Acquired idiopathic generalized anhidrosis (AIGA) is a very rare disease, and its pathogenesis is poorly understood. We here report on a 20-year-old man presenting with a history of inability to sweat, small wheals, and occasional heat intolerance since 3 months. On provocation test, there was no sweating over the entire surface of the body, excluding the palms and axillae. His medical history was unremarkable and laboratory examination findings were all normal. There was no familial history suggestive of neuroendocrine disease. Based on these findings, we diagnosed acquired idiopathic generalized anhidrosis. To our knowledge, this is the first case of AIGA in Korean dermatologic literature. Herein, we report a rare case of AIGA.
Axilla ; Hot Temperature ; Humans ; Hypohidrosis* ; Rare Diseases ; Sweat ; Sweating ; Young Adult

Sudden sensorineural hearing loss (SSNHL) is defined as a sensorineural hearing loss of at least 30 dB in 3 consecutive speech frequencies that occurred within the previous 3 days. In most cases the cause is not identified, although various infective, vascular, and miscellaneous causes have been proposed. It has a reported incidence of 5 to 20 per 100,000 patients per year. Many treatments are used, including corticosteroids, antiviral drugs, hemodilution agents, minerals, vitamins, herbal preparations, batroxobin, carbogen, and oxygen-based treatments. Intra-tympanic dexamethasone injection into the middle ear may be useful to SSNHL patients with profound hearing loss that is intractable to medical treatment or who also have diabetes mellitus. In SSNHL, early treatment with combined modalities that include steroid injection is generally recognized as the current best practice. The spontaneous recovery rates have been reported to be between 32-70%, and although various treatment protocols have been tried, only about half of patients completely recover, usually within 2 weeks. There is much to learn about pathogenesis of sudden sensorineural hearing loss, and more clinical trials are needed to establish evidence-based management.
Adrenal Cortex Hormones ; Antiviral Agents ; Batroxobin ; Clinical Protocols ; Dexamethasone ; Diabetes Mellitus ; Ear, Middle ; Hearing Loss ; Hearing Loss, Sensorineural* ; Hearing Loss, Sudden ; Hemodilution ; Humans ; Incidence ; Minerals ; Plant Preparations ; Practice Guidelines as Topic ; Steroids ; Vitamins

No abstract available.
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Rhabdomyosarcoma is a highly aggressive malignant tumor that originates from mesenchymal cell and invades soft tissue. While the head and neck is the common site of the rhabdomyosarcoma, the parapharyngeal space is rarely reported as a primary site of rhabdomyosarcoma. Therefore, we present a case of rhabdomyosarcoma in the parapharyngeal space.
Head ; Neck ; Pharynx ; Rhabdomyosarcoma*

BACKGROUND AND OBJECTIVES: In this study, we tried to study the criteria and characteristics of patients with noise-induced hearing loss by analyzing the pure tone audiogram such as "C5 dip" or "4 kHz notch". SUBJECTS AND METHOD: Out of 553 patients who complained of tinnitus and hearing loss, medical examination by interview, physical examination, hearing test, brain MRI and survey of tinnitus were performed from January 2010 to December 2012, targeting 81 patients who underwent pure tone hearing test. We analyzed the clinical characteristics of 81 patients with the exception of systemic disease or a history of otologic disease, conductive hearing loss, and vestibular schwannomas. Patients consisted of 70 men and 11 women (34 persons on both sides, 31 persons on right side, and 16 persons on left side), with an average of 48.1+/-12.9 years. 4 kHz notch-type sensorineural hearing loss was compared with contralateral hearing. RESULTS: The audiogram of 4 kHz notch was analyzed, and the results were as follows; 0.5 kHz (11.2+/-9.9 dB HL), 1 kHz (13.2+/-9.9 dB HL), 2 kHz (19.4+/-15.4 dB HL), 3 kHz (38.1+/-18.7 dB HL), 4 kHz (49.3+/-16.2 dB HL), 8 kHz (27.2+/-16.2 dB HL). Among all frequencies of 4 kHz notch audiogram, there was no significant hearing loss in 0.5 kHz and 1 kHz, but significant hearing loss was noted in higher frequencies (paired t-test, p>0.05). The contralateral ear showed a ski-slope hearing loss in about half of the patients. CONCLUSION: Remarkable points of the 4 kHz notch audiogram analysis, known as a typical characteristic of noise-induced hearing loss, were as follows; First, there was hearing loss of about 50 dB HL at 4 kHz. Second, the hearing threshold was improved to about 30 dB HL at 8 kHz. Third, the 4 kHz-notch audiogram showed a significant hearing loss in a high-pitched tone of over 2 kHz, but otherwise there were no significant hearing loss observed in 0.5 kHz and 1 kHz. Fourth, even if there were no symptoms, contralateral ear can still show the ski-slope type of hearing loss.
Brain ; Ear ; Ear Diseases ; Female ; Hearing Loss* ; Hearing Loss, Conductive ; Hearing Loss, Noise-Induced ; Hearing Loss, Sensorineural ; Hearing Tests ; Hearing* ; Humans ; Magnetic Resonance Imaging ; Male ; Neuroma, Acoustic ; Noise* ; Physical Examination ; Tinnitus

OBJECTIVES: The aim of this study was to analyze the incidence of inner ear anomalies in patients with unilateral congenital aural atresia (CAA) combined with microtia. METHODS: We retrospectively reviewed 61 patients with unilateral CAA combined with microtia who underwent high-resolution temporal bone computed tomography (TBCT) and hearing examination. Inner ear anomalies were analyzed using TBCT and evaluated according to the Jahrsdoerfer grading system, Marx classification, and extent of inferior displacement of the mastoid tegmen. RESULTS: Inner ear anomalies were observed in 14 patients (23.0%). Lateral semicircular canal (LSCC) dysplasia was the most common inner ear anomaly, with an incidence of 16.4%. The incidence was significantly higher on the pathologic side than on the unaffected side (P=0.002). All vascular anomalies were observed in the high-riding jugular bulb, with an incidence of 24.6%. The incidence was significantly higher on the pathologic side than on the unaffected side (P<0.001). LSCC dysplasia was significantly more common in patients with a lower Jahrsdoerfer score (odds ratio, 0.66; P=0.004). CONCLUSION: The incidence of inner ear anomalies was relatively high in patients with unilateral CAA combined with microtia; LSCC dysplasia was the most common anomaly and the probability of coexistence was higher in patients with a lower Jahrsdoerfer score.
Classification ; Congenital Microtia ; Ear, Inner ; Hearing ; Humans ; Incidence ; Mastoid ; Retrospective Studies ; Semicircular Canals ; Temporal Bone

Objectives: . This study aimed to investigate the relationship between tinnitus and joint pain from representative samples of Koreans. Methods: . The demographics and the responses to a questionnaire about tinnitus and joint pain severity and mental health status of adults aged ≥50 years in the 2010–2012 Korean National Health and Nutrition Examination Survey were analyzed. Results: . Among 9,032 individuals, 26.7% reported experiencing tinnitus within the past year. Participants with tinnitus were more frequently older, hearing loss, and had lower education levels, income, and body weight. Participants with regular exercise and sleep had a lower tinnitus prevalence. The incidences of stress, depressed mood, and suicidal ideation were significantly higher in the tinnitus group and participants with joint pain. The rates of participants with tinnitus according to the number of joint pain sites (zero, one, two, and three) was 22.1%, 31.4%, 33.3%, and 44.2%, and those of participants with severely annoying tinnitus according to the number of joint pain sites (zero, one, two, and three) were 3.3%, 6.8%, 7.9%, and 10.7%, respectively. Conclusion . Tinnitus prevalence and severity were significantly related to joint pain, and both conditions were related to psychiatric distress. Thus, the authors suggest that psychiatric distress as a common risk factor for tinnitus and joint pain should be considered when deciding treatment strategies and in guiding public health policy.

Background and Objectives: To analyze mapping changes in dynamic range (DR) and neural response threshold (NRT) as prognostic factors for cochlear implant (CI). To analyze whether postoperative speech perception performance could be predicted using DR change and initial NRT. Subjects and Methods: The speech comprehension data of 33 patients with CI were retrospectively analyzed after 1, 3, 6, and 12 months of device use. All subjects were adult, postlingually hearing-impaired, and Cochlear Nucleus CI users. Speech perception performance was evaluated using aided pure tone audiometry, consonant, vowel, one-word, two-word, and sentence tests. Results: The averages of initial NRT and DR changes were 197.8±25.9 CU (104–236) and 22.2±18.4 CU (-15–79), respectively. The initial DR was 40.8±16.6 CU. The postoperative DR was 50.3±16.4 CU at 3 months, 58±12.3 CU at 6 months, and 62.9±10.4 CU at 12 months. A gradual increase of DR was observed during the first year of CI. Compared with the initial DR, significant increases in DR were observed at 3 (p<0.05), 6 (p<0.001), and 12 (p<0.001) months. Compared with initial speech performance outcomes, a significant gain in all performance outcomes was achieved at 12 months (p<0.001). Conclusions Patients with low NRT after CI surgery could initially set DR to a wider range and had better final speech perception outcomes. Conversely, patients with high NRT after CI surgery had to set up a gradual increase in DR while adjusting the T-C level, and the final speech perception outcomes were worse. DR and NRT, the main CI mapping variables, can help predict prognosis related to speech perception outcomes after CI surgery. In conclusion, the post-CI speech perception is better with a lower initial NRT, wider final DR, or younger age.

Background and Objectives: Acute low-tone hearing loss (ALHL) is gaining attention as an independent disease identity with close association with endolymphatic hydrops and early stage Meniere’s disease (MD). This study aims to compare patients of ALHL with patients exhibiting low-tone hearing loss and ear fullness without vertigo in various audio-vestibular assessments and in progression to overt MD.Subjects and Method A total of 249 patients with low-tone hearing loss with ear fullness without vertigo was enrolled in this study. Of these patients, 58 patients met criteria for ALHL, which was defined as having an average hearing loss of ≥30 dB at 125, 250, and 500 Hz and ≤20 dB at 2, 4, and 8 kHz. Demographics, electrocochleography (ECoG) abnormality, rate of hearing improvement, vestibular functions, and progression to MD were analyzed. Results: An average low-tone hearing loss of ALHL patients was 42.8 dB, which recovered to 18.9 dB following a combined treatment of diuretics and oral steroid therapy. The hearing recovery rate of this group was 87.9% and the ECoG abnormality ratio was 42.5%. Also, 15.5% of ALHL patients eventually progressed to MD. Conclusion This study described demographics and characteristics of ALHL, demonstrating a successful response to the combined treatment of diuretics and oral steroid. Also, this report demonstrated a close relationship between the degree of low-tone hearing loss and ECoG abnormality and observed the progression to MD in ALHL patients. These data can be usefully applied in clinical setting to explain clinical outcomes of ALHL.

Background and Objectives: Tinnitus, although being a common chronic disease, can be an intractable disease that causes depression and insomnia. This study aimed to analyze the results of the Tinnitus Handicap Inventory (THI), Beck Depression Inventory (BDI), and Pittsburgh Sleep Quality Index (PSQI) questionnaire surveys before and after clonazepam therapy. In addition, we analyzed the association of three pre-treatment questionnaires and evaluated whether pre-treatment factors could predict the post-treatment THI index.Subjects and Method Patients were selected from those who visited a tertiary hospital from 2019 to 2021 for the treatment of chronic tinnitus they had for more than 3 months and who were over 20 years old. Patients were excluded from the study if they were diagnosed with acute sudden hearing loss, Meniere’s disease, brain/internal auditory canal tumors, or muscular/vascular tinnitus. The questionnaire surveys of THI, BDI, PSQI were conducted before and after 3 months of clonazepam therapy (Rivotril [Roche Inc.] 0.25 or 0.5 mg). Questionnaire scores were compared using the paired t-test. Multiple regression analysis was used to determine the relationships among the three questionnaires. Results: A total of 76 patients (38 males and 38 females) with the mean age of 57.2±9.01 years was analyzed. The average hearing threshold was 30.4±20.67 dB HL on the right and 31.7±17.06 dB HL on the left. The pre-treatment THI, BDI, and PSQI scores were 44.3±23.4, 7.96±2.36, and 6.85±4.68, respectively. The relationships between the THI and BDI and the THI and PSQI were significant (p=0.0027 and p<0.0001, respectively). The pre-THI score showed no significant association with age, sex, or hearing threshold (p=0.91, 0.85, and 0.23, respectively). The post-treatment THI score was 33.6±17.1, which was significantly lower than the pre-THI scores (p<0.0001). Post-BDI and post-PSQI were 7.38±2.25 and 4.04±3.20, respectively. Post-PSQI also significantly decreased compared with pre-PSQI (p=0.0002), but post-BDI did not significantly decrease (p=0.1231). In the THI survey, Question 7 (sleep disturbance) showed decrease the most, followed by Question 25 (unstable mood). The post-treatment THI could be predicted by using the formula, 0.7673+0.6947×pre-THI+0.3572×pre-PSQI. Conclusion The appropriate/optional use of clonazepam at low doses (0.25-0.5 mg) can significantly improve chronic tinnitus and sleep quality. Tinnitus was significantly associated with the scores of THI, BDI, PSQI and the usage of Clonazepam significantly reduced the THI and PSQI scores. However, clonazepam did not affect the BDI score.