BACKGROUND AND OBJECTIVES: We investigated whether the intratympanic steroid injections might be an effective initial treatment for sudden sensorineural hearing loss (SSNHL) in patients with diabetes mellitus (DM). SUBJECTS AND METHOD: We assessed a total of 167 patients with DM and SSNHL who visited a tertiary referral otolaryngology department between January 2010 and April 2018. Forty-two patients with DM and SSNHL received intratympanic steroid injections; 48 patients with DM and SSNHL received systemic steroid treatment; and 77 patients with DM and SSNHL received a combination of systemic and intratympanic treatment. Initial and post treatment hearing levels and fasting blood sugar (FBS) were assessed, and correlations between hearing gain and the duration of DM, HbA1c, FBS were investigated. RESULTS: After steroid treatment, hearing levels were 38.87±25.35 dB in the intratympanic injection group, 41.09±28.49 dB in the systemic steroid treatment group, and 47.81±27.12 dB in the combined treatment group. Final hearing levels and hearing gain in the three groups did not differ significantly. FBS after treatment in the systemic steroid treatment and combined steroid treatment group worsened relative to the intratympanic injection group (202.00±9.40 mg/dL to 326.63±7.85 mg/dL). FBS, duration of DM, and HbA1c levels did not affect the hearing gain in patients with DM and SSNHL. CONCLUSION: Intratympanic steroid injections are comparable to the systemic steroid treatment modality for SSNHL in patients with DM. Thus, intratympanic steroid injections may serve as an effective initial treatment modality for SSNHL in patients with DM.
Blood Glucose ; Diabetes Mellitus ; Fasting ; Hearing ; Hearing Loss, Sensorineural ; Humans ; Injection, Intratympanic ; Methods ; Otolaryngology ; Referral and Consultation ; Steroids

BACKGROUND AND OBJECTIVES: Sensorineural hearing loss (SNHL) in children is associated with neurocognitive morbidity. The cause of SNHL is a loss of hair cells in the organ of Corti. There are currently no reparative treatments for SNHL. Numerous studies suggest that cord blood mononuclear cells (human umbilical cord blood, hUCB) allow at least partial restoration of SNHL by enabling repair of a damaged organ of Corti. Our objective is to determine if hUCB is a safe treatment for moderate to severe acquired SNHL in children. SUBJECTS AND METHODS: Eleven children aged 6 months to 6 years with moderate to severe acquired SNHL were treated with intravenous autologous hUCB. The cell dose ranged from 8 to 30 million cells/kg body weight. Safety was assessed by measuring systemic hemodynamics during hUCB infusion. Infusion-related toxicity was evaluated by measuring neurologic, hepatic, renal and pulmonary function before and after infusion. Auditory function, auditory verbal language assessments and MRI with diffusion tensor imaging (DTI) were obtained before and after treatment. RESULTS: All patients survived, and there were no adverse events. No infusionrelated changes in hemodynamics occurred. No infusion-related toxicity was recorded. Five subjects experienced a reduction in auditory brainstem response (ABR) thresholds. Four of those 5 subjects also experienced an improvement in cochlear nerve latencies. Comparison of MRI with DTI sequences obtained before and after treatment revealed increased fractional anisotropy in the primary auditory cortex in three of five subjects with reduced ABR thresholds. Statistically significant (p < 0.05) reductions in ABR thresholds were identified. CONCLUSIONS: TIntravenous hUCB is feasible and safe in children with SNHL.
Anisotropy ; Auditory Cortex ; Body Weight ; Child ; Cochlear Nerve ; Diffusion Tensor Imaging ; Evoked Potentials, Auditory, Brain Stem ; Fetal Blood ; Hair ; Hearing Loss, Sensorineural ; Hemodynamics ; Humans ; Magnetic Resonance Imaging ; Mesenchymal Stromal Cells ; Organ of Corti ; Umbilical Cord

BACKGROUND AND OBJECTIVES: The cause of idiopathic sudden sensorineural hearing loss (ISSNHL) is still unclear, but recently, chronic inflammation and thrombosis have received attention. The neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) are some of the markers that show the state of inflammation and ischemia, which are measured routinely in the complete blood cell count (CBC) test. The aim of this study were to investigate the relevance of NLR and PLR with ISSNHL. SUBJECTS AND METHOD: Enrolled in our retrospective analysis were 103 patients diagnosed with ISSNHL. Blood samples were taken from the patients and hearing assessments were performed. NLR and PLR were calculated using the CBC results. Then the patients were divided into 4 groups using Sigel's criteria according to their response to the treatment, which were again classified two groups, the “recovered” and “unrecovered” group. RESULTS: NLR, PLR, and neutrophil values of the unrecovered group were significantly higher than the recovered group (p=0.002, p=0.009, and p=0.038, respectively). On the other hand, lymphocyte values were significantly higher in the recovered group (p=0.007). After adjustment in a multivariate logistic regression analysis, NLR was associated with the recovery of ISSNHL (Odds ratio=1.290, p=0.042). In addition, NLR and PLR values were also significantly different between the groups classified by the Sigel's criteria (p=0.009 and p=0.029, respectively). CONCLUSION: PLR values may be useful in predicting hearing recovery after treatment in patients with ISSNH. It is also expected to be a potential marker for predicting the prognosis and determining further treatment options.
Biomarkers ; Blood Cell Count ; Hand ; Hearing ; Hearing Loss, Sensorineural* ; Humans ; Inflammation ; Ischemia ; Logistic Models ; Lymphocytes ; Methods ; Neutrophils ; Prognosis* ; Retrospective Studies ; Thrombosis

BACKGROUNDThe prevalence of sudden sensorineural hearing loss in children (CSSNHL) is consistently increasing. However, the pathology and prognosis of CSSNHL are still poorly understood. This retrospective study evaluated clinical characteristics and possible associated factors of CSSNHL.

METHODSOne hundred and thirty-six CSSNHL patients treated in Department of Otolaryngology-Head and Neck Surgery and Institute of Otolaryngology at Chinese PLA General Hospital between July 2008 and August 2015 were included in this study. These patients were analyzed for clinical characteristics, audiological characteristics, laboratory examinations, and prognostic factors.

RESULTSAmong the 136 patients (151 ears), 121 patients (121 ears, 80.1%) were diagnosed with unilaterally CSSNHL, and 15 patients (30 ears, 19.9%) with bilateral CSSNHL. The complete recovery rate of CSSNHL was 9.3%, and the overall recovery rate was 37.7%. We found that initial degree of hearing loss, onset of treatment, tinnitus, the ascending type audiogram, gender, side of hearing loss, the recorded auditory brainstem response (ABR), and distortion product otoacoustic emissions (DPOAEs) had prognostic significance. Age, ear fullness, and vertigo had no significant correlation with recovery. Furthermore, the relevant blood tests showed 30.8% of the children had abnormal white blood cell (WBC) counts, 22.1% had elevated homocysteine levels, 65.8% had high alkaline phosphatase (ALP), 33.8% had high IgE antibody levels, and 86.1% had positive cytomegalovirus (CMV) IgG antibodies.

CONCLUSIONSCSSNHL commonly occurs unilaterally and results in severe hearing loss. Initial severe hearing loss and bilateral hearing loss are negative prognostic factors for hearing recovery, while positive prognostic factors include tinnitus, gender, the ascending type audiogram, early treatment, identifiable ABR waves, and DPOAEs. Age, vertigo, and ear fullness are not correlated with the recovery. Some serologic indicators, including the level of WBC, platelet, homocysteine, ALP, positive CMV IgG antibody, fibrinogen, and some immunologic indicators, are closely related to CSSNHL.

Adolescent ; Child ; Child, Preschool ; Evoked Potentials, Auditory, Brain Stem ; Female ; Hearing Loss, Sensorineural ; blood ; etiology ; physiopathology ; Humans ; Male ; Otoacoustic Emissions, Spontaneous ; Retrospective Studies

BACKGROUND: Sensorineural hearing loss (SNHL) is a form of diabetic neuropathy. Its prevalence rate varies from 21.7-73.3% among different populations. The association of this complication with long-term glycemic control has not been described extensively.
OBJECTIVES: The study aims to determine the prevalence of SNHL in Filipino patients with diabetes consulting in a tertiary hospital; and to determine the association of SNHL with the degree of blood sugar control as measured by the mean hemoglobin bA1c (HbA1c) for the last five years.
METHODOLOGY: A cross-sectional study of 128 patients in a tertiary hospital was done. Patients were recruited via stratified random sampling with the different clinics as the stratifying variable. They underwent physical examination and pure tone audiometry (PTA) to detect presence of SNHL and presence of distal peripheral neuropathy. Chart review was done to gather the HbA1c levels for the last five years, as well as data on the presence of retinopathy and nephropathy. The average HbA1c levels, and other clinical and demographic factors and their association with SNHL were analyzed using logistic regression.
RESULTS: The prevalence of SNHL among patients with diabetes is 45.31%. Glycemic control does not seem to be associated with SNHL (p value 0.451, OR 1.447). Age was found to be significantly associated with SNHL (p value=0.046, OR=1.035). Among patients age 60 years old and below, retinopathy was significantly associated with SNHL (p value 0.023, OR=3.564). Multivariate analysis did not show any significant predictor for SNHL. There was no observed difference in the proportion of patients with SNHL among males (48.94%) compared to females (43.21%), p value of 0.530. A more advanced age is associated with SNHL among males (p value 0.024, OR=1.095) and a family history of hearing loss is an independent predictor of SNHL (p value 0.047, OR=1.088).
CONCLUSION: There is a high prevalence rate of SNHL among Filipino patients with diabetes. SNHL does not seem to be associated with glycemic control. Screening for SNHL maybe warranted for patients with diabetes due to its high prevalence rate regardless of glycemic control. Hearing care, focusing on prevention of hearing loss, should be advocated for patients with diabetes mellitus

Human ; Male ; Female ; Aged ; Middle Aged ; Adult ; Audiometry, Pure-tone ; Blood Glucose ; Deafness ; Diabetic Neuropathies ; Hearing Loss ; Hearing Loss, Sensorineural ; Diabetes Mellitus ; Patients

BACKGROUND AND OBJECTIVES: Several prognostic factors are known to be related to the recovery of sudden sensorineural hearing loss (SSNHL). Recent studies have suggested that cardiovascular risk factors (CVRFs) are associated with the occurrence of SSNHL. However, the value of CVRFs as a predictor of recovery in patients with SSNHL has been rarely evaluated. We aim to evaluate the prognostic value of CVRFs in relation to hearing recovery of SSNHL. SUBJECTS AND METHOD: A total of 278 patients who were diagnosed and treated for SSNHL and who underwent blood sampling and follow-ups for more than 3 months were reviewed retrospectively. We reviewed CVRFs such as age, body mass index, blood pressure, cholesterol, smoking history, the presence of diabetes mellitus and other related underlying diseases. Patients were divided into three groups (low, medium, and high CVRF groups) according to the CVRF grades. Hearing thresholds were repeatedly measured on the initial visit, 1 week, 1 month and 3 months after treatment. Treatment outcome was analyzed by comparing hearing recovery rate and post-treatment audiometric changes among the three CVRF groups. RESULTS: Seventy (25.2%), 129 (46.4%) and 79 (28.4%) patients were included into the low, medium and high CVRF groups, respectively. The hearing threshold was significantly reduced at 3 months after treatment in all three groups (p<0.001). The hearing recovery rate of the low CVRF group was significantly higher than that of the medium and high CVRF group (p=0.011). On the last visit, the high CVRF group significantly showed more poor hearing improvement than the low CVRF group did (p=0.045). CONCLUSION: Our findings suggest that the presence of CVRFs may be a poor prognostic sign for hearing recovery in patients with SSNHL.
Blood Pressure ; Body Mass Index ; Cholesterol ; Diabetes Mellitus ; Follow-Up Studies ; Hearing Loss, Sensorineural* ; Hearing* ; Humans ; Methods ; Retrospective Studies ; Risk Factors* ; Smoke ; Smoking ; Treatment Outcome

Previously, we reported the expression levels of specific microRNA machinery components, DGCR8 and AGO2, and their clinical association in patients with idiopathic sudden hearing loss (SSNHL). In the present study, we investigated the other important components of microRNA machinery and their association with clinical parameters in SSNHL patients. Fifty-seven patients diagnosed with SSNHL and fifty healthy volunteers were included in this study. We evaluated mRNA expression levels of Dicer and Drosha in whole blood of patients with SSNHL and the control group, using RT & real-time PCR analysis. The Dicer mRNA expression level was down-regulated in patients with SSNHL. However, the Drosha mRNA expression level was not significantly altered in patients with SSNHL. Neither the Dicer nor Drosha mRNA expression level was not associated with any clinical parameters, including age, sex, duration of initial treatment from onset (days), initial Pure tone average, Siegel's criteria, WBC, and Erythrocyte sedimentation rate. However, mRNA expression levels of Dicer and Drosha were positively correlated to each other in patients with SSNHL. In this study, we demonstrated for the first time that the Dicer mRNA expression level was down-regulated in patients with SSNHL, suggesting its important role in pathobiology of SSNHL development.
Acute Disease ; Adult ; Biomarkers ; DEAD-box RNA Helicases/*blood ; Down-Regulation ; Female ; Gene Expression Regulation ; Hearing Loss, Sensorineural/*blood ; Hearing Loss, Sudden/*blood ; Humans ; Male ; MicroRNAs/*metabolism ; Middle Aged ; Ribonuclease III/*blood/*metabolism ; Statistics as Topic

OBJECTIVES: The microRNAs have been implicated in the development and function of the inner ear, especially in contribution to hearing. However, the impact of idiopathic sudden sensorineural hearing loss (SSNHL) on expression of miRNA biogenesis-related components has not been established. To investigate the regulations of microRNA (miRNA) biogenesis-related components, argonaute 2 (AGO2) and DiGeorge syndrome critical region gene 8 (DGCR8) mRNA expression in SSNHL and to evaluate the value of clinical parameters on their expression. METHODS: Thirty-seven patients diagnosed with SSNHL and fifty-one healthy volunteers were included in this study. We measured mRNA expression levels of AGO2 and DGCR8 in whole blood cells but erythrocytes of patients with SSNHL and controls, using reverse transcription and real-time polymerase chain reaction analysis. RESULTS: The mRNA expression level of AGO2 is upregulated in SSNHL. The expression level of AGO2 was significantly correlated with that of DGCR8 in both patients with SSNHL and controls. Expression level of AGO2 in SSNHL was correlated with white blood cell counts. CONCLUSION: This study demonstrated for the first time that the AGO2 mRNA expression level was upregulated in SSNHL, suggesting its important role in pathobiology of SSNHL development.
Blood Cells ; DiGeorge Syndrome ; Ear, Inner ; Erythrocytes ; Healthy Volunteers ; Hearing ; Hearing Loss, Sensorineural* ; Hearing Loss, Sudden ; Humans ; Leukocyte Count ; MicroRNAs ; Real-Time Polymerase Chain Reaction ; Reverse Transcription ; RNA, Messenger ; Social Control, Formal

OBJECTIVES: Korean red ginseng (KRG) has hypoglycemic, antioxidant, antithrombotic, and other beneficial effects in human. The present study evaluate the therapeutic effects of KRG on hearing recovery and glucocorticoid-induced hyperglycemia in patients with idiopathic sudden sensorineural hearing loss (SSNHL) undergoing systemic steroid therapy. METHODS: The patients were divided into 2 groups: the steroid, and the combination of steroid and red ginseng. Pure tone averages (PTA) were assessed at the first visit and 2-month follow-up. All patients underwent fasting blood glucose analyses just before and on the fifth day of treatment. Both groups were treated with a 10-day course of oral methylprednisolone at tapering doses starting from a daily dose of 48 mg. To the combination group, KRG extract was administered by mouth at a daily dose of 3 g for 20 days in addition to methylprednisolone. Hearing gain was calculated comparing the initial PTA and PTA at 2 months' follow-up. Treatment responses were classified according to Siegel's criteria. RESULTS: Pretreatment conditions were similar between the steroid (n=37) and combination groups (n=36). At 2 months after the treatment, PTA improved significantly in both groups, but there was no significant difference in the mean hearing gain & recovery rate. The non-diabetic subjects in the steroid group (n=27) exhibited a 24% increase in the mean blood glucose level during the systemic steroid therapy, while those in the combination group (n=34) showed no changes. CONCLUSION: Although the KRG did not provide greater therapeutic effects on hearing recovery, we suggest that red ginseng can be a useful adjuvant to the current steroid therapy to normalize glucocorticoid-induced hyperglycemia in non-diabetic patients during the treatment of SSNHL.
Blood Glucose* ; Fasting ; Follow-Up Studies ; Glucose ; Hearing Loss, Sensorineural* ; Hearing* ; Humans ; Hyperglycemia ; Methylprednisolone ; Mouth ; Panax* ; Steroids

The autosomal dominant deafness disorder at the DFNA9 locus has been described and the clinical aspects extensively characterized, showing adult-onset, progressive sensorineural hearing loss and vestibular dysfunction. DFNA9 is caused by mutations of the human Coagulation factor C homology (COCH)gene. COCH encodes cochlin, a highly abundant secreted protein of unknown function in the inner ear. Several mutations have been identified so far: P51S, V66G, G87W, G88E, V104del, I109T, I109N, W117R, A119T, M512T, C542F, C542T. We hereby report a case of cochlear implantation in a 55-year-old man with bilateral sensorineural hearing loss, which is caused by a mutation in COCH gene (W117R).
Blood Coagulation Factors ; Cochlear Implantation ; Cochlear Implants ; Deafness ; Ear, Inner ; Hearing Loss, Sensorineural ; Humans ; Middle Aged