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Novel hearing loss (HL) genes are constantly being discovered, and evidence from independent studies is essential to strengthen their position as causes of hereditary HL. To address this issue, we searched our genetic data of families with autosomal dominant HL (ADHL) who had been tested with high-throughput DNA sequencing methods. For CD164, only one pathogenic variant in one family has so far been reported. For LMX1A, just two previous studies have revealed its involvement in ADHL. In this study we found two families with the same pathogenic variant in CD164 and one family with a novel variant in LMX1A (c.686CA; p.(Ala229Asp)) that impairs its transcriptional activity. Our data show recurrence of the same CD164 variant in two HL families of different geographic origin, which strongly suggests it is a mutational hotspot. We also provide further evidence for haploinsufficiency as the pathogenic mechanism underlying LMX1A-related ADHL.

HYPOTHESIS AND BACKGROUND Hearing loss leads to synaptic changes in auditory neurons and their networks, and functions as a consequence of the interplay between genes and proteins. However, cellular and molecular mechanisms leading to deafness-induced plasticity in the auditory cortex (AC) remain unclear. Here, we examined the changes in gene expression and key signaling pathways that regulate differentially expressed genes (DEGs) in the AC following auditory deafferentation using RNA-sequencing (RNA-Seq) analysis. METHODS Cochlear ablation-induced bilaterally deafened Sprague-Dawley rats were maintained for 12 weeks and their ACs were harvested. RNA-seq analysis was performed on each sample to identify which genes were expressed. This information was then used for comparative analysis of DEGs between samples. The statistical significance of DEGs was determined by fold change (|FC| > 1.5) and independent t test (p

Background and Objectives Surgery for bilateral chronic otitis media (COM) is usually performed sequentially, not simultaneously. The main reason is to prevent iatrogenic bilateral conductive hearing loss during recovery period. However, with asymmetric hearing loss, the difference in patient inconvenience between sequential and simultaneous surgery may be the same. This study evaluates the efficacy of simultaneous COM surgery in patients with asymmetric hearing.Subjects Materials and Method From 2012 to 2018, 9 patients underwent simultaneous bilateral COM surgery. The period of patients’ hospital stay, the success rate of tympanoplasty, tolerability for operation, and hearing thresholds were analyzed statistically.Results For the 8 same-day surgery patients, the mean hospital period was 4.4±0.7 days, which was much shorter than that of sequential surgery (8 days). Tympanoplasty was successful in all patients without complications. In the better side, the preoperative/postoperative mean air conduction thresholds (AC) were 62.97±12.89 dB/47.81±19.07 dB (p=0.017), the bone conduction thresholds (BC) were 46.72±10.31 dB/37.66±16.99 dB (p=0.161) and the air-bone gaps (ABG) were 16.25±8.81 dB/10.16±7.78 dB (p=0.176). In the worse side, the preoperative/ postoperative mean AC were 86.56±18.22 dB/72.18±29.43 dB (p=0.035), BC were 53.28± 11.10 dB/48.13±18.41 dB (p=0.173), and ABG were 33.28±11.22 dB/24.06±14.80 dB (p=0.500). In both ear, the postoperative AC and BC were better than or equivocal to those of the preoperative value, and the result was similar with each of the unilateral ear audiological results.Conclusion Despite the fact that there is no complete consensus to date, simultaneous bilateral COM surgery can be an option when patients have asymmetric hearing loss. Simultaneous bilateral COM surgery could save time, cost, and lead to similar results with sequential surgery, so it could be a considerable surgical option for patients with bilateral COM.

For the hearing rehabilitation of patients with profound sensorineural hearing loss, cochlear implants (CIs) are widely used. However, the result of CI is variable, and the central neural plasticity is considered to be a reason for this variability. We hypothesize that the resting-state cortical networks play a role in the condition of profound hearing loss and are affected by CI. To investigate the resting-state neuronal networks after cochlear implantation, we acquired 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET) images in experimental animals.Eight domestic adult cats were enrolled in this study. The hearing threshold of the enrolled animals was within the normal range, as measured by auditory evoked potential. They were divided into two groups: control (n=4) and hearing loss (n=4) groups. Hearing loss was induced by co-administration of ethacrynate and kanamycin. FDG-PET was performed at normal hearing state and at 4 and 11 months after the deafening procedure. Cochlear implantation was performed in the right ear and electrical cochlear stimulation was performed for 7 months (from 4 to 11 months after the deafening procedure). Acquired PET images were analyzed and compared between the two groups and among the three time points.At 4 months after hearing loss, the auditory cortical area's activity decreased, and the associated visual area activity increased. After 7 months of cochlear stimulation, the superior marginal gyrus and cingulate gyrus showed hypermetabolism, and these areas were components of the default mode network. The inferior colliculi showed hypometabolism.Resting-state cortical activity was elevated after cochlear stimulation in the default mode network component. This suggests that the awareness level was elevated after hearing restoration by the CI.

Objective The aims of this multicenter study were to prospectively evaluate the prevalence of dead regions (DRs) in sudden sensorineural hearing loss (SSNHL) and compare the clinical characteristics and hearing outcomes of SSNHL according to the presence of DRs. Study design Prospective study. Setting Multicenter study. Patients and methods The threshold-equalizing noise (TEN) test was prospectively performed on a total of 130 patients diagnosed with SSNHL. All patients received systemic steroid therapy and/or intratympanic steroid injection within 1 month after onset. Pure-tone audiograms and the TEN test were conducted before and after steroid treatment. Age, sex, side of affected ear, recurrence, onset of symptoms, presence of dizziness, and comorbid diseases were also collected. The prevalence of DRs in SSNHL and clinical factors related to the DRs were assessed. Hearing outcomes for SSNHL according to DRs were evaluated in 68 patients who followed a pure-tone audiogram. Results The overall prevalence of one or more DRs in SSNHL evaluated using the TEN test was 20.8% (27/130 subjects) and the overall frequency-specific prevalence of DR was 6.7% (61/910 DRs). Although the DRs (+)and DR (-) groups had similar initial pure-tone thresholds, the DRs (+) group had significantly worse initial WRS compared to the DRs (-) group (p = 0.015). The presence of DRs was not associated with hearing recovery in a multivariate logistic regression model, but it was significantly associated with the degree of hearing gain in a multivariate linear regression model (p = 0.018). Conclusions The presence of DRs can be considered one of the poor prognostic factors for SSNHL and the TEN test may contribute to assess the prognosis of SSNHL in clinical settings.

Objectives To analyze the clinical characteristics of cochlear fistulas (CFs) and propose a new fistula classification system with regard to the cochlea. Materials and methods A retrospective chart review was conducted between January 2008 and December 2015 to identify patients who had undergone surgery for cholesteatoma with an associated CF. The following data were collected: preoperative symptoms, findings of temporal bone computed tomography (TBCT), fistula stage, cholesteatoma classification, surgical technique, and pre- and postoperative pure-tone audiometry. Results We analyzed a total of 159 patients, out of which 9 (5.7%) were diagnosed with a CF. The average duration of the chronic otitis media was 19.8 years. Cholesteatomas that induced CF rarely existed in the nonaggressive state; recurrent otorrhea was observed in all but one of our subjects. All the patients with CF had a distinct origin of cholesteatoma that developed from the retraction of posterior pars tensa; further, 88.9% cholesteatomas extended to and filled the sinus tympani. Preoperative audiometry revealed total hearing loss in 4 (44.4%) patients. Further, five patients with residual hearing before surgery had stage I fistulas, and the bone conduction thresholds remained stable after surgery. Conclusion Cochlear fistulas were often detected in patients with (1) a history of chronic otitis media (exceeding 10 years), (2) frequently recurring otorrhea, and (3) pars tensa cholesteatomas that extended to the posterior mesotympanum and filled the sinus tympani. Such patients can suffer from potentially severe and irreparable sensorineural hearing loss.

Objectives/hypothesis To evaluate the long-term effects of intralesional steroid injections (ILSIs) in patients with otohematoma and to suggest the clinical implications, especially with regard to the duration of otohematoma. Study design Retrospective analysis. Methods Fifty-six patients were enrolled and classified into short-term otohematoma (n = 30) and long-term otohematoma (n = 26) groups according to the period of time after auricular trauma. After the first ILSI, all patients underwent weekly examinations during the 21-day observational period to determine the treatment outcomes and were then followed up for reevaluation for a maximum of 36 months. We considered ILSIs to be ineffective if the otohematoma persisted after the third ILSI, and we opted to perform surgical treatment in such cases. In addition, we evaluated early recurrence, late recurrence, and ILSI-related complications. Results After up to three ILSIs, 29 out of 30 cases (96.7%) with short-term otohematoma and 20 out of 26 cases (76.9%) with long-standing otohematoma were treated without complications. Three patients with long-term otohematoma, however, experienced late recurrences at 4, 15, and 18 months, respectively. Seven patients who showed no response after ILSIs underwent surgical treatment. The duration of otohematoma (P = .043) and a higher initial aspirated fluid volume (P = .014) were shown to significantly increase the risk of treatment failure after ILSIs. Conclusions Multiple and immediate ILSIs in patients with otohematoma appear to be an effective treatment approach with no complications. Patients with short-term otohematoma showed better outcomes following ILSIs. The treatment approach may be optimized based on the duration and degree of otohematoma. Level of evidence 4 Laryngoscope, 129:459-465, 2019.

Objective Although many studies have investigated sudden sensorineural hearing loss (SSNHL) in adults, there were few studies on SSNHL in the pediatric population; especially research on treatment and prognosis of pediatric SSNHL was limited. The aim of this study was to evaluate clinical characteristics, treatment outcomes and prognostic factors in children and adolescents with SSNHL. Methods A retrospective review of medical records of 67 pediatric patients (67 ears) who had diagnosed with SSNHL at our hospitals was performed to analyze patients’ clinical manifestations and audiograms. All patients were treated with high-dose systemic prednisolone (1 mg/kg), and 17 of them underwent intratympanic steroid injection therapy. Audiological evaluation was carried out before and after treatment, and hearing recovery was defined as complete recovery and partial recovery according to Siegel’s criteria. Patients were divided into two groups: childhood group (ages between 4 and 12 years old) and adolescence group (age > 12 years), and clinical characteristics and treatment outcomes were investigated. In addition, patients were divided into two groups according to degree of hearing recovery, and evaluation was made regarding possible prognostic factors. Results The recovery rate in total 67 patients was 55.2%. The recovery rate of the childhood group was significantly lower than that of the adolescence group ( p = 0.038). While the presence of vertigo did not significantly correlate with prognosis ( p = 0.219), the presence of tinnitus was significantly associated with hearing recovery ( p = 0.005). Audiological assessment revealed that a low initial hearing threshold, high speech discrimination score, and descending type of audiogram were positively associated with hearing recovery ( p = 0.002, p = 0.003, and p = 0.029, respectively). Conclusion The childhood group had worse treatment outcomes than the adolescence group. High initial hearing threshold and absence of tinnitus were poor prognostic factors of hearing recovery. Active treatment is required for patients with these poor prognostic factors and childhood patients with SSNHL.

Purpose: The purpose of this study was to investigate the effect of the development and application of the new nurses' core skill program on the clinical skill, confidence, knowledge and performance of new nurses. Methods: This study was a nonequivalent control group post-test design on 46 new nurses with 3 to 6 months work experience in a general hospital. Data were collected from August 2015 to August 2016. A training program of core fundamental nursing skills was applied on 23 new nurses from 2016 in the experiment group, and an existing training method was applied on 23 new nurses from 2015 in the comparison group. The collected data were analyzed using SPSS/WIN 23.0. Results: The experimental group ($89{pm}4.29$) showed higher score in practical skill assessment compared to the control group ($85.09{pm}3.99$) and showed a statistically significant difference (p

Purpose: Although some studies argue that it is worthy for cochlear implant (CI) users to have frequency information above 3,000 Hz for consonant recognition, it is not clear whether CI patients make use of this information, especially in Korean. In this experiment, we tested whether consonant recognition in CI patients depended on the utilization of information from 3,000 to 8,000 Hz. Methods: We conducted consonant recognition tests in Korean [3 syllable structures: vowel-consonant-vowel (VCV), consonant-vowel (CV), and vowelconsonant (VC)] pronounced by one male and one female speakers for 3 normal hearing (NH) and 3 CI adults with 9 low-pass filtering frequency conditions. Results: Both groups showed decreased correction scores as cut-off frequency getting lower. CI subjects scored twice errors to NH subjects across frequency conditions. The cut-off frequency of 1,500 Hz brought a significant decrease of consonant recognition. Consonants in VCV syllable recognized better than VC and CV syllable in both groups. Fricatives and affricates in the manner of articulation and alveolar and palatal in the place of articulation are the ones that benefit from high frequency coding. Alveolar plosive sounds were most difficult sounds in both groups and this confirms previous studies of consonant recognition in Korean. Conclusion: NH and CI groups demonstrated poorer consonant recognition as cut-off frequency decreased with relatively large decrements of CI group. The information above 3,000 Hz conveys little information for consonant recognition in the normal and CI subjects while some CI subjects utilized information up to 6,000 Hz. There were large individual differences, particularly in CI group.

Objective This study aimed to determine the radiation dose of temporal bone computed tomography (TBCT) to detect pediatric congenital cholesteatoma (CC), and suggests strategy for lowering perioperative radiation dose. Methods Fifty-three CC patients followed up for more than 12 months after surgery. Perioperative clinical findings and the effective radiation dose (ERD) of TBCT were investigated. Patients’ mean age at initial TBCT was 60 months; the mean follow-up period was 43 months. Results In 33 (62.3%) of 53 patients, only one TBCT was performed postoperatively. Postoperative CT imaging was performed to evaluate before second-stage ossiculoplasty, due to suspicion of CC recurrence on examinations, or as part of follow-up. When TBCT was performed on more than two occasions after surgery as a part of follow-up, there was no difference in the distribution of stage or type of CC compared to patients subjected to TBCT on less than three occasions ( p = 0.093 and p = 0.744, respectively). During the most-recent follow-up of these 53 cases, there was no CC recurrence. The mean ERD of a single TBCT was 1.2 mSv, and the mean cumulative ERD of TBCT was 2 mSv per patient. Conclusion Mean and cumulative ERD of TBCT performed in CC cases was not too high to consider radiation hazard. However, efforts to minimize the number of CT scans should be taken basically.

Deafness induces many plastic changes in the auditory neural system. For instance, dendritic changes cause synaptic changes in neural cells. SMI-32, a monoclonal antibody reveals auditory areas and recognizes non-phosphorylated epitopes on medium- and high-molecular-weight subunits of neurofilament proteins in cortical pyramidal neuron dendrites. We investigated SMI-32-immunoreactive (-ir) protein levels in the auditory cortices of rats with induced unilateral and bilateral deafness. Adult male Sprague-Dawley rats were divided into unilateral deafness (UD), bilateral deafness (BD), and control groups. Deafness was induced by cochlear ablation. All rats were sacrificed, and the auditory cortices were harvested for real-time quantitative polymerase chain reaction (RT-qPCR) and western blot analyses at 2, 4, 6, and 12 weeks after deafness was induced. Immunohistochemical staining was performed to evaluate the location of SMI-32-ir neurons. Neurofilament heavy chain (NEFH) mRNA expression and SMI-32-ir protein levels were increased in the BD group. In particular, SMI-32-ir protein levels increased significantly 6 and 12 weeks after deafness was induced. In contrast, no significant changes in protein level were detected in the right or left auditory cortices at any time point in the UD group. NEFH mRNA level decreased at 4 weeks after deafness was induced in the UD group, but recovered thereafter. Taken together, BD induced plastic changes in the auditory cortex, whereas UD did not affect the auditory neural system sufficiently to show plastic changes, as measured by neurofilament protein level.

To evaluate the long-term effects of intralesional steroid injections (ILSIs) in patients with otohematoma and to suggest the clinical implications, especially with regard to the duration of otohematoma.Retrospective analysis.Fifty-six patients were enrolled and classified into short-term otohematoma (n = 30) and long-term otohematoma (n = 26) groups according to the period of time after auricular trauma. After the first ILSI, all patients underwent weekly examinations during the 21-day observational period to determine the treatment outcomes and were then followed up for reevaluation for a maximum of 36 months. We considered ILSIs to be ineffective if the otohematoma persisted after the third ILSI, and we opted to perform surgical treatment in such cases. In addition, we evaluated early recurrence, late recurrence, and ILSI-related complications.After up to three ILSIs, 29 out of 30 cases (96.7%) with short-term otohematoma and 20 out of 26 cases (76.9%) with long-standing otohematoma were treated without complications. Three patients with long-term otohematoma, however, experienced late recurrences at 4, 15, and 18 months, respectively. Seven patients who showed no response after ILSIs underwent surgical treatment. The duration of otohematoma (P = .043) and a higher initial aspirated fluid volume (P = .014) were shown to significantly increase the risk of treatment failure after ILSIs.Multiple and immediate ILSIs in patients with otohematoma appear to be an effective treatment approach with no complications. Patients with short-term otohematoma showed better outcomes following ILSIs. The treatment approach may be optimized based on the duration and degree of otohematoma.4 Laryngoscope, 129:459-465, 2019.

Neural plasticity is a characteristic of the brain that helps it adapt to changes in sensory input. We hypothesize that auditory deafferentation may induce plastic changes in the cytoskeleton of the neurons in the inferior colliculus (IC). In this study, we evaluated the dynamic status of neurofilament (NF) phosphorylation in the IC after hearing loss. We induced auditory deafferentation via unilateral or bilateral cochlear ablation in rats, aged 4 weeks. To evaluate cytoskeletal changes in neurons, we evaluated mRNA fold changes in NF heavy chain expression, non-phosphorylated NF protein fold changes using SMI-32 antibody, and the ratio of SMI-32 immunoreactive (SMI-32-ir) neurons to the total neuronal population in the IC at 4 and 12 weeks after deafness. In the bilateral deafness (BD) group, the ratios of SMI-32-ir neurons significantly increased at 4 weeks after ablation in the right and left IC (6.1 ± 4.4%, 5.0 ± 3.4%, respectively), compared with age-matched controls (P 0.01, P 0.01). At 12 weeks after ablation, the ratio of SMI-32 positive neurons was higher (right, 3.4 ± 2.0%; left, 3.2 ± 2.3%) than that in the age-matched control group, albeit not significant in the right and left side (P = 0.38, P = 0.24, respectively). Consistent with the results of the ratio of SMI-32-ir neurons, SMI-32-ir protein expression was increased at 4 weeks after BD, and the changes at 12 weeks after bilateral ablation were not significant in the right or left IC. The age-matched control fold changes of NF mRNA expression after bilateral deafness were not significant at 4 and 12 weeks after deafness in right and left IC. Unilateral deafness did not induce significant change of NF mRNA expression, SMI-32-ir protein expression, and the ratio of SMI-32-ir neurons in the IC at 4 and 12 weeks after hearing loss. Bilateral auditory deafferentation induces structural changes in the neuronal cytoskeleton within the IC, which is prominent at 4 weeks after BD. The structural remodeling of neurons stabilized at 12 weeks after BD. Unlike BD, unilateral auditory deafferentation did not affect the dynamic status of NFs in the IC.

Objective This study analyzed the characteristics of tinnitus identified in anemia patients with cohort- and population-based studies in a single institute and suggests a management algorithm. Methods Fifty patients who were treated for anemia and referred for tinnitus treatment were included in a single institute retrospective study. Characteristics of tinnitus were investigated in a correlation analysis with demographic and audiologic parameters. For the population-based study, data from the Korea National Health and Nutrition Examination Survey collected between 2010 and 2011 were analyzed. The study population consisted of 11,402 individuals aged 20–97 years with complete tinnitus-related data. The prevalence of tinnitus in anemia patients was investigated using the questionnaire, and associations between tinnitus and blood/urine parameters were evaluated by binary logistic regression analysis. Results In a single-institute study, patients with non-pulsatile tinnitus were significantly older and their initial hemoglobin was higher than those with pulsatile tinnitus (p = 0.001, 0.008, respectively). In pulsatile tinnitus, age and difference between initial and post-treatment hemoglobin were significantly associated with a subjective improvement in tinnitus (p = 0.002, 0.016, respectively). There were no significant audiologic or hematologic parameters associated with the improvement of non-pulsatile tinnitus. In the population-based study, there was no significant correlation between anemia and tinnitus (p = 0.064). In a multivariate analysis, age was the only parameter associated with tinnitus in participants with anemia. Conclusion The therapeutic strategy and prognosis of tinnitus in anemia patients differ according to the characteristics of tinnitus and the severity of anemia.

WD40 repeat protein 1 (WDR1) has been suggested as a protective mechanism or a sign of regeneration in avian cochlea. However, its role in mammalian cochlea has yet to be determined. Hence, we investigated WDR1 expression in sound-overstimulated Sprague-Dawley rats. Rats were divided into three groups (the permanent and temporary threshold shift [PTS and TTS] groups and the control group) according to the extent of noise exposure and euthanized immediately, 3, or 7 days after noise exposure for cochlear harvest. Immunocytochemistry localized WDR1 to outer hair cells, Deiter's cells, outer sulcus cells, and Reissner's membrane in the control group, and the PTS and TTS groups exhibited stronger WDR1 expression in the same cochlear regions than the controls. Moreover, WDR1 expression in these noise-exposed groups was extended to inner hair cells and basal cells of the stria vascularis. The expression of WDR1 in the PTS and TTS groups showed differences in intensity and shifts of localization, based on exposure length and recovery duration. Contrary to the avian cochlea, hair cell regeneration does not naturally occur in the acoustically damaged mammalian cochlea. Therefore, elevated WDR1 expression after acoustic overstimulation in the current experiments may provide a mechanism for protection against noise exposure.

The present study aimed to evaluate the effects of systemic lipoic acid on hearing preservation after cochlear implantation. Twelve Dunkin-Hartley guinea pigs were randomly divided into two groups: the control group and the lipoic acid group. Animals in the lipoic acid group received lipoic acid intraperitoneally for 4 weeks. A sterilised silicone electrode-dummy was inserted through the round window to a depth of approximately 5 mm. The hearing level was measured using auditory brainstem responses (ABRs) prior to electrode-dummy insertion, and at 4 days and 1, 2, 3 and 4 weeks after electrode-dummy insertion. The threshold shift was defined as the difference between the pre-operative threshold and each of the post-operative thresholds. The cochleae were examined histologically 4 weeks after electrode-dummy insertion. Threshold shifts changed with frequency but not time. At 2kHz, ABR threshold shifts were statistically significantly lower in the lipoic acid group than the control group. At 8, 16 and 32kHz, there was no significant difference in the ABR threshold shift between the two groups. Histologic review revealed less intracochlear fibrosis along the electrode-dummy insertion site in the lipoic acid group than in the control group. The spiral ganglion cell densities of the basal, middle and apical turns were significantly higher in the lipoic acid group compared with the control group. Therefore, systemic lipoic acid administration appears to effectively preserve hearing at low frequencies in patients undergoing cochlear implantation. These effects may be attributed to the protection of spiral ganglion cells and prevention of intracochlear fibrosis.

Objective Type 3 secretion system (T3SS) is the most important virulence factor in Pseudomonas aeruginosa infection. Of the various T3SS effector genes, exoS and exoU showed mutually exclusive distributions, and these two genes showed varied virulence. In many pseudomonal infections, the distribution of these genes showed different pattern and it influenced severity of infection. This study was aimed to evaluate differences of virulence factors and antibiotics resistance between chronic otitis media and other body infection caused by P. aeruginosa . Methods To estimate the prevalence of effector genes of T3SS, especially the distributions of exoS and exoU genes and their association with antibiotic resistance in COM, we compared the prevalence of T3SS genes in isolates from COM with those from lower respiratory infection and bacteremia. Other virulence genes, including groEL , pilA , ndvB , lasB , rhlI , and apr , were also studied to evaluate prevalence. These isolates were tested for antibiotic susceptibility, and we examined the association between antibiotic susceptibility and the prevalence of T3SS effector genes. Results The COM group showed a significantly higher e xoU -positive rate than the control group (70.6% vs. 6.7%; P exoU showed significant antibiotic resistance to ciprofloxacin and tobramycin ( P = 0.035), whereas there was no significant difference in the control group. Conclusions The high incidence of exoU -positive P. aeruginosa and ciprofloxacin resistance can explain the chronicity and intractability of infection in COM. Elucidation of this pathogenicity will facilitate the development of new treatment options for COM patients.

Objectives/Hypothesis: Herniation of the temporomandibular joint (TMJ) into the external auditory canal (EAC) is a very rare condition, and it mostly occurs in patients with bony wall defects in the anterior EAC. The purpose of this study was to evaluate the clinical and radiological findings and the treatment results of TMJ herniation into the EAC by reviewing a large sample of temporal bone computed tomography (CT) scans. Study Design: A retrospective study. Methods: We reviewed 985 adult temporal bone CT scans and evaluated the presence of defects, the size and location of defects, and TMJ herniation. We enrolled 23 cases with anterior EAC wall defects. Results: Twenty-three (2.3%) patients from the 985 temporal bone CT cases revealed an anterior wall defect involving the EAC. We identified the causes for the defects, such as canal cholesteatoma and trauma, in eight of the 23 cases. We observed 15 (1.5%) patients with patent foramen of Huschke. The mean defect size was 3.06 × 2.69 mm. Among the patients with these lesions, six had a TMJ herniation into the EAC. Four of the six cases were surgically reconstructed using a titanium mesh via a preauricular approach, whereas the remaining two cases were managed with endaural removal and skin graft. Conclusions: TMJ herniation into the EAC is very rare and may not occur even if an anterior wall defect of the EAC is present. Lesions can be successfully managed with surgical reconstruction of the TMJ via a preauricular approach, whereas small lesions can be managed using an endaural approach. Laryngoscope, 2010

Several previous studies have demonstrated that comorbidities, secondary causes, physical inactivity, and osteoporosis may cause recurrence of benign paroxysmal positional vertigo (BPPV). However, there has also been some controversy over the clinical course(s) and cause(s) of recurrent BPPV (rBPPV). We identified clinical features and associated factors, including decreased bone mineral density, in the recurrence of BPPV.In total, 198 patients with idiopathic BPPV, diagnosed at the otolaryngology clinics of Seoul National University Boramae Medical Center, were enrolled. The medical data of these patients were reviewed retrospectively. Recurrent BPPV was defined as the recurrence of BPPV after at least 1 month of a symptom-free interval following previous successful treatment.Of the BPPV patients, 67 (33.8%) were classified as rBPPV. Among them, about 16% showed changes in the involved semicircular canals and about 6% showed multiple semicircular canal involvement. rBPPV was more common in patients with comorbidities (P0.001). Involved semicircular canals showed no statistically significant difference according to the recurrence of BPPV. The mean symptom-free interval of the rBPPV group varied from 1 to 50.2 (mean, 11.6) months; however, 90% of BPPV recurrence occurred within 24 months. Bone mineral density in dual-energy X-ray absorptiometry (DEXA) was markedly decreased in BPPV patients versus normal controls, but there were no significant differences according to BPPV recurrence.The incidence of rBPPV in idiopathic BPPV patients was 33.8% in the present study. The mean period of recurrence after a symptom-free interval was about 11.6 months; most patients showed recurrence within 2 years after the first attack of BPPV. Furthermore, about 16% of patients suffered from rBPPV at a different kind or type of canal from the semicircular canal of the initial BPPV attack. Comorbidities, but not age, gender, or the involved semicircular canal, might be correlated with BPPV recurrence. Decreased bone mineral density did not show significant association with BPPV recurrence, but showed a significant relation with BPPV occurrence.

Objectives: This study was conducted to examine improvements of cognitive abilities after cochlear implantation in deaf children. We also examined the psychosocial factors that predict good outcomes of cochlear implantation. Design: A neuropsychological test battery was administered to 17 deaf children (mean age, 7 yr 2 mo) before receiving the cochlear implant, and they were reassessed with the same test at 6-mo follow-up. Their parents completed questionnaires concerning their medical and educational history, parenting style, and parental emotional problems. Results: Deaf children showed marked improvement in speedy and delicate motor coordination and visual organization abilities. Their cognitive functions of comprehension, concentration, and sequential processing, as measured by nonverbal tests, were found to have improved from the levels of borderline to mild mental retardation to that of near-normal range. However, their performances on Information, Comprehension and Similarity, and Mathematics subtests requiring verbal abilities did not show significant changes. Deaf children's working memory improved significantly after cochlear implantation. However, they showed more omission errors in the visual attention test at follow-up than before cochlear implantation. Such inattentiveness for visual stimuli in children post-implantation could be attributed to distractibility of these children toward external noise. Mothers' depression was negatively correlated with scores of acquired knowledge of deaf children. Conclusion: At the 6-mo follow-up after cochlear implant, deaf children showed marked improvement in nonverbal cognitive functions and working memory. Conversely, their verbal abilities did not significantly change. Maternal factors were found to be important for predicting the prognosis of cochlear implantation. The absence of a control group precludes the possibility of drawing any firm conclusions because the effect of the implant cannot be teased apart from the effects of maturation and training. Future studies should address this question with the use of appropriate control groups.

This study was designed to determine if hearing status monitoring during intravenous infusion of EA reduces individual variability and to evaluate the correlation between EA dose and Bwt.Twenty-five cats with the mean age of 24 ± 3.7 weeks (range = 20.6-28.3) and a mean weight of 3.21 ± 0.84 kg (range = 1.9-5.1) were administered a subcutaneous injection of KM (300 mg/kg) followed by an intravenous infusion of EA (1 mg/min). Click-evoked auditory brainstem responses (ABRs) were recorded to monitor hearing during the infusion. When ABR thresholds exceeded a 90 dB sound pressure level, the infusion of EA was terminated. Histopathology forapex, middle, and base sections of the cochlea were examined after 6 months.The dose of EA was optimized for deafening through simultaneous ABR measurements. Bwt was positively correlated with EA dose (mg) (p 0.001, R(2) = 0.548), which was different from a study previously reported. Cochlear histopathology assessments revealed an absence of organ of Corti in the majority of cochleae.Co-administration of kanamycin (KM) and ethacrynic acid (EA) was an easy and effective method for deafening procedures in adult animals. Body weight (Bwt) was positively correlated with EA dose (mg) and an optimal EA dose can be calculated.

Objectives This study was aimed to assess the relationship between the type of temporal bone area involved and conductive hearing loss. Methods We enrolled 97 patients who visited the otolaryngology clinics of Seoul National University Hospital or Boramae Medical Center, Seoul Metropolitan Government-Seoul National University with temporal bone fracture between January 2004 and January 2014. Audiometric parameters, including initial and improved air-bone (AB) conduction gap values, were reviewed in accordance with the temporal bone computed tomography (external auditory canal [EAC], middle ear [ME], mastoid [M], and ossicle [O]). Results Patients with ossicular chain involvement exhibited a larger AB gap compared to those with no ossicular chain involvement at 250, 1,000, 2,000, and 4,000 Hz. Among the groups without ossicular chain involvement, the initial AB gap was largest in patients with EAC+ME+M involvement, followed by the ME+M and M-only involvement groups. The greatest improvement in the AB gap was observed in the EAC+ME+M group followed by the ME+M and M-only groups, irrespective of ossicular chain involvement. Improvements in AB gap values were smallest at 2,000 Hz. Conclusion Conductive hearing loss pattern differed according to the temporal bone area involved. Therefore, areas such as the hematoma and hemotympanum, as well as the fracture line of the temporal bone area, must be evaluated to predict audiologic patterns with otic capsule preserving temporal bone fracture.

An atraumatic cochlear electrode array has become indispensable to high-performance cochlear implants such as electric acoustic stimulation (EAS), wherein the preservation of residual hearing is significant. For an atraumatic implantation, we propose and demonstrate a new improved design of a cochlear electrode array based on liquid crystal polymer (LCP), which can be fabricated by precise batch processes and a thermal lamination process, in contrast to conventional wire-based cochlear electrode arrays. Using a thin-film process of LCP-film-mounted silicon wafer and thermal press lamination, we devise a multi-layered structure with variable layers of LCP films to achieve a sufficient degree of basal rigidity and a flexible tip. A peripheral blind via and self-aligned silicone elastomer molding process can reduce the width of the array. Measuring the insertion and extraction forces in a human scala tympani model, we investigate five human temporal bone insertion trials and record electrically evoked auditory brainstem responses (EABR) acutely in a guinea pig model. The diameters of the finalized electrode arrays are 0.3 mm (tip) and 0.75 mm (base). The insertion force with a displacement of 8 mm from a round window and the maximum extraction force are 2.4 mN and 34.0 mN, respectively. The electrode arrays can be inserted from 360° to 630° without trauma at the basal turn. The EABR data confirm the efficacy of the array. A new design of LCP-based cochlear electrode array for atraumatic implantation is fabricated. Verification indicates that foretells the development of an atraumatic cochlear electrode array and clinical implant.

Superficial siderosis (SS) of the central nervous system has been thought to be a rare condition that generates progressive hearing loss, ataxia, pyramidal signs, and dementia. The main cause of hearing loss by SS is thought to be neuronal. Because there is no histopathologic report of the human temporal bone in SS, there is a debate about the possibility of cochlear involvement. We present a 25-year-old man who was investigated for bilateral progressive sensorineural hearing loss and vestibular failure after head trauma. On brain MRI, SS of the central nervous system was detected. Distortion product otoacoustic emission (DPOAE) and auditory brainstem response (ABR) showed no response on both sides. However, integrity of the eighth nerve was proved by the electrical ABR test on the right side and the patient benefited significantly from cochlear implantation. The sensorineural hearing loss in SS seems to be related to cochlear damage as well as neuronal damage. So, cochlear implantation would be a hearing rehabilitation modality for the sensorineural hearing loss caused by SS.

Although autologous rib cartilage is a preferred source of graft material in rhinoplasty, rib cartilage for dorsal augmentation has been continuously criticized for its tendency to warp and for high donor-site morbidities. However, no meta-analysis or systemic review on complications associated with autologous rib cartilage use in rhinoplasty has been conducted.To carry out a systematic review and a meta-analysis of available literature to evaluate complications regarding autologous rib cartilage in rhinoplasty.The studies reporting complications associated with the autologous rib cartilage use in rhinoplasty were systematically reviewed by searching the MEDLINE, PubMed, and Embase databases for sources published from 1946 through June 2013.The selected articles included clinical studies conducted with at least 10 patients and at least 1 postoperative long-term complication or donor-site morbidity in rhinoplasty. Excluded were nonhuman studies; review articles; case reports; abstracts; and reports of nasal reconstruction as indication for surgery, use of homologous rib cartilage, and diced or laminated methods.Two investigators independently reviewed all studies and extracted the data using a standardized form. A meta-analysis was performed using a random-effects model.Number of patients; follow-up duration; and rates of complication, donor-site morbidity, and revision surgery. Also noted were study authors and year of publication.Ten studies involving a total 491 patients were identified. Mean follow-up across all studies was 33.3 months. In meta-analysis, the combined rates were 3.08% (95% confidence interval [CI], 0%-10.15%) for warping, 0.22% (95% CI, 0%-1.25%) for resorption, 0.56% (95% CI, 0%-2.61%) for infection, 0.39% (95% CI, 0%-1.97%) for displacement, 5.45% (95% CI, 0.68%-13.24%) for hypertrophic chest scarring, 0% (95% CI, 0%-0.32%) for pneumothorax, and 14.07% (95% CI, 6.19%-24.20%) for revision surgery.The overall long-term complications and donor-site morbidity rates associated with autologous rib cartilage use in rhinoplasty were low. Warping and hypertrophic chest scarring showed relatively higher rates, warranting a surgeon's attention. Because a limited number of studies and patients were eligible, and consistent definitions of complications were lacking in this meta-analysis, future studies with a larger series of patients and objective outcome measurements are needed to obtain more reliable results.4.

The dichotomy between acoustic temporal envelope (ENV) and fine structure (TFS) cues has stimulated numerous studies over the past decade to understand the relative role of acoustic ENV and TFS in human speech perception. Such acoustic temporal speech cues produce distinct neural discharge patterns at the level of the auditory nerve, yet little is known about the central neural mechanisms underlying the dichotomy in speech perception between neural ENV and TFS cues. We explored the question of how the peripheral auditory system encodes neural ENV and TFS cues in steady or fluctuating background noise, and how the central auditory system combines these forms of neural information for speech identification. We sought to address this question by (1) measuring sentence identification in background noise for human subjects as a function of the degree of available acoustic TFS information and (2) examining the optimal combination of neural ENV and TFS cues to explain human speech perception performance using computational models of the peripheral auditory system and central neural observers. Speech-identification performance by human subjects decreased as the acoustic TFS information was degraded in the speech signals. The model predictions best matched human performance when a greater emphasis was placed on neural ENV coding rather than neural TFS. However, neural TFS cues were necessary to account for the full effect of background-noise modulations on human speech-identification performance.

This study was conducted to investigate long-term neurocognitive outcomes and to determine associated risk factors in a cohort of Korean survivors of childhood acute lymphoblastic leukemia (ALL). Forty-two survivors of ALL were compared with 42 healthy controls on measures of a neurocognitive test battery. We analysed potential risk factors (cranial irradiation, sex, age at diagnosis, elapsed time from diagnosis, and ALL risk group) on neurocognitive outcomes. ALL patients had lower, but non-significant full-scale intelligence quotient (FSIQ, 107.2±12.2 vs. 111.7±10.2), verbal intelligence quotient (VIQ, 107.7±13.6 vs. 112.2±11.4), and performance intelligence quotient (PIQ, 106.3±14.2 vs. 110.1±10.7) scores than healthy controls. However, patients treated with cranial irradiation performed significantly lower on FSIQ (102.2±8.1), VIQ (103.3±11.7), and PIQ (101.4±13.2) compared to non-irradiated patients and healthy controls. ALL patients also had poor attention, concentration, and executive functions. Among ALL survivors, cranial irradiation was a risk factor for poor FSIQ, being male was a risk factor for poor PIQ, and younger age was a risk factor for poor attention. Therefore, the delayed cognitive effects of ALL treatment and its impact on quality of life require continuing monitoring and management. Graphical Abstract

OBJECTIVE Compared with conventional cochlear electrode arrays, which are hand assembled and wire-based, polymer-based implants have several advantages. They are very precise, and their fabrication is inexpensive because of the use of thin-film processes. In the present study, a cochlear electrode array based on a high-performance liquid crystal polymer material is devised. Furthermore, the device is encapsulated in silicone elastomer. METHODS The fabrication steps introduced here include thin-film processes with liquid crystal polymer (LCP) films and customized self-aligning molding processes for the electrode array. To assess the feasibility of the proposed electrode array, the charge storage capacitance and impedance were measured using a potentiostat. Vertical and horizontal deflection forces were measured using a customized fixture and a force sensor. Insertion and extraction forces were also measured using a transparent human cochlear plastic model, and five cases involving human temporal insertion trials were undertaken to assess the level of safety during the insertion process. RESULTS The charge storage capacity and impedance at 1 kHz were 33.26 mC/cm and 1.02 kΩ, respectively. Likewise, the vertical force and horizontal force of the electrode array were 3.15 g and 1.07 g. The insertion force into a transparent plastic cochlear model with displacement of 8 mm from a round window was 8.2 mN, and the maximum extraction force was 110.4 mN. Two cases of human temporal bone insertion showed no observable trauma, whereas 3 cases showed a rupture of the basilar membrane. CONCLUSION An LCP-based intracochlear electrode array was fabricated, and its electrical and mechanical properties were found to be suitable for clinical use.

Temporal modulation detection ability matures over many years after birth and may be particularly sensitive to experience during this period. Profound hearing loss during early childhood might result in greater perceptual deficits than a similar loss beginning in adulthood. We tested this idea by measuring performance in temporal modulation detection in profoundly deaf children and adults fitted with cochlear implants (CIs). At least two independent variables could constrain temporal modulation detection performance in children with CIs: altered encoding of modulation information due to the CI-auditory nerve interface, and atypical development of central processing of sound information provided by CIs. The effect of altered encoding was investigated by testing subjects with one of two different hearing mechanisms (normal hearing vs. CI) and the effect of atypical development was studied by testing two different age groups. All subjects were tested for their ability to detect acoustic temporal modulations of sound amplitude. A comparison of the slope, or cutoff frequency, of the temporal modulation transfer functions (TMTFs) among the four subject groups revealed that temporal resolution was mainly constrained by hearing mechanism: normal-hearing listeners could detect smaller amplitude modulations at high modulation frequencies than CI users. In contrast, a comparison of the height of the TMTFs revealed a significant interaction between hearing mechanism and age group on overall sensitivity to temporal modulation: sensitivity was significantly poorer in children with CIs, relative to the other three groups. Results suggest that there is an age-specific vulnerability of intensity discrimination or non-sensory factors, which subsequently affects sensitivity to temporal modulation in prelingually deaf children who use CIs.

We aim to evaluate the incidence and clinical manifestations of sensorineural hearing loss (SNHL) in adult patients with acute otitis media (AOM). Seventy-five patients (age > 18 years; 83 ears) diagnosed with AOM between January 2008 and March 2011 at our clinic were enroled and retrospectively reviewed. We detected audiometrically confirmed SNHL during the course of AOM in eight patients. The clinical course, treatment, and audiometric final outcome of each case were reviewed. SNHL was associated with AOM in 8 out of 83 ears (9.3 %). The mean age of patients was 57.5 years, and the mean follow-up period was 21.1 months (range 0.6–46.3 months). The most common symptom was tinnitus. Mean bone conduction hearing threshold was 39.5 dB in pure tone audiometry. All patients showed high-frequency HL, and three showed pan-frequency HL. All patients were treated with oral antibiotics at the initial visit. Seven ears were treated with a combination of oral steroids. Myringotomy was also performed. Seven of eight patients showed improvement; however, 8 kHz thresholds were not improved. This suggested that the inflammation spread through the round window. The mean duration of recovery was 18.6 days. SNHL associated with AOM in adult patients occurs during the early phases of the disease course. High-frequency hearing was commonly affected and was well treated with oral antibiotics, myringotomy, and steroid therapy. Audiometry can be helpful for treating adult patients with AOM. Active treatment, including myringotomy, should be performed during the early phase, if SNHL is suspected.

Functional changes of mitochondrial respiratory chain (MRC) and hearing threshold shifts were observed after noise exposure and the pattern was different depending on the extent of the noise damage.The aim of this study was to reveal any functional changes of the MRC enzyme in outer hair cells after noise damage.Sprague-Dawley rats were used and divided into three groups including normal control group and noise exposure for 1 h and 5 h. White band noise was used with 120 dB SPL. We measured hearing threshold and activity of succinate dehydrogenase (SDH) before noise exposure, and immediately and 1, 3, 7, and 14 days after noise exposure in each group. Immunostaining using monoclonal anti-MRC complex V subunit α was performed. Transmission electron microscopy (TEM) was carried out.The hearing threshold level changed depending on the exposure time in both the noise-exposed groups. In the 1 h group, hearing was improved after 1 day, and in the 5 h group, hearing was measured after 7 days. Changes in SDH activity were observed at the third day in the 1 h group and at the seventh day in the 5 h group. The expression level of MRC complex V was increased after noise exposure. TEM showed mitochondrial damage.

The objective of the study was to determine the therapeutic efficacy of early combined treatment with intratympanic steroid injection (ITSI) in patients with severe to profound sudden sensorineural hearing loss (SSNHL), who did not respond to initial systemic steroid treatment (SST). The study design included historical controlled trials (retrospective analysis for the SST group). Patients (n = 73; 38 women and 35 men) diagnosed with severe to profound SSNHL at the time of their first visit in tertiary referral centers were recruited. Among these 73 patients, 30 patients who showed no early response within a week after the start of initial SST were prospectively included as the ITSI group. ITSI was performed twice a week for two consecutive weeks. In contrast, 43 patients with the same condition who did not receive ITSI were retrospectively included as the SST group. For each group, pure-tone audiogram (PTA) and speech discrimination score (SDS) tests were performed before SST and more than 2 months after termination of treatment in each group. After termination of each treatment, the final average gain on PTA showed no significant difference between the two groups; however, the final average gain of SDS demonstrated a significantly better recovery in the ITSI group. ITSI as part of an early combined therapy represents an effective treatment in terms of the improvement of SDS in severe to profound SSNHL showing no early response to initial SST.