pure tone audiometry were performed in children with skeletal dysplasias. To explore the potential associations with hearing loss, we studied various craniofacial anomalies., Results: A total of 125 children (mean age 9.2 years, range 5-18 years) with skeletal dysplasia were enrolled, and otoscopic examination identified eardrum abnormalities in 45.6% of the children. Audiologic analysis revealed 14 cases (11.2%) with conductive hearing loss (CHL) and 22 cases (17.6%) with sensorineural hearing loss (SNHL).