A Case of Familial Otosclerosis / 대한이비인후과학회지

Otosclerosis is a primary metabolic bone disease of the otic capsule and ossicles. It is one of the causes of acquired hearing loss, with clinical manifestations occurring in approximately 1% of individuals in some populations in the Western countries. Although the cause of otosclerosis is undetermined, the disease has a well established hereditary predisposition, with approximately half of all affected individuals having family members known to be affected. Many genetic studies of otosclerosis support an autosomal dominant mode of inheritance with penetrance in the range of 20-40%. There have been a few reports of the clinically suspicious otosclerosis cases in Korea, but otosclerosis having familial forms have not been reported. We report one case of familial otosclerosis.

Clinical Characteristics of Thyroid Micropapillary Carcinoma / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Papillary microcarcinoma of the thyroid gland (PMC) is defined as a papillary thyroid cancer (PTC) measuring less than 1 cm in its diameter. PMC is frequently diagnosed today, mainly as a result of the wide use of neck ultrasonography and fine needle aspiration biopsy. Despite the overall excellent prognosis for patients with PMC, the extent of thyroid resection and the necessity of lymph node dissection have become the issues of controversy. The rising incidence of the PMC among PTC necessitates the identification of prognostic factors and standardization of treatment protocols. Therefore, to evaluate the characteristics of PMC, we reviewed our experiences. SUBJECTS AND METHOD: Between 2000 and 2005, 265 patients underwent thyroid surgery and 194 patients showed PTC. Of these patients, 53 patients (27.3%) had PMC (9 male, 44 female, mean age 50.1 years). The data from these patients were retrospectively analyzed. RESULTS: The mean tumor size was 7.19 mm. The most frequently presenting symptom was thyroid mass only (77%) and the combined thyroid diseases were found in 35 patients (66%). Twelve patients (27.9%) had multifocal microcarcinomas and in 11 patients (20.8%) had extrathyroidal invasion. Lymph node metastasis was found in 18 patients (33.9%). Lymph node metastasis was significantly associated with the presence of extrathyroidal invasion (p=0.031), lymph node metastasis (p=0.008) and extrathyroidal invasion are more frequently observed in PMCs over 5 mm in size. CONCLUSION: These clinical characteristics may be considered as prognostic factors of the PMC. So the treatment modalities should be chosen taking into consideration of the various clinical factors.

Clinical Manifestations of Headache in Meniere's Disease

BACKGROUND AND OBJECTIVES: A possible link between Meniere's disease (MD) and headache was originally suggested by Prosper Meniere. We aimed to analyze the clinical manifestation of headache in definite MD compared with benign paroxysmal positional vertigo (BPPV) as a control group. MATERIALS AND METHODS:We examined headache in 67 patients with definite MD according to the criteria of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS). Sex- and age-matched 67 patients with BPPV served as a control group. Information was obtained concerning the prevalence, localization, severity, character, sequence of headache and response to therapy. RESULTS: Altogether 60 MD patients (90%) and 47 BPPV patients (70%) was reply the questionnaire. 46 MD patients (69%) and 15 BPPV patients (22%) reported headache. Headache was moderate to severe in 39 MD patients (85%) and as a whole more severe than that of the BPPV patients (P<0.05). Temporal area was the most frequently involved region in MD group, whereas posterior neck area was most frequent in BPPV group. The sequence of headache and vertigo attack was pre-(23%), intra-(33%), after-(44%). The 39 patients (89%) of headache in MD was response to the vestibular suppressants, diuretics, calcium channel blocker. CONCLUSION: It is concluded that high incidence of headache and migraine in combination with MD seems to common pathophysiology with migraine. Therefore, our results could provide predictive value in the treatment and follow up of MD patients with headache.

Clinical Manifestations of Cerebellar Infarction Mimicking Unilateral Vestibulopathy

BACKGROUND AND OBJECTIVES: Pseudo-labyrinthine symptom without any other accompanying neurologic symptoms or signs occur with cerebellar infarction. The prognosis and management of cerebellar infarction differ from those of vertigo associated with unilateral vestibulopathy. The objective of study was to analyze the clinical symptoms of cerebellar infarction mimicking unilateral vestibulopathy according to the infarction territory. MATERIALS AND METHOD: Among 273 patients who showed pseudo-labyrinthine symptoms or signs, 28 patients were diagnosed cerebellar infarction on MRI between January 2003 and October 2006. Out of the 28 patients, 9 patients accompanying with focal neurologic symptoms or signs in early stage were excluded, and a retrospective analysis of total 19 patients was done through chart reviews. Using diffusion-weighted imaging, we divided 19 patients into two groups, AICA and PICA territory infarction. Clinincal features, outcome of audiologic and vestibular function test were compared between the groups. RESULTS: Among the 19 subjects, 10 were males and 9 were females, the mean age was 63.1+/-.0. On MRI, 5 (26.3%) subjects showed infarction in unilateral AICA territory, 14(73.7%) in unilateral PICA territory. In the patients with AICA territory infarction, audiological tests confirmed unilateral sensorineuronal hearing loss in all 5 subjects. On V-ENG, 4/5 (80%) demonstrated horizontal or mixed horizontal torsional spontaneous nystagmus, and 4/5 (80%) had a canal paresis to caloric stimulation. In several days of hospital stay, 3/5 (60%) showed new neurologic signs, facial numbness. In the patients with PICA infarction, there was no hearing change. On V-ENG, 12/14(85.7%) demonstrated horizontal or mixed horizontal torsional spontaneous nystagmus, and 7/14 (50%) had a canal paresis to caloric stimulation. In several days of hospital stay, 10/14 (71.4%) accompanied with cerebellar ataxia. CONCLUSION: Cerebellar infarction simulating unilateral vestibulopathy is more common than previously thought. Early recognition of the cerebellar infarction showing pseudo-vestibular symptom may allow specific management. Also taking into account that clinical features differ by infarction territory will assist in determining the patient's status of the disease.

A Case of Crocodile Tear Syndrome / 대한이비인후과학회지

The syndrome of crocodile tear refers to an abnormal gusto-lacrimal reflex where any gustatory stimulation produces excessive tearing as well as salivation. It is a relatively rare syndrome that has mainly been observed after a recovery from a peripheral facial nerve palsy, surgery of the acoustic neuroma or in a congenital syndrome. The most commonly accepted mechanism for the pathogenesis of the crocodile tear syndrome is that of aberrant regeneration of nerve fibers or that of transaxonal transmission. Crocodile tear syndrome may be effectively treated by intraglandular injections of botulinum toxin. Authors experienced a case of crocodile tear syndrome that occurred after the removal of acoustic neuroma; it was treated successfully by injection of botulinum toxin into the lacrimal gland. We report this case with the related literatures.

24 hr Ambulatory ECG and Schellong Test for the Diagnosis of Cardiovascular Origin Dizziness

BACKGROUND AND OBJECTIVES:Dizziness is a vague symptom of disease. Dizziness of cardiovascular origin is associated with decreased cardiac output and usually presented as light-headed sensation of an impending faint. This study aims to provide the clinician with a logical approach to identifying the cardiovascular causes of dizziness. MATERIALS AND METHOD:From February 2002 to June 2004, we sampled the 30 dizzy patients who complained light-headed sensation of an impending faint with underlying disease of arrhythmia or abnormal blood pressure. The 11 patients with arrhythmia were monitored by 24 hr ambulatory ECG monitoring and all were examined with Schellong test. RESULTS:Among the 11 patients with arrhythmia, 7 patients were diagnosed as cardiovascular origin dizziness by 24hr ambulatory ECG monitoring and 10 patients as orthostatic hypotension by Schellong test. CONCLUSION:The results of this study indicated that 24hr ambulatory ECG monitoring and Schellong test had efficacy for the diagnosis of dizzy patients who complained light-headed sensation of an impending faint with underlying disease of arrhythmia or abnormal blood pressure