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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.

To investigate HLA-associated genetic susceptibility to Meniere's disease in relation to type II collagen (CII) autoimmunity status, HLA-DRB1 genotyping and ELISA measurement of anti-CII antibody were performed in 41 Korean patients with Meniere's disease. In the anti-CII positive subgroup (20%) of patients, the frequency of HLA-DRB1*0405 was significantly increased (uncorrected) compared with both controls (63% vs 16%) and anti-CII negative patients (63% vs 12%). In the anti-CII negative subgroup, HLA-DRB1*1201 was significantly increased (uncorrected) (27% vs 10%) and DRB1*13 was decreased (6% vs 24%) compared with controls; these alleles appeared to confer susceptibility and resistance to the development of the disease. Association of HLA-DRB1*0405 with anti-CII positive Meniere's disease in this study suggests that it shares a specific HLA-DR sequence, QRRAA, as a genetic susceptibility factor with the anti-CII positive rheumatoid arthritis. In conclusion, whilst type II collagen autoimmunity may have a partial role in Meniere's disease, different HLA-DR alleles may also be associated with either susceptibility or resistance to the development of the disease in relation to anti-CII antibody status.

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.

We used the IL088 Otodynamic Analyzer system to study click-evoked otoacoustic emissions (CEOAEs) in 30 healthy guinea pigs. The animals were anesthetized and patterns of the CEOAEs were evaluated before manipution, after the tympanic bulla was opened, and after formation of a microfistula on the basal turn of the cochlea. The animals then were divided into three pressure loading groups (10, 20, and 30 cm H2O). CEOAEs were recorded with a capillary manometer at pretest, 5, 10, 20, 30, 45, and 60 min after perilymphatic pressure loading to the basal turn of cochlea, and 10 and 20 min after pressure unloading. As perilymphatic pressure increased, all three pressure groups showed maximum decreases in both echo response and reproducibility 5 min after pressure loading. In the 10 cm H2O pressure group, emissions recovered 10 min after pressure loading, and this tendency continued. However, in the 20 and 30 cm H2O pressure groups, no recovery of emissions was seen throughout the 60 min observation period, except for emissions after pressure unloading. The results suggest that the echo response and reproducibility may be sensitive indicators of cochlear function and perilymphatic pressure regulation capacity.

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.

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.

Claudius' cells absorb Na(+) through the amiloride-sensitive epithelial sodium channel (ENaC). Transepithelial ion transport through ENaC and possibly a Cl(-) secretory pathway is regulated by P2Y purinergic signaling.The purpose of this study was to investigate ion transport in Claudius' cells and its purinergic regulation.Young adult Sprague-Dawley rats and gerbils were studied. The Claudius' cell layer on the basilar membrane was dissected from the basal turn of the cochlea. A voltage-sensitive vibrating probe was used to measure transepithelial short circuit current (I(sc) ). The baseline I(sc) of Claudius' cells was measured in the perilymph-like control solution and the change of I(sc) after application of amiloride (10 μM) or uridine triphosphate (UTP, 100 μM).A negative baseline I(sc) was observed in the control solution (-12.50 ± 3.95 μA/cm(2), n = 8) and the addition of amiloride resulted in a decrease of I(sc) by 75.8%. The application of UTP, an agonist for P2Y purinergic receptors, led to a partial inhibition of I(sc) (by 38.2 ± 3.2%, n = 5), and subsequent addition of amiloride abolished the remaining I(sc).

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.

Cerebellar Purkinje cells (PCs) are the sole output of the cerebellar cortex and function as key to a variety of learning-related behaviours by integrating multimodal afferent inputs. Intrinsic membrane excitability of neurons determines the input-output relationship, and therefore governs the functions of neural circuits. Cerebellar vermis consists of ten lobules (lobules I-X), and each lobule receives different sensory information. However, lobule-specific differences of electrophysiological properties of PC are incompletely understood. To address this question, we performed a systematic comparison of membrane properties of PCs from different lobules (lobules III-V vs. X). Two types of firing patterns (tonic firing and complex bursting) were identified in response to depolarizing current injections in lobule III-V PCs, whereas four distinct firing patterns (tonic firing, complex bursting, initial bursting and gap firing) were observed in lobule X. A-type K(+) current and early inactivation of fast Na(+) conductance with activation of 4-aminopyridine-sensitive conductances were shown to be responsible for the formation of gap firing and initial bursting patterns, respectively, which were observed only in lobule X. In response to current injection, PCs in lobule X spiked with wider dynamic range. These differences in firing pattern and membrane properties probably contribute to signal processing of afferent inputs in lobule-specific fashion, and particularly diversity of discharge patterns in lobule X, as a part of the vestibulocerebellum, might be involved in strict coordination of a precise temporal response to a wide range of head movements.

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.

Iwasaki S, Atsumi K, Ocho S, Mizuta K (1998) Facial nerve stimulation by a cochlear implant in a hemodialysis patient with bone of low mineral density. European Archives of Otorhinolaryngology 225: 352–354. Muckle RP, Levine SC (1994) Facial nerve stimulation produced by cochlear implants in patients with cochlear otosclerosis. American Journal of Otology 15(3): 394–398. Valvassori GE, Dobben GD (1985) CT densitometry of the cochlear capsule in otosclerosis. American Journal of Neuroradiology 6: 661–667. Weber BP, Lenarz T, Battmer RD, Hartrampf R, Dahm MC, Dietrich B (1995) Otosclerosis and facial nerve stimulation. Annals of Otology Rhinology and Laryngology Suppl. 166: 445–447.

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.

Perilymphatic fistula (PLF) is an uncommon disease characterized by abnormal leakage of the perilymph from the inner ear into the middle ear. The symptoms of PLF include sudden-onset hearing loss, tinnitus, ear fullness, and vertigo resulting from inner-ear trauma, stapedial surgery or barotrauma such as valsalva, and nose blowing. As nystagmus can be induced by the affected-ear in down position, benign positional paroxysmal vertigo (BPPV) should be considered for differential diagnosis. About 40-50% of PLF patients have experienced spontaneous healing, but surgical repair should be considered when the hearing symptom and dizziness are not alleviated or are aggravated within a few days. Reported herein, with a review of the relevant literature, is a case of PLF initially misdiagnosed as BPPV but where successful fistula repair was finally achieved.

KCNQ1/KCNE1 K(+) channels and P2Y(4) receptors are expressed in the apical membrane of rat strial marginal cells from postnatal day 1 (P1) and maintained throughout development.The purpose of the present study was to investigate the developmental expression of KCNQ1/KCNE1 K(+) channel and of P2Y(4), which is an important metabotropic regulator of KCNQ1/KCNE1 K(+) channel in strial marginal cells.Sprague-Dawley rats at different stages of development (P1, P3, P5, P7, P14, and P21) were studied. The spiral ligament with the stria vascularis was detached from the cartilaginous or bony cochlea and prepared for a voltage-sensitive vibrating probe and immunohistochemistry.Chromanol 293B, a blocker of KCNQ1/KCNE1 K(+) channel, inhibited short-circuit currents (I ( sc )) from P1 to P21. Similarly, I ( sc ) were found to be decreased by uridine 5'-triphosphate at all ages. The antagonist profiles indicated that the apical P2Y receptor is P2Y(4) subtype. KCNQ1, KCNE1, and P2Y(4) were immunolocalized in the apical region of stria vascularis at P1.

This is a short overview on imaging techniques in pre-, peri- and postoperative evaluation of cochlear-implant patients. Surgery techniques are described as well as possible complications and how to avoid them.

Objectives: Careful surgical candidate selection guarantees a high probability of serviceable hearing postoperatively in congenital aural atresia (CAA) patients. The authors analyzed hearing results after CAA surgery with long-term follow-up (F/U) with respect to several clinical factors. Study Design: This was a retrospective study. Methods: The medical records of 93 CAA patients (100 ears) who underwent operations from January 1987 through December 2002 at Seoul National University Hospital were reviewed. Mean duration was 56.9 months. The authors evaluated the results of hearing after surgery over 3 year F/U with a view to clarifying the factors accounting for unsuccessful results. Results: Approximately 64% of patients treated surgically achieved a considerable hearing gain over long-term F/U. Postoperative hearing remained relatively stable over the period from 6 months to 3 years postoperatively, yielding only 2.75 dB of aggravation. However, hearing results in revision cases deteriorated with time, which led to statistically higher air-conduction thresholds than those of primary cases at the 1 and 3 year F/Us. Resultantly, only 26.6% of patients having achieved a poor hearing gain post first surgery benefited from revision audiologically. The severity of microtia was found to help predict poor long-term hearing outcomes after CAA surgery. Conclusions: Nonrevision cases and cases with mild microtia appear to have acceptable and stable long-term hearing results. Disappointing long-term hearing results in revision, and severe microtia cases should lead to considerations of alternative options in these cases, such as bone-anchored hearing aids, which offer reliable and stable results.

Extracellular ATP decreases K+ secretion in strial marginal cells via apical P2Y4 receptors. We investigated the effect of reactive blue 2 (RB-2), an antagonist of rat P2Y4, on rat strial marginal cells using a voltage-sensitive vibrating probe. The application of RB-2 increased K+ secretion in a dose-dependent manner, and this increase was characterized as a peak followed by a partial relaxation to a steady-state. Moreover, this response was similar to that caused by 4,4′-diisothiocyanatostilbene-2,2′-disulfonic acid (DIDS). Suramin had no similar effect, except at high concentration. Thus, we tested the effects of these chemicals on P2Y4 receptors in strial marginal cells. Both RB-2 and DIDS had antagonistic activities at P2Y4, and the antagonist potency at P2Y4 paralleled the potency of K+ secretion. Interestingly, 2′- and 3′-O-(4-benzoyl-benzoyl)adenosine 5′-triphosphate (BzATP) exhibited an agonistic effect at P2Y4 receptor, which was blocked by RB-2, but not by pyridoxalphosphate-6-azophenyl-2′,4′-disulfonic acid (PPADS). Based on these results, we speculate that direct and/or indirect inhibitory mechanisms between P2Y4 and KENQ1/KCNE1 K+ channels exist in strial marginal cell.

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.

Approximately one child in a thousand is reported to be born with profound hearing impairment. A study by Ajou University Hospital in Korea from March 1998 through Dec. 2001 reported that 0.05% of normal neonates and 1.11% of NICU neonates are born with moderate or severe hearing impairment [1]. According to the registry of handicapped persons in Korea in 2003, the number of hearing impaired persons with a severe handicap, to the extent that normal social life is impossible is listed in Table 1.The analysis of data collected by Gallaudet University in the 1969–1970 Annual Survey of Hearing Impaired Children and Youth showed that genetic causes of hearing impairment were 50.7% of children, while analysis of the survey data collected two decades later (1988–1989) estimated a genetic etiology in 62.8% of cases.

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.

Facial nerve paralysis is a visible complication after acoustic neuroma surgery. In this study, the prognostic factors that can predict postoperative facial nerve function after acoustic neuroma surgery were investigated. The medical records of 68 patients with acoustic neuroma who underwent surgery with neurotological approaches from March 1987 to May 2001 at Seoul National University Hospital were reviewed retrospectively. The influence of surgical approach, preoperative facial nerve function, and tumor size on postoperative facial nerve function was determined by examining facial nerve function preoperatively, immediately postoperatively, and at the time of the last follow-up using the House-Brackmann grading system. Spearman correlation analysis was used for the statistical evaluation. Surgical approach for tumor removal and tumor extension had no influence on preserving the facial nerve. Preoperative and immediate postoperative facial nerve function and tumor size were important prognostic factors in long-term facial nerve function.

This study compared long-term speech performance after cochlear implantation (CI) between surgical strategies in patients with chronic otitis media (COM). Thirty patients with available open-set sentence scores measured more than 2 yr postoperatively were included: 17 who received one-stage surgeries (One-stage group), and the other 13 underwent two-stage surgeries (Two-stage group). Preoperative inflammatory status, intraoperative procedures, postoperative outcomes were compared. Among 17 patients in One-stage group, 12 underwent CI accompanied with the eradication of inflammation; CI without eradicating inflammation was performed on 3 patients; 2 underwent CIs via the transcanal approach. Thirteen patients in Two-stage group received the complete eradication of inflammation as first-stage surgery, and CI was performed as second-stage surgery after a mean interval of 8.2 months. Additional control of inflammation was performed in 2 patients at second-stage surgery for cavity problem and cholesteatoma, respectively. There were 2 cases of electrode exposure as postoperative complication in the two-stage group; new electrode arrays were inserted and covered by local flaps. The open-set sentence scores of Two-stage group were not significantly higher than those of One-stage group at 1, 2, 3, and 5 yr postoperatively. Postoperative long-term speech performance is equivalent when either of two surgical strategies is used to treat appropriately selected candidates. Graphical Abstract Keywords: Cochlear Implantation, Otitis Media, Tympanomastoidectomy, Speech Performance INTRODUCTION Profound sensorineural hearing loss (SNHL) can occur in patients with chronic otitis media (COM) as a secondary effect of labyrinthitis, a labyrinthine fistula, or iatrogenic injury during surgery. Cochlear implantation (CI) has become the mainstay of auditory rehabilitation in patients with profound SNHL. However, CI was initially contraindicated in COM patients, because insertion of foreign material through a contaminated field into a space featuring potential intracranial communication was thought to be inappropriate (1). As the audiological benefits afforded by CI became better understood, and as surgical skills and radiological techniques improved, CIs have been increasingly performed in patients with COM. Recently, many reports on CI in patients with COM have appeared, addressing several issues, including candidate selection, choice of surgical strategy, and postoperative complications (2, 3, 4, 5, 6, 7, 8, 9, 10). In patients with COM, complete eradication of infected foci in the middle ear cleft prior to CI is essential to avoid development of serious complications and subsequent explantation (8). Therefore, it is essential to consider disease activity status when planning the surgical strategy; either one- or two-stage surgery may be appropriate. Postoperative findings after CI in patients with COM have been evaluated in those treated with one- or two-stage procedures, but among-study differences in results are apparent (2, 3, 7, 9). Hearing rehabilitation is the most important goal of CI, so the evaluation of postoperative speech performance is essential. However, such performance after CI in COM patients has been evaluated in only a few reports (4, 10) and comparisons of speech performance after application of various surgical strategies have not been performed. Hence, in the present study, we analyzed the postoperative complication and post-CI long-term speech performance of patients with COM treated via one- or two-stage surgery.

The objective of this study was to evaluate postoperative meatal stenosis after surgery for congenital aural atresia using anteriorly and inferiorly based periosteal flaps (AIPFs). These were compared with the groups that did not use these flaps.This was a retrospective clinical study.The study was conducted at the tertiary referral hospital.There were 133 patients (151 ears) who had undergone surgical correction for congenital aural atresia from November 1987 to March 1999.The anterior approach surgical method was used to correct the congenital aural atresia.A comparison between the 2 groups, 1 using the AIPFs and the other that did not use the AIPFs, was performed to evaluate both the incidence and the interval of postoperative meatal stenosis. The correlation between the age of the first operation to correct congenital aural atresia and the incidence of postoperative meatal stenosis was also investigated.Meatal stenosis was the most common postoperative complication (23.8%) found. The incidence of meatal stenosis was much lower in the group using AIPFs (n = 105) than in the group that did not (n = 46) (19.0% versus 35.0%). The interval for the development of postoperative meatal stenosis showed similar distribution in both groups. In age distribution, the younger the age of the first operation, the more frequent the occurrence of postoperative meatal stenosis.AIPF is an effective surgical method for reducing the incidence of postoperative meatal stenosis.