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Background A considerable number of patients with sudden sensorineural hearing loss (SSNHL) have been reported to have dizziness. Objective To analyze vestibular functions and identify the clinical characteristics of SSNHL with dizzinessMETHODS:71 patients with SSNHL who complained of dizziness were investigated retrospectively. The patients underwent vestibular function tests consisting of video-nystagmography, video head impulse test and vestibular evoked myogenic potentials. Results Among 35 patients with spontaneous nystagmus (SN), 21 showed ipsilesional posterior canal gain deficit, 11 showed a gain deficit in the posterior canal only and 9 showed both horizontal and posterior canal gain deficit in video head impulse test. In only one subject with bilateral horizontal canal gain deficit with contralesional SN, AICA infarction was observed.Among 36 patients without SN, 10 were diagnosed as benign paroxysmal positional vertigo and 5 had ipsilesional canal paresis in caloric test. Other 21 patients showed no abnormal signs in vestibular function tests. Conclusions In VFT analysis of SSNHL with dizziness, diverse patterns were identified. In the absence of SN, no definite vestibular organ involvement was the most frequent. But in the case with SN, posterior canal deficit was most common and cerebellar ischemic stroke was rare.

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.

OBJECTIVE To compare the results of suppression head impulse paradigm (SHIMP) and head impulse paradigm (HIMP) in acute vestibular neuritis (AVN). STUDY DESIGN Retrospective study. SETTING Tertiary otology clinic. PATIENTS AND INTERVENTIONS We tested 21 patients with AVN with the HIMP, SHIMP, and caloric tests, and we analyzed the relationships between the tests' results. MAIN OUTCOME MEASURES For this study, we adopted vestibulo-ocular reflex (VOR) gains in the SHIMP and HIMP, peak saccade velocity (PSV) of SHIMP which is another indicator of residual vestibular function, and canal paresis of caloric test. RESULTS VOR gains showed significant correlation (R = 0.926, p

OBJECTIVE The purpose was to evaluate brain plasticity that contributes to speech performance after cochlear implantation (CI) in postlingual elderly (>60 years) patients. MATERIALS AND METHODS Fifteen elderly postlingual deaf patients who underwent preoperative brain fluorodeoxyglucose positron emission tomography (FDG-PET) and were followed-up for more than 1 year after CI were included. The mean age of these patients was 64.6 years (range, 60-80 years). Based on their sentence score at 1 year after CI surgery, the patients were classified into two groups: poor performers (CID score of

Evaluation of the characteristic differences between click-and CE-Chirp-evoked auditory brainstem responses (ABRs) in normal hearing and sensorineural hearing loss.A prospective study. Ears with normal hearing and with sensorineural hearing loss were evaluated. Pure-tone audiometry and click-and CE-Chirp evoked ABRs exams were conducted for all ears. Visual detection levels, wave-V amplitudes, and latencies of the ABRs were assessed.Twenty-two ears with normal hearing and 22 ears with sloping type sensorineural hearing loss were examined.In normal-hearing ears, mean amplitudes were larger for CE-chirps than for clicks at all intensities until 80 dB nHL, at which the amplitudes dropped off, presumably due to upward spread of excitation. In ears with sensorineural hearing loss, however the drop-off was less significant at 80 dB nHL. Comparisons with pure-tone audiometry findings revealed ABRs to CE-Chirps to correlate at 0.5, 1, 2, and 3 kHz, and to clicks at 1, 2, 3, and 4 kHz.The CE-Chirp has advantages over clicks for examining normal ears. However, under high-level stimulation, these advantages are no longer present. In ears with sensorineural hearing loss, the upward spread of excitation is less prominent. The CE-Chirps results correlate significantly to low frequency audiometric findings at 0.5 kHz, while clicks do not.

To evaluate the efficacy of tinnitus retraining therapy (TRT), especially in patients who did not revisit the clinic after starting the program, and to determine the current status of patients who were lost to follow-up.Telephone survey.Tertiary referral center.Forty-seven patients enrolled in a TRT program at Seoul National University Hospital. Twenty-four patients who regularly visited the clinic were deemed the good follow-up (GF) group, and 23 patients who did not return after the initial counseling were deemed the follow-up loss (FL) group.Post-TRT questionnaires with the Tinnitus Handicap Inventory (THI) and a tinnitus visual analog scale (VAS), performed with a telephone survey.TRT efficacy, evaluated via the THI, and tinnitus VAS scores. A questionnaire on reasons for non-attendance after TRT was used.Pre-TRT VAS and THI scores showed no significant difference between groups. After TRT, both groups showed significant decreases in tinnitus VAS and THI scores. Post-TRT VAS scores for awareness, effects on daily life, and post-TRT THI scores were significantly lower in the FL group.Treatment outcomes were better in the FL group than in the GF group in several parameters. The FL group was greatly influenced by the first TRT counseling and obtained sufficient relief that they no longer felt the need for additional treatment. Treatment outcomes in patients lost to follow-up may not be as bad as presumed.

A narrow bony cochlear nerve canal (BCNC) is associated with sensorineural hearing loss necessitating cochlear implantation (CI). This study evaluated the implications of BCNC width for post-CI outcomes. A total of 56 children who had received CIs were included. The patients were divided into three groups according to the width of the BCNC (Group 1: diameter < 1.4 mm, n=17; Group 2: diameter 1.4–2.0 mm, n=14; Group 3: diameter > 2.0 mm, n=25). The post-CI speech performances were compared among the three groups according to BCNC width. The correlation between BCNC width and post-CI speech performance was evaluated. Logistic regression analysis was also performed to investigate factors that can impact post-CI speech performance. Cochlear nerve deficiency (CND) occurred more frequently in Group 1. Groups 1 and 2 had significantly worse post-CI outcomes. Patients with intact cochlear nerves had significantly better post-CI outcomes than those with CND. When the cochlear nerve was intact, patients with a narrower BCNC showed less favorable results. Therefore, patients with either a narrow BCNC or CND seemed to have poorer outcomes. A narrow BCNC is associated with higher CND rates and poor outcomes. Measurement of BCNC diameter may help predict CI outcomes.

Objective To extend our preliminary study by comparing the hearing outcomes of handle-malleostapedotomy (handle-MS; connecting the prosthesis to the malleus handle) and neck-MS (connecting the prosthesis to the malleus neck) in a larger case series. Patients Thirty-five patients who underwent MS at Seoul National University Hospital from January 1983 to December 2013. Intervention Review of pre- and postoperative audiometric data, intraoperative findings. Main outcome measures Postoperative air-bone gap (ABG) and closure of ABG. Results In 11 of 20 patients (55.0%) in the handle-MS group and 12 of 15 (80%) in the neck-MS group, the postoperative ABG closed to within 20 dB HL. For frequencies of 0.25, 0.5, 1, 2, 3, and 4 kHz, the ABGs did not differ significantly between the two groups. The postoperative ABG closure was lower in the neck-MS group (22.3 ± 12.6 dB HL) than in the handle-MS group (23.5 ± 11.6 dB HL), but without statistical significance (p = 0.78). Four patients in each group underwent MS as a revision procedure. One of them underwent neck-MS as revision surgery because of prosthesis extrusion after handle-MS. The mean ABG closure at 1 year postoperatively in patients who underwent revision neck-MS (19.9 ± 11.9 dB HL) was not different from that of the patients who underwent revision handle-MS (20.9 ± 8.7 dB HL). Conclusion As neck-MS provided results comparable to those of handle-MS in our extended case series, it might be a useful procedure in selected cases of otosclerosis or stapes fixation with an incus anomaly, especially in revision cases after handle-MS.

Appropriate customized auditory rehabilitation for hearing impaired subjects requires prediction of residual hearing and progression of hearing loss. Mutations in TMPRSS3 encoding a transmembrane serine protease were reported to be associated with two different autosomal recessive nonsyndromic hearing loss (arNSHL) phenotypes, DFNB8 and DFNB10, in terms of residual hearing that may mandate different rehabilitation. We aimed to reveal the genetic contribution of TMPRSS3 mutations among Korean populations and to correlate the clinical phenotype with TMPRSS3 genotypes. Fifty families that segregated arNSHL and have visited our clinic recently for 2 years were recruited for TMPRSS3 screening. Novel TMPRSS3 variants detected in our cohort were modeled using a predicted three-dimensional (3D) structure of the serine protease domain. The prevalence reached up to 11.2 % (3/27) among subjects with either prelingual hearing loss but retaining some degree of language development or with postlingual ski-slope hearing loss. We also found that a p.A306T allele is a founder allele in this population. Based upon the 3D modeling, we were able to correlate significant retention of residual low-frequency hearing and slower progression of its loss to this novel variant p.T248M that was predicted to have milder pathogenicity. A yeast-based protease assay confirmed a mild pathogenic potential of the p.T248M variant and a tight correlation between the protease activity and the residual hearing. Preservation of this low-frequency hearing should be of utmost importance when considering auditory rehabilitation. Our results significantly narrow down the candidate population for TMPRSS3 sequencing for more efficient genetic diagnosis. More importantly, genotype-phenotype correlation of this gene observed in our cohort suggests that TMPRSS3 can be an appropriate candidate for personalized and customized auditory rehabilitation.The prevalence of TMPRSS3 mutations among Korean postlingual hearing loss is 8.3 %. The p.A306T variant of TMPRSS3 is the common founder allele in Koreans. A novel variant, p.T248M of TMPRSS3, was predicted to have milder pathogenicity. There was a genotype-phenotype correlation of this gene in Koreans. Our data support implication of this gene for personalized rehabilitation.

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.

Objective: To determine the optimal surgical approach for cochlear implantation (CI) preoperatively based on the spatial relation of a displaced facial nerve (FN) and middle ear structures and to analyze clinical outcomes of CHARGE syndrome. Methods: Facial nerve displacement and associated deviation of inner ear structures were analyzed in 13 patients (17 ears) with CHARGE syndrome who underwent CI. Surgical accessibility through the facial recess was assessed based on anatomical landmarks. Postoperative speech performance and associated clinical characteristics were analyzed. Results: The most consistently identified ear anomalies were semicircular canal aplasia (100%), ossicular anomaly (100%), and vestibular hypoplasia (88%). Facial nerve displacement was found in 77% of cases (anteroinferior: 47%, anterior: 24%, inferior: 6%). The width of available surgical space around facial recess was significantly greater in cases of facial recess approach (2.85 ± 0.9 mm) than those of alternative approach (0.12 ± 0.29 mm, P = .02). Postoperatively, 53% achieved better than category 4 on the categories of auditory perception (CAP) scale. The CAP category was significantly correlated with internal auditory canal diameter ( P = .025) and did not differ according to the applied surgical approach. Conclusion: Preoperative determination of surgical accessibility through facial recess would be useful for safe surgical approach, and successful hearing rehabilitation was achievable by applying appropriate surgical approaches.

Objectives/Hypothesis To identify the clinical and laboratory characteristics of bilateral vestibulopathy (BVP) on the video head impulse test (vHIT). Study Design Retrospective chart analysis. Methods During 23 months, 1,789 patients with dizziness underwent vHIT in our tertiary referral hospital. Of these patients, 65 (3.6%) patients had bilaterally positive catch-up saccades. Based on the caloric test, 15 (group 1) had bilateral caloric weakness, 13 (group 2) had unilateral caloric weakness, and 37 (group 3) had normal caloric responses on both ears. We collected data on these patients regarding demographics, symptoms, gain, and type of saccade on horizontal canal plane vHIT, as well as gain and time constant on velocity step of the rotatory chair test. Results The average age of group 2 (70.38 ± 11.96 years) and group 3 (69.03 ± 11.01 years) were significantly older than that of group 1 (54.80 ± 11.96 years) (P = 0.029, P = 0.003, respectively). Although all patients had bilaterally positive vHIT, 10 of 15 in group 1 were finally diagnosed as classical BVP by clinical features. On comparison of average gain on bilateral horizontal vHIT, groups 2 (0.71 ± 0.17) and 3 (0.80 ± 0.14) had higher gain compared to group 1 (0.45 ± 0.22) (P = 0.001, P = 0.000, respectively). On velocity step test, time constant and gain of group 3 (11.60 ± 3.07, 0.49 ± 0.13) was significantly higher than those of group 1 (4.92 ± 1.36, 0.22 ± 0.17) (P = 0.000, P = 0.004, respectively). On the receiver operating characteristic curve analysis, vHIT alone seemed to be a discordant method for diagnosis of BVP compared to the caloric and step velocity tests. Conclusion About 3.6% patients with dizziness showed bilateral vestibular ocular reflex deficit during high-frequency acceleration, which was prevalent especially in elderly patients. Also, positive bilateral vHIT does not always correlate with caloric or rotatory chair test results. This may imply that a diverse spectrum of vestibulopathies exist according to the stimulation frequency of deficit. Level of Evidence 4. Laryngoscope, 2016

Most X-linked nonsyndromic hearing loss is caused by various types of mutations of the POU domain class 3 transcription factor 4 gene (POU3F4). We found five unique missense and frameshift truncation and extension mutations in Korean patients. Two missense mutations (p.Thr211Met and p.Gln229Arg) disturbed transcriptional activity. Two frameshift extension mutations (p.Thr354GlnfsX115 and p.X362ArgextX113) were located outside of POU domain and nuclear localization signal (NLS) at the C-terminus. POU3F4 protein levels were low and could be restored by MG132, a proteasome inhibitor, in vitro. These mutant POU3F4 proteins were exclusively localized to the cytoplasm and did not have transcriptional activity. Frameshift mutation (p.Leu317PhefsX12) in POU3F4 leads to the truncation of the C-terminal 44 amino acids spanning the POU domain and NLS. This frameshift truncation mutant protein was located in both the nucleus and cytoplasm and was present at low protein levels. This mutant was also transcriptionally inactive, even in the presence of MG132. From these results, we conclude that frameshift truncation and extension mutations in the C-terminus of POU3F4 lead to cytoplasmic localization and subsequent proteosomal degradation due to structural aberrations, which cause transcriptional inactivity and thus nonsyndromic hearing loss.

Systemic application of human mesenchymal stem cells (hMSCs) may be another effective tool for stem cell supply to the inner ear.Most researchers have used local application to provide the inner ear with stem cells. In spite of their efficacy, these methods can potentially cause irreversible damage to the inner ear. A homing phenomenon of systemically administered MSCs to the ischemic myocardium and brain was recently reported. Moreover, hMSCs have several advantages over conventional neural or embryonic stem cells. Thus we hypothesized that intense noise or ototoxic injury to the cochlea could induce the homing of hMSCs.We harvested hMSCs from bone marrow of the iliac crest of five normal individuals. Then hMSCs at a dose of 4 × 10(6) cells were given via intravenous injection of cell suspension into rats with cochleae damaged by noise or ototoxic drugs. Histological analysis was undertaken 30 days later.Systemically delivered hMSCs were usually largely entrapped in the lungs. However, we documented the homing of some hMSCs to the cochlea with degenerated inner hair cells. The recruitment of hMSCs was limited to the spiral ganglion area only. The migration of donor cells into the cochlea was accompanied by the expression of brain-derived neurotrophic factor (BDNF).

This study aimed to investigate the most prevalent stage in each age-group of children with congenital cholesteatoma (CC) and verify the correlation between the stage and the age of the patients for the type of CC ("closed" keratotic cyst and "open" infiltrative types). Patients diagnosed with CC between 2004 and 2009 (n = 156; 116 boys and 40 girls; mean age, 5 years and 5 months; range, 12 months-16 years and 7 months) were enrolled retrospectively. Assessment of stage and type of CC was performed with preoperative high-resolution temporal bone computed tomography and intraoperative findings. The stage of CC was determined using Potsic's staging system classified into four stages according to ossicular involvement and mastoid extension. The patients consisted of groups divided on the basis of a 2-year interval. The prevalence of stage I began to decline from the age-group of 1-2 years. In contrast, the prevalence of stages III increased from the age-group of 3-4 years and that of stage IV from the age-group 5-6 years. The prevalence of "open" infiltrative type CC increased from approximately the age of 7 years, thus showing some correlation with age but not with stage. The prevalence of CC with the advanced stage increased since the age of 2 years. Therefore, the importance of early diagnosis and treatment of CC should be more emphasized.

Mean incudostapedial joint (ISJ) angulation of atretic ears was statistically wider than ISJ angulation of non-atretic ears. Postoperative hearing results in the narrow tympanoplasty type II (T2) subgroup were better than those in the wide T2 subgroup. In cases of abnormally wide ISJ angulation, we recommend that partial ossicular reconstruction (POR) be used as a surgical means of achieving hearing improvement.To report the results of ISJ angulation in patients with congenital aural atresia and its effects on postoperative hearing improvement.Patients undergoing canaloplasty with T2 (149 ears) and canaloplasty with POR (32 ears) were enrolled. The T2 group was dichotomized according to an ISJ cut-off of an angle of 120° into the 'narrow' T2 and 'wide' T2 subgroups. The pre- and postoperative air-bone gap (ABG) and ABG change were analyzed.Mean ISJ angulation of non-atretic ears was 93.06 ± 13.21° and that of atretic ears in the T2 group was 118.39 ± 19.60° (p0.001). Mean short- and long-term postoperative ABGs were better in the narrow T2 subgroup than in the wide T2 subgroup (p = 0.01 and 0.03, respectively). The short-term postoperative ABG and ABG change in the POR showed superior results to those of the wide T2 subgroup (p0.001 and 0.041, respectively).

Context: Enlargement of the vestibular aqueduct (EVA) is a commonly detected inner ear anomaly related to hearing loss and often associated with mutations of SLC26A4 encoding pendrin, a transmembrane exchanger of Cl−, I−, and HCO3−. Here we describe the phenotypes of 27 Korean EVA subjects and their SLC26A4 genotypes determined by bidirectional nucleotide sequencing. Results: The detected variants include two novel missense substitutions (p.V138L and p.P542R). We characterized the ability of p.V138L and p.P542R pendrin products to traffic to the plasma membrane in COS-7 cells and to transport Cl−, I−, and HCO3− in Xenopus oocytes. The results indicate that p.P542R is a benign polymorphic variant, whereas p.V138L is a pathogenic mutation. Since this and other studies of East Asian EVA cohorts show that the majority of SLC26A4 mutations affect either or both of two amplicons (exons 7–8 and 19), we developed a hierarchical protocol that integrates direct sequencing with denaturing high-performance liquid chromatography analyses for detection of SLC26A4 mutations in these populations. We validated the cost efficiency of the integrated protocol by a simulated screen of published East Asian EVA cohorts with known SLC26A4 genotypes. Conclusions: Our study further defines the spectrum of SLC26A4 mutations among East Asians and demonstrates a rapid and efficient protocol for their detection.

The first objective of this study was to identify rates, types, and laterality of clinically relevant inner ear malformations in children with unilateral sensorineural hearing loss (USNHL). The second objective was to assess the change of the ipsilesional and contralesional hearing thresholds of the patients with USNHL and the association between hearing change with time and the findings on high-resolution temporal bone computed tomography (TBCT).Retrospective case review.Tertiary referral center.A total of 322 children diagnosed with USNHL on initial audiometry were included.Otologic examination, pure-tone audiometry or auditory brainstem response, and high-resolution TBCT.Radiologic findings demonstrating clinically relevant bony or soft tissue malformations of the inner ear. Audiologic findings of change in thresholds.In a series of 322 consecutively investigated children with USNHL, 93 TBCT scans (28.9%) were identified as abnormal. The abnormal CT findings included cochleovestibular malformations (49 cases; 52.7%), vestibular malformations (27 cases; 29.0%), and malformations of vestibular or cochlear aqueducts (17 cases; 18.3%). Of these abnormal CT findings, 18 cases (19.4%) showed bilateral malformations. Incomplete partition type II was the most common type of malformation (28 cases), followed by narrow internal auditory canal (23 cases) and subsequently followed by enlarged vestibular aqueduct syndrome (17 cases). Of 244 patients, 77 (31.6%) in the profound USNHL group accompanied malformations, which was significantly higher than 23.8% (15 of 78) in the nonprofound (mild to severe) USNHL group (p = 0.036). Of 115 patients who were regularly followed up for more than 6 months, 7 (6.1%) had hearing decrement (including 3 cases of bilateral decrement). Of 85 patients in the normal TBCT group, 4 (4.7%) revealed hearing decrement, and considering there were only 27 patients with nonprofound USNHL in this group, the rate of decrement was as much as 14.8% (4 of 27). Of 24 patients in the unilateral malformation group, 1 patient (4.2%) showed worsening of hearing, and 2 of 6 (33.3%) patients showed worsening of hearing in the bilateral malformation group.Based on the results, we propose all children with USNHL should have a TBCT scan because management, including genetic counseling and prognostic predictions of these cases, may be significantly influenced by the CT outcome. Moreover, the non-negligible rate of progressive nature of ipsilateral and contralateral SNHL in normal-looking TBCT group and in groups with inner ear malformations mandates longitudinal audiologic assessments for both ears

Benefits of the use of anteriorly and inferiorly based periosteal flaps (AIPFs) in congenital aural atresia (CAA) patients was found to be effective at reducing canal stenosis (CS) occurrence by long term F/U. However, in terms of minimizing CS, in addition to recruitment of AIPFs, considerations of patient factors, such as degree of microtia and age are mandatory.AIPF during canaloplasty were evaluated with a specific focus on whether this technique can offset the negative effects of several risk factors for postoperative CS after CAA surgery.The authors undertook a retrospective review of the medical records of 164 congenital aural atresia patients (190 ears) who had undergone surgery at Seoul National University Hospital. Median follow up period was 54 months. The anterior approach surgical method with and without the use of AIPFs were utilized in 111 and 79 ears respectively. Comparison of the influences of several factors on CS occurrence was undertaken by statistical analyses to evaluate whether this AIPFs technique can counterbalance the negative effect of patient factors in postoperative CS.Those with a younger age (12 yrs), moderate to severe microtia (grade II, III), or those in whom AIPF was not used in surgery (non AIPF group) were found to show statistically significant higher frequency of CS(+). Nevertheless, the positive effect of AIPF was not able to completely counterbalance the effects of negative patient factors on CS development. A protocol compatible with the results of this study that minimizes CS is presented in the discussion.

To review surgical findings and hearing outcomes of incus footplate assembly (IFA) for the patients with conductive hearing loss due to missing stapes superstructure with a mobile stapes footplate.Retrospective case review and survey.Pre- and postoperative audiometric data and intraoperative findings were reviewed. Postoperative air-bone gap (ABG) and ABG closure (postoperative air-conduction threshold-preoperative bone-conduction threshold) were analyzed.The causes of missing stapes superstructure and conductive hearing loss were congenital ossicular anomaly (n = 5), chronic otitis media (n = 2), and congenital cholesteatoma (n = 1). The prosthesis was designed to fit between the medial side of the incus and stapes footplate and had a mean length of 3.6 ± 0.5 mm. The mean pre- and postoperative ABG were 38.3 ± 4.8 and 13.3 ± 10.0 dB, respectively. The postoperative ABG at frequencies of 0.25, 0.5, 1.0, 2.0, 3.0, and 4.0 kHz were 20.0 ± 15.4, 16.9 ± 11.9, 16.3 ± 10.3, 10.6 ± 7.3, 12.9 ± 14.0, and 23.1 ± 16.2 dB, respectively. The mean ABG closure was 9.5 dB (range, -1.3∼35.8 dB). Seven cases obtained the best results (mean ABG closure ≤10 dB). In the remaining patient, the mean ABG closure was 9.5 dB until 6 months after surgery, but was 35.8 dB 1 year after surgery.IFA seems to be a reasonable surgical option in patients with missing the stapes superstructure, but with a mobile footplate in which the long process of incus is preserved.4 Laryngoscope, 126:2569-2573, 2016.

To evaluate the correlation between a narrow bony cochlear nerve canal (BCNC) and facial nerve stimulation (FNS) after cochlear implantation (CI) and their underlying mechanisms and to predict the risk of FNS preoperatively.Retrospective cohort study.A total of 64 pediatric cases that underwent CI were included. Among them, 32 cases experienced FNS after CI, and another 32 cases were selected from 817 pediatric implantees by stratified random sampling. The width of the BCNC, the status of the cochlear nerve (CN) and the internal auditory canal (IAC), T level, and C level were compared and analyzed. Strategies for eliminating FNS were also analyzed.The FNS group showed a narrower BCNC (1.09 ± 0.52 mm) than the control group (1.99 ± 0.61 mm; P.01), a lower CN/facial nerve ratio (0.32 ± 0.36) than the control group (1.34 ± 0.76; P.01), and narrower IAC diameter (4.06 ± 1.71 mm) than the control group (5.66 ± 1.36 mm; P.01). The FNS group also showed higher T level (165.7 ± 28.3 μA) than the control group (142.2 ± 21.2 μA; P.01). Adjustment of the C levels and/or pulse width and switching off offending electrodes were attempted to eliminate FNS, with a 75.0% success rate. The FNS group still showed low Categories of Auditory Performance scores (3.00 ± 1.90) compared with the control group (5.94 ± 1.41, P.01) after adjustment.A narrow BCNC could be a cause of FNS after CI. Therefore, careful selection of the side for CI and programming strategies are required to reduce FNS.4. Laryngoscope, 126:1433-1439, 2016.

Objectives (1) To assess the long-term effects of cochlear implantation (CI) on speech perception and school life, and (2) to evaluate behavioral and emotional development, including social skills, post-implantation. Methods We telephoned caregivers and asked them a standardized questionnaire. We used the data to explore the daily lives (including school life) of children who had undergone CI before the age of 4 years and who had used the device for >10 years. We also evaluated behavioral/emotional development. Results Most children used CI devices for virtually all their waking moments and attended mainstream schools. Moreover, more than 75% of them could comprehend common phrases or conversations without lip-reading and carry out a telephone conversation with a person known to them. The mean T-scores for all scales of behavioral/emotional assessment, including those concerning social skills, were within the normal ranges, although scores on the competence scale were lower than those on other scales. Conclusions This long-term study of early-CI children shows that CI improves not only speech perception, but also behavioral/emotional development, including social skills.

To analyze the clinical manifestations and genetic features of patients with hearing loss (HL) and incomplete partition (IP) type III malformation, and to evaluate speech performance after cochlear implantation (CI) in these patients.Individual retrospective cohort study.Of 206 probands with inner ear malformations (IEMs), we constructed a homogeneous cohort of 11 genetically documented IP type III (DFNX2). Mutations affecting POU3F4 were classified as extension (n = 2), truncation (n = 3), large genomic deletion (n = 2), or missense substitution (n = 4). Postoperative outcomes were rigorously assessed with focus on POU3F4 genotypes and compared with 80 age-matched implantees without IEMs.HL in our cohort was prelingual in onset irrespective of degree. Serviceable hearing was obtained by wearing conventional hearing aids in three, and eight subjects required CI. No correlation was found between mutation types and initial auditory phenotype. As for the eight cochlear implantees, average Categories of Auditory Perception score was1 preoperatively and 3.9 at 2 years post-CI. Speech performances improved over the first 3 months postoperatively in a manner comparable to implantees without IEMs. However, it then tended to slow down until 1 year postoperatively, leading to worse scores at 2 years than implantees without IEMs. Furthermore, this was more evident in those with a truncation or deletion mutation.CI surgeons should be aware that postoperative auditory performance may be not as good in IP type III patients as in patients without IEMs. In particular, our study implies that certain types of POU3F4 mutations may have poorer prognoses than other types after CI.4. Laryngoscope, 126:E123-E128, 2016.

To propose management options for cochlear implantation in chronic otitis media based on our experiences.A retrospective review of 418 cochlear implantations performed by the 2 senior authors between November 1988 and February 2004 was conducted. Nine patients who had chronic otitis media in the ear to be implanted were included. Of these, three showed active inflammation at presentation; the other six cases had undergone previous tympanomastoidectomy surgery and did not show active inflammation at presentation.Five patients with active inflammation or without an adequate soft tissue layer in the mastoid bowl underwent a two-stage procedure. Four cases who showed inactive inflammation and had an adequate tissue layer to protect the electrode array underwent a single-stage technique, although two of them showed dry tympanic membrane perforation. No local or intracranial inflammation recurred. The electrode was exposed in the mastoid bowl in one case, who was managed with revisional mastoid obliteration with soft tissue.Complete eradication of inflammation and the securing of a strong protective soft tissue layer over the electrode are prerequisites for cochlear implantation in ears with chronic otitis media.

Objective: To analyze the results on a series of pediatric cholesteatomas, using various preoperative factors, including some of the proposed classification systems, to identify those factors that influence the postoperative result. Method: Retrospective review of 118 children with acquired cholesteatoma who underwent surgery from January 1978 to October 1999. The median observation period was 5 years, range 1–20 years. The rate of recidivism calculated using the Kaplan–Meier method and relative risk by the Cox regression model. Results: Cholesteatoma in patients less than 8 years old with mastoid involvement had significantly higher risk of recidivism than the other groups examined. Conclusions: In this study, cholesteatoma recurred up to 5 years after operation, and long-term close follow-up is important especially in cases involving higher risk such as younger child with mastoid extension of cholesteatoma.

Objective This study was designed to evaluate the binaural effects from bimodal hearing according to the aided hearing threshold in the nonimplanted ear. Materials and methods Subjects included 17 individuals who continued to use a hearing aid (HA) in the nonimplanted ear for more than 6 months postoperatively. Speech perception and sound localization were tested with unilateral cochlear implantation (CI) and bimodal hearing with and without background noise. Materials were presented at an average of 70 dB sound pressure level from a front loudspeaker in a quiet condition and then with background noise at a signal-to-noise ratio of +10 dB HL. Speech perception scores were based on percent-correct performance of repeating a spoken word under each condition. Sound localization scores were obtained by averaging the sum of angle differences between the active loudspeaker and the loudspeaker indicated by the subject. Results Speech perception scores (mean ± SD) of unilateral CI and bimodal hearing were 63.3% ± 17.7% and 73.1% ± 18.5% under the quiet condition (p = 0.029) and 65.5% ± 21.9% and 70.9% ± 23.6% under the noisy condition (p = 0.01), respectively. Angle differences (mean ± SD) of unilateral CI and bimodal hearing were 72.8 ± 27.4 degrees and 84.1 ± 29.9 degrees under the quiet condition (p = 0.052) and 79.3 ± 26.9 degrees and 77.3 ± 22.0 degrees under the noisy condition (p = 0.906), respectively. Patients were divided into two groups according to their aided hearing thresholds: Group 1 (aided hearing threshold ≤50 dB HL; n = 8) and Group 2 (aided hearing threshold >50 dB HL; n = 9). The speech perception scores of bimodal hearing in each group were 85.3% ± 13.3% and 60.8% ± 17.5% (p = 0.023) under the quiet condition and 82.7% ± 9.0% and 59.4% ± 26.8% under the noisy condition (p = 0.052), respectively. For sound localization, the angle differences of bimodal hearing in each group were 54 ± 28.6 degrees and 83.9 ± 11.9 degrees under the quiet condition (p = 0.042) and 63.0 ± 23.5 degrees and 89 ± 22.6 degrees under the noisy condition (p = 0.049), respectively. Conclusion Based on the relationship between the aided hearing level and bimodal hearing performance, this current study suggests that bimodal benefits for sound localization and speech perception in noise are significant but only when sound detection is adequate for the hearing aids. Therefore, bimodal hearing could be applied to selective patients with favorable aided hearing levels.

OBJECTIVE: The aim of this study was to understand the prevalence and molecular genetic etiology of incomplete partition type III (IP type III) anomaly in Koreans. We also attempted to verify the prevalence of genomic deletions in the DFNX2 locus and to look for association between inheritance patterns and mutation type in East Asian IP type III subjects. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Subjects with IP type III anomaly and their biological mothers. INTERVENTIONS: Sanger sequencing, array-comparative genomic hybridization (aCGH), and PCR were performed. We also analyzed the type and inheritance of the causative genetic abnormality in East Asian DFNX2 patients. MAIN OUTCOME MEASURE: Mutation type and occurrence. RESULTS: We identified IP type III in 10 (4.8%) of 206 patients with an inner ear abnormality. We confirmed an etiologic homogeneity, DFNX2, of the IP type III in this Korean population. Two (20%) of the 10 DFNX2 carried a large genomic deletion affecting POU3F4, as proved by aCGH. PCR confirmed that the 2 deletions occurred de novo. Genetic alteration occurred de novo in 29.4% (5/17) of all reported Korean IP type III cases. From this study and literature review, we observed a striking difference of de novo occurrence rate (75% versus 12.5%, p = 0.032) between large genomic deletions and point mutations in East Asian population. CONCLUSIONS: Our data suggest that different POU3F4 mutations might show different recurrence rate in siblings of the IP type III families, especially in East Asian population. Genetic counseling should be provided accordingly.

In order to assess the applicability of transient evoked otoacoustic emissions (TEOAEs) measurements on the sequential monitoring of children with middle ear effusion (MEE), the effects of MEE on TEOAEs response were investigated. TEOAEs responses were recorded on 56 ears of 33 patients before and after tympanostomy tube placement. In the 37 ears with MEE seen at myringotomy, the average band reproducibility below 2 kHz recovered significantly after tube placement, whereas the average band reproducibility at 5 kHz diminished. In the 19 ears without MEE, no significant changes in echo amplitude and band reproducibility were noted in the post-operative TEOAEs measurements compared with pre-operative ones. The results of this study suggest that an improvement in echo response and band reproducibility below 2 kHz in serial TEOAEs measurements may indicate resolution of MEE in children with otitis media with effusion.

While deafness-induced plasticity has been investigated in the visual and auditory domains, not much is known about language processing in audiovisual multimodal environments for patients with restored hearing via cochlear implant (CI) devices. Here, we examined the effect of agreeing or conflicting visual inputs on auditory processing in deaf patients equipped with degraded artificial hearing. Ten post-lingually deafened CI users with good performance, along with matched control subjects, underwent H 2 15 O-positron emission tomography scans while carrying out a behavioral task requiring the extraction of speech information from unimodal auditory stimuli, bimodal audiovisual congruent stimuli, and incongruent stimuli. Regardless of congruency, the control subjects demonstrated activation of the auditory and visual sensory cortices, as well as the superior temporal sulcus, the classical multisensory integration area, indicating a bottom-up multisensory processing strategy. Compared to CI users, the control subjects exhibited activation of the right ventral premotor-supramarginal pathway. In contrast, CI users activated primarily the visual cortices more in the congruent audiovisual condition than in the null condition. In addition, compared to controls, CI users displayed an activation focus in the right amygdala for congruent audiovisual stimuli. The most notable difference between the two groups was an activation focus in the left inferior frontal gyrus in CI users confronted with incongruent audiovisual stimuli, suggesting top-down cognitive modulation for audiovisual conflict. Correlation analysis revealed that good speech performance was positively correlated with right amygdala activity for the congruent condition, but negatively correlated with bilateral visual cortices regardless of congruency. Taken together these results suggest that for multimodal inputs, cochlear implant users are more vision-reliant when processing congruent stimuli and are disturbed more by visual distractors when confronted with incongruent audiovisual stimuli. To cope with this multimodal conflict, CI users activate the left inferior frontal gyrus to adopt a top-down cognitive modulation pathway, whereas normal hearing individuals primarily adopt a bottom-up strategy.

A p.V37I variant of GJB2 has been reported from subjects with moderate or slight hearing loss especially in East Asian populations. This study aimed to estimate the prevalence of the p.V37I variant among such subjects and prove, epidemiologically, its pathogenic potential to cause mild hearing loss. A total of 380 subjects from 201 families with hearing loss were enrolled. From them, 103 families were selected who had autosomal recessive inheritance or sporadic occurrence of hearing loss and who were younger than 15 years old. GJB2 sequencing was carried out for the probands of all 103 families. The prevalence of the p.V37I variant was compared between the subtle, mild or moderate hearing loss (group I) and the severe or profound hearing loss (group II) groups. Where possible, a targeted next generation sequencing of 82 deafness genes was performed from the p.V37I carrier to exclude the existence of other pathogenic genes. Five (4.8%) of 103 probands were found to carry p.V37I. The carrier frequency of p.V37I among group I (18.2%) was significantly higher than that of group II (1.2%) or the reported Korean normal hearing control group (1.0%). Detection of the p.V37I variant of GJB2 in 18.2% of Koreans with mild hearing loss strongly suggests its contribution to the pathogenesis of milder hearing loss, which might justify sequencing of GJB2 from these subjects in the Korean population.

Objective To compare the hearing outcomes between 2 malleostapedotomy (MS) procedures, handle-MS, connecting the prosthesis with the malleus handle and neck-MS, connecting the prosthesis with the malleus neck. Patients Fourteen individuals having undergone MS in the setting of otosclerosis or congenital ossicular fixation from January 1983 through December 2009. Intervention Review of preoperative and postoperative audiometric data, ossicular abnormalities, and postoperative complications. Main outcome measures Postoperative air-bone gap (ABG), closure of ABG, and postoperative changes in bone conduction thresholds. Results Of 14 patients, 7 underwent handle-MS, and 7 underwent neck-MS. Morphologic or functional abnormalities of the incus were identified in all cases. There was no significant sensorineural hearing loss. The mean postoperative ABGs were 19.8 ± 11.9 dB in the handle-MS group and 14.7 ± 5.5 dB in the neck-MS group. The postoperative ABGs for single frequencies revealed better results for neck-MS at all frequencies (0.25, 0.5, 1, 2, 3, and 4 kHz) without statistical significance. The functional success rate (ABG closure, ≤ 10 dB) was 28.6% for the handle-MS group and 42.9% for the neck-MS group (p > 0.05). Conclusion Inasmuch as neck-MS is easy to perform and yields comparable results to those of handle-MS, it may be an alternative procedure of use in selected cases of otosclerosis or stapes fixation with incus anomaly.

The objective of this study was to clarify the cause of the air–bone gap in incomplete partition (IP) type III cases according to the POU3F4 gene (DFNX2) mutation type. A retrospective analysis of patient medical records was done in a tertiary referral medical center. Five IP type III patients proved to be carrying a mutation in or affecting POU3F4. The hearing and the middle ear status at either exploratory tympanotomy or cochlear implantation from these DFNX2 cases was reviewed. Four of five unrelated IP type III patients harbored a point mutation of POU3F4 and the fifth patient carried a large genomic deletion upstream to POU3F4. Two of the four DFNX2 patients carrying a point mutation had moderate to severe mixed hearing loss with a substantial amount of air–bone gap. These patients underwent exploratory tympanotomy to identify the cause of their hearing loss. The other three patients, including one carrying a large deletion, had profound hearing loss at presentation and received a cochlear implant. In the exploratory tympanotomy group with a substantial amount of air–bone gap and a point mutation (n = 2), one patient had a perfect ossicular chain with normal mobility, a positive ipsilateral stapedial reflex, and a positive round window reflex. In the cochlear implantation group (n = 3), we found a stapes with normal mobility and a positive round window reflex in one patient who harbored a large genomic deletion upstream to POU3F4. We concluded that the probable presence of the third window effect is not limited to the particular type of POU3F4 mutation.

Objectives/Hypothesis To evaluate whether there is a genetic contribution to the development of narrow bony cochlear nerve canal and analyze the differences between unilateral and bilateral narrow bony cochlear nerve canal. Study Design Retrospective review. Methods Patients diagnosed with unilateral (n=44) or bilateral (n=13) sensorineural hearing loss with narrow bony cochlear nerve canals from October 2004 to July 2011 were evaluated. The segregation ratio of hearing loss among siblings of probands was calculated. The differences in segregation ratio and other clinical characteristics were also compared. Results The segregation ratio of hearing loss in siblings of patients with unilateral narrow bony cochlear nerve canal was close to zero, whereas the ratio in siblings of patients with bilateral canals was 20%. Internal auditory canal narrowing was significantly more frequently combined in unilateral than bilateral narrow bony cochlear nerve canal cases. Cochlear nerve deficiency based on magnetic resonance imaging was found in 63.6% of bilateral narrow bony cochlear nerve canal cases. Milder hearing loss was exclusively detected in bilateral cases. Conclusions Narrow bony cochlear nerve canal is not likely to have a simple Mendelian inheritance pattern, especially in unilateral cases. The contribution of a Mendelian genetic etiology to bilateral narrow bony cochlear nerve canals remains elusive. More frequent coexistence of internal auditory canal narrowing among unilateral narrow bony cochlear nerve canal cases suggests that bilateral narrow bony cochlear nerve canals are not just a duplicate of the unilateral narrow bony cochlear nerve canal. Level of Evidence 4. Laryngoscope, 123:1996–2000, 2013

Conclusion: The transcanal approach could be an effective and time-saving method for selected cochlear implantation (CI) candidates. Objective: This study was designed to introduce the method of CI via the transcanal approach used in patients with abnormal temporal bone structure or chronic otitis media (COM). Methods: The medical records of six patients who underwent CI via the transcanal approach were reviewed retrospectively. The electrode was inserted through the external auditory canal (EAC) after tympanomeatal flap elevation and cochleostomy. Results: Three patients had a history of inactive COM. In the remaining patients, cochleostomy via the transmastoid approach was difficult due to aberrant anatomic structures such as vascular anomaly, narrow aditus, and anteriorly displaced sigmoid sinus. All patients underwent CI via the transcanal approach successfully. There was no serious complication postoperatively.

In patients with cochleovestibular malformations, surgeons should always consider the possibility of an aberrant course or shape of the facial nerve (FN). In our series, the majority of cases could be successfully implanted without major complications through careful handling of the FN and ossicles.To assess the frequency, type, and surgical implications of FN aberrations encountered during cochlear implantation (CI).Medical records and radiologic findings of 972 patients who underwent CI from 1988 to 2009 were reviewed retrospectively. Radiologic and intraoperative findings demonstrating malformations of the FN, ossicles, and inner ear were evaluated. Intraoperative events and postoperative FN outcome were reviewed.Seven patients (0.7%) had an aberrant course or shape of the FN during CI. All these patients were pediatric. Four showed abnormal anterior or inferior location of the vertical segment, two had a bifurcated horizontal or vertical segment, and one had an inferiorly located horizontal segment. All seven patients displayed combined malformations of the cochlea and had malformed or missing ossicles. CIs were successfully performed via the facial recess approach and cochleostomy. There was no postoperative FN paralysis except in one case with immediate iatrogenic FN paralysis that recovered completely 1 month after FN decompression.

Objectives/Hypothesis: The aim of this study was to investigate associations between tinnitus and stress factors including anxiety in elementary school students. Study Design: Cross-sectional study. Methods: We conducted a cross-sectional questionnaire survey in 940 students aged from 10 to 12 years. Data on 928 students were collected. The questionnaire comprised 96 questions that were classified into six categories: subjects' symptoms, stress factors, State Anxiety (transitory emotional condition characterized by feeling of tension and apprehension) Inventory for Children (SAIC), Trait Anxiety (general tendency to respond with anxiety to environmental threat) Inventory for Children (TAIC), visual analog scale of tinnitus, and Tinnitus Handicap Inventory (THI). Results: Four hundred thirty-five students (46.9%) had experienced tinnitus more than once, and 41 (4.4%) suffered from it continuously. Self-perception of hearing loss, dizziness, headache, and concerns about obesity had significant differences between tinnitus and nontinnitus groups, whereas other stress factors did not show any difference. TAIC scores showed statistically significant differences according to the frequency of tinnitus in children experiencing tinnitus, whereas SAIC scores did not. Annoyance, influence on daily life, disturbance of sleep, and study by tinnitus and THI scores showed significant differences according to the frequency of tinnitus. Conclusions: The present study confirms that many children are aware of tinnitus and that they may be susceptible to stressful environments. In particular, trait anxiety may be associated with tinnitus. Because both tinnitus and anxiety can affect the daily lives and health of children—as with adults—a detailed strategy for the management of tinnitus in children should be established.

This study was designed to evaluate the dimensions of the bony cochlear nerve canal (BCNC) in congenital unilateral sensorineural hearing loss (USNHL) patients with normal inner ears, and to analyze the correlation between BCNC and hearing status. Medical records and temporal bone computed tomography (TBCT) findings of 380 patients who visited Seoul National University Hospital between January 1999 and December 2007 were reviewed retrospectively. The length and width of the BCNC at the fundus of the internal auditory canal were measured in millimeters based on the axial view of TBCT for three ear groups: normal inner ears of control group subjects (group A, 179 ears), normal inner ears on the contralateral side of USNHL patients (group B, 201 ears), and the affected inner ears in USNHL (group C, 201 ears). The mean values of length and width in group C (0.79 ± 0.36 and 1.58 ± 0.83 mm, respectively) were significantly smaller than those in group A (1.07 ± 0.10 and 2.38 ± 0.28 mm, respectively; p < 0.001) and group B (1.04 ± 0.23 and 2.33 ± 0.39 mm, respectively; p < 0.001). The receiver operating characteristic curves for BCNC were conducted to estimate the cutoff values from which the proportions of profound hearing loss increased, compared to those of mild to severe hearing loss. The areas under the curve were 0.487 ± 0.044 (p = 0.781) and 0.622 ± 0.041 (p = 0.011) for length and width, respectively. The cutoff value of width with a sensitivity of 90% for profound USNHL was 1.16 mm in otherwise normal inner ears. Clinicians would be recommended to take a close look at BCNC as one of the possible causes of hearing loss in otherwise normal inner ears of USNHL on TBCT.

Postoperative external auditory canal (EAC) stenosis is the most common complication after congenital aural atresia (CAA) surgery. The authors applied an ear mold or hearing aid as an EAC stent following surgery and analyzed the impact on postoperative EAC stenosis.Historical cohort study.A tertiary hospital.Ninety-six patients who underwent canaloplasty between 1996 and 2010 were included in this study. To discover factors contributing to postoperative EAC stenosis, clinical parameters, including age, sex, Marx grading, Schuknecht classification, Jahrsdoerfer score, surgical approach, triamcinolone injection, and use of stenting with an ear mold or hearing aid, were reviewed and analyzed. Each stent was used for at least 6 months postoperatively. Pure-tone audiometry was performed preoperatively and 3, 6, and 12 months after canaloplasty.Postoperative EAC stenosis was the most common postoperative complication, occurring in 8 (8.2%) cases with a mean time interval of 4.1 months. For patients who did not use an ear mold or a hearing aid during the postoperative follow-up period, the relative risk for the development of postoperative EAC stenosis was 5.125 (95% confidence interval, 1.428-18.400; P = .023). Other factors did not show an association with postoperative stenosis. Preoperative air-bone gap (ABG) was 49.00 dB, and closure of the ABG within 30 dB was obtained in 56.9%, 58.1%, and 48.7% of patients at the 3-, 6-, and 12-month follow-up, respectively.Stenting with an ear mold or hearing aid might be a useful method for preventing postoperative EAC stenosis in CAA patients.

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.

In cases of petrous apex (PA) cholesteatoma, radical removal should be prioritized over an unreasonable sparing of hearing or facial symmetry. Nevertheless, for patients with serviceable hearing and spared inner ear structures, conservative approaches may be applicable. Restoration of facial nerve (FN) function is achievable by reanimation procedures.To analyze clinical manifestations, surgical techniques, and postoperative functional results of PA cholesteatoma.From 1987 to 2010, 13 cases of PA cholesteatoma underwent operations. Clinical, audiological, and radiological findings, surgical approach, and postoperative functional outcomes were analyzed retrospectively.The most common symptoms were hearing loss and FN paralysis. All PA cholesteatomas extended to the middle ear; 10 (76.9%) through the anterior-superior route, whereas the other 3 (23.1%) were through the posterior-superior route. In 10 patients, labyrinthectomy was inevitable because of inner ear invasion. However, three limited cholesteatomas were removable via the middle cranial fossa or transmastoid approach without changing the bone-conduction threshold. Among six cases with FN paralysis, one underwent interposition graft, two FN decompression, and the others hypoglossal-FN anastomosis. One FN decompression case recovered to House-Brackmann grade I, and all other reanimation cases finally presented with grade IV. There were no major complications or recurrences during the follow-up period.

Objectives/Hypothesis: Ramsay Hunt syndrome is known to be accompanied with cranial polyneuropathy very occasionally. We reviewed our experience to analyze the clinical manifestations and prognosis of these cases. Study Design: Retrospective review. Methods: Eleven patients with Ramsay Hunt syndrome presenting as cranial polyneuropathy between 1999 and 2009 were included in this study retrospectively. All patients were treated with systemic steroids and antiviral agents. Assessment of recovery from facial nerve palsy (FNP) using the House-Brackmann (HB) grading system was performed, and the follow-up period was 4 to 28 months. Results: The patient numbers for the initial degree of FNP were five for HB grade II, one for grade III, two for grade IV, and three for grade V. The cochleovestibular nerve was involved in 10 cases, and hearing loss was observed in nine patients. Other involved cranial nerves were IX in seven cases, X in five cases, V in three cases, and III/XII in one case each. Antiviral agents and steroids were administered within 3 days after the onset of symptoms for about 1 to 2 weeks in most cases. After the treatment the degree of FNP improved to HB grade I or II in nine of 11 patients (81.8%). Symptoms of other cranial nerves also improved in most patients, whereas recovery for hearing loss showed poor results. Conclusions: Ramsay Hunt syndrome associated with cranial polyneuropathy has a good prognosis for FNP and other cranial nerve palsies with a combination therapy of antiviral agents and steroids, but not for recovery of hearing loss. Laryngoscope, 2010

Surgical treatment of fibrous dysplasia of the temporal bone (TBFD) revealed relatively good outcomes. Also, active surgical intervention is necessary to prevent the formation of secondary cholesteatoma or other complications. In particular, FD involving the inner ear may require greater caution when deciding on the resection area and timing of surgery.The purpose of this study was to analyze clinical manifestations of TBFD and its surgical outcomes.Nine patients diagnosed with TBFD after surgery at three tertiary referral centers were included. Pre- and postoperative clinical, audiological, and radiological findings were analyzed retrospectively.Most patients (88.9%) showed FD involving the mastoid and adjacent area. Radiological patterns consisted of six pagetoid and three sclerotic forms; seven patients were monostotic, whereas two patients were polyostotic. Canalplasty (CP) was performed in six cases and intact canal wall mastoidectomy was performed in three of them; hearing gain was achieved in five and one patient maintained preoperative normal hearing. Two patients underwent revision CP and showed no recurrence. In addition, one case had secondary cholesteatoma formed by an obstruction of the external auditory canal; another with a fast-growing cystic mass in the petrous bone underwent transmastoid labyrinthectomy and had no recurrence for 11 months.

Objectives Cholesteatoma has a tendency to recur if not properly eradicated. This study sought to investigate and compare the outcome of the canal wall up (CWU) versus the canal wall down (CWD) procedure for recurrent cholesteatoma after initial canal preserving surgery. Methods Between January 1990 and August 2007, 42 patients who underwent a revision tympanomastoidectomy for a recurred cholesteatoma were analyzed retrospectively. All patients initially underwent the canal wall up procedure. Recurrence rates, audiologic outcomes, and the extent of recurrent/residual cholesteatoma were investigated, and the revision surgical methods were compared. Results The mean follow-up duration was 10 years (range, 13 months–15.6 years). The CWD procedure was performed in 29 (69%) patients with a recurred cholesteatoma and the CWU procedure in 13 (31%) as a first revision procedure. CWD surgeries were performed in more severe cases. A second revision surgery was required in five (12%) patients. Extended cholesteatoma recurrences were observed even among cases with a lower disease stage at the time of primary surgery. The second recurrence rate was significantly higher in the CWU group than the CWD group (p = 0.026). The 8-year disease-free follow-up rate in the CWD group was significantly higher than the CWU group (p = 0.002). Postoperative AB gap closure was significantly better in the CWU group than CWD group (p = 0.001). Conclusion The CWD procedure is a safer and more successful method for controlling recurrent cholesteatoma. Thus, surgeons should not be hesitant to perform the CWD procedures for revision cases.

OBJECTIVES To compare partial ossicular replacement (POR) with type II tympanoplasty (T2) with regard to the postoperative hearing outcomes in patients with congenital aural atresia. STUDY DESIGN A retrospective review of medical records. SETTING Tertiary referral center. PATIENTS Thirty-four ears undergoing POR were compared with matched 34 ears by age at operation, grade of microtia, and the Jahrsdoerfer grading scale score that underwent T2. METHODS The medical records of the patients that underwent surgery for congenital aural atresia were reviewed. MAIN OUTCOME MEASURES The preoperative and postoperative air-conduction threshold, air-bone gap, and air-bone gap closure were compared between the 2 groups. RESULTS Thirty-four ears in each group were compared. PORs were performed in cases of a incudostapedial joint problem, ossicular anomaly, or ossicular fixation. The preoperative and 6-month postoperative mean air-conduction thresholds and air-bone gaps were not significantly different in the comparisons between the 2 groups. The mean values for the postoperative air-bone gap closure were not significantly different in the 2 groups. The hearing results 3 years after surgery were available in 13 patients in each group. In these patients, the mean values of the preoperative and the 3-year postoperative air-conduction threshold and air-bone gap were not statistically different in the 2 groups. However, the mean air-bone gap closure in the POR group was 28.6 +/- 10.2 dB hearing level compared with 19.0 +/- 12.9 dB hearing loss in the T2 group, which was statistically significant (p = 0.034). CONCLUSION POR can be a good surgical option in cases of a poor incudostapedial joint connection, ossicular anomaly, or ossicular fixation.

OBJECTIVES The goal of this study was to characterize the signal intensity (SI) pattern in the endolymphatic duct/sac from T2-weighted spin echo magnetic resonance (MR) images and T2*-weighted 3-dimensional (3-D) constructive interference in steady state (CISS) MR images that were obtained from a homogenous group of patients with an enlarged vestibular aqueduct (EVA) associated with SLC264 (PDS) mutations. STUDY DESIGN A retrospective study. SETTING Tertiary referral center. PATIENTS Eleven subjects (22 ears), with at least 1 SLC26A4 mutation and bilateral EVA confirmed by temporal bone computed tomography, were enrolled. INTERVENTION The MR images of 22 ears were reviewed retrospectively. MAIN OUTCOME MEASURES The SI of the endolymphatic sac (ES) from 2 sequences were compared and reviewed. The presence of anomalies of the vestibule were also evaluated. In addition, the caloric test results were also reviewed to characterize the vestibular function in a subset of our cohort (12 ears). RESULTS T2*-weighted 3-D CISS MR images revealed a more variable SI in the enlarged endolymphatic system than did the T2-weighted images. There was a hierarchy for the order of SI loss from the endolymphatic system on the T2*-weighted 3-D CISS MR images. There were 3 cases where the SI loss from the intraosseous ES, on the T2*-weighted 3-D CISS images, was associated with an abnormal caloric response. CONCLUSION The SI of the ES observed on T2*-weighted 3-D CISS images of EVA patients, with SLC26A4 mutations, showed a distinctive pattern, and the results from this study suggest a potential role for the MR imaging in providing physicians with better information about the functional status of the inner ear.

Conclusion Cochlear implantation (CI) remains a safe procedure with a low complication rate. Nevertheless, advances in surgical techniques and the optimization of treatment modalities would further reduce complications. Objective To assess the complications of CI and describe the management of each complication encountered at our hospital. Patients and methods This study involved 720 patients that underwent implantation from November 1988 through April 2007. Mean age at implantation was 13.6 years (range 12 months to 83 years). Patients were followed up regularly with a mean follow-up of 42 months (range 4-81 months). Results No death was attributed to device implantation. Major complications included: device failure in 12 patients, misplaced electrodes in 4, hematoma in 3, flap necrosis in 3, magnet displacement in 2, facial nerve twitching in 2, electrode exposure in 2, external auditory canal keratoma in 1, immediate facial nerve paralysis in 1, and skin flap irritation due to the suture material in 1. The overall major complication rate was 4.2% (30/720), and there were 37 minor complications (5.1%), which were resolved by conservative treatment or minor intervention. Minor complications included temporary vertigo in 17 patients, facial twitching in 11, hematoma in 4, subcutaneous emphysema in 3, and temporary facial nerve paralysis in 2.

Objective Evaluation of head-position dependency during the ice-water test is essential when the test is indicated. Because ice-water irrigation (IWI) in the prone position is frequently skipped in many laboratories, we investigated the importance of evaluating position dependency in an ice-water test. Design and setting Twenty-five unilateral peripheral vestibulopathy patients showing no nystagmus during warm irrigation were included. IWI was performed in supine and prone positions, and maximum slow-phase velocity and direction of nystagmus were assessed by using video nystagmography. Results Eleven cases showed head-position dependency and were interpreted as hypofunctional. Fourteen cases showed no dependency, suggesting the absence of end organ function. In the latter group, 6 patients showing definite nystagmus in supine position could have been misinterpreted as hypofunctional, had it not been for prone-position results. Conclusions Evaluation of head position dependency by using IWI is important in the determination of lateral semicircular canal function.

To evaluate the long-term speech perception of cochlear implantees and to compare the developing auditory performance patterns of prelingual children and postlingual deaf adults.Twenty-nine prelingually deaf children and 17 postlingually deaf adults who had been followed up for 4 years were included in the study. Speech perception ability was assessed by means of vowel and consonant confusion tests and the Korean version of the Central Institute of Deafness (K-CID) test (performed without visual cues). The test results were analyzed at 3 and 6 months after implantation and then annually.In the prelingually deaf children, the average results continuously improved over the 4-year period. In the postlingually deaf adults, the average results did not improve further after the first 2 years. Individuals with5 years of deafness had a faster rate of recovery of speech perception than those who had been deaf for5 years. The K-CID scores were negatively correlated with age at implantation for the prelingually deaf group and with the duration of deafness in the postlingually deaf group. Children fitted with implants at a younger age showed better speech perception ability than those fitted with implants at an older age. Interestingly, prelingually deaf children aged 5-7 years at implantation showed the widest variation in individual outcomes. Amongst this group of children with highly variable outcomes, the metabolic status of brain cortices determined by means of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) was available for three patients. The individual with the widest hypometabolic area had the best speech perception ability.The extent of hypometabolism as assessed by FDG-PET seemed to be one of the major factors predicting the outcome of cochlear implantation.

The aim of this study was to report a series of 18 facial nerve schwannomas, including 2 infantile cases.Retrospective case review.Tertiary referral center.Eighteen patients with facial nerve schwannoma, operated on between 1980 and 2000.Surgical treatments were performed in all cases.The presenting symptoms and facial nerve function were graded using the House-Brackmann scale and eye closure. RESULTS Facial nerve paralysis was the most common symptom, presenting in 94% of cases, followed by hearing loss and mass lesion. In one case, the tumor was shaved, leaving the facial nerve intact. In the other cases, the facial nerve reconstruction with hypoglossal-facial anastomosis or interposition graft was performed. The postoperative facial function was House-Brackmann grade IV in most cases (88.2%). In terms of the functional recovery classified by complete or incomplete eye closure, the moderate preoperative facial nerve palsy group showed a better functional outcome than severe group.In cases with good facial nerve function, it would be better to consider an alternative method for preserving the facial nerve. Furthermore, when facial nerve paralysis has developed to more than House-Brackmann grade III, an immediate operation is recommended to obtain a good postoperative facial functional recovery.