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.
Warts

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: This study aimed to analyze postoperative performance and mapping characteristics of cochlear implants (CIs) by comparing patients with autism spectrum disorder (ASD) to those without ASD, and to suggest CI mapping solutions in patients with ASD.Subjects and Method This retrospective study enrolled 10 children with ASD and hearing disabilities, who received simultaneous bilateral CI (ASD group), and 20 children with bilateral hearing disabilities, who received simultaneous bilateral CI at the same age (control group). CI performance was analyzed using speech perception tests (categorical auditory performance score and monosyllable, bisyllable, and Ling’s 6 tests) and a sound field test. The mapping characteristics focused on variables related to stimulus intensity and fine-tuning. Results: The performance of the ASD group was significantly poorer than that of the control group in all speech perception and sound field tests. At the comfortable (C) and threshold (T) levels, the ASD group scored significantly lower than the control group. The dynamic range of ASD group was significantly narrower than the control group. The ASD group had significantly lower pulse width, sensitivity, and volume than control group. Conclusion CI mapping in the ASD group showed practical limitations. To avoid overstimulation in patients with ASD, the dynamic range should be set narrow, or the C/T level should be set lower than normal. Key control factors, such as pulse width, sensitivity, and volume, should be set lower than the control group. Although lower performance from CI is generally expected in the ASD group, CI mapping in the ASD group requires a long-term approach with dedicated efforts and patience.

Background and Objectives: This study aimed to analyze postoperative performance and mapping characteristics of cochlear implants (CIs) by comparing patients with autism spectrum disorder (ASD) to those without ASD, and to suggest CI mapping solutions in patients with ASD.Subjects and Method This retrospective study enrolled 10 children with ASD and hearing disabilities, who received simultaneous bilateral CI (ASD group), and 20 children with bilateral hearing disabilities, who received simultaneous bilateral CI at the same age (control group). CI performance was analyzed using speech perception tests (categorical auditory performance score and monosyllable, bisyllable, and Ling’s 6 tests) and a sound field test. The mapping characteristics focused on variables related to stimulus intensity and fine-tuning. Results: The performance of the ASD group was significantly poorer than that of the control group in all speech perception and sound field tests. At the comfortable (C) and threshold (T) levels, the ASD group scored significantly lower than the control group. The dynamic range of ASD group was significantly narrower than the control group. The ASD group had significantly lower pulse width, sensitivity, and volume than control group. Conclusion CI mapping in the ASD group showed practical limitations. To avoid overstimulation in patients with ASD, the dynamic range should be set narrow, or the C/T level should be set lower than normal. Key control factors, such as pulse width, sensitivity, and volume, should be set lower than the control group. Although lower performance from CI is generally expected in the ASD group, CI mapping in the ASD group requires a long-term approach with dedicated efforts and patience.

Background and Objectives: This study aimed to analyze postoperative performance and mapping characteristics of cochlear implants (CIs) by comparing patients with autism spectrum disorder (ASD) to those without ASD, and to suggest CI mapping solutions in patients with ASD.Subjects and Method This retrospective study enrolled 10 children with ASD and hearing disabilities, who received simultaneous bilateral CI (ASD group), and 20 children with bilateral hearing disabilities, who received simultaneous bilateral CI at the same age (control group). CI performance was analyzed using speech perception tests (categorical auditory performance score and monosyllable, bisyllable, and Ling’s 6 tests) and a sound field test. The mapping characteristics focused on variables related to stimulus intensity and fine-tuning. Results: The performance of the ASD group was significantly poorer than that of the control group in all speech perception and sound field tests. At the comfortable (C) and threshold (T) levels, the ASD group scored significantly lower than the control group. The dynamic range of ASD group was significantly narrower than the control group. The ASD group had significantly lower pulse width, sensitivity, and volume than control group. Conclusion CI mapping in the ASD group showed practical limitations. To avoid overstimulation in patients with ASD, the dynamic range should be set narrow, or the C/T level should be set lower than normal. Key control factors, such as pulse width, sensitivity, and volume, should be set lower than the control group. Although lower performance from CI is generally expected in the ASD group, CI mapping in the ASD group requires a long-term approach with dedicated efforts and patience.