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Chronic myelomonocytic leukemia is a myeloid stem cell disease characterized by an abnormal production and accumulation of monocytic cells in association with other signs of myeloproliferation. Extramedullary manifestations of CMML are common and can affect the spleen, liver skin, and lymph nodes. However, otologic manifestations are extremely rare and could have occurred from either direct leukemic infiltration, hemorrhage of the cochlea, labyrinth, leukostasis, or infection. There is no standard treatment protocol for sensorineural hearing loss in CMML patients. More research is needed to improve the understanding of the pathogenesis of this condition, in order to provide better treatment options., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Introduction: Intra-cochlear hemorrhage is a rare cause of sudden sensorineural hearing loss (SSNHL) which may be accompanied by diverse labyrinthine symptoms. In these cases, we expect magnetic resonance imaging (MRI) to demonstrate a high signal intensity in the labyrinth on unenhanced T1-weighted images as well as in fluid-attenuated inversion recovery (FLAIR) images., Aims: To describe an experience with a case of intra-cochlear hemorrhage in a patient treated with anticoagulation, causing SSNHL and vertigo., Methods: Case report and literature review., Results: An 85-year old patient treated with anticoagulation therapy presented with right SSNHL, tinnitus and vertigo. Physical examination revealed: bilateral normal otoscopic examination, lateralized left Weber tuning fork test and a spontaneous left horizontal nystagmus. MRI performed demonstrated a high signal intensity inside the cochlea on unenhanced T1-weighted images., Conclusions: Performing an MRI is necessary in order to rule out frequent causes of SSNHL including benign as well as malignant tumors, malformations, trauma and more. The finding of an intra-labyrinthine hemorrhage causing SSNHL is rare, and should be taken into consideration when treated by anticoagulation therapy.

Objectives: To assess the clinical value of periventricular white matter hyperintensity (PWMH) found on brain magnetic resonance imaging (MRI) in patients with sudden sensorineural hearing loss (SSNHL)., Methods: In this prospective study, 115 patients who were diagnosed with SSNHL aged between 55 and 75 years were analyzed. All subjects underwent brain MRI and were divided into a PWMH and control groups, depending on the presence of PWMH on MRI. PWMH was subdivided into 3 groups according to severity. Pure-tone average results and hearing gain were compared between the 2 groups before treatment and 2 months after treatment. Hearing improvement was assessed using Sigel's criteria., Results: A total of 106 patients (43 in the PWMH group and 63 in the control group) finally completed the 2-month follow-up. Average hearing gain in the PWMH group was significantly higher than in the control group (34.8 ± 20.3 and 25.9 ± 20.3, respectively, P  = .029). PWMH score 1 showed significantly better hearing levels and hearing gain compared to PWMH score 3 and the control group. Multivariate analysis revealed that younger age, better initial hearing level, and the presence of PVWM score 1 were associated with good recovery., Conclusions: The presence of PWMH score 1 on brain MRI in patients with SSNHL was associated with better treatment response and was a good prognostic factor in a multivariate analysis while the hearing recovery in more severe PWMH (scores 2, 3) was not different from the control group.

Objective: To identify genes implicated in sudden sensorineural hearing loss (SSNHL) and localize their expression in the cochlea to further explore potential pathogenic mechanisms and therapeutic targets., Study Design: Systematic literature review and bioinformatics analysis., Data Sources: The following sources were searched from inception through July 2, 2020: PubMed-NCBI, MEDLINE, Embase, CINAHL, Cochrane Library, ClinicalTrials.gov, OpenGrey, GreyNet, GreyLiterature Report, and European Union Clinical Trials Registry. PubMed-NCBI and MEDLINE were additionally searched for human temporal bone histopathologic studies related to SSNHL., Methods: Literature review of candidate SSNHL genes was conducted according to PRISMA guidelines. Existing temporal bone studies from SSNHL patients were analyzed to identify the most commonly affected inner ear structures. Previously published single-cell and single-nucleus RNA-Seq datasets of the adult mouse stria vascularis, as well as postnatal day 7 and 15 mouse cochlear hair cells and supporting cells, were utilized for localization of the SSNHL-related genes curated through literature review., Conclusions: We report 92 unique single nucleotide polymorphisms (SNPs) in 76 different genes that have been investigated in relation to SSNHL in the literature. We demonstrate that a subset of these genes are expressed by cell types in the adult mouse stria vascularis and organ of Corti, consistent with findings from temporal bone studies in human subjects with SSNHL. We highlight several potential genetic targets relevant to current and possible future SSNHL treatments., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2021, Otology & Neurotology, Inc.)

Background: The previous 30 years have provided information on the mechanisms of cell death in the inner ear after noise exposure, ototoxic drug injury, and during aging, and clinical trials have emerged for all of these acquired forms of hearing loss. Sudden hearing loss is less well understood, but restoration of hearing after sudden hearing loss is also a long-standing drug target, typically using steroids as an intervention but with other agents of interest as well., Purpose: The purpose of this review was to describe the state of the science regarding clinical testing of investigational medicinal products for the inner ear with respect to treatment or prevention of acquired hearing loss., Data Collection and Analysis: Comprehensive search and summary of clinical trials listed in the National Library of Medicine (www., Clinicaltrials: gov) database identified 61 clinical trials., Results: Study phase, status, intervention, and primary, secondary, and other outcomes are summarized for studies assessing prevention of noise-induced hearing loss, prevention of drug-induced hearing loss, treatment of stable sensorineural hearing loss, and treatment of sudden sensorineural hearing loss., Conclusion: This review provides a comprehensive summary of the state of the science with respect to investigational medicinal products for the inner ear evaluated in human clinical trials, and the current challenges for the field., Competing Interests: None declared., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

Recent studies suggest that even moderate sudden sensorineural hearing loss (SSNHL) causes reduction of gray matter volume in the primary auditory cortex, diminishing its ability to react to sound stimulation, as well as reorganization of functional brain networks. We employed resting-state functional MRI (rs-fMRI), in conjunction with graph-theoretical analysis and a newly developed functional "disruption index," to study whole-brain as well as local functional changes in patients with unilateral SSNHL. We also assessed the potential of graph-theoretical measures as biomarkers of disease, in terms of their relationship to clinically relevant audiological parameters. Eight patients with moderate or severe unilateral SSNHL and 15 healthy controls were included in this prospective pilot study. All patients underwent rs-fMRI to study potential changes in brain connectivity. From rs-fMRI data, global and local graph-theoretical measures, disruption index, and audiological examinations were estimated. Mann-Whitney U tests were used to study the differences between SSNHL patients and healthy controls. Associations between brain metrics and clinical variables were studied using multiple linear regressions, and the presence or absence of brain network hubs was assessed using Fisher's exact test. No statistically significant differences between SSNHL patients and healthy controls were found in global or local network measures. However, when analyzing brain networks through the disruption index, we found a brain-wide functional network reorganization (p < 0.001 as compared with controls), whose extent was associated with clinical impairment (p < 0.05). We also observed several functional hubs in SSNHL patients that were not present in healthy controls and vice versa. Our results demonstrate a brain involvement in SSNHL patients, not detectable using conventional graph-theoretical analysis, which may yield subtle disease clues and possibly aid in monitoring disease progression in clinical trials., (© 2021 John Wiley & Sons, Ltd.)

Objective: To investigate the genetic causes of sudden sensorineural hearing loss (SSNHL) patients in China. This study focused on analyzing variations of coding sequence of common genes related to deafness, revealing the molecular pathogenesis of sudden deafness from a genomics perspective, discovering molecular markers associated with the onset of deafness, and then supplying prevention to high-risk populations, classifying disease according to accurate etiology, and choosing a much more precision therapy., Methods: We retrospectively analyzed the clinical characteristics of 51 patients diagnosed as SSNHL with vertigo treated in the Chinese PLA General Hospital. In this study, mutation screening of 307 nuclear genes and mitochondrial genome responsible for human or mouse deafness was performed on the 51 cases of unilateral sudden deafness patients with vertigo., Results: We identified 51 cases of unilateral sudden deafness, including 2 cases of low-mid frequency hearing impairment, 18 cases of mid-high frequency hearing loss, 11 cases of flat-type hearing loss, and 20 cases of all frequency hearing loss. Among the 51 cases, 8 (15.69%) cases of GJB2 heterozygous variations, 1 (1.96%) case of GJB3 heterozygous variations, 5 (9.8%) cases of SLC26A4 heterozygous variations, 2 (3.92%) cases of COCH heterozygous variations, 14 (27.45%) cases of CDH23 heterozygous variations, 14 (27.45%) cases of OTOF heterozygous variations, 1 (1.96%) case of SLC17A8 heterozygous variations and 2 (3.92%) cases of KCNE1 heterozygous variations. No mtDNA gene variations were identified., Conclusion: SSNHL has some relationship with hereditary in Chinese population, but its complex genetic pathogenic mechanisms need further study., (© 2021. Huazhong University of Science and Technology.)

Migraine is one of the most common and highest burdens of disease. As a primary cerebral dysfunction illness, migraine might exhibit other system-related symptoms, including vestibular and cochlear symptoms. With the publication of the diagnostic criteria of vestibular migraine, the link between migraine and vestibular symptoms became clear. However, the relationship between migraine and cochlear symptoms is far from straightforward. Therefore, we focus on the correlation between migraine and deafness, sudden sensorineural hearing loss, acute tinnitus, and chronic tinnitus to better understand the relationship between migraine and cochlear symptoms., (© 2021. The Author(s).)

Background: Sudden sensorineural hearing loss (SSNHL) is considered an otologic emergency globally. Aetiology is unknown in most cases but still the disease is believed to be caused by inflammation of the cochlea; therefore, steroids are considered beneficial due to their anti-inflammatory effect., Methods: This study was conducted on 62 patients of sudden sensorineural hearing loss (SSNHL) in Ayub Medical Complex, Abbottabad. The patients were given prednisolone and their response to the therapy was monitored. Factors like age of the patient, gender, degree of hearing loss and duration of symptoms at initial presentation were recorded and their effect of response of the patient was also noted., Results: The research subjects were 62 patients. Majority of the patients suffered from moderate to moderately severe hearing loss. Age and gender did not influence the response to the treatment. While the patients who presented earlier after the onset of disease and the patients who had milder degree of hearing loss at presentation had a better response to therapy., Conclusions: Oral corticosteroid therapy is a good therapeutic option for the treatment of sudden sensorineural hearing loss (SSHNL). The response to therapy is better in patients with milder hearing loss and those who present early to the otologist for treatment.

Introduction: Etiology of ISSNHL includes cessation of vascular perfusion, viral infection and cochlear membrane injury. Precise location of injury should be defined for a target-oriented treatment. Vestibular complaints in ISSNHL are hypothesized as involvement of vestibule. Vestibular complaints can be either due to involvement of inner ear or neural tract at any level., Objectives: In the present study we aimed to demonstrate involvement of vestibular organs in the absence of vestibular symptoms. It was aimed to evaluate superior and inferior vestibular neural pathways., Methods: c-vemp and o-vemp were applied to patients suffering ISSNHL without vertigo. Pure tone averages, audiogram configurations, degree of hearing loss were analyzed. Latencies of P1 and N1 waves, amplitudes of P1-N1 waves were evaluated. Asymmetrical vemp wave patterns were compared between two ears regarding difference of PTA., Results: Latencies of c-vemp waves were longer and amplitudes were smaller. o-vemp parameters were similar on both sides. Positive correlation was observed between c-vemp latencies and degree hearing loss., Conclusion: Inferior vestibular nerve pathway is affected in the absence of vertigo in ISSNHL with spared superior vestibular nerve pathway. Damage in IVN pathway correlates with degree of ISSNHL.

Sudden sensorineural hearing loss (SSNHL) is a medical emergency, making detailed examination to determine possible causes and early treatment important. However, etiological examinations in SSNHL do not always reveal a cause, and several factors have been found to affect treatment outcomes. Various studies are being performed to determine the prognosis and effects of treatment in patients who experience sudden hearing loss, and to identify biomarkers associated with this condition. Embase, PubMed, and the Cochrane database were searched using the key words SSNHL, prognostic, and biomarker. This search identified 4 articles in Embase, 28 articles in PubMed, and 36 in the Cochrane database. Of these 68 articles, 3 were duplicates and 37 were unrelated to the research topic. After excluding these articles, the remaining 28 articles were reviewed. Factors associated with SSNHL were divided into six categories: metabolic, hemostatic, inflammatory, immunologic, oxidative, and other factors. The associations between these factors with the occurrence of SSNHL and with patient prognosis were analyzed. Low monocyte counts, low neutrophil/lymphocyte ratio (NLR) and monocyte/high-density lipoproteins (HDL) cholesterol ratio (MHR), and low concentrations of fibrinogen, platelet glycoprotein (GP) IIIa, and TNF-α were found to be associated with good prognosis. However, these factors alone could not completely determine the onset of and recovery from SSNHL, suggesting the need for future basic and clinical studies.

Objective: The role of magnetic resonance (MR) imaging in idiopathic sudden sensorineural hearing loss (ISSHL) is controversial due to the inhomogeneity of clinical and MR protocols. The aim of this work is to relate early MR findings obtained immediately after the admission, with the clinical presentation, the audiological findings, and the outcomes of treatment., Study Design: Prospective observational study., Setting: Tertiary referral university center., Patients: Forty-seven patients (22 M, 25 F; age: 54.4 ± 17.5 yr) consecutively referred to the Department of Emergency for ISSHL., Interventions: All patients underwent the diagnostic and therapeutic work-up for ISSHL, and MR imaging within 72 hours from the admission, independently of the symptoms onset. All patients received the same treatment (systemic steroid therapy, intratympanic steroid injection, and hyperbaric oxygen therapy)., Main Outcome Measure(s): MR patterns, clinical, and laboratory findings., Results: MR imaging was positive in 25 of 47 cases (53%), with a perfect agreement between clinical and MR examinations (Cohen K = 1) upon the affected ear. Three different radiological patterns were observed: labyrinthine haemorrhage (n = 5), acute inflammatory process (n = 14), isolated blood-labyrinth barrier breakdown (n = 6). By binary logistic regression, only vertigo was associated with a positive MR imaging [B = 2.8; p = 0.011; OR = 9.5 (95% CI: 2.2-40.8)] and the latter was the only variable associated with an unfavorable outcome [(B = 2.8; p = 0.02 OR = 12.8 (95% CI: 2.9-56.7)]., Conclusion: Patients affected by ISSHL with associated vertigo show a higher likelihood of having a positive MR imaging, which, in turn, seems to predict an unfavorable outcome.

Objective: To investigate the clinical characteristics, etiology, treatment outcomes, and prognostic factors of sudden sensorineural hearing loss (SSNHL) in children to guide the clinical diagnosis and treatment of SSNHL in the pediatric population., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Patients diagnosed with SSNHL from November 2011 to December 2017 with relatively complete clinical data., Intervention: Diagnosis and systemic treatment of SSNHL., Main Outcome Measures: Patients' clinical characteristics, etiology, laboratory tests, imaging, pure-tone audiometry at admission, and discharge were analyzed., Results: A total of 25 children and 149 adults with SSNHL were included. Recent or previous viral infection rates (81.8%) and fasting blood glucose level (5.23 + 1.47 mmol/L) in children with SSNHL were lower than those in adult SSNHL patients (p = 0.033, p = 0.033). Autoimmune abnormalities (90.0%) and plasma fibrinogen abnormalities (27.3%) were higher in children with SSNHL than those in adult SSNHL patients (40.0%, 8.8%, respectively, p < 0.05). The recovery rate in children (38.4%) with SSNHL is comparable to that in adults (22.6%), but children have a higher complete rate compared to adults (26.9%, 11.3%, respectively, p < 0.05). Children with a profound audiometric curve had a worse prognosis in comparison to other types of audiometric curves (p = 0.041)., Conclusions: Children with SSNHL have a lower rate of viral infection in comparison to adults with SSNHL. Fasting blood glucose levels, complement C3, C4, and fibrinogen may be closely related to childhood SSNHL. The recovery rate in children with SSNHL is comparable to that in adults, but children have a higher complete rate compared to adults. A profound hearing curve is an unfavorable prognostic factor in both children and adults with SSNHL.

Background and Purpose: A detailed knowledge of the normal Magnetic Resonance (MR) anatomy of the vestibular endolymphatic space (ES) could be useful to understand the linkage between endolymphatic hydrops (EH) and Ménière's disease (MD). Our aim was to describe the MR anatomy of the vestibular ES as depicted by MR imaging in healthy ears., Methods: This report describes a single-center retrospective study. Three readers analyzed the healthy ears of 22 consecutive patients who had undergone MRI for unilateral sudden hearing loss. The readers described the vestibular ES based on a delayed post-contrast 3D-FLAIR sequence according to six well-defined planes, three oblique sagittal (lateral, intermediate and medial) planes and three axial (superior, intermediate and inferior) planes., Results: On sagittal lateral and intermediate planes, we identified the SSC ampulla combined with the utricle in 22/22 ears. On the sagittal medial plane, the saccule was detectable in 15/22 (68%) ears, having a club shape with the long axis oriented cranio-caudally; in 7/22 (32%) ears, the saccule presented an oval/round shape that appeared more conspicuously on the axial intermediate plane. The ES occupied the half superior portion of the vestibule in 22/22 ears, never contacting the round and oval windows. On the axial plane, in 17/22 cases, the ES showed a Y-shaped arrangement, while in 5/22 ears (23%), the ES presented a more globular shape., Conclusion: MR imaging represents a valid tool to explore the in vivo anatomy of the vestibular ES and to highlight its variability in normal ears., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objective: To calculate the spiral ganglion neural decay rate among patients diagnosed with idiopathic sudden sensorineural hearing loss (ISSNHL)., Study Design: Retrospective., Setting: Temporal bone histopathology bank., Patients: Subjects diagnosed during life with unrecovered unilateral ISSNHL., Intervention(s): Spiral ganglion cell count in the bilateral reconstructed Rosenthal canal., Main Outcome Measure(s): Decay rate of spiral ganglion cells., Results: Nine patients were enrolled. The average age of ISSNHL diagnosis and death was 52 and 63 years, respectively. The total and segmental SGCC decay shows a temporal dependency. The time lag between the ISSNHL event and death was linearly proportional to the SGCC decay. Subjects who died less than 5 years from diagnosis of ISSNHL had no more than 13% loss of their total SGCC, and no more than 14% of SGCC per Rosenthal canal segment. When a longer period passed from ISSNHL diagnosis to death (19-20 yr), a loss of 16% and 13 to 18% of total and segmental SGCC occurred, respectively., Conclusions: SGCC decline from ISSNHL diagnosis through life.

A female patient with unilateral enlarged vestibular aqueduct (EVA) demonstrated scala vestibuli dilatation on that side while on the contralateral side both vestibular aqueduct and scala vestibuli were normal. This important radiological finding demonstrates that modiolar defects (hence 'cystic apex') observed in Incomplete partition-II is due to pressure transfer via EVA during embryological development. Therefore, it supports the previous histopathological ideas radiologically. Depending on the patency of EVA, variety of modiolar defects may arise.

Objective: To evaluate whether Sudden Sensorineural Hearing Loss (S-SNHL) may be an early symptom of Multiple Sclerosis (MS)., Materials and Methods: A systematic review was conducted using the following keywords: "Multiple sclerosis, hearing loss, sudden hearing loss, vertigo, tinnitus, magnetic resonance imaging, otoacoustic emission, auditory brainstem responses, white matter lesions, sensorineural hearing loss, symptoms of MS and otolaryngology, nerve disease and MS". Only the articles that included results of at least one auditory test and MRI were considered. We evaluated the prevalence of SNHL in patients with MS, the presence of different forms of SNHL (S-SNHL and Progressive SNHL (P-SNHL)) and their correlation with the stage of MS, the results of electrophysiological tests, and the location (if any) of MS lesions as detected by white matter hyperintensities in the MRI., Results: We reviewed a total of 47 articles, which included 29 case reports, 6 prospective studies, 6 cohort studies, 4 case-control studies, and 2 retrospective studies. 25% of patients suffered from SNHL. S-SNHL typically occurred in the early stage of the disease (92% of patients) and was the only presenting symptom in 43% of female subjects. Instead, P-SNHL occurred in the late stage of MS (88% of patients). Auditory Brainstem Responses (ABR) were abnormal in all MS patients with S-SNHL. When S-SNHL appeared during the early stage of the disease, MS lesions were found in the brain in 60% of patients and in the Internal Auditory Canal in 40% of patients. ABR remained abnormal after recovery., Conclusions: S-SNHL can be an early manifestation of MS and should always be considered in the differential diagnosis of this condition, especially in women. The pathophysiology can be explained by the involvement of microglia attacking the central and/or peripheral auditory pathways as indicated by WMHs.

The present study aimed to determine the effects of ischemia/reperfusion (IR) injury for the carotid system on hearing, particularly, the role of autophagy in this process. Sixty-three Sprague-Dawley rats were divided randomly into three groups: sham surgery animals (S), temporary carotid artery occlusion (ischemia) for 30 min (I30), and temporary carotid artery occlusion for 60 min (I60). Auditory brainstem response measurements were performed on mice. After 72 h of reperfusion, the microcirculation was measured in mice after ischemia injury. Immunofluorescence was used to examine the expression of caspase-3 and light chain 3B in the cochlear sections. Temporary carotid artery occlusion lasting for 30 (I30) or 60 min (I60) caused significant hearing loss in the ischemia phase. Following a recovery during the postreperfusion phase, the temporal threshold shift occurred in the I30 group, whereas a permanent threshold shift occurred in the I60 group. Moreover, both microcirculation and autophagy affected hearing 24 h after reperfusion, whereas at 72 h, autophagy works as an intrinsic cellular process that protects against death from the IR effect. These results suggest that the sooner the reperfusion, the better the hearing recovery. In conclusion, autophagy promotes cell survival in the cochlea; however, excessive IR damage counteracts the beneficial potential of autophagy protection and leads to a permanent threshold shift.

Introduction Sudden sensorineural hearing loss (SSNHL) involves an acute unexplained hearing loss, nearly always unilateral, that occurs over less than a 72-hour period. SSNHL pathogenesis is not yet fully understood. Cochlear vascular occlusion has been proposed as a potential mechanism of hearing damage and cochlear ischaemia has been related to alterations of cochlear microvessels. In addition, some researchers have focused their attention on the rheological alterations and blood hyperviscosity. Erythrocyte deformability plays a key role in determining blood viscosity, and it is critical to cochlear perfusion. It has been shown that oxidative stress-induced erythrocyte membrane fluidity alterations are linked to the progression of cardiovascular diseases. Methods To determine whether erythrocytes from SSNHL patients show signs of oxidative stress, and whether this condition can modify the haemorheologic profile in these patients, we analysed haemorheologic profile and erythrocyte oxidative stress in 35 SSNHL patients and 35 healthy subjects, matched for age and sex. Fluorescence anisotropy was used to evaluate the fluidity of erythrocyte membranes. Results Our results show a significant structural and functional involvement of erythrocyte membrane alterations in SSNHL, as well as elevated levels of membrane lipid peroxidation and intracellular reactive oxygen species (ROS) production. In addition, erythrocyte-derived ROS and erythrocyte lipid peroxidation positively correlated with whole blood viscosity and erythrocyte deformability. Moreover, in vitro experiments demonstrated that ROS display a key role in erythrocyte membrane fluidity. Conclusion These findings indicate that erythrocyte oxidative stress plays a key role in the pathogenesis of SSNHL and pave the way to new therapeutic interventions., Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Immune checkpoint inhibitors have emerged as a promising therapeutic option for metastatic cancers. However, they have been associated with inflammatory adverse reactions in various organ systems. A recent article reported a case of sudden bilateral hearing loss that occurred in a patient with metastatic melanoma being treated with pembrolizumab. The authors attributed that complication to an autoimmune reaction secondary to the treatment. This commentary discusses the importance of considering the diagnosis of leptomeningeal metastasis in patients with metastatic melanoma who present with new cranial nerve deficits.

Acute vertigo with sudden sensorineural hearing loss (SSNHL) is a rare clinical emergency. Here, we report the audio-vestibular test profiles of 27 subjects who presented with these symptoms. The vestibular test battery consisted of a three-dimensional video head impulse test (vHIT) of semicircular canal function and recording ocular and cervical vestibular-evoked myogenic potentials (oVEMP, cVEMP) to test otolith dysfunction. Unlike vestibular neuritis, where the horizontal and anterior canals with utricular function are more frequently impaired, 74 % of subjects with vertigo and SSNHL demonstrated impairment of the posterior canal gain (0.45 ± 0.20). Only 41 % showed impairment of the horizontal canal gains (0.78 ± 0.27) and 30 % of the anterior canal gains (0.79 ± 0.26), while 38 % of oVEMPs [asymmetry ratio (AR) = 41.0 ± 41.3 %] and 33 % of cVEMPs (AR = 47.3 ± 41.2 %) were significantly asymmetrical. Twenty-three subjects were diagnosed with labyrinthitis/labyrinthine infarction in the absence of evidence for an underlying pathology. Four subjects had a definitive diagnosis [Ramsay Hunt Syndrome, vestibular schwannoma, anterior inferior cerebellar artery (AICA) infarction, and traction injury]. Ischemia involving the common-cochlear or vestibulo-cochlear branches of the labyrinthine artery could be the simplest explanation for vertigo with SSNHL. Audio-vestibular tests did not provide easy separation between ischaemic and non-ischaemic causes of vertigo with SSNHL.

Hearing loss caused by cochlear damage is the main symptom of sudden deafness (SD). Some patients also suffer from vestibular symptoms. In recent years, more attention has been paid to the vestibular dysfunction in patients with SD. The lesions could involve the whole inner ear in SD patients with and without vertigo. Comprehensive evaluation of vestibular function may help us understand the extent of lesions in sudden deafness and analyze the pathogenesis of disease. A less involvement of inner ear lesion may indicate a better hearing recovery.

Objective: To study the clinical characteristics of early tinnitus in patients with sudden deafness., Method: Forty-seven patients with sudden deafness and early tinnitus recieved subjective review and filled out classified questionnaire of tinnitus. By pure tone audiometry and tinnitus matching test, we obtain the distribution, quantity and matching loudness of tinnitus patients., Result: (1) Objective examination: the most common frequencies of tinnitus were 8000 Hz and 4000 Hz. The most common numbers were two frequencies. The matching loudness was (16.63 ± 8.37) dB. (2) Subjective examination: the tinnitus was monotone (74.0%), sustained (89.0%), third level loudness (45.0%), third level annoying (60.0%), high frequency (83.0%), sound of cicadas (66.7%). The most influencing factors were hearing (28.2%) and mood (29.5%). (3) Relationship between tinnitus and pure tone hearing threshold curve: there was a significant correlation between the frequency of pure tone audiometry and the matching loudness of tinnitus (r = -0. 370, P < 0.01). There was no significant correlation between the frequency of pure tone tinnitus and the matching loudness of tinnitus (r = -0.083, P > 0.05)., Conclusion: (1) The results of subjective examination were consistent with those of objective examination. (2) The tinnitus should be considered in the early treatment of sudden deafness.

Objective: To investigate the correlation between findings of three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging (3D FLAIR MRI) and baseline as well as outcome variables of idiopathic sudden sensorineural hearing loss (ISSHL)., Data Sources: Publications of all languages listed in PubMed and EMBASE STUDY SELECTION: Studies of ISSHL patients without primary treatment and precontrast 3D FLAIR MRI scan was performed., Data Extraction: Demographical and clinical characteristics of patients were extracted and the quality of studies was evaluated using the Newcastle-Ottawa Scale., Data Synthesis: Continuous and dichotomous data was synthesized in Inverse Variance and Mantel-Haenszel models, respectively. The aggregate results were estimated and displayed in forest plots., Conclusions: The presence of high signal (HS) in inner ear on 3D FLAIR MRI indicated more severe initial hearing loss, and the existence of HS in inner ear on 3D FLAIR MRI increased the incidence of vertigo by 2.88 times. Meta-analysis of the dichotomous data of hearing recovery rate showed the hazard of recovery in HS group was significantly less than the one in no signal (NS) group. The pooled hearing improvement in decibels also favored NS group, but statistically the difference was not significant.

Objective: To demonstrate that sudden sensorineural hearing loss is possibly of viral origin rather than vascular., Study Design: The histopathologic morphology in 7 temporal bones with known vascular impairment due to surgical interventions was compared with that of 11 bones with a history of idiopathic sudden sensorineural hearing loss (ISSNHL). Attention was paid to the spiral ligament, stria vascularis, organ of Corti hair cells, tectorial membrane, ganglion cell population, and degree of perilymph fibrosis and the auditory nerve., Setting: A temporal bone laboratory that has been in operation for more than 50 years and includes a database consisting of clinical and histopathological information that facilitates quantitative and qualitative analysis., Subjects: Eight hundred forty-nine individuals who pledged their temporal bones for scientific study, of which 18 were selected for this study by means of the database criteria of sudden sensorineural hearing loss and postmiddle fossa and retro sigmoid sinus tumor removal or vestibular nerve section., Results: Sudden sensorineural hearing loss bones exhibited no perilymph fibrosis compared with 6 of 7 vascular cases with fibrosis (P ≤ .001), exhibited less loss of ganglion cells (P ≤ .026), exhibited greater survival of spiral ligament (P ≤ .029), and averaged twice the survival of hair cells and more widespread tectorial membrane abnormalities., Conclusion: Analysis of human temporal bones from patients with a sudden sensorineural hearing loss does not support a vascular insufficiency but is more suggestive of a viral etiology.

Objective: We aimed to determine: (1) whether mean platelet volume was elevated in patients with sudden sensorineural hearing loss, compared with healthy controls; and (2) whether mean platelet volume level was related to hearing loss severity., Materials and Methods: The study included 31 patients with sudden sensorineural hearing loss and 31 age- and sex-matched, healthy controls. Peripheral venous blood samples were taken from subjects and mean platelet volume and levels of glucose, total cholesterol, high-density lipoprotein, low-density lipoprotein and triglyceride were measured., Results: Mean platelet volume was significantly greater in the sudden sensorineural hearing loss group compared with the control group. However, there was no significant correlation between mean platelet volume level and hearing loss severity., Conclusion: Mean platelet volume, a determinant of platelet activation, is elevated in patients with sudden sensorineural hearing loss. To our knowledge, this is the first report investigating mean platelet volume levels in such patients. Our findings indirectly support the hypothesis of vascular impairment as a pathogenetic factor in sudden sensorineural hearing loss.

Objective: To explore the characteristics of vertebral artery system in the patients with sudden deafness by using digital subtraction angiography (DSA)., Method: Thirty-four cases of sudden deafness with vertebrobasilar artery ischemia confirmed by the color doppler ultrasound were undergone DSA in both side. The characteristics of vertebral artery, basal artery and before-cerebellum artery were analysis before specific therapy., Result: There is no related complication were occurred among 34 cases. Side vertebral artery was blocked in 2 cases, atherosclerosis was found in 5 cases. The right cerebellar artery anterior and the left cerebellar artery posterior were found filling defect or minor change in 29.4% (10/34) and 35.3% (12/34) of the patients, respectively. The right and the left arteria auditiva interna were found filling defect or minor change in 64.7% (22/34) and 73.5% (25/34) of the patients, respectively. After specific therapy, 8 cases were cured, the hearing of 9 cases were markedly improved, the hearing of 12 cases were improved efficient and 5 patients have no hearing improvement, the total effective rate was 85.3%., Conclusion: The arteria auditiva interna and inferior anterior arteria cerebelli caused inner ear ischemia were found in the patients with sudden deafness. The using of vasodilator may have satisfactory and positive curative effect in the therapy of sudden deafness.

Background: Pathological levels of blood lipids could be one of the causes of sudden sensorineural hearing loss (SSNHL). The objective of this manuscript is therefore to evaluate the relationship between blood lipid content and sudden sensorineural hearing loss (SSNHL)., Methods: The correlation between serum lipid parameters, including total cholesterol (TC), triglycerides, high-density lipoprotein cholesterol (HDL-C), low density lipoprotein cholesterol (LDL-C), apolipoprotein AI (apo AI), apolipoprotein B (apo B), and lipoprotein A (Lp(a)), and the onset of SSNHL was analyzed from a data set of 250 patients and an age, gender and weight matched control group of 250 subjects., Results: The result of single factor logistic regression shows that TC (p<0.01), LDL-C (p<0.01), and apo B (p=0.03) of SSNHL group were significantly higher than those of the control group. The odds ratio of TC, LDL, and apo B are higher than 1, while the confidence intervals of the odds rations do not include 1. No significant difference was found with the prevalence of hypertension (P=0.818), diabetes (P=0.869) and smoking habits (P=0.653) between SSNHL group and control group., Conclusion: Total cholesterol, low density lipoprotein cholesterol and apolipoprotein B concentrations may be important factors in the pathogenesis of sudden sensorineural hearing loss, and should be assessed during the investigation of patients with this condition.

Objective: To evaluate the prevalence of labyrinthine ossification, and especially cochlear ossification, in a cohort of patients with unilateral sudden deafness or severe sensorineural hearing loss., Design: Retrospective data collection., Study Sample: Sixty-four consecutive patients with unilateral sudden deafness or severe sensorineural hearing loss and either high-resolution CT (HRCT) of the temporal bone (isotropic spatial resolution ≤ 0.8 mm; n = 18) or high resolution CISS MRI (isotropic spatial resolution ≤ 1 mm; n = 55) were included. Nine patients underwent both imaging modalities. A standardized reading regarding labyrinthine ossifications was performed by an experienced head and neck radiologist blinded to clinical symptoms., Results: Radiologic signs of cochlear ossification were present in 14 patients (12 CT and 2 MRI). Eight patients showed unilateral and six patients bilateral signs of cochlear ossification. In all except one of the unilateral cases, the deafened ear was affected., Conclusions: Signs of cochlear ossification were found in an unexpectedly high rate (14/64, 22%) of patients with acute deafness. The data suggest HRCT of the temporal bone to be more sensitive to detect labyrinthine ossification than MRI. HRCT of the temporal bone should therefore be considered in patients with impaired recovery of acute deafness to exclude cochlear ossification; if present, and, in cases of early signs, the patient should be evaluated further to facilitate early cochlear implantation before progression impedes electrode insertion, reflecting latest developments considering cochlea implants for single-sided deafness to be effective.

Introduction: Recently, several studies and case reports have dealt with the topic of cerebral sinus venous thrombosis (CSVT) and focused on sudden hearing loss as an early and rare symptom, to diminish the delay in diagnosing this serious disease., Materials and Methods: We conducted a retrospective analysis over 3 years and investigated MRIs of all inpatients who were treated for sudden sensorineural hearing loss. The aim of the study was to evaluate whether sudden hearing loss could be an early indicator, or the first sign, of CSVT., Results: In total, 554 patients were included. Only 2 patients with CSVT could be identified. In both, sudden unilateral sensorineural hearing loss was not the only symptom. They also reported headache, and 1 patient also reported tinnitus and vertigo., Conclusion: In our opinion, sudden unilateral sensorineural hearing loss alone is not a reliable indicator of CSVT. In combination with headache or visual impairment, this rare vascular disease should be taken into account.

Objective: Positively related to functional connectivity using resting state fMRI functional connectivity method to observe the right of sudden deafness of auditory cortex in patients with brain., Method: We selected the right side of the 12 cases of patients with sudden deafness resting state fMRI data acquisition, positive correlation function for the observation about the right of sudden deafness patients using the method of functional connectivity brain auditory cortex and the brain regions to connect brain map, and matched the normal hearing group the difference., Result: The right side of sudden deafness in patients with valid data for the seed point of A I bilateral. The brain was activated network included bilateral transverse gyrus, superior temporal gyrus, insula, cingulate gyrus and supplementary motor area. Brain networks were activated like a normal person, but there were differences between the two., Conclusion: The right side of the deafness of A I seed point the functional connectivity of the auditory system is still mainly confined to the auditory system, but the local auditory cortex functional reorganization occurs.

Objective: To investigate the vestibular system changes in sudden deafness with vertigo (SDwV) and sudden deafness without vertigo (SDwoV) and the cause of persistent canal paresis (CP) in SDwV patients., Study Design: Retrospective study., Materials and Methods: Four temporal bones from the affected ear in 4 patients with unilateral sudden deafness (SD), 2 SDwV and 2 SDwoV, were selected. Four contralateral temporal bones with normal-hearing ears were defined as the control. Morphologic findings of the labyrinth, the number of Scarpa's ganglion cells, and the density of vestibular hair cells were investigated in all temporal bones. Clinical data and the results of vestibular tests of 11 patients with unilateral SD, as a separate group, also were investigated., Results: Atrophic change of the organ of Corti, tectorial membrane, and stria vascularis in cochlea, and deposits and atrophic otoconial membrane in vestibular sense organs were seen on affected ears more than control ears. The density of Type I hair cells seemed to decrease on the saccular macula and the posterior semicircular canal crista on affected ears, and there was no remarkable difference between SDwV and SDwoV. In 1 patient with SDwoV who died 10 months after the onset of SD, there were large amount of deposits on the cupula, the atrophied otoconial membrane was peeling off from the saccular macula, and the saccular membrane collapsed to the saccular macula in the affected ear. In the clinical data, all SDwV who were examined within 2 years from the onset had CP, and all SDwV had profound hearing loss., Conclusion: There is no remarkable difference between SDwV and SDwoV in the number of Scarpa's ganglion cells and the density of vestibular hair cells. The damage of the extracellular superstructure is seen in SD with or without vertigo. The damage of extracellular superstructure is potentially one of the causes of persistent CP in patients with SD.

The pathogenesis of sudden sensorineural hearing loss (SSNHL) is unclear, though some researchers postulate the major mechanism of onset to be via circulatory disturbance or cochlear inflammation. SSNHL can represent the sole manifestation of anterior inferior cerebellar artery infarction, and patients with a SSNHL may have higher than normal risk of future stoke. According to a vascular remodeling theory, vertebral arteries (VAs) are typically asymmetric with the basilar artery (BA) gradually curving in the opposite direction of the larger VA. Decreased wall shear stress on the inner surface of the curvature (weaker side of the vertebral artery) gives rise to an atherothrombogenic environment. It is hypothesized that angulation of the BA could contribute to the decline of anterior inferior cerebellar artery (AICA) flow or to the development of atheroma formation in the AICA orifice. Vertebrobasilar junction angulation could represent a simple and useful marker of SSNHL caused by a vascular compromise of the cochlea., (Copyright © 2012. Published by Elsevier Ltd.)

Objectives: (1) To study the prevalence and characteristics of large endolymphatic sac internal compartments on thin-section T2- and T2*-weighted magnetic resonance imaging, and to relate these to other large endolymphatic sac magnetic resonance imaging features, and (2) to correlate the compartment imaging features, endolymphatic sac size and labyrinthine anomalies with the patients' clinical and audiological data., Method: Magnetic resonance imaging studies for 38 patients with large endolymphatic sac anomalies were retrospectively reviewed in a tertiary referral centre. Endolymphatic sac compartment presence, morphology and imaging signal were assessed. Endolymphatic sac size and labyrinthine anomalies were also recorded. Endolymphatic sac compartments and other imaging features were correlated with clinical and audiological data., Results: Compartments were present in 57 per cent of the imaged endolymphatic sacs, but their presence alone did not correlate with other imaging features or clinical data. The endolymphatic sac : internal auditory meatus signal ratio was associated with a history of sudden or fluctuating hearing loss. Hearing loss correlated with opercular and extraosseous endolymphatic sac size measurements. A larger midpoint intraosseous endolymphatic sac size was associated with clear fluid loss at cochlear implantation., Conclusion: The magnetic resonance imaging characteristics of large endolymphatic sac compartments have been defined. The endolymphatic sac size and distal compartment signal should be recorded, as these provide prognostic information and assist the planning of appropriate interventions.

Objective: To test the therapeutic effect on moderate and severe sudden deafness treated with low-energy laser irradiation on acupoint and external auditory canal combined with auricular point sticking (APS) and as compared with electroacupuncture., Methods: Two hundred and fifty-eight cases of moderate and severe sudden hearing loss were randomly divided into an observation group 1, an observation group 2 and a control group, 86 cases in each group. In three groups, 10% low molecular Dextran 500 mL were used for intravenous infusion. Based on the above treatment, the observation group 1 was treated with low-energy laser irradiation on acupoint and external auditory canal (such as Ermen (TE 21), Tinggong (SI 19) and Tinghui (GB 2)), combined with APS at Gan (liver), Shen (kidney) and Neifenmi (endorine), etc. The observation group 2 was treated with electroacupuncture at the same acupoints as those point irradiation in observation group 1. Fifteen days made one session. The therapeutic effects were evaluated after one and two sessions., Results: After two sessions, The cured rate was 40.7% (35/86) in observation group 1 and 38.4% (33/86) in observation group 2,which were superior to 25.6% (22/86) in control group (both P < 0.05). Compared with one session, the therapeutic effects after two sessions were better in two observation groups (both P < 0.05), but there was no significant difference between two groups (both P > 0.05). In comparison of the improvements of frequency audiometry and auditory function, the two observation groups were better than those in control group (P < 0.05, P < 0.01), and the improvements after two sessions were better in two observation groups (both P < 0.01)., Conclusion: Both of low-energy laser irradiation on acupoint and external auditory canal combined with APS and electroacupuncture are able to decrease frequency audiometry, improve auditory function, and the therapeutic effects are better with prolongation of treatment time. The clinical efficacy of above two the rapies on moderate and severe sudden deafness is superior remarkably to that of conventional treatment. The therapy of low-energy laser irradiation on acupoint and external auditory canal combined with APS can replace the electroacupuncture therapy in treating moderate and severe sudden deafness.

Epidermoid tumors of the cerebellopontine angle are associated with a variety of symptoms, usually attributed to compression and displacement of involved cranial nerves. The authors present a case of a large epidermoid tumor in the left cerebellopontine angle with sudden hearing loss and tinnitus. The intraoperative finding of migration of two tumor pearls into the cochlear nerve was the origin of the clinical sign. The patient improved remarkably after removal of the tumor. This case demonstrates the mechanism for selective hearing loss associated with the large cerebellopontine angle., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Introduction: Sudden sensorineural hearing loss (SSNHL] can be the presenting symptom of an acoustic neurinoma. High sensitivity and specificity rates define magnetic resonance imaging (MRI) of the brain and internal acoustic canal as the gold standard for the diagnosis of retro-cochlear pathologies. A previous study revealed low compliance rates (49%] for performing an MRI after an episode of SSNHL; 15% of patients who had an MRI were diagnosed with a retro-cochlear pathology., Aim of Study: To evaluate current compliance rates for performing an MRI of the brain and internal acoustic canal after an episode of SSNHL., Material and Methods: The study cohort included 41 patients, 24 females and 17 male, with an average age of 49.8 +/- 16.8 years (range, 16-78 years), who were diagnosed with SSNHL and admitted for treatment between December 2009 and June 2010., Results: Two months after discharge from hospitalization, only 29 patients (70.7%) returned for the scheduled followup. Only 21 patients (51.2% of those admitted) had an MRI of the brain and internal acoustic canal, as was recommended upon their discharge. Three of those who had an MRI were diagnosed with a retro-cochlear pathology. In 20 patients [48.8%) who did not perform an MRI, a retro-cochlear pathology could not be ruled out., Conclusions: Compliance rates for performing an MRI after an episode of SSNHL haven't changed in recent years. The main reason seems to be poor patients' compliance, as well as their general practitioners and health insurance companies. Ruling out retro-cochlear pathologies by means of MRI is highly important and considered common medical practice. Guidelines regarding the importance of MRI after an episode of SSNHL should be updated and reinforced.

Protein kinase C-eta (PRKCH) gene has been recently identified as a susceptible risk locus for cerebral infarction and hemorrhage in the Asian populations. The inner ear artery, a usual branch of anterior inferior cerebellar artery, is an end artery with minimal collaterals, therefore, the inner ear is particularly vulnerable to ischemia. The potential association between the development of stroke and sudden sensorineural hearing loss (SSNHL) has been implied. The authors hypothesized that the PRKCH polymorphism predisposing to stroke is associated with SSNHL risk, in view of brain magnetic resonance imaging (MRI) findings. The authors compared 33 cases of prevalent SSNHL with other cases among 2188 adults aged 40 to 79 years who participated in the Study of Aging, to assess the impact of PRKCH 1425G/A polymorphism in consideration of brain MRI findings. Multiple logistic regression was used to obtain odds ratios (ORs) for SSNHL, with adjustment for other possibly influential factors under additive model of minor allele. The per-allele OR for SSNHL risk was 1.770 (95% confidence interval: 1.024-3.060) after adjustments. The effect of the 1425A-allele varied by white matter lesion (WML) status. A significant impact of the A-allele on SSNHL risk increment was observed in higher-WML group, but not in no- or mild-WML group. The 1425A-allele of PRKCH has probably contributed to the susceptibility to SSNHL, despite the etiological heterogeneity of SSNHL, and the impact of the PRKCH 1425A variation observed in this study may imply underlying vascular pathogenesis of SSNHL., (Copyright © 2011 Informa Healthcare USA, Inc.)

Objective: To investigate inner ear of patients with sudden deafness with three-dimensional fluid attenuated inversion recovery (3D FLAIR) MRI, and the relationship between the results of 3D FLAIR and the prognosis., Methods: Twenty-three patients with sudden deafness received 3D FLAIR at 3T MRI, and the signals of inner ear were recorded. Hearing levels were evaluated at initial visit and after treatment. The relationship between 3D FLAIR findings and hearing prognosis was evaluated., Results: Eight patients with sudden deafness showed high signals in the affected cochlea on 3D FLAIR, the others of affected cochlea and all of contralateral cochlea showed no signal on 3D FLAIR. The age, sex, affected side, period to initial visit and initial audiogram had no difference between cochlea no signal group and high signal group. The average auditory threshold (x±s) in cochlea high signal group (90±21) dB HL was significant higher than that in cochlea no signal group (60±28) dB HL, P<0.05 at patients' discharge. After treatment, in cochlea no signal group, two cases' hearing was complete recovered, remarkable improvement in five cases, slight improvement in two cases and no change in six cases. In cochlea high signal group, hearing was slight improvement in one case and no change in seven cases. The prognosis was significant difference between two groups. Five of seven patients with vertigo and sudden deafness showed high signal in affected side vestibule on 3D FLAIR, and the hearing of whom had no change after treatment., Conclusion: 3D FLAIR can show high signal in affected inner ear in sudden deafness patients, and which is related to a poor hearing prognosis.

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

Conclusion: The findings suggest that alterations in the composition of inner ear fluid play important roles in the development of low-tone sudden deafness (LTSD). High-intensity signals on three-dimensional fluid attenuated inversion recovery (3D-FLAIR) may reflect an increased concentration of protein in the inner ear due to the increased permeability of blood vessels. Disordered blood flow in the inner ear is associated with an increased permeability of the blood-labyrinth barrier. Therefore, the disordered blood flow in the cochlea may be closely related to the pathophysiological mechanisms of LTSD., Objectives: The 3D-FLAIR sequence has been used to detect alterations in the composition of inner ear fluid. The purpose of this study was to report imaging findings in cases of LTSD., Methods: 3D-FLAIR magnetic resonance imaging was performed in five women with nonrecurrent-type LTSD., Results: Three of the five patients (60%) showed high-intensity signals in the cochlear basal turn on precontrast 3D-FLAIR. Postcontrast enhancement was not prominent in any patient. In patient 1, the cochlea of the unaffected side showed high-intensity signals. No patients had such signals in the vestibulae or the semicircular canals.

Conclusions: Contralateral hearing loss is significantly correlated with poor hearing outcomes in patients with idiopathic sudden sensorineural hearing loss (ISSNHL)., Background: The hearing outcome in patients with ISSNHL was analyzed using multiple variables., Methods: A retrospective chart review was conducted using 89 patients with ISSNHL. Patients within 40 dB HL of average hearing levels and/or patients whose hearing loss was restricted to low frequencies were excluded. The influence of pre-existing conditions on hearing outcome was analyzed using a polytomous universal model. Pre-existing conditions analyzed included hyperglycemia, hypercholesterolemia, hypertension, and contralateral hearing loss. In addition, the severity of hearing loss, age group, and the existence of vertigo were analyzed concomitantly., Results: Hearing recovery was significantly reduced in patients with a past history of contralateral hearing loss.

Objectives: The purpose of this research is to understand the etiology of sudden hearing loss due to genetic factors in Turkish people. Determination of these genetic factors and better understanding of molecular pathogenesis may guide more realistic planning and treatment recommendations., Patients and Methods: Forty patients (Group 1; 19 males, 21 females; mean age 37.9+/-15.6 years; range 9 to 76 years) who presented with sudden hearing loss to the Ear, Nose and Throat Clinic of Medical Faculty Hospital of Cumhuriyet University between January 2008 and June 2009, and were diagnosed with sudden hearing loss through history, physical examination and review of audiometric findings, and 20 healthy volunteers (Group 2; 14 males, 6 females; mean age 31.7+/-4.4 years; range 24 to 43 years) for the control group were included in this study. All Patients were evaluated by the genetic clinic for the GJB2, GJB3, GJB6 and WFS1 gene using multiplex ligation-dependent probe amplification (MLPA) method mutation analysis., Results: No difference was found in the peripheral blood sample analyses of the two groups at WFS1 exon 8 and connexin 26, 30 and 31 gene zones using the MLPA method with respect to heterozygous mutation (p=0.291, p>0.05). In four patients in group 1 heterozygous mutation was detected at the target gene zone. Heterozygous mutation was in the WFS1 exon 8 zone in two patients; and in the WFS1 exon 1 zone in other two patients., Conclusion: Sudden hearing loss studies in the future should include connexin 26, connexin 30 and other gene mutations that may affect the function of the gap-junction located in the region of the cochlea stria vascularis (stV), basal membrane (BM), spiral limbus (Li) and spiral ligament (SL). These studies should be performed on larger series, and should include family members of patients with sudden hearing loss.

Conclusion: 3 Tesla (3T) magnetic resonance imaging (MRI) performed 4 h after intravenous gadolinium (Gd) injection provides sufficient anatomic resolution of the inner ear fluid spaces in sudden deafness. The signal intensity ratio (SIR) between the cochlea and cerebellum may be a good indicator of disruption of the blood-labyrinthine barrier., Objectives: We evaluated the inner ear 4 h after intravenous Gd injection to determine whether 3T MRI enables the acquisition of images of the affected inner ear in sudden deafness., Methods: Ten patients underwent 3T MRI scanning 4 h after intravenous Gd injection. Three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) MRI was performed., Results: The SIR varied from 0.45 to 2.17 in 11 affected ears and from 0.43 to 1.48 in 9 unaffected ears. The difference of contrast (affected ear vs unaffected ear) could be detected in five of the nine patients with unilateral sudden deafness. The Gd distribution was recognized in the vestibule of 10 affected ears and in the cochlea of 5 affected ears, in which no significant hydrops was observed. In the remaining vestibules and cochleas of affected ears, the Gd enhancement was too faint to evaluate the endolymphatic hydrops.