Jae-Jin Song

Unilateral sensorineural hearing loss in children: the importance of temporal bone computed tomography and audiometric follow-up.

Objectives: 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). Study Design: Retrospective case review. Setting: Tertiary referral center. Patients: A total of 322 children diagnosed with USNHL on initial audiometry were included. Interventions: Otologic examination, pure-tone audiometry or auditory brainstem response, and high-resolution TBCT. Main Outcome Measures: Radiologic findings demonstrating clinically relevant bony or soft tissue malformations of the inner ear. Audiologic findings of change in thresholds. Results: 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. Conclusion: 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.

Effects of congruent and incongruent visual cues on speech perception and brain activity in cochlear implant users

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 H215O-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.

Comorbid Benign Paroxysmal Positional Vertigo in Idiopathic Sudden Sensorineural Hearing Loss: An Ominous Sign for Hearing Recovery

Objective To determine characteristics and their prognostic value in idiopathic sudden sensorineural hearing loss (ISSNHL) with comorbid ipsilateral benign paroxysmal positional vertigo (BPPV). Study Design Retrospective chart review. Setting Tertiary referral center. Patients Of the 374 patients with a diagnosis of ISSNHL at Seoul National University Bundang Hospital from January 2004 to December 2009, 32 patients (8.6%) with comorbid BPPV were recruited and compared with matched ISSNHL patients without BPPV. Interventions Otologic and neurotologic examinations, pure-tone audiometry (PTA), vestibular function tests, and brain magnetic resonance imagings. Main Outcome Measures Comparison of the findings of serial audiograms for 6 months and vestibular function tests in patients with ISSNHL and concurrent BPPV with those in age-matched ISSNHL patients without BPPV. Results Patients with BPPV showed higher PTA averages than those without BPPV on initial and follow-up audiograms. Moreover, the improvement in PTA was less in the BPPV group than in the control. BPPV most commonly involved the posterior 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 and another 2 within 3 months. Conclusion Comorbid BPPV is a negative prognostic indicator of auditory function in ISSNHL. Concurrent BPPV in ISSNHL suggests combined damage to the utricle and may indicate severe and widespread labyrinthine damage, leading to the poor prognosis.

Pediatric tracheostomy revisited: A nine-year experience using horizontal intercartilaginous incision

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.

Anxiolytics reduce residual dizziness after successful canalith repositioning maneuvers in benign paroxysmal positional vertigo.

Abstract Conclusions: 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). Objective: 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. Methods: 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. Results: 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.

Sinus Wall Resurfacing for Patients With Temporal Bone Venous Sinus Diverticulum and Ipsilateral Pulsatile Tinnitus

BACKGROUND Pulsatile tinnitus (PT) caused by venous sinus diverticulum is a relatively common, potentially incapacitating condition. Although treatment via an external approach or endovascular coiling has been reported, much remains unknown about the possible pathophysiological mechanisms and appropriate management of PT. OBJECTIVE To review our case series of PT resulting from either sigmoid sinus diverticulum (SSD) or middle cranial fossa venous sinus diverticulum (MFD-VS) and to discuss the possible pathophysiological mechanisms and desirable treatment options. METHODS Four PT patients with either SSD or MFD-VS were treated with transmastoid resurfacing. In 1 case, a revision operation was performed as a result of recurrence of PT 4.5 years after the initial operation. The medical records and temporal bone imaging findings were retrospectively reviewed. RESULTS PT was resolved in all cases immediately after transmastoid resurfacing, but 1 patient in whom bone wax was used for initial resurfacing experienced PT 4.5 years later. The PT was successfully managed with revision resurfacing with autologous bone chips/bone cement. In the other cases, the resolution of PT lasted throughout a median follow-up of 5.75 years. Notably, 2 of 4 cases had preoperative low-frequency hearing loss (LFHL) and experienced immediate postoperative improvement in LFHL. CONCLUSION PT resulting from either SSD or MFD-VS can be treated successfully with transmastoid resurfacing of the venous wall. Preoperative ipsilesional LFHL and the improvement of hearing threshold after surgical intervention may be preoperative and postoperative surrogate objective signatures of PT. To ensure the resolution of symptoms, secure reconstruction with firm materials and long-term follow-up are mandatory.

Morphological brain network assessed using graph theory and network filtration in deaf adults

Prolonged deprivation of auditory input can change brain networks in pre- and postlingual deaf adults by brain-wide reorganization. To investigate morphological changes in these brains voxel-based morphometry, voxel-wise correlation with the primary auditory cortex, and whole brain network analyses using morphological covariance were performed in eight prelingual deaf, eleven postlingual deaf, and eleven hearing adults. Network characteristics based on graph theory and network filtration based on persistent homology were examined. Gray matter density in the primary auditor cortex was preserved in prelingual deafness, while it tended to decrease in postlingual deafness. Unlike postlingual, prelingual deafness showed increased bilateral temporal connectivity of the primary auditory cortex compared to the hearing adults. Of the graph theory-based characteristics, clustering coefficient, betweenness centrality, and nodal efficiency all increased in prelingual deafness, while all the parameters of postlingual deafness were similar to the hearing adults. Patterns of connected components changing during network filtration were different between prelingual deafness and hearing adults according to the barcode, dendrogram, and single linkage matrix representations, while these were the same in postlingual deafness. Nodes in fronto-limbic and left temporal components were closely coupled, and nodes in the temporo-parietal component were loosely coupled, in prelingual deafness. Patterns of connected components changing in postlingual deafness were the same as hearing adults. We propose that the preserved density of auditory cortex associated with increased connectivity in prelingual deafness, and closer coupling between certain brain areas, represent distinctive reorganization of auditory and related cortices compared with hearing or postlingual deaf adults. The differential network reorganization in the prelingual deaf adults could be related to the absence of auditory speech experience.

Malleus neck-anchoring malleostapedotomy: preliminary results.

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.

Increased intracranial pressure after surgical treatment of pulsatile tinnitus from a prominent sigmoid sinus.

A 58-year-old man presented with a 2-year history of pulsatile tinnitus in his left ear. The sound was coincident with his heartbeat, and the intensity was increased with head turn to the right and decreased during a Valsalva or turning the head to the left. The tinnitus became progressively louder and interfered with his hearing and sleep. He was frustrated with intractable tinnitus, and quality of life was deteriorated. Subjective severity of the tinnitus was assessed through a Korean version of Tinnitus Handicap Inventory score (1). On physical examination, the left tympanic membrane was normal. Using a Toynbee’s tube, the tinnitus was auscultated as a low-pitched tone synchronous with his heartbeat. Pure tone audiometry revealed mild, low-frequency sensorineural hearing loss on the left side. Noncontrast temporal bone computed tomography demonstrated the dominant sigmoid sinus (SS) on the left side (Fig. 1A). The patient elected to proceed with mechanical SS compression to reduce turbulent venous flow because of incapacitating symptoms. A cortical mastoidectomy was performed to expose the prominent SS. A 1.5 2Ycm area of the prominent SS was exposed, leaving a bony bridge traversing the exposed sinus. Three pieces of cortical bone were harvested and were inserted under the bony bridge (Fig. 1B). Immediately after the surgery, the tinnitus decreased markedly. However, the patient complained of a pulsating headache. Magnetic resonance venography (MRV) of the brain demonstrated near-complete obstruction of the SS (Fig. 2A). The fundus photograph revealed mild papilledema (Fig. 3). The patient was managed with intravenous steroids for elevated intracranial pressures, and revision surgery was planned to partially decompress the obstructed SS. The 3 previously inserted pieces of bone were removed, and the return of venous flow was identified. Two of the 3 pieces of bone were reinserted (Fig. 1C), and approximately half of the cross-sectional diameter of the SS was maintained. Immediately after the surgery, the patient’s headache subsided. Follow-up pure tone audiometry indicated improvement in the lowfrequency threshold. Follow-up MRV (Fig. 2B) demonstrated resumed patency of the SS. Four months

Prognostic Value of Extension Patterns on Follow-up Magnetic Resonance Imaging in Patients With Necrotizing Otitis Externa

OBJECTIVES To analyze the clinical characteristics of necrotizing otitis externa (NOE) and to evaluate the prognosis according to the progression of disease in terms of extension patterns on follow-up magnetic resonance images. DESIGN A retrospective clinical study. SETTING Tertiary academic center. PATIENTS We reviewed medical records of 36 patients with NOE followed up by temporal bone magnetic resonance images on a regular basis from January 1, 1992, through December 31, 2008. MAIN OUTCOME MEASURES The initial compartments affected by NOE were defined as 4 categories: anterior, medial, midline, and intracranial and extracranial. The extensions of NOE were evaluated by comparison between initial and follow-up magnetic resonance images 6 months later and defined by the direction of spread from one to another compartment and/or disease progression within the same compartment. The patients were divided into 3 groups (limited, single, and multiple extension groups) on the basis of the multiplicity of extension routes. The clinical characteristics and prognostic factors were investigated, and overall survival rates were compared according to extension patterns. RESULTS Retrocondylar fat infiltration (86%) was the most common finding, followed by parapharyngeal fat infiltration (81%) and ipsilateral nasopharyngeal musculature thickening (75%). Anterior and medial extension patterns were observed in 3 (8%) and 5 (14%) patients, respectively. Eighteen patients (50%) with combined extension patterns showed a significantly lower overall survival rate than those with single and limited extension patterns (P = .01). CONCLUSION The retrocondylar fat infiltration was the earliest change in NOE, and combined extension patterns may be a poor prognostic factor in patients with NOE.