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Sudden Sensorineural Hearing Loss in Children: Clinical Characteristics, Etiology, Treatment Outcomes, and Prognostic Factors

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (4)

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.

JIF:2.06

Clinical Features of Ears With Otosclerosis and Endolymphatic Hydrops

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (4)

Objective: Endolymphatic hydrops (EH) has been reported in ears with otosclerosis. The objective of this study was to investigate the clinical features of ears with otosclerosis and EH on magnetic resonance imaging (MRI) and identify predictors for the presence of EH. Study Design: Retrospective study. Setting: University hospital. Materials and Methods: Forty-six ears from 37 patients with otosclerosis were included in the present study. Interventions: The subjects were divided into three groups, those with no, mild, or significant EH, based on 3-T MRI with intravenous injection of gadolinium. Hearing levels and the extent of otosclerotic lesions graded based on the computed tomography (CT) findings were compared among the groups. Moreover, to examine the vascular activity of the disease, intraoperative measurements of blood flow were also evaluated. Main Outcome Measures: Imaging, hearing levels, and blood flow values. Results: The overall rate of EH was 58.7% (27 of 46 ears); cochlear EH (52.2%) was more frequent than vestibular EH (26.1%). Average thresholds in ears with significant EH were significantly higher at several frequencies, both on air and bone conduction, than those with no or mild EH. Significant EH was more frequently observed in ears with advanced stages on CT than in those without advanced stages. The values of blood flow in the area anterior to the oval window were higher in some ears with EH than in ears without EH. Conclusion: EH was frequently present in ears with otosclerosis, especially those with severe hearing loss or advanced disease on CT.

JIF:2.06

The Clinical Outcomes After Intratympanic Gentamicin Injection to Treat Meniere's Disease: A Meta-analysis

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (4)

Objectives: In recent decades, intratympanic gentamicin (ITG) has increasingly been used to treat intractable Meniere's disease (MD). We performed a meta-analysis of pooled clinical outcomes, exploring whether ITG was effective and safe. Data Sources: Cochrane Library database, Embase, and Medline. Study Selection: We searched scientific and medical databases to March 2018 for articles evaluating clinical outcomes after ITG treatment of intractable MD according to the American Academy of Otolaryngology Head and Neck Surgery (AAO-HNS) guidelines. Data Extraction: We performed a meta-analysis to evaluate treatment efficacy and safety. Quantitative and descriptive information of included RCTs was obtained. Data Synthesis: We ultimately evaluated 49 of the initially retrieved 1,062 citations (the 49 articles included data from a total of 2,344 MD patients). In almost all studies, patients served as their own controls; "before-and-after'' clinical outcomes were reported. The I-2 metric was used to explore heterogeneity. Conclusion: Overall, our results seem to provide the limited evidence about efficacy and toxicity effects of ITG. However, clinical outcomes require further confirmation; many included studies were poorly designed, less than 2 years for reporting results in MD are in the majority of patients. More long-term prospective follow-up, high-quality, large-scale, randomized controlled trials are needed to confirm that ITG is safe and effective when used to treat intractable MD.

JIF:2.06

Auditory Selective Attention Hindered by Visual Stimulus in Prelingually Deaf Children With Cochlear Implants

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (5)

Objective: To compare the influence of visual distractors on the performance of auditory selective attention between prelingually deaf children with a CI (cochlear implant) and children with normal-hearing. Design: Twenty-two patients who had a cochlear implant device (10 males and 12 females, aged 6.64 +/- 0.99 yrs) and 16 normal-hearing children (6 males and 10 females, aged 6.09 +/- 0.51 yrs) were recruited. Half of the auditory stimuli were presented together with visual stimuli, and participants were required to complete an auditory identification task. Reaction times and discriminability (d') for these two groups were recorded and calculated. Results: The normal-hearing group had shorter mean reaction times than the CI group in detecting auditory targets. With visual distraction, the d' of the normal-hearing group was significantly better than that of CI group (t = 2.649, p = 0.012), while no statistical significance was found between the two groups without visual distraction (t = 0.693, p = 0.493). Conclusion: Enhanced processing of visual stimuli interferes with auditory perception in CI users by occupying the capacity-limited attention.

JIF:2.06

Altered Gray Matter Volume and White Matter Integrity in Sensorineural Hearing Loss Patients: A VBM and TBSS Study

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (6)

Objective: The purpose of the present study was to detect structural changes in the brains of patients with sensorineural hearing loss (SNHL) by combining voxel-based morphometry (VBM) and tract-based spatial statistics (TBSS). Methods: Thirty-five patients with SNHL (mean age: 39.72 +/- 1.81 yr) and 23 age-matched control subjects (mean age: 39.83 +/- 1.96 yr) were assessed using three-dimensional, T1-weighted imaging, and diffusion tensor imaging. TBSS and VBM analyses were performed to evaluate grey matter (GM) volume changes and white matter (WM) alternations, as measured by mean diffusivity (MD), fractional anisotropy (FA), axial diffusivity (AD), and radial diffusivity (RD). Results: VBM showed decreased GM volume in patients with SNHL in the fusiform gyrus of the right temporal lobe and right middle occipital gyrus. TBSS revealed WM integrity changes, including decreased FA and RD and increased RD in several WM regions. However, MD showed no significant difference between patients with SNHL and age-matched controls. Conclusion: Patients with SNHL showed smaller GM volume and WM integrity changes in several regions.

JIF:2.06

Endoscopic-Assisted Repair of Spontaneous Temporomandibular Joint Herniation Through a Transcanal Approach

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (6)

Objective: To report of the presentation, diagnosis, and management of a spontaneous temporomandibular joint (TMJ) herniation. Patients: A 69-year-old man with a spontaneous TMJ herniation. Interventions: Diagnostic evaluation and surgical approaches. Main Outcome Measures: Imaging, and medical and surgical treatment outcomes. Results: At 14 months follow-up, the patient reported improved tinnitus and on examination there was no evidence of a hypertrophic scar or canal stenosis, no recurrence of TMJ herniation, no signs of otorrhea, otalgia; and with normal TMJ function. Conclusions: The advantages of repairing spontaneous TMJ herniation through a transcanal approach using otoendoscopy include a high definition video of surgical field, minimal invasiveness, and good postoperative cosmetic outcome.

JIF:2.06

Serum Bilirubin Level as a Potential Marker for the Hearing Outcome in Severe-Profound Bilateral Sudden Deafness.

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (6)

Objective: To investigate the association of serum bilirubin level with hearing outcomes in bilateral sudden sensorineural hearing loss (BSSHL) patients. Participants: One hundred thirteen in-patient BSSHL patients were consecutively enrolled between July 2008 and December 2015 in a tertiary center. Main Outcome Measures: Multivariable linear regression, generalized estimating equations (GEE), and stratified analyses were applied to examine the association between serum bilirubin level and hearing outcome measures such as final hearing threshold and absolute and relative hearing gains in BSSHL. Results: After full adjustment for potential confounders, total bilirubin levels (TBIL) were observed to be positively and independently associated with hearing outcomes as measured by final hearing (beta [95% confidence interval {CI}]: -1.5 [-2.7, -0.2] dB HL per 1 mu mol/L increase in TBIL) and absolute and relative hearing gains (beta [95% CI]: 1.4 [0.2, 2.7] dB and 1.6 [0.2, 3.1] dB, respectively) in the severe to profound hearing loss subpopulation. Conclusions: Higher TBIL levels, within the normal or mildly elevated ranges, were independently and significantly associated with better hearing outcome in BSSHL patients with severe to profound hearing loss. Given bilirubin elevation treatments exist, our finding suggests a novel pharmacological strategy for this specific subpopulation.

JIF:2.06

Effect of Oval Window Blockage on Bone Conduction in Cadaver Heads

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (7)

Objective: This study aimed to explore the feasibility of medical adhesive in the molding of oval window (OW) blockage in cadaver heads and to study the effect on bone conduction (BC). Methods: Four cadaver heads were selected to establish OW blockage model. The daub type of medical adhesive was used to immobilize OW. The vibration properties of the round window membrane (VRWM) in response to the acoustic stimulation, and the vibration properties of the round window membrane and cochlear promontory (VCP) in response to the BC transducer B-71 stimulation were assessed by laser Doppler vibrometer in both pre-OW blockage and post-OW blockage. Results: After blocking the oval window, the mean values of the sound-induced velocities amplitude responses of the round window membrane by air conduction were decreased significantly beyond 30 dB in all measured frequencies (p<0.05). The round window membrane relative velocity (VRWM/VCP) shows a decrease of about 1 dB at 1 and 3 kHz frequencies and a slight increase of around 0.5 dB from 4 to 8 kHz frequencies in post-OW blockage. However, it should also be noted that the VRWM/VCP is a significant decrease of 1.2 dB at 3 kHz in post-OW blockage compared with preOW blockage (p<0.05). Conclusion: Medical adhesive was available for the immobilization of oval window. In cadaver heads, the effect of OW blockage on the BC was the notching at 3 kHz.

JIF:2.06

EPO Attenuates Cisplatin-Induced Ototoxicity in HEI-OC1 Auditory Cell Via the Nrf2-ARE Signaling Pathway

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (7)

Hypothesis: Erythropoietin (EPO) may protect against cisplatin-induced ototoxicity in HEI-OC1 auditory cell line. Background: Cisplatin is a widely used chemotherapeutic agent for the treatment of human solid tumors limited by its high incidence of ototoxicity. Currently, there are no clinical solutions. EPO has been reported to have varieties of neuroprotective effects. However, nothing has yet been reported on its potential to prevent cisplatin ototoxicity in auditory cell lines and possible mechanism. Methods: HEI-OC1 cells were incubated with 20mM of cisplatin for 48 hours, after application of various concentrations of EPO for 24 hours. Cell viability was determined using a Cell Counting Kit-8 (CCK-8) assay. Oxidative stress and apoptosis were assessed by reactive oxygen species (ROS) measurement, Hoechst 33258 staining, and flow cytometry (FC). Western blot (WB) and real-time quantitative PCR were used to detect nuclear factor erythroid 2-related factor 2 (Nrf2), NAD (P) H quinone oxidoreductase 1 (NQO1), heme oxygenase-1 (HO-1), Bcl-2 and Bcl-xl protein and mRNA expression levels. Results: Pretreatment with 40, 8, and 1.6 ng/mL of EPO for 24 hours before application of 20mM cisplatin increased cell viability in HEI-OC1 cells. Besides, EPO enhanced the expression of Nrf-2, HO-1, and NQO1. Moreover, upregulation of the expression of Bcl-2 and bcl-xl were also observed. Conclusion: Our results suggest that EPO alleviates cisplatin- induced ototoxicity by activating Nrf2-ARE signaling.

JIF:2.06

Prognostic Factors of Long-Term Hearing Preservation in Small and Medium-Sized Vestibular Schwannomas After Microsurgery

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (7)

Objective: The authors evaluated the long-term hearing outcomes of patients with vestibular schwannoma (VS) to explore appropriate surgical treatment. Study Design: Retrospective study. Setting: Tertiary referral center. Patients: A total of 138 patients diagnosed with small and medium-sized VS with serviceable hearing from January 2006 to December 2015. Interventions: All patients underwent microsurgery via retrosigmoid (RSA) or middle cranial fossa approach (MFA) and were followed up for over 2 years. Main Outcome Measures: Pre-and postoperative hearing, including pure tone audiometry, speech discrimination score, and auditory brainstem response (ABR), were analyzed. Results: The mean tumor size and volume were 16.6 +/- 3.4mm and 1711.8 +/- 918.5mm(3), respectively. Preoperative hearing levels were Class A in 42, Class B in 67, and Class C in 29 patients. Patients with a tumor from the superior vestibular nerve (SVN) had better hearing at diagnosis. Postoperative hearing levels were Class A, B, C, and D for 28, 17, 32, and 61 patients. Hearing outcomes were significantly better in patients with normal intraoperative I wave on ABR. Hearing loss within 6 months had a positive effect on postoperative hearing. Better preoperative hearing and tumors from SVN were correlated with better postoperative hearing outcomes. Tumor size, cystic variation, or extension to the fundus of internal auditory canal had no influence on hearing preservation. Conclusions: Better preoperative hearing, shorter hearing loss period, tumors from SVN, and normal intraoperative I wave are prognostic factors for serviceable hearing. RSA and MFA are effective and safe for tumor removal and hearing preservation.

JIF:2.06

Long-term Outcomes of Bone Conduction Hearing Implants in Patients With Bilateral Microtia-atresia

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (8)

Objectives: To evaluate the long-term outcomes of three different types of bone conduction hearing implants (BCHI)-BAHA, Ponto, and Bonebridge-in Mandarin-speaking patients with bilateral microtia-atresia. Methods: This cohort study enrolled 59 patients affected by bilateral microtia-atresia, with an upper bone conduction threshold limit of 30 dB HL at frequencies of 0.5 to 4 kHz. All subjects underwent unilateral BCHI surgery, including 26 (18 males, 8 females, of mean age 8.7 +/- 1.9 yr) implanted with BAHA devices; 10 (7 males, 3 females, of mean age 11.7 +/- 2.8 yr) implanted with Ponto devices; and 23 (14 males, 9 females, of mean age 9.0 +/- 1.8 yr) implanted with Bonebridge devices. The main outcome measures included long-term audiological benefits, patient satisfaction, and complications. Each subject acted as his or her own control. Results: Two years after BCHI surgery, the mean hearing thresholds in the BAHA, Ponto, and Bonebridge groups had improved to 22.6 +/- 1.6 dB HL, 21.6 +/- 1.2 dB HL, and 22.5 +/- 1.5 dB HL, respectively. The mean percentages of subjects in these three groups recognizing speech at 65 dB SPL under quiet conditions were 97.7 +/- 4.2%, 96.3 +/- 1.1%, and 94.4 +/- 9.4%, respectively, whereas the mean percentages recognizing speech under noise conditions (signal:noise ratio +5) were 87.0 +/- 1.8%, 89.3 +/- 9.3%, and 85.3 +/- 4.7%, respectively. Questionnaires revealed patients' benefits and satisfaction with this surgery. Three (11.5%) of 26 patients in the BAHA group and 1 (10%) of 10 in the Ponto group experienced skin irritation, but all recovered after local treatment. Five (19.2%) patients in the BAHA group and two (20%) in the Ponto experienced abutment extrusion about 6 months postoperatively, with all achieving good results after revision surgery to replace the abutment. One (3.8%) patient in the BAHA group experienced local chronic inflammation and underwent surgery to replace the BAHA with a Bonebridge implant. One (4.3%) patient in the Bonebridge group developed a local infection 3 months postoperatively and underwent implant removal. Conclusions: All three BCHIs were well tolerated after long-term follow-up, and all improved audiometric thresholds and the intelligibility of speech in the presence of both quiet and noise. These implants should be considered valid and safe options for the functional rehabilitation of patients with bilateral microtia-atresia.

JIF:2.06

The Influence of Metabolic Syndrome on the Prognosis of Idiopathic Sudden Sensorineural Hearing Loss

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (8)

Objectives: We aimed to verify the hypothesis that metabolic syndrome (MetS) and its components affect the prognosis of idiopathic sudden sensorineural hearing loss (ISNNHL). Study Design: A retrospective cohort study. Setting: Tertiary otology referral center. Patients: We divided patients who were diagnosed with ISSNHL between January 2015 and January 2018 into a MetS group and a Non-MetS group according to the diagnostic criteria of MetS. Interventions: We diagnosed ISSNHL patients by using pure-tone audiometry and treated them with oral steroids, blood flow promoting agents, and hyperbaric oxygen therapy. Main Outcome Measures: We used multivariate logistic analysis to identify prognostic factors of ISSNHL. Results: The MetS group comprised 94 patients, and the Non-MetS group comprised 162 subjects. Despite the rate of hypertension, diabetes mellitus, hyperlipidemia, and BMI, no significant difference was observed between the two groups (p > 0.05). The rates of complete recovery and partial recovery of the MetS group were significantly lower than those of the Non-MetS group (p < 0.05). According to the multivariate analysis, MetS was significantly correlated with a poor prognosis (OR = 2.912, p = 0.008), and the OR increased with an increase in the number of MetS components. Late onset of treatment, high initial hearing threshold, and presence of diabetes mellitus and hyperlipidemia were associated with a poor prognosis (p < 0.05). Conclusions: The presence of MetS may negatively affect the recovery of Chinese patients with ISSNHL, and the prognosis was poorer with an increase in the number of MetS components. Early onset of treatment, low initial hearing threshold and absence of diabetes mellitus, and hyperlipidemia are associated with favorable hearing recovery.

JIF:2.06

Predictors of Distant Metastasis and Survival in Adenoid Cystic Carcinoma of the External Auditory Canal

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (10)

Objective: To analyze the predictors of both distant metastasis and survival in patients with adenoid cystic carcinoma of the external auditory canal. Study Design: Retrospective patient review. Setting: A single university hospital. Patients: Eighty-two cases with adenoid cystic carcinoma of the external auditory canal were referred to our institution between 2004 and 2016. Main Outcome Measures: Distant metastasis was detected by lung computed tomography, proton emission tomography computed tomography, or histopathologic examination of tissue samples. Distant metastasis predictors were analyzed using Student's t tests and chi(2) tests. The log-rank tests of Kaplan-Meier survival curves were used to evaluate survival differences. Results: During a median follow-up of 36 months (range, 6-162 mo), distant metastasis developed in 25 patients. The occurrence of distant metastasis was significantly associated with histopathologic subtype, T classification, and local recurrence (p < 0.05). The 1-, 10-, 20-, and 25-year cumulative survival rates in the patents with DM were 95.7, 95.7, 71.7, and 0%, respectively, and all survival rates were 100% for the 57 patients without distant metastasis (p = 0.115). Median survival time after occurrence of distant metastasis was 13 months (range, 1-120 mo). Prognosis was better with solely lung metastasis than with metastases to other visceral organs or bone (p <0.05). Conclusions: Distant metastasis appeared to result in a poorer prognosis, occurrence of distant metastasis was significantly associated with local recurrence, extensive surgery is recommended to achieve local control and reduce distant metastasis risk. Routine follow-up investigations for detecting distant metastasis are warranted for patients with an increased risk for distant metastasis.

JIF:2.06

Role of Autophagy in Acquired Cholesteatoma

期刊: OTOLOGY & NEUROTOLOGY, 2019; 40 (10)

Hypothesis: Autophagy and its enhancement may have a role in the pathogenesis of acquired cholesteatoma. Background: The etiopathogenesis of acquired cholesteatoma remains unclear. Some clinical features of cholesteatoma are similar to those of cancer. The study of autophagy in cancer has indicated that enhanced autophagy enables tumor cell survival and growth. Methods: Cholesteatoma epithelium and normal external auditory canal (EAC) epithelium were obtained from patients with acquired cholesteatoma, and marginal epithelium of the tympanic membrane perforation was obtained from patients with chronic otitis media (COM). Immunohistochemistry (IHC) was performed to detect the expression of light chain 3 (LC3) in cholesteatoma and EAC epithelium. Western blotting (WB) was performed to detect the expression of LC3, Beclin-1, or the PI3K/AKT pathway in cholesteatoma, EAC, and COM epithelium. Results: LC3 staining of IHC was stronger in cholesteatoma epithelium compared with normal EAC epithelium. The ratios of LC3-II/I and Beclin-1 expression on WB were significantly higher in cholesteatoma epithelium compared with EAC epithelium or COM epithelium, and there was a significantly higher ratio of p-PI3K/PI3K and p-AKT/AKT in cholesteatoma epithelium compared with EAC epithelium. Conclusions: Enhanced autophagy might play a role in the pathogenesis of acquired cholesteatoma. PI3Ks might have different regulatory functions on autophagy in the cholesteatoma epithelium.

JIF:2.06

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