Hyun Woo Lim

Congenital cholesteatoma: clinical features and growth patterns

OBJECTIVE The clinical features and patterns of growth, relative to age, were examined in patients with congenital cholesteatoma to investigate disease progression and site of origin. PATIENTS AND METHODS We retrospectively reviewed 72 children younger than 15 years with intraoperatively confirmed congenital cholesteatoma diagnosed using the inclusion criteria of Levenson et al. Patient demographics, history, and otoscopic and operative findings were evaluated. Correlation between mass volumetric and operation age was analyzed in patients with closed-type masses. Parameters of disease extent of middle ear quadrant, ossicular erosion, attic involvement, invasion of the mastoid cavity, and type of mass were assessed relative to age at operation. RESULTS Mean patient age was 63.1 months (range, 20-179 months), with 51 patients (70.8%) being asymptomatic and diagnosed incidentally. Nineteen patients (26.4%) had closed-type cystic masses, and 53 (73.6%) had open-type lesions. The volume of closed-type masses was linearly correlated with age at operation. Logistic regression showed that increased age at operation was associated with a lower proportion of anterosuperior quadrant lesions, resulting in uncertainty about the site of origin. The possibility of open-type masses also increased according to age at operation. CONCLUSION Congenital cholesteatoma shows growth and extension over time. Early detection and intervention are necessary to avoid advanced disease.

A Multicenter Randomized Double-Blind Study: Comparison of the Epley, Semont, and Sham Maneuvers for the Treatment of Posterior Canal Benign Paroxysmal Positional Vertigo

We evaluated the short-term efficacy of Epley, Semont, and sham maneuvers for resolving posterior canal benign paroxysmal positional vertigo (BPPV) in a prospective multicenter randomized double-blind controlled study. Subjects were randomly divided into three groups: Epley (36 patients), Semont (32 patients), and sham (Epley maneuver for the unaffected side, 31 patients). Out of 14 institutes which participated in this study, 5 institutes had previous experience of the Epley but not the Semont maneuver and the other 9 had previous experience of both maneuvers. Each maneuver was repeated twice if there was still positional vertigo or nystagmus on day 0, and the presence of nystagmus and vertigo on positional testing were evaluated immediately, 1 day, and 1 week after treatment. After the first maneuver, the Epley group showed a significantly higher resolution rate of positional nystagmus than the Semont or sham groups (63.9, 37.5, and 38.7%, respectively). After the second maneuver, the resolution rate (83.3%) of the Epley group was significantly higher than that (51.6%) of the sham group. At 1 day and 1 week after treatment, the resolution rate of the Epley group was significantly higher than those of the other groups. Similar results were seen for the resolution of positional vertigo. The Epley maneuver showed persistent resolution rates of positional vertigo and nystagmus without a fatigue phenomenon. The Epley maneuver was significantly more effective per maneuver than Semont or sham maneuvers for the short-term treatment of posterior canal BPPV. The Semont maneuver showed a higher success rate than the sham maneuver, but it was not significantly different.

Hearing improvement after cochlear implantation in common cavity malformed cochleae: long-term follow-up results

Abstract Conclusion: We suggest that cochlear implantation (CI) should be a good therapeutic modality for hearing restoration in patients with common cavity malformed ears. Objective: To analyze hearing improvement from CI performed in common cavity malformed cochleae. Methods: A total of 11 patients (5 male and 6 female, mean age 4.5 ± 2.8 years) and 12 ears were enrolled in this study. During the insertion of electrodes, we used C-arm fluoroscopy to avoid intrameatal placement. We evaluated hearing improvement every 6 months and the mean follow-up period was 80.5 ± 24.1 months (53–125 months). Results: During the operation, there were only four cases with fully inserted electrodes. Cerebrospinal fluid gushed out in two cases during the cochleostomy and postoperative meningitis occurred in two patients. One patient had to undergo reimplantation 4 years later due to device failure and recurrent meningitis. During the 48 months follow-up hearing evaluation, the ability of hearing increased along with the age. The final average MAIS, CAP, SIR, and open set one- and two-syllable word scores were 90.3 ± 18.1%, 4.9 ± 1.6, 3.1 ± 0.9, 24.1 ± 25.9%, and 48.6 ± 38.7%, respectively.

Expression of matrix metalloproteinase 2 and 9 and tissue inhibitor of metalloproteinase 1 in nonrecurrent vs recurrent nasal polyps

BACKGROUND Matrix metalloproteinases (MMPs) 2 and 9 are a group of Zn2+-dependent endopeptidases to remodel the extracellular matrix. The association of these 2 endopeptidases with the development of morphologic changes in nasal polyps was previously described. OBJECTIVE To determine whether MMP-2, MMP-9, and tissue inhibitor of MMP-1 (TIMP-1) play a role in the recurrence of nasal polyps. METHODS To compare MMP-2, MMP-9, and TIMP-1 expression in recurrent vs nonrecurrent polyps, nasal polyps (NPs) and recurrent nasal polyps (RNPs) were obtained from 15 NP patients with chronic rhinosinusitis (CRS) undergoing endoscopic sinus surgery (ESS) and 15 RNP patients with CRS undergoing revision ESS. Fifteen specimens of inferior turbinate mucosa from patients undergoing nasal septal surgery were used as control. Through real-time polymerase chain reaction and immunohistochemistry, MMP-2, MMP-9, and TIMP-1 expressions were measured among controls, NP patients, and RNP patients. RESULTS Expression of MMP-9 messenger RNA in the NP patients was significantly higher than in the controls. In addition, expression of MMP-9 messenger RNA in the RNP patents was significantly increased compared with NP patients. With immunohistochemistry, a more increased expression of MMP-9 was observed in NP patients than controls. Expression of MMP-9 in RNP patients was also significantly high compared with NP patients, particularly in stroma. CONCLUSION Expression of MMP-9 is increased in NP patients, and it is so more in the mucosa of RNP patients. Increased expression of MMP-9 is particularly found in the stoma of RNP patients, and it may contribute to the recurrence of NP.

Recurrent Bilateral Branch Retinal Artery Occlusion with Hearing Loss and Encephalopathy: The First Case Report of Susac Syndrome in Korea

We report the first case of Susac syndrome in Koreans, in a 23-yr-old female patient who presented with sudden visual loss and associated neurological symptoms. Ophthalmic examination and fluorescein angiography showed multiple areas of branch retinal artery occlusion, which tended to recur in both eyes. Magnetic resonance imaging showed dot-like, diffusion-restricted lesions in the corpus callosum and left fornix, and audiometry showed low-frequency sensory hearing loss, compatible with Susac syndrome. She received immunosuppressive therapy with oral steroid and azathioprine. Three months later all the symptoms disappeared but obstructive vasculitis have been relapsing. This patient demonstrated the entire clinical triad of Susac syndrome, which tends to occur in young females. Although this disorder has rarely been reported in Asian populations, a high index of suspicion is warranted for early diagnosis and timely treatment.

Interaural Differences of Distortion Product Otoacoustic Emission Amplitudes in Patients with Unilateral Tinnitus

Objective We aimed to determine whether abnormalities in outer hair cell (OHC) function were related to tinnitus through interaural comparison of distortion product otoacoustic emissions (DPOAEs). Study Design Cross-sectional study. Setting Tertiary care university teaching hospital. Participants Twenty-seven patients with unilateral tinnitus and pure-tone average of both ears ≤25 dB hearing loss (HL) at 500, 1000, 2000, and 4000 Hz were included. Subjects and Methods Pure-tone thresholds observed at 500 to 16,000 Hz and DPOAE amplitudes at f2 frequencies of 1001 to 6348 Hz were compared between the tinnitus ears and nontinnitus ears in patients with unilateral tinnitus. Results The pure-tone averages (13 ± 6 dB HL) in the nontinnitus ears were similar to those (15 ± 6 dB HL) in the tinnitus ears. There were no differences in pure-tone averages at all frequencies tested. While the DPOAE amplitudes measured at f2 frequencies of 1001 to 3174 Hz in tinnitus ears were not different from those in the nontinnitus ears, the tinnitus ears showed significantly reduced DPOAE amplitudes when compared with the nontinnitus ears at frequencies of 4004 to 6348 Hz. Conclusion OHC dysfunction was correlated with tinnitus at high frequencies, and DPOAE amplitudes can provide additional information about cochlear dysfunction, which is complementary to pure-tone audiometry.

Effects of a zinc-deficient diet on hearing in CBA mice.

This study investigated the effect of a zinc-deficient diet on the hearing in CBA mice and aimed to verify whether this hearing change is reversible by supplementation of zinc afterwards. We assessed hearing through an auditory brainstem response (ABR) with tone burst stimulation in 4, 8, 16, and 32 kHz and distortion product otoacoustic emissions in 5.6, 8, 11.3, and 16 kHz every week. The ABR threshold started to increase after 4 weeks on a zinc-deficient diet. The difference in the threshold between control and zinc-deficient animals became greater over time and plateaued at about 6 weeks. The ABR threshold differences between control and zinc-deficient mice were greater at higher frequencies. Four weeks of normal diet, following 8 weeks of a zinc-deficient diet, restored the ABR threshold to normal at all measured frequencies. Zinc-deficient mice did not show any distortion product otoacoustic emission threshold change at all frequencies. This finding suggests that a zinc-deficient diet increased the ABR threshold in CBA mice and a zinc-adequate diet restored the ABR threshold to normal.

Surgical treatment of posterior semicircular canal dehiscence syndrome caused by jugular diverticulum

OBJECTIVE We report a rare case of posterior semicircular canal dehiscence caused by a jugular diverticulum, and we describe its surgical treatment using a dehiscence resurfacing manoeuvre. METHOD The clinical findings, surgical procedure and outcomes are presented. RESULTS A 66-year-old man presented with disequilibrium, sound-induced vertigo, a reduced ocular vestibular evoked myogenic potential threshold, and pressure-induced vertical and torsional nystagmus. Computed tomography revealed a right posterior semicircular canal dehiscence caused by a diverticulum of the jugular bulb. The defect in the posterior semicircular canal was localised and resurfaced with bone paté, temporalis muscle fascia and conchal cartilage, under direct visualisation. Post-operatively, the patients symptoms disappeared and his ocular vestibular evoked myogenic potential threshold normalised. CONCLUSION This case illustrates that posterior semicircular canal dehiscence can be surgically managed by resurfacing the defect site via a transmastoid approach.

Vegetable or Pulse Granuloma in the Nasal Cavity

Vegetable granuloma or pulse granuloma results from the implantation of food particles of vegetable origin. Pulse granulomas have mainly been reported in association with lung aspirations, the oral cavity with a history of oral procedures and less frequently in gastrointestinal tracks. We report a 31-year-old woman who presented with right nasal obstruction and was found to have a firm mass in the right nasal cavity. Paranasal sinus computerized tomography scans identified a calcified ring lesion in her right nasal cavity. Endoscopic sinus surgery was performed, and pathology examination revealed a lesion consistent with a pulse granuloma that contains starch granules with cellulose envelopes appearing as hyaline rings surrounded by inflammation cells and concentrically arranged delicate connective tissue. Pulse granuloma is a well described entity with distinct histopathology. However, pulse granulomas are rare, and especially extraoral pulse granulomas are extremely rare. We found that pulse granuloma can be occurred in the nasal cavity through regurgitation.

Hearing loss following ventriculoperitoneal shunt in communicating hydrocephalus patients: A pilot study

Hearing loss can be associated with a decrease in cerebrospinal fluid (CSF) pressure because changes in CSF pressure induce changes in perilymph pressure. Hearing loss after neurosurgical procedures have been reported, but clinical information on hearing loss after the placement of ventriculoperitoneal (VP) shunts, the most commonly used CSF shunt for hydrocephalus patients, is limited. This study is aimed to show the relationship between VP shunt and hearing loss.