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Background Understanding the characteristics of residual hearing at low frequencies and its natural course in relation to molecular genetic etiology may be important in developing rehabilitation strategies. Thus, we aimed to explore the characteristics and natural course of residual hearing at low frequencies associated with the two most frequent deafness genes: GJB2 and SLC26A4. Methods Initially, 53 GJB2 and 65 SLC26A4 subjects were enrolled, respectively. Only those whose audiograms exhibited hearing thresholds ≤70 dB at 250 and 500 Hz, and who had at least 1-year follow-up period between the first and last audiograms, were included. Collectively, the clinical characteristics of 14 ears from eight subjects with GJB2 variants, and 31 ears from 22 subjects with SLC26A4 variants fulfilled the strict criteria. In this study, a dropout rate refers to an incidence of dropping out of the cohort by cochlear implant surgery due to severe hearing deterioration. Results Among the ears with complete serial audiogram data set, significant residual hearing at low frequencies at the time of inclusion was observed in 18.8% of those with GJB2 variants (15 out of 80 ears) and 42.6% of those with SLC26A4 variants (46 out of 108 ears), revealing a difference between two deafness genes. Subsequently, ears with SLC26A4 variants (11 of 46 ears, 23.9%) turned out to have a higher dropout rate for cochlear implantation due to hearing deterioration within the first year than those with GJB2 variants (1 of 15, 6.7%), albeit with no statistical significance. Throughout the follow-up period (mean: 37.2 ± 6.8, range: 12 to 80 months), deterioration of residual hearing at low frequencies at 250 Hz (dB HL/y) and 500 Hz (dB HL/y) of those with GJB2 variants exhibited 3.1 ± 1.3 (range: 0 to 15) and 5.2 ± 1.6 (range: 0 to 20), respectively, suggesting the deterioration of residual hearing in GJB2 variants was rather slow and gradual. Specifically, GJB2 p.Leu79Cysfs*3 show less remarkable residual hearing at low frequencies, but then a relatively stable nature. In contrast, SLC26A4 variants demonstrated a significantly higher dropout rate due to severe hearing deterioration requiring cochlear implantation compared with the GJB2 variants. This trend was observed not only in the first-year follow-up period but also in the follow-up periods thereafter. The p.His723Arg;c.919-2A>G genotype of SLC26A4, in particular, was associated with a high propensity for sudden hearing deterioration, as indicated by the dropout rate, which was as high as 46.2% for cochlear implantation due to hearing deterioration during the first year follow-up period. Furthermore, the dropout rate for cochlear implantation was observed in 7.1% of those with GJB2 variants (one out of 14 ears) and 30.3% of those with SLC26A4 variants (10 out of 33 ears) throughout the entire follow-up period. Conclusions Our results suggest that there is a difference with respect to the progressive nature of residual hearing at low frequencies between the two most common genes responsible for hearing loss, which may provide clinical implications of having individualized rehabilitation and timely intervention.

We present diagnostic criteria for mal de débarquement syndrome (MdDS) for inclusion into the International Classification of Vestibular Disorders. The criteria include the following: 1] Non-spinning vertigo characterized by an oscillatory perception (‘rocking,’ ‘bobbing,’ or ‘swaying’) present continuously or for most of the day; 2] Onset occurs within 48 hours after the end of exposure to passive motion, 3] Symptoms temporarily reduce with exposure to passive motion (e.g. driving), and 4] Symptoms persist for >48 hours. MdDS may be designated as “in evolution,” if symptoms are ongoing but the observation period has been less than 1 month; “transient,” if symptoms resolve at or before 1 month and the observation period extends at least to the resolution point; or “persistent” if symptoms last for more than 1 month. Individuals with MdDS may develop co-existing symptoms of spatial disorientation, visual motion intolerance, fatigue, and exacerbation of headaches or anxiety. Features that distinguish MdDS from vestibular migraine, motion sickness, and persistent postural perceptual dizziness (PPPD) are reviewed. Motion-moderated oscillatory vertigo can also occur without a motion trigger, typically following another vestibular disorder, a medical illness, heightened psychological stress, or metabolic disturbance. Terminology for this non-motion triggered presentation has been varied as it has features of both MdDS and PPPD. Further research is needed into its phenomenological and biological relationship to MdDS, PPPD, and other vestibular disorders.

Objectives Individuals with early- and late-onset deafness showed different functional and morphological brain changes, but white matter alterations in both deaf groups still need to be elucidated. This study aimed to investigate changes in white matter integrity and white matter anatomical connectivity in both early- and late-onset deaf groups compared with hearing group. Design Diffusion tensor imaging data from 7 early-onset deaf (50.7 ± 6.5 years), 11 late-onset deaf (50.9 ± 12.3 years), and 9 hearing adults (48.9 ± 9.5 years) were preprocessed using FSL software. To find changes in white matter integrity, tract-based spatial statistics was used, which implemented on FSL software. Fractional anisotropy (FA), axial diffusivity (AD) and radial diffusivity (RD) were calculated and compared among the groups with age as a nuisance variable. To find out the effect of onset age or duration of deafness to the white matter integrity, onset-age or duration of deafness was treated as a variable of interest in the general linear model implemented on tract-based spatial statistics. White matter connectivity was constructed by a deterministic tractography and compared among the groups. Results In comparison to the hearing group, the early-onset deaf group did not show any significant changes but the late-onset deaf group showed decreased FA and increased RD in the several white matter areas. AD in the late-onset deaf group was not significantly different compared with the hearing group. The regions included the corpus callosum, posterior and superior corona radiata, internal capsule, posterior thalamic radiation, superior longitudinal fasciculus, and tapetum of the right hemisphere. Increased RD was also additionally observed in the right external capsule, fornix, and cerebral peduncle. The onset age or duration of deafness was not significantly correlated with the white matter integrity in the early-onset deaf group. In contrast, the onset age showed a significantly positive correlation with the RD, and a negative correlation with the FA, in the late-onset deaf group. The correlated white matter areas were also similar to the findings of comparison with the hearing group. In comparison to the hearing group, the early-onset deaf group did not show altered white matter connectivity, while the late-onset deaf group showed decreased white matter connectivity in between the right lingual and hippocampal areas. Conclusions The present results suggest that late-onset deaf adults showed decreased FA and increased RD, and early-onset deaf adults showed no difference compared with the hearing group. In the late-onset deaf adults, onset-age showed a significantly positive correlation with RD and negative correlation with FA. Duration of deafness was not significantly correlated with the changes. Increased RD indicating demyelination occurred in the brain, and the changes were not limited to the auditory cortex but expanded to almost whole brain areas, suggesting significant effect of auditory deprivation on the brain later in life. The altered white matter connectivity in between the right limbic-occipital areas observed in the late-onset deaf group might be caused by altered language functions after auditory deprivation. Future studies are necessary incorporating functional and anatomical aspects of the brain changes in deaf group.

Objectives. Although unilateral hearing loss (UHL) has been proven to be associated with educational and behavioral problems, few studies have investigated psychopathological abnormalities in this population. The aim of this study was to evaluate the psychopathological influence of UHL among Korean 19-year-old males.Methods. The authors retrospectively compared the objective personality test profiles of 602 subjects with UHL with those of 23,790 peers with normal hearing. All participants in the current study were 19-year-old males who underwent a physical examination and completed the Korean Military Multiphasic Personality Inventory for conscription at the Military Manpower Administration from February 2015 to December 2016.Results. Significantly higher scores were found on neurosis scales in the UHL group than in the normal-hearing group (50.9± 10.8 vs. 44.9±6.0 for anxiety; 51.0±10.5 vs. 44.9±5.2 for depression; 51.1±10.4 vs. 45.1±6.81 for somatization, all PP

Introduction: It was previously demonstrated that tinnitus due to profound unilateral hearing loss can be treated by the use of electrical stimulation via a cochlear implant (CI) with long-lasting positive effects. In cases where patients are not suitable for cochlear implantation due to aplasia/hypoplasia, cochlear malformations etc., an auditory brainstem implant (ABI) may be a solution. While auditory performance with ABI is well investigated, it is currently unknown whether stimulation through ABI also renders tinnitus reduction in patients with incapacitating tinnitus. The current case study reports on the subjective tinnitus perception during a 5-year follow-up period. In addition, a first H215O PET imaging study in an ABI patient is carried out revealing underlying neural substrates of tinnitus. Methods: A 56-year-old male single-sided deaf patient with incapacitating tinnitus received an ABI after insufficient auditory performances and only minor tinnitus reduction with CI. Audiological follow-up was carried out during a 5-year follow-up period comprising pure-tone audiometry, speech-in-quiet testing, speech-in-noise testing, tinnitus questionnaires (tinnitus questionnaire and numeric rating scale) and the HISQUI19 questionnaire. To investigate the neural substrates of tinnitus in this subject, H215O PET tomography scans were acquired in three different conditions: 1) ABI switched off which was considered as the resting-state measurement rendering the loudest possible tinnitus for the patient (ABI OFF); 2) ABI switched on causing a small suppression of tinnitus due to electrical stimulation (ABI ON); 3) ABI switched on and 70 dB SPL white noise presented directly to the external audio processor through a direct audio cable providing the maximum tinnitus suppression for the patient (NOISE). Results: Subjectively the patient reported a significant tinnitus reduction after implantation which remained stable over time with a decrease in tinnitus questionnaire from grade 4 to grade 2 and a 50% reduction in the numeric rating scale (from 8 to 4) during the 5-year period. Comparing the ABI OFF and ABI ON conditions, significant increase in regional cerebral blood flow (rCBF) was observed in brain areas involved in the salience network showing already suppression of tinnitus only by electrical stimulation in the absence of auditory stimuli. The NOISE condition showed relatively decreased rCBF in the insula (as well as in the orbitofrontal cortex) as compared with the ABI OFF condition. Abnormally activated areas comprising the salience network may have been significantly suppressed by the NOISE condition both by acoustic and electrical stimulations of the auditory pathway. Moreover, the NOISE condition showed significantly decreased rCBF in the parahippocampus as compared with the ABI OFF condition. This finding supports the idea of distinct tinnitus generators depending on the amount of hearing loss. Conclusion: The reduction of tinnitus in the current ABI subject may be attributable to partial peripheral reafferentation-induced deactivation of the parahippocampus-based tinnitus generator as well as the salience network. Further validation is required by the use of a follow-up study with a larger number of subjects.

OBJECTIVE To investigate the effects of cavitating lesions involving the internal auditory canal (IAC) in subjects with cochlear otosclerosis with regard to poststapedotomy hearing outcome. STUDY DESIGN Retrospective study. SETTING Tertiary referral center. PATIENTS A retrospective chart review of 134 subjects with otosclerosis treated from January 2011 to June 2017 at Seoul National University Bundang Hospital was conducted. Sixteen subjects (23 ears) with temporal bone computed tomography (TBCT)-confirmed cochlear otosclerosis who underwent stapedotomy were included in the study. MAIN OUTCOME MEASURES Pure tone audiometry (PTA) (i.e., air and bone conduction; AC and BC, respectively) thresholds and air-bone gap (ABG), measured at 6 months postoperatively were compared between cochlear otosclerosis with and without IAC involvement (IAC group and non-IAC group, respectively). RESULTS A total of 14 of 23 ears showed involvement of the IAC. There were no significant differences in age, side of otosclerosis, or preoperative hearing threshold between the two groups. The mean postoperative AC and BC thresholds and ABG of the IAC group were significantly poorer (45.7 dB, 33.8 dB, and 11.8 dB, respectively) than those of the nonIAC group (24.1 dB, 20.0 dB, and 4.1 dB, respectively). CONCLUSIONS Cochlear otosclerosis with cavitating lesions involving the IAC showed significantly poorer postoperative audiological outcomes than those without any cavitating lesion. Cavitation extending to the IAC may act as a third window providing a route for sound energy shunting, and thus precluding successful hearing outcome in some subjects with cavitating otosclerosis after stapedotomy.

Objective To discuss the possible pathophysiologic mechanism of pulsatile tinnitus (PT) perception due to high jugular bulb with bony dehiscence (HJBD) and its improvement after the dehiscent jugular bulb (JB) resurfacing using bone cement, and to describe the efficacy of an objective measure of PT using transcanal sound recording and spectro-temporal analysis (TSR/STA). Study design Retrospective case series study. Setting A university-based, tertiary care cancer center. Patients Three PT patients with HJBD. Main outcome measure(s) All patients underwent transtympanic resurfacing after the source of PT was confirmed by temporal bone imaging and TSR/STA. Improvement of symptom and the changes in the TSR/STA were analyzed. Results In the first case, a revision operation was performed due to slightly improved but persistent PT after initial resurfacing with bone pate and a piece of conchal cartilage. Revision transtympanic JB resurfacing was performed in this case using bone cement, and PT resolved immediately after the surgery. In the second and third cases, PT resolved completely, or was much abated, immediately after transtympanic resurfacing with bone cement. The TSR/STA also revealed improvement of PT. The median follow-up duration was 28 months, and all three patients remained asymptomatic or much improved compared with their preoperative status. Conclusions Transtympanic resurfacing with bone cement, reinforcing the dehiscent JB to reduce focal turbulent flow, is a simple and effective surgical treatment option in patients with PT due to HJBD. In patients with HJBD, the objective measurement of PT by TSR/STA may be of help in selecting appropriate surgical candidates and objective evaluation of the treatment outcome.

OBJECTIVES Recent studies have shown that cochlear duct length (CDL) varies among individuals and could significantly influence the final position of the electrode and its trajectory in the cochlea. Given this, we hypothesized that the degree of modiolar proximity of novel slim modiolar electrodes, such as CI532 and CI632, can also be affected by CDL. To test this hypothesis, we retrospectively evaluated individual CDL to determine if there is any significant correlation of CDL with degree of modiolar proximity. METHODS Fifty-one ears from 38 subjects implanted with slim modiolar electrodes by a single surgeon through the round window approach using the pull-back technique were included. Our cohort was classified according to the deafness onset (congenital versus postlingual) and the degree of modiolar proximity (less versus tight) with reference to the spiral diameter made by the slim modiolar electrodes in situ on transorbital x ray. We then analyzed the CDL and its metrics using a readily available surgical preplanning tool (OTOPLAN) to obtain comparable data. RESULTS Among 30 ears associated with congenital deafness, 9 ears (30%) showed less modiolar proximity, while none of the 21 ears from 19 subjects with postlingual deafness exhibited "less modiolar proximity" based on our criteria. In this study, CDL showed significant variation among subjects. Importantly, a significant inverse correlation between spiral diameter and CDL (ρ = -0.581, p < 0.001) was found, showing that shorter CDLs have longer spiral diameter and less modiolar proximity. Moreover, further pull-back technique characterized by pulling out the electrode a little bit more in cases with shorter CDL, if not always, exhibited tighter modiolar proximity. CONCLUSION A preponderance of less modiolar proximity of the electrode was observed exclusively among congenital deafness cases, demonstrated by a less tight spiral configuration even under the pull-back technique. Our data suggest that shorter CDL is associated with a less tight spiral configuration of slim modiolar electrodes postoperatively. Depending on the insertion technique, the differential degree of modiolar proximity of slim modiolar electrodes can be alleviated in cases with short CDL, which justifies cochlear duct length-based customized insertion of slim modiolar electrodes.

Objective Distinguishing intralabyrinthine schwannoma (ILS) from labyrinthitis is crucial in deciding patient management between surgery and medication; however, the clinical and radiological differentiation between ILS and labyrinthitis is difficult, especially when labyrinthitis shows contrast-enhancement mimicking ILS on magnetic resonance imaging (MRI). This study aimed to evaluate the quantitative characteristics of signal intensity on 3T MRI for differentiating ILS from contrast-enhancing labyrinthitis (CEL). Study design Retrospective study. Setting Tertiary referral center. Patients Between July 2003 and June 2017, 9 subjects diagnosed with ILS (16 MRI cases) and 9 subjects diagnosed with CEL (9 MRI cases) were enrolled in the study. Main outcome measures Normalized signal intensity on contrast-enhanced T1-weighted image (nCE-T1), normalized signal intensity on T2-weighted image (nT2), and normalized signal intensity on contrast-enhanced fluid-attenuated inversion recovery image (nFLAIR) were measured and compared between ILS and CEL. The diagnostic performance of these values was assessed using receiver operating characteristic (ROC) analysis. Results In ILS, the nCE-T1 and the nT2 were significantly higher and lower, respectively, than in CEL (all, p 0.05). The maximal nCE-T1 achieved the highest area under the curve (AUC) in differentiating ILS and CEL, followed by the mean nCE-T1, the mean nT2, and the maximal nT2. Combination of the nCE-T1 and the nT2 showed higher AUC than the nCE-T1 alone, but without statistical significance (p = 0.340). Conclusions Quantitative measurement of the signal intensity on MRI can be a viable imaging tool for differentiating ILS from CEL.

Objective To describe the results of a thorough evaluation in a large series of patients with an enlarged vestibular aqueduct (EVA), focusing on vestibular manifestations with etiological considerations. Study design Retrospective chart review of patients with EVA. Setting Tertiary referral center. Patients A total of 22 EVA patients with a median age of 8 years (6 mo-35 yr) who underwent both audiovestibular and radiologic examinations. Main outcome measures Patient demographics, radiologic findings, audiologic results, vestibular symptoms, findings of neurotologic examinations, and laboratory evaluations were collected and analyzed. Standard descriptive statistics were used to summarize patient characteristics. Subjects who had a history of vertigo attack were categorized as "vestibulopathy group," while subjects without any history of vertigo as "non-vestibulopathy group." Results Of the 41 ears included, 37 (90.2%) had hearing loss on initial audiometric evaluations. Among the 22 patients, 14 (63.6%) complained of dizziness. Of the 14 vertiginous patients, seven had recurrent episodes, five had a history of single attack, and two presented with postural imbalances. There were no significant differences between vestibulopathy and non-vestibulopathy groups with regard to the relationship between the development of vestibular symptoms and aqueductal size, hearing threshold, or age at first visit. Four of the 22 (18.2%) patients developed secondary benign paroxysmal positional vertigo (BPPV) and all patients complained of simultaneous decreases in hearing. Conclusions Our results demonstrate that patients may develop vestibular symptoms during their clinical course, and all patients with an enlarged vestibular aqueduct should be cautioned regarding the potential development of vestibular pathology. Moreover, the non-negligible incidence of secondary BPPV mandates positional tests when evaluating EVA patients with vertigo.

We aimed to evaluate the subjective satisfaction after incus vibroplasty and to determine predictive factors affecting patient satisfaction in sensorineural hearing loss. A retrospective review of audiological data and an additional survey about subjective satisfaction after surgery were performed in 14 patients who underwent incus vibroplasty surgery. A numeric rating scale reflecting the degree of satisfaction after incus vibroplasty, compared with experiences using a conventional hearing aid, was used. Patients who showed median or better satisfaction were deemed the highly satisfied (HS) group, and the others were deemed the less satisfied (LS) group. To find the predictive factors correlated with satisfaction for incus vibroplasty, comparative analysis between two groups was performed. We found that the numeric rating scale for satisfaction was variable, ranged from 0 to 10, and was negatively correlated with age at operation (p

The present study aimed to evaluate and compare the outcome of different bone conduction hearing implants (BCHIs) in subjects with mixed hearing loss (MHL) and single-sided deafness (SSD) in terms of audiometric results and compliance. Twenty-one subjects with MHL and 18 subjects with SSD undergoing implantation of Baha connect, Baha attract, or Bonebridge were enrolled. Functional gain, effective gain, and usage rate of BCHIs were retrospectively reviewed. As for MHL, the functional gain of three devices was not significantly different (p = 0.477), while the effective gain of Bonebridge was higher (− 8.8 [− 15.0, − 3.5] dB) than that of Baha connect (− 20.0 [− 26.3, − 11.3] dB, p = 0.037), especially at 0.5 kHz (p = 0.010) and 1 kHz (p = 0.014). In SSD subjects, the effective gain of Bonebridge was significantly higher than that of Baha attract (− 11.3 [− 15.0, − 7.5] vs − 21.3 [− 21.3, − 16.3] dB, p = 0.012), while the functional gain of Bonebridge and Baha attract was not different. The constant usage rate of BCHIs tends to be higher in MHL subjects [17/21 (82%)] than that in SSD subjects [10/18 (56%)]. In SSD subjects, the constant user group showed higher functional gain than the non-constant user group, with a significant difference at 3 kHz (35.0 [33.8, 45.0] vs 17.5 [10.0, 27.5] dB, p = 0.006). Bonebridge shows a higher effective gain than Baha connect in the MHL group and Baha attract in the SSD group. The usage rate of BCHIs is lower in SSD than that in MHL. In SSD subjects, the constant user group tended to show higher functional gain than the non-constant user group. Irrespective of the device type, the tendency of higher functional gain of BCHIs, especially at mid frequencies, may potentially lead to yield good compliance in SSD, mandating a meticulous fitting strategy ensuring a sufficient mid-frequency functional gain in SSD.

Although frequently classified as "objective tinnitus," in most cases vascular pulsatile tinnitus (VPT) is not equal to objective tinnitus because it is not easy to objectively document VPT. The present study was conducted to develop a novel transcanal sound recording and spectrotemporal analysis method for the objective and differential diagnosis of VPT.A case series with a control group.Tertiary referral center.Six VPT subjects with radiological abnormalities and six normal controls.The method was tested based on recordings obtained from the ipsilateral external auditory canal (EAC) using an insert microphone with the subject's head in four different positions. The recorded signals were first analyzed in the time domain, and short-time Fourier transform was performed to analyze the data in the time-frequency domain.From the temporal analysis, the ear canal signals recorded from the VPT subjects exhibited large peak amplitudes and periodic structures, whereas the signals recorded from the control subjects had smaller peak amplitudes and weaker periodicity. From the STA represented by two-dimensional spectrograms and three-dimensional waterfall diagrams, all of the VPT subjects demonstrated pulse-synchronous acoustic characteristics that were representative of their respective presumptive vascular pathologies, whereas the control subjects did not display such characteristics.The present diagnostic approach may provide additional information regarding the origins of VPT cases as well as an efficient and objective diagnostic method. Furthermore, this approach may aid in the determination of appropriate imaging modalities, treatment planning, and evaluation of treatment outcomes.

Objectives To investigate longitudinal changes in behavior and quality-of-life (QoL) in children with sleep disordered breathing (SDB) after adenotonsillectomy (AT). Methods This study prospectively enrolled 148 children who underwent AT for SDB. Caregivers filled out Korean attention deficit hyperactivity disorder (ADHD) rating scale (K-ARS) and Korean version of the obstructive sleep apnea-18 (KOSA-18) at preoperative 1 day, postoperative 1 month, and postoperative 6 months. Longitudinal changes in K-ARS and KOSA-18 were evaluated and compared among subgroups. Results Both K-ARS and KOSA-18 scores improved immediately at postoperative 1 month and were maintained at postoperative 6 months. The mean preoperative K-ARS scores of the non-ADHD, ADHD-trait, and overt-ADHD groups showed uniformly immediate and sustained improvements. Also, the mean preoperative KOSA-18 scores of the small, moderate, and large impact groups were significantly improved both at postoperative 1 month and 6 months. The changes in behavior and QoL showed no significant differences with regard to the aforementioned factors. Conclusion After AT, we may expect immediate and sustained improvements in behavior and QoL in children with SDB regardless of preoperative symptom severity. Moreover, by performing AT, improvements in behavior and QoL are expected regardless of sex, age, allergic trait, or concurrent coblation-assisted turbinoplasty. These findings may help our colleague physicians counselling pediatric SDB patients and their caregivers.

Recent studies have suggested that hyperacusis, an abnormal hypersensitivity to ordinary environmental sounds, may be characterized by certain resting-state cortical oscillatory patterns, even with no sound stimulus. However, previous studies are limited in that most studied subjects with other comorbidities that may have affected cortical activity. In this regard, to assess ongoing cortical oscillatory activity in idiopathic hyperacusis patients with no comorbidities, we compared differences in resting-state cortical oscillatory patterns between five idiopathic hyperacusis subjects and five normal controls. The hyperacusis group demonstrated significantly higher electrical activity in the right auditory-related cortex for the gamma frequency band and left superior parietal lobule (SPL) for the delta frequency band versus the control group. The hyperacusis group also showed significantly decreased functional connectivity between the left auditory cortex (AC) and left orbitofrontal cortex (OFC), between the left AC and left subgenual anterior cingulate cortex (sgACC) for the gamma band, and between the right insula and bilateral dorsal anterior cingulate cortex (dACC) and between the left AC and left sgACC for the theta band versus the control group. The higher electrical activity in the SPL may indicate a readiness of “circuit-breaker” activity to shift attention to forthcoming sound stimuli. Also, because of the disrupted salience network, consisting of the dACC and insula, abnormally increased salience to all sound stimuli may emerge, as a consequence of decreased top-down control of the AC by the dACC and dysfunctional emotional weight attached to auditory stimuli by the OFC. Taken together, abnormally enhanced attention and salience to forthcoming sound stimuli may render hyperacusis subjects hyperresponsive to non-noxious auditory stimuli.

Objectives/Hypothesis 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. Study Design Individual retrospective cohort study. Methods 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. Results 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 was

Perioperative Minnesota Multiphasic Personality Inventory (MMPI) scores may be beneficial for predicting prognosis of cochlear implantation (CI). A positive attitude for social interaction in particular correlates with a better speech outcome. Proper perioperative psychological management may, therefore, assist in the auditory rehabilitation of CI patients.To determine the perioperative psychological state of CI patients and its relationship with patient prognosis after CI.This study prospectively enrolled 29 patients who underwent CI from 2005-2013. The MMPI was administered to assess psychosocial and emotional issues surrounding CI and the Korean version of the Central Institute of Deafness (K-CID) score was used to measure speech perception.CI resulted in a significant improvement on the MMPI Paranoia scale (p = 0.02). Patients with abnormal pre-operative and post-operative MMPI scores also had an earlier onset of deafness, longer duration of deafness, and lower K-CID scores than patients with normal MMPI scores (all p 0.05). The post-CI K-CID score had a significant negative correlation with the pre-operative MMPI Schizophrenia score (p 0.01) and significant negative correlations with the post-operative MMPI Paranoia (p = 0.02), Psychasthenia (p = 0.02), Schizophrenia (p = 0.04), Hypomania (p = 0.02) and Social Introversion (p = 0.03) scores.

Psychosocial factors should be considered during cochlear implantation (CI). There were differences in psychosocial characteristics according to the etiology of deafness. The outcomes may be affected by psychosocial variables such as the severity of mental distress and social problems as well as duration of deafness.To evaluate the psychosocial characteristics of deaf people undergoing CI and to determine which psychosocial factors affect performance after CI.A total of 289 subjects who underwent CI were enrolled. The participants were classified into prelingually deaf (pre-LD) and postlingually deaf groups (post-LD), including progressive and sudden deafness subgroups. The Minnesota Multiphasic Personality Inventory (MMPI) was administered before CI to measure psychosocial and emotional problems. To measure CI outcomes, speech perception ability was assessed by the open-set Korean version of the Central Institute of Deafness (K-CID) test and categories of auditory performance (CAP) scores before and after CI.Approximately 45% of subjects experienced psychological problems before undergoing CI. Subjects in the Pre-LD group had more psychosocial distress and were more likely to be oversensitive in interpersonal situations, while those in the post-LD group were more depressed. Deafness duration and psychosocial factors significantly predicted hearing ability after CI. Deafness duration directly and indirectly affected the outcome of CI. That is, duration of deafness caused psychosocial problems, which may have resulted in negative effects on outcomes of CI.

Introduction Auricular Arteriovenous Malformation of the external ear is a rarely encountered disease; in particular, arteriovenous malformation arising from the auricle, with spontaneous bleeding, has seldom been reported. Objective In the current study, we report an unusual case of late-onset auricular arteriovenous malformation originating from the posterior auricular artery that was confirmed by computed tomographic angiography. The case was successfully managed by pre-surgical intravascular embolization followed by total lesion excision. Prompted by this case, we also present a scoping review of the literature. Methods A case of a 60 year-old man with right auricular arteriovenous malformation treated in our tertiary care center, and 52 patients with auricular arteriovenous malformation described in 10 case reports and a retrospective review are presented. Auricular arteriovenous malformation can manifest as swelling of the ear, pulsatile tinnitus, pain, and/or bleeding. On physical examination, a pulsatile swelling and/or a tender mass is evident. When arteriovenous malformation is suspected, the lesions should be visualized using imaging modalities that optimally detect vascular lesions, and managed via embolization, mass excision, or auricular resection. Effectiveness of the various diagnostic methods used and the treatment outcomes were analyzed. Results Various imaging modalities including Doppler sonography, computed tomographic angiography, magnetic resonance angiography, and/or transfemoral cerebral angiography were used to diagnose 38 cases reported in the literature. In another 15 cases, no imaging was performed; treatment was determined solely by physical examination and auscultation. Of the total of 53 cases, 12 were not treated (their symptoms were merely observed) whereas 20 underwent therapeutic embolization. In total, 32 patients, including 1 patient who was not treated and 10 with persistent or aggravated arteriovenous malformation after previous embolization, underwent mass excision or auricular resection depending on the extent of the lesion. No major postoperative complication was recorded. The postoperative follow-up duration varied from 1 month to 19 years, and only one case of unresectable, residual cervicofacial arteriovenous malformation was recorded. Conclusion Auricular arteriovenous malformation is a rarely encountered disease, but should be suspected if a patient presents with a swollen ear and pulsatile tinnitus. Appropriate imaging is essential for diagnosis and evaluation of the extent of disease. As embolization affords only relatively poor control, total surgical removal of the vascular mass is recommended.

Objectives Globally, newborn hearing screening (NHS) is variably incorporated into national healthcare systems. The authors reviewed the set-up and evolution process of a hospital-based NHS program in South Korea, where screening costs for low-income families are paid by the National Health Authority. Methods The NHS process for 13805 newborns delivered in a tertiary referral center of South Korea from 2005 through 2014 was reviewed. Hearing screening was conducted using automated auditory brainstem response (AABR); hearing loss was confirmed by auditory brainstem response for newborns who did not pass the screening test. Results The mean screening rate for hearing loss was 53.6% (7403 of 13805 newborns), which plateaued at 79.6% over time. Of the 14806 ears (7403 newborns), 1030 (7.0%) were assessed as “refer” on the first AABR, with 204 (1.4%) being assessed as “refer” on the second AABR. In hearing confirmation tests, 74 infants (1.0% of 7403 newborns) were diagnosed with hearing loss, including 13 infants (0.2%) with bilateral moderate to profound sensorineural hearing loss (SNHL). Hearing rehabilitation with long-term follow-up was confirmed in 11 infants. Conclusions In this hospital-based NHS program, the screening rate plateaued at ∼50% when the National Health Authority was not involved, but increased to ∼70% when the cost for low-income families was covered by the government. Among infants needing active hearing rehabilitation due to bilateral moderate to profound SNHL, 15% were lost to follow-up. These results demonstrate the need for a universal, mandatory NHS program to systematically register hearing-impaired infants within the government-sponsored public healthcare system.

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.

Objectives/Hypothesis To describe our tracheostomy procedure using a vertical skin incision and a horizontal intercartilaginous incision and to compare our postoperative results with those in the recent literature. Study Design Retrospective chart review and literature review. Methods One hundred eleven children underwent tracheostomy using a vertical skin incision and a horizontal intercartilaginous incision over a 9-year period. A retrospective chart review was undertaken with respect to the following variables: demographics, weight, primary diagnosis, surgical indication, follow-up duration, decannulation, interval between tracheostomy and decannulation, and complications experienced. In addition, the outcomes were compared to results from a literature review. Results Of the 111 patients, 56 had a history of prolonged intubation, 52 had upper airway obstruction, and three underwent tracheostomy for tracheobronchial toileting. Three (2.7%) patients experienced early major complications without major sequelae, and 14 (12.6%) experienced late major complications. Twenty-one (18.9%) patients were decannulated during the follow-up period, whereas 17 (17.8%) died of their primary disease or tracheostomy-related complications (15 died of their primary disease and two died of tracheostomy-related complications). The complication rates showed overall no differences, but our patient series showed significantly less stomal granulation tissue formation and non-significant trend for less suprastomal collapse as compared with previous literature. Conclusions Pediatric tracheostomy using a vertical skin incision and horizontal intercartilaginous incision is comparable with the conventional procedure in terms of complication and mortality rates. Future randomized controlled studies are mandatory to directly compare conventional tracheotomy using a vertical skin incision and a horizontal intercartilaginous incision with our procedure. Level of Evidence 4. Laryngoscope, 125:485–492, 2015

Objective In patients with pulsatile tinnitus (PT), physical examination such as auscultation with head position change or digital compression over the ipsilateral jugular vein provides physicians with important information. However, objective diagnosis of PT is sometimes limited because 1) audible bruit is absent on auscultation in some patients, 2) abnormal vascular structures found in radiologic evaluation is not always pathognomonic because they can be found in asymptomatic subjects as well, and 3) although an objective diagnostic tool using transcanal sound recording has recently been introduced, special equipment is needed. In this regard, recent studies that have reported ipsilateral low-frequency hearing loss (LFHL) on pure-tone audiometry (PTA) in some patients with PT, and its recovery after successful management, prompted us to conduct a retrospective observational study on the characteristics of the audiometric profile, the association between the audiometric profile and radiologic findings, and pre- and posttreatment changes in low-frequency hearing thresholds in PT patients. We tested two hypotheses: PT patients with marked vascular pathologies located close to the cochlea may show ipsilateral pseudo-LFHL (PLFHL) because of the masking effects of the PT itself, and their PLFHL may disappear if their vascular pathology is successfully managed by surgical or endovascular intervention. Study design Retrospective case review. Setting Tertiary referral center. Patients A total of 85 PT subjects who underwent both audiologic and radiologic examinations. Main outcome measure All patients' pre- and posttreatment PTA thresholds and radiologic findings were analyzed. By comparing the LFHL (an ipsilateral hearing threshold greater than 10 dB HL at both 250 and 500 Hz or greater than 20 dB HL at either 250 or 500 Hz compared with the contralateral side) group and a non-LFHL group with regard to the incidence of vascular structural abnormalities, we evaluated the incidence of abnormal vascular structures in the head and neck between the LFHL and non-LFHL groups. In addition, by comparing pre- and posttreatment PTA thresholds of seven PT patients with ipsilateral LFHL, we further evaluated the changes in low-frequency hearing thresholds and their role as an objective sign for diagnosis and outcome evaluation. Results Of 85 patients, 22 (25.9%) presented with ipsilateral LFHL. Compared with patients without this condition, patients with ipsilateral LFHL showed a significantly higher rate of abnormal vascular structure. In addition, most of the radiologic abnormalities found in the LFHL group were highly suspicious causative lesions that are responsible for the perception of PT according to the previous literature. In eight PT patients with ipsilateral LFHL who underwent both pre- and posttreatment audiograms, the average posttreatment pure-tone threshold at 250 Hz showed significant improvement compared with the pretreatment threshold. Conclusion PT patients presenting with ipsilateral LFHL have higher possibility of having a discrete vascular pathology near the cochlea on radiologic evaluation. As ipsilateral LFHL improves in most patients after treatment, LFHL in patients with PT may be PLFHL because of the masking effects of the pulsatile sound, and the changes in the low-frequency thresholds may be applicable for objective diagnosis and evaluation of the effects of the treatment.

Objective Although vascular pulsatile tinnitus (VPT) has been classified as “objective”, VPT is not easily recognizable or documentable in most cases. In response to this, we have developed transcanal sound recording (TSR) and spectro-temporal analysis (STA) for the objective diagnosis of VPT. By refining our initial method, we were able to apply TSR/STA to post-treatment outcome evaluation, as well as pre-treatment objective diagnosis. Methods TSR was performed on seven VPT patients and five normal controls before and after surgical or interventional treatment. VPT was recorded using an inserted microphone with the subjects placed in both upright and supine positions with 1) a neutral head position, 2) head rotated to the tinnitus side, 3) head rotated to the non-tinnitus side, and 4) a neutral position with ipsi-lesional manual cervical compression. The recorded signals were analyzed in both time and time-frequency domains by performing a short-time Fourier transformation. Results The pre-treatment ear canal signals of all VPT patients demonstrated pulse-synchronous periodic structures and acoustic characteristics that were representative of their presumptive vascular pathologies, whereas those the controls exhibited smaller peaks and weak periodicities. Compared with the pre-treatment signals, the post-treatment signals exhibited significantly reduced peak- and root mean square amplitudes upon time domain analysis. Additionally, further sub-band analysis confirmed that the pulse-synchronous signal of all subjects was not identifiable after treatment and, in particular, that the signal decrement was statistically significant at low frequencies. Moreover, the post-treatment signals of the VPT subjects revealed no significant differences when compared to those of the control group. Conclusion We reconfirmed that the TSR/STA method is an effective modality to objectify VPT. In addition, the potential role of the TSR/STA method in the objective evaluation of treatment outcomes in patients with VPT was proven. Further studies incorporating a larger sample size and more refined recording techniques are warranted.

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 suggests that adjuvant anti-anxiety medication may be helpful for patients with benign paroxysmal positional vertigo (BPPV) even after a successful canalith repositioning procedure (CRP).Although the CRP is an effective treatment for BPPV, many patients suffer from persistent dizziness despite successful CRPs. The aim of this study was to evaluate the effect of adjuvant anxiolytic medication on residual dizziness after successful CRP.Between January 2011 and March 2011, 73 patients were diagnosed with BPPV and they underwent successful treatment with CRPs. The patients were randomly assigned to either the medication group or the control group. The patients in the medication group were prescribed low dose etizolam for 2 weeks whereas the patients in the control group were not prescribed any medication. All patients were scheduled to return 2 weeks after evaluation of subjective visual vertical. Subjective symptoms before and after CRP were measured using the Dizziness Handicap Inventory (DHI) and the Activities-specific Balance Confidence (ABC) scale.Both groups demonstrated a significant improvement in DHI scores. However, the medication group showed significantly greater decrease in the functional (p = 0.018) and emotional (p = 0.030) subscale scores, as well as in the total DHI (p = 0.038) score.

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).

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.

Objective Conversion deafness is characterized by sudden hearing loss without any identifiable cause. In the current study, we investigated presumed conversion deafness in a cochlear implant user using H215Opositron emission tomography (PET) scan with speech and noise stimuli in conjunction with audiologic tests such as impedance test and auditory response telemetry. Also, by performing a follow-up PET scan after recovery and comparing prerecovery and postrecovery scans, we attempted to find possible neural substrates of conversion deafness. Patient A 51-year-old man with conversion deafness after 4 years of successful cochlear implant use. Intervention Supportive psychotherapy. Main Outcome Measures Prerecovery and postrecovery H215O-PET scans Results The prerecovery H215O-PET scan revealed auditory cortex activation by sound stimuli, which verified normal stimulation of the central auditory pathway. Notably, compared with the prerecovery state, the postrecovery state showed relative activation in the right auditory cortex both under the speech and noise stimulus conditions. Moreover, the bilateral prefrontal and parietal areas were activated more in the postrecovery state than in the prerecovery state. In other words, relative deactivation of the prefronto-parieto-temporal network, a network responsible for conscious sensory perception, or relative dysfunction of top-down and bottom-up attention shifting mediated by the ventral and the dorsal parietal cortices, may have resulted in conversion deafness in the patient. Conclusion Relative deactivation of the prefronto-parieto-temporal network or dysfunction in the ventral and the dorsal parietal cortices may be related to the development of conversion deafness.

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.

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.

Enlarged vestibular aqueduct (EVA) may precipitate secondary benign paroxysmal positional vertigo (BPPV), especially in pediatric populations, as well as paretic dysfunction of the vestibular end organ.EVA is characterized by the early onset of sudden or progressive sensorineural hearing loss with or without vestibular dysfunction. However, vestibular dysfunction in patients with EVA has not been described as frequently as hearing loss, and an association with BPPV has not been previously reported. By reviewing the cochleo-vestibular status of three children and two young adults with EVA who had been diagnosed with BPPV, characteristic features of BPPV in EVA patients were investigated and possible pathogenetic mechanisms are speculated.A retrospective review of medical records was conducted for five patients with EVA who presented with BPPV from January 2004 to July 2009. Clinical courses, characteristics of vertigo and audiovestibular laboratory findings were reviewed.Among 26 patients with radiologically confirmed EVA, 5 (19.2%) exhibited positional nystagmus compatible with BPPV through Dix-Hallpike and head-roll tests. Hearing loss usually accompanied BPPV attacks, and BPPV was recurrent in three patients. Multiple semicircular canals were frequently involved in each episode, and different canals were also involved in recurrent cases. Canalith repositioning procedures were usually successful without difficulty.

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.

*Department of OtorhinolaryngologyYHead and Neck Surgery, Seoul National University Bundang Hospital,Seongnam; ÞDepartment of Otorhinolaryngology, Eulji University College of Medicine; þDepartment ofOtorhinolaryngology, National Medical Center, Seoul; §Department of Neurology, Seoul NationalUniversity Bundang Hospital, Seongnam; and kSensory Organ Research Institute, Seoul NationalUniversity Medical Research Center, Seoul, KoreaObjective: To determine characteristics and their prognostic valuein idiopathic sudden sensorineural hearing loss (ISSNHL) withcomorbidipsilateralbenign paroxysmal positionalvertigo (BPPV).Study Design: Retrospective chart review.Setting: Tertiary referral center.Patients: Of the 374 patients with a diagnosis of ISSNHL atSeoulNationalUniversityBundangHospitalfromJanuary2004to December 2009, 32 patients (8.6%) with comorbid BPPVwere recruited and compared with matched ISSNHL patientswithout BPPV.Interventions: Otologic and neurotologic examinations, pure-tone audiometry (PTA), vestibular function tests, and brain mag-netic resonance imagings.MainOutcomeMeasures:Comparisonofthefindingsofserialaudiograms for 6 months and vestibular function tests in patientswith ISSNHL and concurrent BPPV with those in age-matchedISSNHL patients without BPPV.Results: Patients with BPPV showed higher PTA averages thanthose without BPPV on initial and follow-up audiograms.Moreover,theimprovement inPTAwaslessintheBPPVgroupthan in the control. BPPV most commonly involved the pos-terior canal (17/32, 53.1%), followed by the horizontal canal(8/32, 25%), both the posterior and horizontal canals (6/32,18.8%), and the anterior canal (1/32, 3.1%). Thirteen (40.6%)of 32 patients had recurrences of BPPV, 9 within a week andanother 2 within 3 months.Conclusion: ComorbidBPPV isa negativeprognostic indicatorof auditory function in ISSNHL. Concurrent BPPV in ISSNHLsuggests combined damage to the utricle and may indicate se-vere and widespread labyrinthine damage, leading to the poorprognosis. Key Words: Benign paroxysmal positional vertigoVHearing lossVSudden.Otol Neurotol 33:137Y141, 2012.

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.

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.