Won Ho Chung

Diagnostic criteria for Menière's disease

This paper presents diagnostic criteria for Menières disease jointly formulated by the Classification Committee of the Bárány Society, The Japan Society for Equilibrium Research, the European Academy of Otology and Neurotology (EAONO), the Equilibrium Committee of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) and the Korean Balance Society. The classification includes two categories: definite Menières disease and probable Menières disease. The diagnosis of definite Menières disease is based on clinical criteria and requires the observation of an episodic vertigo syndrome associated with low- to medium-frequency sensorineural hearing loss and fluctuating aural symptoms (hearing, tinnitus and/or fullness) in the affected ear. Duration of vertigo episodes is limited to a period between 20 minutes and 12 hours. Probable Menières disease is a broader concept defined by episodic vestibular symptoms (vertigo or dizziness) associated with fluctuating aural symptoms occurring in a period from 20 minutes to 24 hours.

Inflammatory pseudotumor involving the skull base: response to steroid and radiation therapy.

OBJECTIVE: To evaluate the response to steroid and radiation therapy in inflammatory pseudotumor (IPT) involving the skull base. STUDY DESIGN AND SETTING: Eight patients with IPT of the skull base were evaluated. Six patients were diabetic. All patients underwent initial high-dose steroid therapy. Seven of 8 patients underwent subsequent low-dose steroid maintenance. Additional low-dose radiation therapy was performed in 6 patients. We reviewed the medical records and radiologic findings of the patients to evaluate their responses to the steroid and radiation therapy. RESULTS: In all patients, the initial response to steroid therapy was fair. However, 7 patients showed symptom recurrence after cessation of steroid therapy. In 5 of 6 patients, low-dose radiation therapy resulted in recurrence. On follow-up MRI, none of the patients showed complete remission of the disease. CONCLUSION: This study shows aggressive clinical behavior of IPT involving the skull base and its poor response to steroid therapy and low-dose radiation therapy. For improving control, more aggressive initial efforts including high-dose radiation therapy with or without concurrent steroid therapy might play a role. EBM rating: C-4

Inflammatory pseudotumour involving the skull base and cervical spine

Inflammatory pseudotumour (IPT) is an idiopathic condition characterized by sclerosing inflammation, which mimics a neoplastic process. IPT involving the skull base and cervical spine is distinctly rare and usually indistinguishable from aggressive neoplasms or infection. We report a case of IPT involving the skull base and cervical spine. Initially the patient complained of headache and hearing loss without other neurological dysfunction. Two cycles of oral systemic steroid therapy resulted in only partial responses. Low dose radiotherapy was followed by quadriparesis as the lesion infiltrated into the cervical vertebral bodies. Subsequently a second course of radiation was administered to the whole cervical spine. Marked improvement was observed clinically and radiologically; however, the patient gradually deteriorated and died of sepsis. Given the aggressive nature of disease and the complications related to the long-term treatment, we suggest that a more aggressive therapeutic approach is suitable in extensive IPT of the skull base.

Hearing Outcomes According to the Types of Mastoidectomy: A Comparison between Canal Wall Up and Canal Wall Down Mastoidectomy

Objectives The aim of this study was to compare the hearing outcomes between canal wall up mastoidectmy (CWUM) and canal wall down mastoidectmy (CWDM). Methods One hundred seventy one chronic suppurative otitis media (CSOM) patients were enrolled in this retrospective study. The patients who underwent the second staged ossiculoplasty at least 6 months after mastoidectomy and who had an intact, well aerated tympanic cavity as well as intact mobile stapes at the time of operation were selected from the medical record. Based on the type of mastoid surgery, the patients were categorized into two groups: the CWUM (n=38) and CWDM groups (n=133). The hearing results of the CWUM and CWDM groups were compared using the pre- and post-operative air-bone gap (ABG) at 3 months after ossiculoplasty. Results The preoperative ABG in both groups (CWUM and CWDM) were 28.4±15.6 dB and 31.8±14.5 dB, respectively (P=0.18). Both groups didnt show any significant difference (10.9 dB vs. 13.5 dB, respectively) (P=0.21) for the postoperative ABG closure. The proportion of patients with an ABG less than 20 dB was 58.6% of the CWDM patients and 68.4% of the CWUM patients (P=0.25). Conclusion The type of mastoid surgery (CWUM and CWDM) did not affect the hearing results of CSOM patients. When choosing the type of mastoidectomy procedure for CSOM surgery, the hearing outcomes are basically the same for both types of procedure.

Criterios diagnósticos de enfermedad de Menière. Documento de consenso de la Bárány Society, la Japan Society for Equilibrium Research, la European Academy of Otology and Neurotology (EAONO), la American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) y la Korean Balance Society

This paper presents diagnostic criteria for Menières disease jointly formulated by the Classification Committee of the Bárány Society, The Japan Society for Equilibrium Research, the European Academy of Otology and Neurotology (EAONO), the Equilibrium Committee of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) and the Korean Balance Society. The classification includes 2 categories: definite Menières disease and probable Menières disease. The diagnosis of definite Menières disease is based on clinical criteria and requires the observation of an episodic vertigo syndrome associated with low-to medium-frequency sensorineural hearing loss and fluctuating aural symptoms (hearing, tinnitus and/or fullness) in the affected ear. Duration of vertigo episodes is limited to a period between 20 min and 12h. Probable Menières disease is a broader concept defined by episodic vestibular symptoms (vertigo or dizziness) associated with fluctuating aural symptoms occurring in a period from 20 min to 24h.

Comparison of Endoscopic Tympanoplasty to Microscopic Tympanoplasty

Objectives This study aimed to compare the outcome of endoscopic and microscopic tympanoplasty. Methods This was a retrospective comparative study of 73 patients (35 males and 38 females) who underwent type I tympanoplasty at Samsung Medical Center from April to December 2014. The subjects were classified into two groups; endoscopic tympanoplasty (ET, n=25), microscopic tympanoplasty (MT, n=48). Demographic data, perforation size of tympanic membrane at preoperative state, pure tone audiometric results preoperatively and 3 months postoperatively, operation time, sequential postoperative pain scale (NRS-11), and graft success rate were evaluated. Results The perforation size of the tympanic membrane in ET and MT group was 25.3%±11.7% and 20.1%±11.9%, respectively (P=0.074). Mean operation time of MT (88.9±28.5 minutes) was longer than that of the ET (68.2±22.1 minutes) with a statistical significance (P=0.002). External auditory canal (EAC) width was shorter in the ET group than in the MT group (P=0.011). However, EAC widening was not necessary in the ET group and was performed in 33.3% of patients in the MT group. Graft success rate in the ET and MT group were 100% and 95.8%, respectively; the values were not significantly different (P=0.304). Pre- and postoperative audiometric results including bone and air conduction thresholds and air-bone gap were not significantly different between the groups. In all groups, the postoperative air-bone gap was significantly improved compared to the preoperative air-bone gap. Immediate postoperative pain was similar between the groups. However, pain of 1 day after surgery was significantly less in the ET group. Conclusion With endoscopic system, minimal invasive tympanoplasty can be possible with similar graft success rate and less pain.

Signal intensity change of the labyrinth in patients with surgically confirmed or radiologically diagnosed vestibular schwannoma on isotropic 3D fluid-attenuated inversion recovery MR imaging at 3 T

ObjectiveThe purpose of this study was to evaluate the signal intensity (SI) change of the labyrinth in patients with vestibular schwannoma on isotropic 3D fluid-attenuated inversion recovery (FLAIR) imaging at 3xa0T.MethodsThirty-four patients with surgically confirmed or radiologically diagnosed vestibular schwannoma were included in this study. Retrospectively, we visually and quantitatively compared the SIs of the cochlea and vestibule on the affected side with those on the unaffected side. We also investigated whether there was correlation between the SI ratios (SIRs) of the labyrinth and the audiometric findings.ResultsOn 3D FLAIR images, the SI of the cochlea and vestibule on the affected side was significantly increased in 97% (33/34) and 91% (31/34), respectively. While the SI of the cochlea was diffusely increased, that of the vestibule was only partially increased. Quantitative study also revealed statistically significantly higher SIRs of the cochlea (0.99u2009±u20090.29) and vestibule (0.73u2009±u20090.31) on the affected side, compared with the cochlea (0.47u2009±u20090.20) and vestibule (0.19u2009±u20090.10) on the unaffected side. There was no significant correlation of the SIRs of the labyrinth with the degree of hearing loss.ConclusionIn patients with vestibular schwannoma, isotropic 3D FLAIR imaging is a useful method for the evaluation of the SI changes of the labyrinth.

Clinical assessment of music perception in Korean cochlear implant listeners

Conclusion: The Korean version of the Clinical Assessment of Music Perception (K-CAMP) test is an efficient, self-administrable test for discrimination of a wide range of music perception ability in adult Korean cochlear implant (CI) users. Objectives: The aims of the study were the development of a clinically practical test of music perception for adult Korean CI lisetners and evaluation of its effectiveness. Methods: Twelve adult post-lingually deafened patients and 12 normal-hearing (NH) controls were tested with the K-CAMP test, comprising pitch direction discrimination, melody identification, and timbre identification tests. Results: Pitch discrimination, melody identification, and timbre identification in CI users showed a wide range of perceptual abilities. The mean pitch change discrimination difference limen (DL) for the base frequency of 262 Hz (middle C) was 2.7 ± 1.7 semitones: 4.4 ± 4.2 semitones for 330 Hz (E4) and 8.1 ± 3.0 semitones for 391 Hz (G4) in CI listeners. The DL widened as the base frequency increased. The melody identification test produced 21.1 ± 21.7% correct answers, and the timbre identification test recorded 25.7 ± 8.5% correct answers in CI listeners. Pitched percussion instruments (piano, guitar) were better for timbre identification. Speech performance scores had a positive correlation with the pitch discrimination DL (p <u20090.05).

Effects of Early Surgical Exploration in Suspected Barotraumatic Perilymph Fistulas

Objectives Treatment of traumatic perilymph fistula (PLF) remains controversial between surgical repair and conservative therapy. The aim of this study is to analyze the outcomes of early surgical exploration in suspected barotraumatic PLF. Methods Nine patients (10 cases) who developed sudden sensorineural hearing loss and dizziness following barotrauma and underwent surgical exploration with the clinical impression of PLF were enrolled. Types of antecedent trauma, operative findings, control of dizziness after surgery, postoperative hearing outcomes, and relations to the time interval between traumatic event and surgery were assessed retrospectively. Results All patients had sudden or progressive hearing loss and dizziness following trauma. Types of barotrauma were classified by the origin of the trauma: 4 external (car accident, slap injury) and 6 internal traumas (lifting, nasal blowing, straining). Surgical exploration was performed whenever PLF was suspected with the time interval of 2 to 47 days after the trauma. The possible evidence of PLF was found during surgery in 9 cases: a fibrous web around the oval window (n=3), fluid collection in the round window (RW; n=6) and bulging of the RW pseudomembrane (n=1). In every patient, vestibular symptoms disappeared immediately after surgery. The hearing was improved with a mean gain of 27.0±14.9 dB. When the surgical exploration was performed as early as less than 10 days after the trauma, serviceable hearing (≤40 dB) was obtained in 4 out of 7 cases (57.1%). Conclusion Sudden or progressive sensorineural hearing loss accompanied by dizziness following barotrauma should prompt consideration of PLF. Early surgical exploration is recommended to improve hearing and vestibular symptoms.

Syndromic Hearing Loss in Association with PTPN11-Related Disorder: The Experience of Cochlear Implantation in a Child with LEOPARD Syndrome.

Hearing loss (HL) is one of the most frequent clinical manifestations of patients who suffer with multi-systemic genetic disorders. HL in association with other physical stigmata is referred to as a syndromic form of HL. LEOPARD syndrome (LS) is one of the disorders with syndromic HL and it is caused by a mutation in the PTPN11 or RAF1 gene. In general, 5 year old children who undergo cochlear implantation usually show a marked change in behavior regarding sound detection within the first 6 months of implant use, but word identification may not be exhibited for at least another 6-12 months of implant use. We herein report on a 5-year-old girl with LS. Her clinical manifestations including bilateral sensorineural HL, which indicated the diagnosis of LS. We confirmed the diagnosis by identifying a disease-causing mutation in the PTPN11 gene, which was a heterozygous missense mutation Ala461Thr (c.1381G>A). She underwent cochlear implantation (CI) without complications and she is currently on regular follow-up at postoperative 1 year. This is the first reported case of CI in a patient with LS in the medical literature.