Unilateral Sensorineural Hearing Loss Research Articles

The impairment of speech perception in noise following pure tone hearing recovery in patients with sudden sensorineural hearing loss

To explore whether patients with unilateral idiopathic sudden sensorineural hearing loss (uISSNHL) have normal speech in noise (SIN) perception under different masking conditions after complete recovery of pure tone audiometry. Eight completely recovered uISSNHL patients were enrolled in ISSNHL group, while 8 normal-hearing adults matched with age, gender, and education experience were selected as the control group. Each group was tested SIN under four masking conditions, including noise and speech maskings with and without spatial separation cues. For both ISSNHL and control groups a two-way ANOVA showed a statistically significant effect of masking type (p = 0.007 vs p = 0.012). A significant effect of perceived spatial separation (p < 0.001 vs p < 0.001). A significant interaction between masking type and perceived spatial separation was found (p < 0.001 vs p < 0.001). A paired sample T-test showed that the SIN perception of the control group was statistically significant lower than that of ISSNHL patients only under speech masking without spatial separation cues (p = 0.011). There were still abnormalities in the auditory center shortly after complete recovery in the ISSNHL group (within 2 weeks). However, the auditory periphery and higher-level ability to use spatial cues was normal.

Caracterização dos pacientes com zumbido atendidos por serviço de Saúde Auditiva

ABSTRACT Purpose: to establish the profile of individuals with tinnitus treated at a Hearing Health service and to associate the symptom with gender, audiological profile, and presence of vertigo. Methods: a descriptive, retrospective study with an analysis of 6,000 medical records of individuals treated at a hearing health service was carried out. The data collected from the medical records were: sociodemographic data, characteristics of hearing impairment, characteristics of tinnitus and vertigo, speech therapy interview, and otorhinolaryngological, audiological, otoneurological assessments, characteristics of life style and risk factors. An descriptive analysis was performed using the Chi-square test of association, adopting a statistical significance level of 5% (p&lt;0.05) for the inferential analysis. Results: there was a statistically significant association between the presence of tinnitus and gender, normal hearing, and vertigo, and a significant association was also found between tinnitus and unilateral and bilateral conductive hearing loss, and unilateral and bilateral sensorineural hearing loss. Conclusion: there was a significant association, in the population evaluated, for the presence of tinnitus and female gender, normal hearing, unilateral and bilateral conductive hearing loss, unilateral and bilateral sensorineural hearing loss, and the presence of vertigo.

Treatment of Meniere's disease by Multi-session Dexamethasone Injections.

Meniere's Disease (MD) is a complex and multifactorial inner ear problem characterized by episodic vertigo, unilateral sensori-neural hearing loss and tinnitus or fullness in the ear due to Endolymphatic hydrops. There are multiple treatment options available for this disease; from which cortisone therapy can be given in different routes and in different doses. is to identify the successfulness of treatment of MD by multi-session intra-tympanic dexamethasone injection compared to the single-session method. Interventional prospective study of study 70 cases of definite MD treated by 4 sessions of intra-tympanic injection weekly and compared to a control group of 66 cases of age matched patients with definite MD treated by the traditional single intra-tympanic injection. Patients In the study and the control group were observed for 6 months after the treatment for the development of any vertiginous attacks. only 3 patients in the study group and 30 patients in the control group failed to respond to treatment and developed vertigo in the period of 6 months after treatment. The difference between the success rate in the two groups was statistically highly significant. multi-session Intra-tympanic dexamethasone injection is superior to the single injection in control of vertigo in patients with definite MD.

Direction-changing spontaneous nystagmus in cerebellopontine angle tumour

The most common symptoms of tumours involving the cerebellopontine angle (CPA) are unilateral sensorineural hearing loss, dizziness, and asymmetric tinnitus. While the clinical manifestations have been well documented in previous studies, the nystagmus findings in these patients have not been thoroughly investigated yet. This study aimed to investigate the incidence of direction-changing spontaneous nystagmus in patients with CPA tumours, evaluate their radiologic characteristics, and gain insight into the mechanisms underlying nystagmus. Direction-changing spontaneous nystagmus was observed in 6 out of 83 patients (7%) with CPA tumours during the 7-year period. Temporal bone magnetic resonance imaging findings revealed the presence of an intrameatal mass in CPA tumours in all six patients with direction-changing spontaneous nystagmus. Vestibular schwannomas were confined within the internal auditory meatus in four patients, and petroclival meningiomas extended into the internal auditory meatus in two patients. The mechanism of direction-changing spontaneous nystagmus may be explained as paroxysmal secondary central hyperactivity in the vestibular nucleus due to the long-standing pressure effect in the vestibular nerve by tumours, or by ephaptic discharges in the vestibular nerve.

Multicenter Results With an Active Transcutaneous Bone Conduction Implant in Patients With Single-sided Deafness.

Objective:To evaluate the audiological and subjective benefit from hearing rehabilitation with an active bone conduction implant in subjects with single-sided sensorineural deafness (SSD).Study Design:Prospective, multicenter, single-subject repeated measures.Setting:Tertiary referral center, five clinics in Germany and Switzerland.Patients:Seventeen subjects aged 18 years and older with severe to profound unilateral sensorineural hearing loss and contralateral normal hearing were followed up for 24 months.Intervention:Active bone conduction implant.Main Outcome Measures:Speech understanding in noise was assessed in three situations: with signal from front, deaf, or normal hearing side (with noise from front in all set-ups). Subjective benefit was evaluated using the Speech, Spatial, and Qualities of Hearing (SSQ-B) and Bern Benefit in Single-Sided Deafness (BBSS) questionnaire.Results:When the signal was coming from the deaf side the mean improvement of the speech reception threshold in noise ranged from 1.5 up to 2.2 dB with the device and was statistically and clinically significant at all tested timepoints. No significant difference between the aided and unaided situation was found when signal and noise were coming from the front. With the signal from the normal hearing side no clinically significant difference, that is, greater than 1 dB between the aided and unaided situation was found. The SSQ-B and BBSS questionnaire showed an overall improvement with no significant difference between time points.Conclusions:The study demonstrates long-term efficacy and benefit of the device in adults with SSD. Patients reported substantial and persistent subjective benefit from the active bone conduction implant.

Diotic and Antiphasic Digits-in-noise Testing as a Hearing Screening and Triage Tool to Classify Type of Hearing Loss.

The digits-in-noise test (DIN) is a popular self-test measure that has traditionally been used to screen for hearing loss by providing either a pass or refer result. Standard approaches either tested each ear monaurally or used a binaural diotic version where identical digits and noise were presented simultaneously to both ears. Recently, a dichotic, antiphasic version was developed, increasing sensitivity of the DIN to unilateral or asymmetric sensorineural hearing loss (SNHL) and conductive hearing loss (CHL). The purpose of this study was to determine predictors and normative ranges of the antiphasic and diotic DIN and to determine if a combination of diotic and antiphasic DIN could accurately categorize hearing into (1) normal, (2) bilateral SNHL, or (3) unilateral SNHL or CHL. The analytical sample consisted of 489 participants between the ages of 18 and 92 years with varying types, symmetry, and degrees of hearing loss. Degree and type of hearing loss were determined based on standard clinical four-frequency (0.5-4 kHz) pure-tone air and bone conduction threshold averages. The sample consisted of bilateral normal hearing (n = 293), bilateral SNHL (n = 172), unilateral SNHL (n = 42), and CHL (n = 32). All participants (n = 489) first completed an antiphasic DIN (digit stimuli 180° out-of-phase between ears), while 393 of the sample also completed a diotic DIN. Two procedures were assessed for their ability to categorize hearing into one of the three hearing groups. The first used a fixed antiphasic cutoff combined with a cutoff formed by a linear combination of antiphasic and diotic speech recognition threshold (SRT) or binaural intelligibility-level difference. Poorer ear pure-tone average was the strongest predictor of antiphasic DIN score, whereas better ear pure-tone average explained more of the variance in diotic SRT. The antiphasic DIN sensitivity and specificity was 90% and 84%, respectively, for detecting hearing loss, with outstanding area under the receiver operating characteristics values exceeding 0.93 to identify hearing loss in the poorer ear. The first fixed SRT cutoff procedure could categorize 75% of all participants correctly, while the second procedure increased correct categorization to 79%. False negative rates for both procedures were below 10%. A sequential antiphasic and diotic DIN could categorize hearing to a reasonable degree into three groups of (1) normal hearing; (2) bilateral SNHL; and (3) unilateral asymmetric SNHL or CHL. This type of approach could optimize care pathways using remote and contactless testing, by identifying unilateral SNHL and CHL as cases requiring medical referral. In contrast, bilateral SNHL cases could be referred directly to an audiologist, or nontraditional models like OTC hearing aids.

A Predictive Model of Bilateral Sensorineural Hearing Loss in Meniere Disease Using Clinical Data.

Meniere disease (MD) is defined by a clinical syndrome of recurrent attacks of spontaneous vertigo associated with tinnitus, aural fullness, and sensorineural hearing loss (SNHL). Most patients have unilateral SNHL, but some of them will develop contralateral SNHL during the course of the disease. Several studies have reported a frequency of 2 to 73% SNHL in the second ear, according to the duration of disease and the period of follow-up. We hypothesize that unilateral and bilateral MD are different conditions, the first would initially involve the apical turn of the cochlea, while bilateral MD would affect the entire length of the cochlea. The aim of the study is to search for clinical predictors of bilateral SNHL in MD to build a predictive model of bilateral involvement. A retrospective, longitudinal study including two cohorts with a total of 400 patients with definite MD was carried out. The inception cohort consisted of 150 patients with MD and the validation cohort included 250 cases. All of the cases were diagnosed of unilateral MD according to their hearing loss thresholds. The following variables were assessed as predictors of bilateral SNHL for the two cohorts: sex, age of onset, familiar history of MD, migraine and high-frequency hearing loss (HFHL, defined if hearing threshold >20 dB in two or more consecutive frequencies from 2 to 8 KHz). A descriptive analysis was carried out according to the presence of HFHL in the first audiogram for the main variables. By using multiple logistic regression, we built-up several predictive models for the inception cohort and validated it with the replication cohort and merged dataset. Twenty-three (19.3%) and 78 (41%) of patients with HFHL developed contralateral SNHL during the follow-up, in the inception and validation cohorts, respectively. In the inception cohort, the best predictive model included HFHL in the first audiogram (OR = 6.985, p = 0.063) and the absence of migraine (OR = 0.215, p = 0.144) as clinical predictors for bilateral SNHL [area under the curve (AUC) = 0.641, p = 0.002]. The model was validated in the second cohort (AUC = 0.621, p < 0.001). Finally, we merged both datasets to improve the precision of the model including HFHL in the first audiogram (OR = 3.168, p = 0.001), migraine (OR = 0.482, p = 0.036) and age of onset >35 years old (OR = 2.422, p = 0.006) as clinical predictors (AUC = 0.639, p < 0.001). A predictive model including the age of onset, HFHL in the first audiogram and migraine can help to assess the risk of bilateral SNHL in MD. This model may have significant implications for clinical management of patients with MD.

Otological Surgery of Acoustic Neuroma

Acoustic neuroma is the most common benign tumor in the internal auditory canal and cerebellopontine region． Its common clinical symptoms include unilateral sensorineural hearing loss, tinnitus and so on． Surgical resection is the predominant treatment of acoustic neuroma. The common approaches dominated by otologists include translabyrinthine approach, enlarged translabyrinthine approach, transotic approach, modified transotic approach, and middle cranial fossa approach; the approach dominated by neurosurgeons is retrosigmoid (suboccipital) approach.. For small tumors with intact hearing, it is recommended to choose the middle cranial fossa approach. Those with large tumors who wish to preserve their hearing can adopt the suboccipital retrosigmoid approach; those who do not consider retaining hearing and have medium or small acoustic neuromas can adopt the translabyrinthe approach or through the transotic approach. With the development of microsurgical technology and the wide application of intraoperative nerve monitoring equipment, the retention rate of facial/cochlear nerve function for surgery of small and medium-sized acoustic neuroma has been significantly improved. In the future, more patients with acoustic neuroma are expected to be completely cured on the basis of preserving facial/cochlear nerve function.

Evaluation of hematological parameters of patients with profound unilateral and mild to profound bilateral sensorineural hearing loss

OBJECTIVE: This study aimed to investigate whether changes in blood cells have an effect on sensorineural hearing loss (SNHL) and compare the hematological parameters of profound unilateral and mild to profound bilateral SNHL patients and the normal population. METHODS: This retrospective study included 100 SNHL patients (81 males, 19 females; mean age: 37.8±16.8 years; range, 18 to 83 years) and 32 healthy volunteers (22 males, 10 females; mean age: 32.8±5.7 years; range 22 to 46 years) between January 2018 and February 2019. Patients who underwent pure tone audiometry and auditory brainstem response (ABR) tests for any reason and were found to have SNHL with no accompanying disease, and those that are non-smokers and without medication were included. Hematological parameters were evaluated in all three groups. RESULTS: Of the 100 SNHL patients, 59 had bilateral and 41 had profound unilateral SNHL. Among the evaluated hematological parameters, the difference in red blood cell count, hematocrit, hemoglobin, platelet distribution width, and neutrophil to lymphocyte ratio (NLR) between the groups was statistically significant (p&lt;0.001). CONCLUSION: Erythrocyte values were high in patients with profound unilateral SNHL, whereas platelet-related values [platelet count, PDW, and platelet to lymphocyte ratio) were higher in patients with bilateral SNHL. In addition, the NLR was found to be high in these patient groups. These findings may provide a treatment target, particularly for profound unilateral SNHL, and may shed light on further studies on SNHL.

A High Risk of Missing Congenital Cytomegalovirus-Associated Hearing Loss through Newborn Hearing Screening in Japan.

It remains unclear to what extent newborn hearing screening (NHS) detects congenital cytomegalovirus (cCMV)-associated sensorineural hearing loss (SNHL) in Japan. This study aimed to clarify the NHS results and audiological characteristics of patients with cCMV-associated SNHL. A total of 541 individuals with unilateral or bilateral hearing loss of unknown etiology were examined for cCMV infection. cCMV infection was defined by the presence of CMV DNA in the dried umbilical cord detected using real-time quantitative PCR. NHS results and audiological data were retrospectively obtained from medical records. Forty-four cases (8.1%) were positive for cCMV infection. Of them, 33 cases underwent NHS and 13 cases (39.4%) passed NHS bilaterally. The pure-tone audiograms of 21 patients were obtained. There were seven cases of unilateral SNHL, five cases of asymmetric bilateral SNHL, and nine cases of symmetric bilateral SNHL. cCMV-related hearing loss is highly heterogeneous, and there is a high risk of missing this condition through NHS.

MRI characteristics of intralabyrinthine schwannoma on post-contrast 4 h-delayed 3D-FLAIR imaging

PurposeThe purpose of this study was to describe the MRI characteristics of intralabyrinthine schwannoma (ILS) on post contrast three-dimensional (3D) fluid-attenuation-inversion-recovery (FLAIR) images obtained four hours after intravenous administration of a gadolinium-based contrast agent (4h-3D-FLAIR). Materials and methodsThis IRB-approved retrospective multi-center study included patients presenting with typical ILS from January 2016 to October 2020. All medical charts were systematically collected. All MRI examinations, including 4h-3D-FLAIR images, were reviewed by two board-certified neuroradiologists. Main outcome measures were location, signal intensity and associated anomalies of ILS. ResultsTwenty-seven out of 8730 patients (0.31%) referred for the investigation of a cochleovestibular disorder had a final diagnosis of ILS. There were 13 men and 14 women with a mean age of 52 ± 17 (SD) years (age range: 20–86 years). The most common clinical presentation was unilateral progressive sensorineural hearing loss (16/27; 59%). All ILS were unilateral and 15 (15/27; 55%) were intracochlear. All ILS presented as a hypointense filling defect within the labyrinth on T2-weighted images that enhanced on post-contrast T1-weighted images. On 4h-3D-FLAIR images, all ILS presented as a hypointense filling defect, associated with diffuse perilymphatic hyperintensity. Two patients (2/27; 7%) presented with ipsilateral endolymphatic hydrops. ConclusionILS displays consistent features on post-contrast 4h-3D-FLAIR images. ILS should not be confused with endolymphatic hydrops and requires a systematic analysis of the corresponding T2-weighted images.

Unilateral Sensorineural Hearing Loss in Children Associated With Sjögren's Syndrome

The occurrence of unilateral sensorineural hearing loss (SNHL) during school age is relatively rare and accounts for approximately 6% of all deafness in childhood. We present two cases involving children who were diagnosed with SNHL associated with Sjögren's syndrome (SS). Case 1: An eight-year-old girl with an approximately two-year clinical history of gradual hearing loss was diagnosed with SNHL associated with SS based on histological findings of inflammation in the salivary glands and the presence of serum anti-Sjögren's syndrome-A antibody. Case 2: An eight-year-old boy with acute idiopathic thrombocytopenic purpura in whom unilateral hearing loss, which was not associated with any problems in daily life, was detected during hospitalization and who was finally diagnosed with SNHL and SS. Steroid treatment was ineffective for both patients. The previously unrecognized combination of SNHL with SS should be considered in the diagnosis of unilateral SNHL, even in children.

1262 Audit of MRI IAM (Internal Auditory Meatus) Requests at Darlington Memorial Hospital and Review of The Literature

Abstract Aim To assess if the MRI IAM scans are being requested as suggested in NICE guidelines Draft 2017 for consultations. Educate and implement any changes if needed to minimise unnecessary investigations. Method Retrospective data collection of 1000 data. Variables included hearing loss (HL), tinnitus (T), dizziness (D) and other lateralized symptoms. Audiograms assessed for asymmetrical HL at decibel level ≥20 Db at a frequency of 0.5 kHz, 1.0 kHz, 2.0 kHz. MRI of IAM assessed if a cerebellar pontine angle (CPA) lesion was presented. Results 853 (85.3%) met criteria. 16/1000 (1.6%) MRI showed CPA lesion. All 16 cases had unilateral sensorineural hearing loss (SNHL) and met NICE guidelines criteria. Discussion Limited value in the scanning of asymptomatic patients, those who do not fit audiometric guidelines, those bilateral symptoms or those with unilateral tinnitus without audiometric asymmetry. Very few studies were found in the literature in relation to symptoms associated with positive findings of CPA lesion. Our results indicate unilateral SNHL were linked with positive findings of MRI and it is similar findings in the literature. We recommend that patients who present with asymmetrical SNHL with this audiometric asymmetrical characteristic of 20Db or more will need an urgent MRI IAM as part of their diagnostic workup.

Spectrum of Handicap in Unilateral Sensorineural Hearing Loss.

Unilateral Sensorineural Hearing Loss (USNHL) can present with varying symptoms apart from hearing loss, such as dizziness and tinnitus. Impaired sound localization, inability to identify speech in noise and decreased temporal summation can significantly impair the daily activities of an individual. This along with dizziness and tinnitus affects the physical, mental and social health as well. The purpose of this study is to assess and correlate the spectrum of handicap faced by USNHL patients. Validated questionnaires were used to evaluate handicap in 42 USNHL patients due to hearing loss (Hearing Handicap Inventory for Adults-HHIA), dizziness (Dizziness Handicap Inventory-DHI) and tinnitus (Tinnitus Handicap Inventory-THI). These handicaps were then correlated with each other using Spearman's correlation coefficient. Statistically significant strong positive correlation of HHIA was seen with DHI (rho = 0.60, p ≤ 0.001) and THI (rho = 0.74, p ≤ 0.001). Similarly, strong positive correlation was seen between emotional subscales of HHIA and DHI (rho = 0.73, p ≤ 0.001). USNHL can lead to significant emotional and social handicap which is compounded in the presence of hearing loss, dizziness and tinnitus. These handicaps are strongly correlated to one another. Hence, a holistic approach is needed for USNHL rehabilitation. There exists a need to develop a comprehensive measure to assess and grade the handicap faced by these patients.

Transcutaneous Osseo-integrated Auditory Devices: Analysis of Two Different Implants in Adults With Different Audiological Inclusion Criteria.

To determine and compare audiological and speech/language discrimination results in patients with osteo-integrated auditory devices, by comparing two different systems including different audiological indications in adult population. Descriptive and analytic, cross-sectional, cohort, and retrospective case review. Tertiary referral center. Fifty patients >14 years old, with conductive, mixed, or unilateral sensorineural hearing loss were compared. The variables studied were the following: pure tone audiometry results, air-bone gap, and the percentage of speech/language discrimination using Disyllabic Word Test. The patients were tested preoperative and 12 months after surgical procedure. Subsequently, a comparative analysis of the both systems was carried out. With both implants, pure tone audiometry results and the difference in auditory thresholds and between the air-bone gap improved significantly. Speech discrimination increased significantly after implantation too. A significant difference was observed after implantation of both devices. Patients implanted with one of the implants showed better audiological results, but no significant differences were found with the other device. So, it was shown that the type of device and the type of hearing loss have no influence on the audiological results or complications. Both implants represent a good choice for audiological rehabilitation in patients with transmissive hearing loss, mixed hearing loss, or unilateral sensorineural hearing loss with a high success and low complication rates.

The newborn hearing screening results in Kastamonu Education and Research Hospital

Purpose: The purpose of this study is to report the results of newborn hearing screening performed in 2017-2020 at the Kastamonu Education and Research Hospital, Turkey. Material and Method: Six thousand seven hundred and ninety newborns undergoing hearing screening between January 2017 and December 2019 at the Kastamonu Education and Research Hospital were included in this retrospective study. The screening ABR test was performed with all babies. Test findings retrieved from our hospital and the central database were subjected to analysis. Results: 4701 babies passed the 1st test, 1920 babies passed the 2nd test, 139 babies passed the 3rd test. Of the 30 babies who could not pass these 3 screening ABR tests and were referred to the reference center, 19 passed the test. Of the remaining 11 babies (0.162%), 7 (0.103%) had unilateral sensorineural hearing loss (SNHL) and 4 (0.058%) had bilateral SNHL. Conclusion: It is very important for hearing loss to be detected via screening tests in the first three months and for treatment and rehabilitation to be started within six months at the latest. These babies can thus acquire normal hearing and not lag behind their peers in terms of linguistic, social, and cognitive skill development.

Challenges of Cochlear Implantation in Intralabyrinthine Schwannoma Patients: Surgical Procedures and Auditory Outcome.

Intralabyrinthine schwannoma (ILS) is a rare benign tumor of the inner ear potentially causing unilateral sensorineural hearing loss and vertigo. This study evaluated the outcome of one surgical session comprising microsurgical ILS resection and cochlear implantation in terms of surgical feasibility, complications, and auditory outcome. Ten clinically and histologically confirmed ILS patients included in this study (three women and seven men; mean age 56.4 ± 8.6) underwent surgery between July 2015 and February 2020. Eight patients had intracochlear tumor location; the remaining two had vestibulocochlear and intravestibular ILS. One of the three following methods was used for tumor removal: an extended cochleostomy, subtotal cochleoectomy, or a translabyrinthine approach. Although negligible improvement was observed in two of the patients, two patients were lost to follow-up, and one opted out from using CI, the speech perception of the five remaining ILS patients improved as per the Freiburg Monosyllable Test (FMT) from 0% before surgery to 45– 50% after the implantation. Our study supports the presented surgical approach’s feasibility and safety, enabling tumor removal and hearing restoration shortly after surgery.

Ear fullness characteristics and prognosis in patients with all-frequency sudden sensorineural hearing loss.

This study investigated the characteristics and prognosis of the feeling of ear fullness in patients with unilateral all-frequency sudden sensorineural hearing loss. Our study included 56 patients with a diagnosis of unilateral all-frequency sudden sensorineural hearing loss accompanied by a feeling of ear fullness and 48 patients without a feeling of ear fullness. The condition of these patients was prospectively observed. Positive correlations were observed between grading of feeling of ear fullness and hearing loss in patients with a feeling of ear fullness (r = 0.599, p < 0.001). No significant differences were observed in the total effective rate of hearing recovery between patients with and without a feeling of ear fullness after one month of treatment (Z = -0.641, p = 0.521). Eighty-six per cent of patients (48 out of 56) showed complete recovery from the feeling of ear fullness. There was no correlation between feeling of ear fullness recovery and hearing recovery (r = 0.040, p = 0.769). The prognosis of feeling of ear fullness is good. There was no correlation between feeling of ear fullness recovery and hearing recovery for all-frequency sudden sensorineural hearing loss patients.

MR Imaging of Cochlear Modiolus and Endolymphatic Hydrops in Patients With Menière's Disease.

Background: Menière's disease (MD) is an inner ear disorder characterized by recurrent episodes of spontaneous vertigo, unilateral low-frequency sensorineural hearing loss, tinnitus, and aural fullness. Current diagnosis still often has to rely on subjective and audiometric criteria only, although endolymphatic hydrops is recognized as the pathophysiological substrate of the disease, having been demonstrated in anatomical pathological studies and by magnetic resonance (MRI). The modiolus has a close functional and anatomical relationship with the cochlear nerve and membranous labyrinth and can be evaluated with MRI but no data exist on the modiolar size in MD.Purpose: Our purpose is to examine the following hypothesis. Is cochlear modiolus smaller in symptomatic ears in MD?Methods: We used a retrospective 3 Tesla MR study (heavily T2-weighted 3D fast asymmetric spin-echo images and 0.5 mm slice thickness) comparing the mean modiolar area (MMA) in the index and best ears of eight patients with definite MD based on audiometric data. The obtained MMA values were compared against the audiometric data and the presence of vestibular endolymphatic hydrops.Results: No differences were seen in MMA between best and worst ears. Ears with a pure tone average (PTA) ≥25 dB and more pronounced endolymphatic hydrops showed lower MMA (not statistically significant). Two patients with extreme endolymphatic hydrops showed a noteworthy ipsilateral decrease in the cochlear modiolus area.Conclusion: No differences were seen in MMA between best and worst ears in definite MD. Worse hearing function (PTA ≥ 25dB) and more pronounced endolymphatic hydrops seem to be associated with lower MMA. This might be related to bone remodeling as a consequence of endolymphatic hydrops. Further research is needed to corroborate and explore these findings.

Disrupted Topological Organization in White Matter Networks in Unilateral Sudden Sensorineural Hearing Loss.

Sudden sensorineural hearing loss (SSNHL) is a sudden-onset hearing impairment that rapidly develops within 72 h and is mostly unilateral. Only a few patients can be identified with a defined cause by routine clinical examinations. Recently, some studies have shown that unilateral SSNHL is associated with alterations in the central nervous system. However, little is known about the topological organization of white matter (WM) networks in unilateral SSNHL patients in the acute phase. In this study, 145 patients with SSNHL and 91 age-, gender-, and education-matched healthy controls were evaluated using diffusion tensor imaging (DTI) and graph theoretical approaches. The topological properties of WM networks, including global and nodal parameters, were investigated. At the global level, SSNHL patients displayed decreased clustering coefficient, local efficiency, global efficiency, normalized clustering coefficient, normalized characteristic path length, and small-worldness and increased characteristic path length (p < 0.05) compared with healthy controls. At the nodal level, altered nodal centralities in brain regions involved the auditory network, visual network, attention network, default mode network (DMN), sensorimotor network, and subcortical network (p < 0.05, Bonferroni corrected). These findings indicate a shift of the WM network topology in SSNHL patients toward randomization, which is characterized by decreased global network integration and segregation and is reflected by decreased global connectivity and altered nodal centralities. This study could help us understand the potential pathophysiology of unilateral SSNHL.