Young Joon Seo

Visualization of endolymphatic hydrops and correlation with audio-vestibular functional testing in patients with definite Meniere's disease

OBJECTIVES Visulization of endolymphatic hydrops has been reported using magnetic resonance imaging (MRI) in patients with Menieres disease. However, the relationship between the endolymphatic hydrops visualized by MRI and audio-vestibular functional tests have not been sufficiently investigated, such as pure tone audiometry (PTA), electrocochleography (EcoG), and vestibular evoked myogenic potential (VEMP). METHODS Here we attempted to visualize endolymphatic hydrops in patients with Menieres disease using 3Tesla (3T)-MRI following intratympanic gadolinium-diethylene-triamnie petaaetic acidbis (Gd-DTPA) injection and correlated the presence of hydrops with auditory testing. Following intratympanic Gd-DTPA contrast injections in 26 patients diagnosed as having definite Menieres disease, 3D-fluid-attenuated inversion recovery (3D-FLAIR) sequence imagings were performed. The PTA, EcoG, and VEMP testing was done bilaterally. Patients had typical results of auditory testing for Menieres disease. RESULTS The 3D-FLAIR MRI clearly demonstrated endolymphatic hydrops with signal voids in the cochlea (81%) and saccule (69%) in the definite Menieres disease group. Auditory tests verified that the visualized cochlear hydrops (basal turn) was correlated with a high tone hearing threshold in PTA and an abnormal EcoG. Though the absence of VEMP did not correlate with vestibular hydrops on MRI, large vestibular hydrops were detected by imaging. This study demonstrates that endolymphatic hydrops could be visualized by 3D-FLAIR MRI in Menieres disease patients. CONCLUSION Cochlea hydrops and vestibular (saccular) hydrops are readily visualized using these techniques. Hydrops, as visualized on MRI, may be a reliable means to diagnosis Menieres disease; this is supported by appropriate correlations with auditory vestibular functional testing.

Genetic aspects and clinical characteristics of familial meniere's disease in a South Korean population

This study was undertaken to investigate the prevalence, inheritance patterns, and clinical characteristics of familial Menieres disease (MD) in a South Korean population.

Cochlear implantation in a bilateral sensorineural hearing loss patient with relapsing polychondritis

Relapsing polychondritis (RP) is a rare disease which presents chondritis in multiple organs. Characteristic features include auricular chondritis, arthritis, nasal chondritis, ocular inflammation, respiratory tract involvement and audiovestibular damage. Fifty percent of cases of RP are associated with inner ear symptoms such as dizziness and hearing loss. We have recently encountered a case of RP in a 34-year-old man who had recurrent chondritis of both auricles and progressive bilateral profound sensorineural hearing loss; he had been treated many times with steroids, immunosuppressants, plasmapheresis treatments. We perfomed a successful cochlear implant surgery on the left ear of this patient. This raises the possibility of using cochlear implants in treating patients with immune-mediated inner ear disease as well as such RP patients.

Clinical Features and Prognosis of Sudden Sensorineural Hearing Loss Secondary to Intralabyrinthine Hemorrhage

Background and Objectives A number of etiologies of idiopathic sudden sensorineural hearing loss (ISSNHL) have been proposed, including viral infection, vascular disturbance, and immune-mediated mechanisms. Intralabyrinthine hemorrhage (ILH) as a cause of SSNHL is extremely rare, and there have been no studies defining the characteristics of hearing impairment and prognosis in patients with ISSNHL due to ILH. This study aimed to investigate the difference in impaired hearing patterns and prognosis for hearing recovery between patients with ISSNHL due to ILH confirmed by magnetic resonance imaging (MRI) and sex- and age-matched patients with ISSNHL due to causes other than ILH. Subjects and Methods We compared the results of audiometry and MRI in 12 patients who had ILH on MRI (hemorrhage group) and in 23 sex- and age-matched controls without abnormal findings related to their hearing loss on MRI (non-hemorrhage group). Initial hearing impairment, progression, and recovery of hearing loss were compared between the two groups. Results A majority of patients (92%) in the hemorrhage group complained of dizziness. Initial hearing impairment was more frequent in the hemorrhage group than in the non-hemorrhage group (94.09±35.9 vs. 66.66±30.1, p-value=0.036). The final recovery threshold in the hemorrhage group was worse (78.19±46.26 vs. 37.17±31.96, p-value=0.014) than that in the non-hemorrhage group. In the hemorrhage group, hearing recovery seemed to occur less often at high frequencies (2,000, 4,000, and 8,000 Hz) than at low frequencies (250, 500, and 1,000 Hz). Conclusions The presence of ILH was associated with poor hearing prognosis and the occurrence of vertigo. The abrupt onset of hearing loss associated with vertigo and the presence of hyperresonance on fat-suppressed T1-weighted MRI images of labyrinthic fluid strongly suggests acute intralabyrinthine hemorrhage, and is predictive of considerable hearing impairment and poor prognosis.

Predictive value of neutrophil to lymphocyte ratio in first-time and recurrent idiopathic sudden sensorineural hearing loss

OBJECTIVE To determine if the neutrophil to lymphocyte ratio (NLR) and platelet to lymphocyte ratio (PLR) in patients with recurrent idiopathic sudden sensorineural hearing loss (ISSNHL) differ from those in healthy people and non-recurrent ISSNHL patients. METHODS A retrospective review of 16 patients with recurrent ISSNHL was performed with strict exclusion criteria. Independent variables were NLR and PLR, which yielded four groups (control, non-recurrent, recurrent-first attack, and recurrent-second attack groups). RESULTS Compared with the control group, non-recurrent and recurrent attack groups showed significantly higher NLR values (p<0.001, respectively). The NLR (odds ratio [OR] 2.178, 95% confidence interval [CI] 1.587-2.988; p<0.001) and PLR (OR, 1.013, 95% CI, 1.006-1.020; p<0.001) were found to be independent risk factors only between the control and recurrent-first attack groups. CONCLUSION Evidence of increased NLR and PLR is shown in recurrent ISSNHL and non-recurrent ISSNHL, and these increased levels are shown in the second attack of recurrent ISSNHL. Clinicians are advised to monitor the NLR and PLR at the clinic to predict recurrent ISSNHL even after hearing is restored.

Resistance to hypertension and high Cl(-) excretion in humans with SLC26A4 mutations.

Pendrin is a membrane transporter encoded by solute carrier family26A4 (SLC26A4). Mutations in this gene are known to cause hearing loss, and recent data from animal studies indicate a link between pendrin expression and hypertension; although, this association in humans is unclear. To clarify this issue, we investigated the influence of pendrin on blood pressure by analyzing demographic and biochemical data – including blood pressure and urinary electrolyte excretion – in patients with bi‐allelic SLC26A4 mutations. Systolic and diastolic blood pressure and the left ventricular hypertrophy index were lower in subjects with pendrin mutations than in controls. In addition, fractional excretion of Na+ and Cl− was increased and serum renin, angiotensin I and II levels were higher in subjects with pendrin mutations as compared to controls. Thus, patients with impaired pendrin function are likely to be resistant to high blood pressure due to enhanced urinary Na+/Cl− excretion. These results suggest that pendrin may regulate blood pressure through increased urinary salt excretion.

Correlation of vestibular aqueduct size with air–bone gap in enlarged vestibular aqueduct syndrome

Patients with enlarged vestibular aqueduct (EVA) often demonstrate an air–bone gap (ABG) at low frequencies on audiometric testing. The mechanism for this has not been well elucidated. We investigated vestibular aqueduct (VA) size and ABG in patients with EVA, and assessed the relationship between VA diameter and ABG.

Expression and Localization of Aquaporin Water Channels in Human Middle Ear Epithelium.

Background Although aquaporins (AQPs) are known to play critical roles as the basis for water and solute transport in water homeostasis, AQPs in normal human middle ear epithelium (NHMEE) has not previously been investigated. Objectives/Hypothesis To investigate the expressions of AQP water channels in NHMEE in situ, in proliferating epithelial cell cultures in vitro. Methods AQP 0–12 expressions by cultured NHMEE cells in situ were assessed by reverse transcriptase–polymerase chain reaction. Normal middle ear epithelial tissue was harvested and investigated for expressions of AQPs (1, 3, 4, and 5) by immunohistochemistry. Expression screening was also carried out on the differentiated NHMEE cells. Results Transcripts for AQP 1, 2, 3, 4, 5, 6, 8, 10, and 11 were expressed consistently in cultured NHMEE cells; however, AQP 0, 7, 9, and 12 subtypes were not expressed. Immunochemistry confirmed the expressions of AQP 1, 3, and 5 at the protein level. AQP 1 was localized at capillary endothelial cells and fibroblasts in lamina propria mucosae; AQP 3 was present solely at the basolateral membrane of ciliated cells, whereas AQP 5 was on the apical surface of ciliated cells. AQP 3 and 5 were intensely expressed in both cultured NHMEE cells in situ and NHMEE tissue in vitro. Conclusion This is the first study to demonstrate that AQPs are expressed by human middle ear epithelium in situ and in vitro, suggesting a potential role in otitis media with effusion. Our study suggests that the presence of AQP 1, 3, and 5 in the middle ear cavity may be to have an important role for water transportation.

Enhanced Homing Technique of Mesenchymal Stem Cells Using Iron Oxide Nanoparticles by Magnetic Attraction in Olfactory-Injured Mouse Models

Intranasal delivery of mesenchymal stem cells (MSCs) to the olfactory bulb is a promising approach for treating olfactory injury. Additionally, using the homing phenomenon of MSCs may be clinically applicable for developing therapeutic cell carriers. Herein, using superparamagnetic iron oxide nanoparticles (SPIONs) and a permanent magnet, we demonstrated an enhanced homing effect in an olfactory model. Superparamagnetic iron oxide nanoparticles with rhodamine B (IRBs) had a diameter of 5.22 ± 0.9 nm and ζ-potential of +15.2 ± 0.3 mV. IRB concentration of 15 µg/mL was injected with SPIONs into MSCs, as cell viability significantly decreased when 20 μg/mL was used (p ≤ 0.005) compared to in controls. The cells exhibited magnetic attraction in vitro. SPIONs also stimulated CXCR4 (C-X-C chemokine receptor type 4) expression and CXCR4-SDF-1 (Stromal cell-derived factor 1) signaling in MSCs. After injecting magnetized MSCs, these cells were detected in the damaged olfactory bulb one week after injury on one side, and there was a significant increase compared to when non-magnetized MSCs were injected. Our results suggest that SPIONs-labeled MSCs migrated to injured olfactory tissue through guidance with a permanent magnet, resulting in better homing effects of MSCs in vivo, and that iron oxide nanoparticles can be used for internalization, various biological applications, and regenerative studies.

Early deterioration of residual hearing in patients with SLC26A4 mutations.

To compare changes in hearing in patients with SLC26A4 during early and late childhood.