• Journal of Audiology & Otology
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• v.23 no.3
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• pp.153-159
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• 2019

Background and Objectives: We aim to explore the effects of residual auditory steady state response (ASSR) on cochlear implantation (CI) outcomes in children lacking auditory brainstem responses (ABRs). Subjects and Methods: We retrospectively reviewed the data of child CI recipients lacking ABRs. All ears were divided into two groups: with residual ASSR and without ASSR. For each frequency, the T- and C-levels and the electrical dynamic ranges of postoperative 3-month and 1-year mappings were compared between the groups. To evaluate speech perception, patients who received simultaneous bilateral CIs were divided into two groups: group 1 exhibited responses at all frequencies in both ears; in group 2, at least one ear evidenced no response. The Categories of Auditory Perception (CAP) and Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) scores were compared between the groups. Results: We enrolled 16 patients. At 2 kHz, the postoperative 3-month and 1-year T-levels of patients with residual hearing were lower than those of hearing loss group (p=0.001, p=0.035). In residual hearing group, the ASSR threshold correlated positively with the postoperative 1-year T-level (p=0.012, R²=0.276) and C-level (p=0.002, R²=0.374). Of 10 simultaneous bilateral CI recipients, 5 exhibited ASSRs at all frequencies and the other 5 showed no response at ≥1 frequency. The latter had higher CAP scores at the postoperative 1-year (p=0.018). Conclusions: In children exhibiting hearing loss in ABR testing, residual hearing at 2 kHz ASSR correlated positively with the post-CI T-level. Those with ASSRs at all frequencies had significantly lower CAP scores at the postoperative 1year. CI should not be delayed when marginal residual hearing is evident in ASSR.

• Korean Journal of Audiology
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• v.23 no.3
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• pp.153-159
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• 2019

Background and Objectives: We aim to explore the effects of residual auditory steady state response (ASSR) on cochlear implantation (CI) outcomes in children lacking auditory brainstem responses (ABRs). Subjects and Methods: We retrospectively reviewed the data of child CI recipients lacking ABRs. All ears were divided into two groups: with residual ASSR and without ASSR. For each frequency, the T- and C-levels and the electrical dynamic ranges of postoperative 3-month and 1-year mappings were compared between the groups. To evaluate speech perception, patients who received simultaneous bilateral CIs were divided into two groups: group 1 exhibited responses at all frequencies in both ears; in group 2, at least one ear evidenced no response. The Categories of Auditory Perception (CAP) and Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) scores were compared between the groups. Results: We enrolled 16 patients. At 2 kHz, the postoperative 3-month and 1-year T-levels of patients with residual hearing were lower than those of hearing loss group (p=0.001, p=0.035). In residual hearing group, the ASSR threshold correlated positively with the postoperative 1-year T-level (p=0.012, R²=0.276) and C-level (p=0.002, R²=0.374). Of 10 simultaneous bilateral CI recipients, 5 exhibited ASSRs at all frequencies and the other 5 showed no response at ≥1 frequency. The latter had higher CAP scores at the postoperative 1-year (p=0.018). Conclusions: In children exhibiting hearing loss in ABR testing, residual hearing at 2 kHz ASSR correlated positively with the post-CI T-level. Those with ASSRs at all frequencies had significantly lower CAP scores at the postoperative 1year. CI should not be delayed when marginal residual hearing is evident in ASSR.

• Journal of Audiology & Otology
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• v.25 no.4
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• pp.171-177
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• 2021

Background and Objectives: The purpose of the present study was to validate the performance and diagnostic efficacy of the Korean digits-in-noise (K-DIN) test in comparison to the Korean speech perception-in-noise (K-SPIN) test, which is the representative speech-in-noise test in clinical practice. Subjects and Methods: Twenty-seven subjects (15 normal-hearing and 12 hearing-impaired listeners) participated. The recorded Korean 0-9 digits were used to form quasirandom digit triplets; 50 target digit triplets were presented at the most comfortable level of each subject while presenting speech-shaped background noise at various levels of signal-to-noise ratios (-12.5, -10, -5, or +5 dB). Subjects were then instructed to listen to both target and noise masker unilaterally and bilaterally through a headphone. K-SPIN test was also conducted using the same procedure as the K-DIN. After calculating their percent correct responses, K-DIN and K-SPIN results were compared using a Pearson-correlation test. Results: Results showed a statistically significant correlation between K-DIN and K-SPIN in all hearing conditions (left: r=0.814, p<0.001; right: r=0.788, p<0.001; bilateral: r=0.727, p<0.001). Moreover, the K-DIN test achieved better testing efficacy, shorter average listening time (5 min vs. 30 min), and easier performance of task according to participants' qualitative reports than the K-SPIN test. Conclusions: In this study, the Korean version of digit triplet test was validated in both normal-hearing and hearing-impaired listeners. The findings suggest that the K-DIN test can be used as a simple and time-efficient hearing-in-noise test in audiology clinics in Korea.

• Korean Journal of Audiology
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• v.25 no.4
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• pp.171-177
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• 2021

Background and Objectives: The purpose of the present study was to validate the performance and diagnostic efficacy of the Korean digits-in-noise (K-DIN) test in comparison to the Korean speech perception-in-noise (K-SPIN) test, which is the representative speech-in-noise test in clinical practice. Subjects and Methods: Twenty-seven subjects (15 normal-hearing and 12 hearing-impaired listeners) participated. The recorded Korean 0-9 digits were used to form quasirandom digit triplets; 50 target digit triplets were presented at the most comfortable level of each subject while presenting speech-shaped background noise at various levels of signal-to-noise ratios (-12.5, -10, -5, or +5 dB). Subjects were then instructed to listen to both target and noise masker unilaterally and bilaterally through a headphone. K-SPIN test was also conducted using the same procedure as the K-DIN. After calculating their percent correct responses, K-DIN and K-SPIN results were compared using a Pearson-correlation test. Results: Results showed a statistically significant correlation between K-DIN and K-SPIN in all hearing conditions (left: r=0.814, p<0.001; right: r=0.788, p<0.001; bilateral: r=0.727, p<0.001). Moreover, the K-DIN test achieved better testing efficacy, shorter average listening time (5 min vs. 30 min), and easier performance of task according to participants' qualitative reports than the K-SPIN test. Conclusions: In this study, the Korean version of digit triplet test was validated in both normal-hearing and hearing-impaired listeners. The findings suggest that the K-DIN test can be used as a simple and time-efficient hearing-in-noise test in audiology clinics in Korea.

• Annals of Clinical Neurophysiology
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• v.24 no.2
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• pp.84-89
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• 2022

Vestibular dysfunction has rarely been reported in MELAS syndrome. A 40-year-old male with long-term diabetes and hearing loss experienced a stroke-like episode with hemisensory disturbance and lactic acidosis. Brain MRI showed temporo-parieto-occipital cortical lesions, and a final diagnosis was made of MELAS syndrome with the mitochondrial 3243A>G mutation. Neuro-otologic evaluations revealed anterior-canal-sparing bilateral impairments of the vestibulo-ocular reflex in the video head impulse test and no caloric paresis. This unique pattern of vestibular dysfunction may aid in diagnosing MELAS syndrome.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1981.05a
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• pp.40.3-41
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• 1981

The ichthyosiform dermatosis is consisted of a heterogenous group of hereditary disorders. Authors report a case of 26 year old female patient who has congenital ichthyosiform dermatosis associated with sensorineural hearing loss, neovascularization of cornea, partial alopecia and abnormalities of nails and teeth. The audiological findings of this patient were as follows: 1. Bilateral severe sensorineural hearing loss. 2. SISI scores: 100% at 1, 000 Hz and 4, 000 Hz on both ear. 3. Tone decay test: 25 dB decay at 1, 000 Hz and 4, 000 Hz respectively on both ear. 4. SRTs : 85 dB on both ear. 5. Speech discrimination scores : 80% at 95 dB hearing level(MCL) on both ear. With above results, cochlear lesion is suspected in this syndrome.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.49 no.3
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• pp.157-162
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• 2023

Elongated styloid syndrome (ESS) can present with myriad symptoms that mimic common features of orofacial pain, such as temporomandibular joint disorders (TMJDs), often causing a challenge and delay in diagnosis. We report the case of a 52-year-old male with a three-year history of non-painful clicking during jaw movement initially diagnosed as TMJD-related internal derangement. The patient presented with a history of annoying jaw sounds for three years, described as a popping sound without bilateral clicking or crepitation. Tinnitus and progressive hearing loss were observed in the right ear, and a hearing aid was recommended by an otolaryngologist. The patient was initially diagnosed with TMJD and managed accordingly; nevertheless, his symptoms persisted. Imaging revealed prominent bilateral styloid process elongation that exceeded the recognized cut-off level of >30 mm for elongation. The patient was informed of his diagnosis and its treatment but opted only for further swallowing and auditory assessments of his ear and nose symptoms. Clinicians should consider including ESS as a differential diagnosis in patients presenting with non-specific chronic orofacial symptoms for timely diagnosis and favorable clinical outcomes.

• Journal of Korean Neurosurgical Society
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• v.49 no.6
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• pp.377-380
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• 2011

A case of intradural extramedullary cavernous angioma is presented with headache, dizziness, and bilateral sensorineural hearing loss caused by an intracranial superficial hemosiderosis. It was incidentally found in a patient with a 3-month history of sustained headache, dizziness and a 3-year history of hearing difficulty. The neurological examination was unremarkable in the lower extremity. MR images showed an intracranial superficial hemosiderosis mostly in the cerebellar region. Myelography and MR images of the thoracolumbar spine revealed an intradural extramedullary mass, which was pathologically proven to be a cavernous angioma. T12 total laminoplastic laminotomy and total tumor removal were performed without any neurologic deficits. The patient's symptoms, including headache and dizziness, have been absent for three years. Intradural extramedullary cavernous angioma can present with an intracranial superficial hemosiderosis as a result of chronic subarachnoid hemorrhage.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1976.06a
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• pp.86.2-86
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• 1976

Authors have performed the audiological analysis in accordance with age, sex, site, duration, cause, ear drums, curve pattern, and hearing level taken from 165 patients (Male; 101, Female; 64) with non-perforated ear drums who had complained hearing impairment. The results are as follows. 1. The audiographic pattern has classified into Normal (30 cases, 18.18%), Flat form (46 cases, 20.6%), Ascending form (5 cases, 3%), Mountain form (9 cases, 5.4%), Basin form (7 cases, 4.2%), Total deaf (24 cases, 14.55%) and Others (10 cases, 6%). 2. In age distribution, the highest is in 3rd decade (50 cases, 30.3%), and the next is in 2nd (43 cases, 26%), 4th (27 cases, 16.3%) in order. 3. Male (101 cases) is more than female (64 cases) giving ratio of 1.9 : 1 4. Bilateral involvement is in 58 cases and unilateral is in 77 5. 45 cases (27.3%) in duration of hearing loss is as short as 3 months and the next is overs; 28 cases (16.9%) 6 to 10 years; 21 cases (12. 7%). And in cases of total deaf, nearly all cases is over 10 years. 6. The suspected cause of the hearing loss is unknown (99 cases, 60%) and the next is following head trauma by traffic accidents in 31 cases (18.8 %) and is related to the middle ear diseased in 12. cases (7.3%). Other causes like senile, noise etc. are in few. 7. The most common findings in ear drums is normal in 177 ears (79.4%) and the next is retracted drums in 28 ears (12.6%). 8. The average hearing level is widely distributed from mild to profound hearing loss which was not related to the audigraphic pattern.

• Korean Journal of Bronchoesophagology
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• v.11 no.2 s.22
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• pp.36-39
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• 2005

Neurofibromatosis type II is an autosomal dominant, highly penetrant disease whose hallmark is bilateral vestibular schwannoma. Hearing loss is the most symptom in Neurofibromatosis type II. The patient can also present with tinnitus, disequilibrium, and headache. Cranial nerve symptoms, such as facial numbness or weakness, dysphagia, or hoarseness, can also be present. The authors experienced a case of neurofibromatosis type II having huge retropharyngeal mass, retropharyngeal abscess, and bilateral acoustic neuromas. The infection was controled with aggressive antibiotics with drainage. The huge neurilemmoma in retropharyngeal space was removed successfully via transoral approach. The authors report the case with literature review.