ABSTRACT
OBJECTIVE: To investigate the positive rate for the Cochlin tomo-protein (CTP: an inner ear-specific protein) detection test among patients with inner ear-related clinical manifestations and evaluate the clinical characteristics of definite perilymphatic fistula (PLF). METHODS: We have performed an ELISA-based CTP detection test using middle ear lavage (MEL) samples from 497 cases of suspected PLF enrolled from 70 clinical centers nationwide between 2014 and 2015. In addition to the CTP-positive rate, audio-vestibular symptoms were compared between CTP-positive and -negative cases. RESULTS: 8-50% of patients in category 1 (trauma, middle and inner ear disease cases), and about 20% of those in categories 2, 3 and 4 (external origin antecedent events, internal origin antecedent events, and without antecedent event, respectively) were positive for CTP. In category 1 cases, the earlier tested samples showed a higher CTP-positive rate, whereas no differences were observed in categories 2, 3 or 4. The characteristic clinical features in the earlier tested cases were nystagmus and fistula sign in CTP test-positive cases in category 1, and streaming water-like tinnitus in those in categories 2, 3 and 4. CONCLUSION: The present study clarified that CTP detection test-positive patients exist at considerable rates among patients with inner ear-related manifestations.
Subject(s)
Ear Diseases/diagnosis , Extracellular Matrix Proteins/analysis , Fistula/diagnosis , Female , Humans , MaleABSTRACT
We report here a case with unidirectional abnormalities of smooth eye movements without gaze nystagmus. Abnormalities of eye movements were confined to unidirectional (leftward) horizontal pursuit and slow phase of OKN; however, horizontal VOR (slow phase of caloric nystagmus) and saccade were normal, and vertical eye movements were also normal. No lesions were detected in the central nervous system, and any history of drug intake was denied. Although the cause of the unidirectional abnormality in eye movement of this case is still not clear, a congenital origin seems to be the most probable.
Subject(s)
Ocular Motility Disorders/physiopathology , Pursuit, Smooth , Adult , Electronystagmography , Humans , Male , Nystagmus, Physiologic/physiology , Ocular Motility Disorders/congenital , Saccades/physiologyABSTRACT
INTRODUCTION: We report three cases of hyper-IgG4 disease with synchronous or asynchronous lymphocytic infiltration onset, IgG4 positive plasma cell infiltration, and fibril formation in multiple exocrine glands and extranodal organs. IgG4-related sialadenitis attracting recent attention has yet to be clarified as a clinical entity. CASE REPORT: Case 1, a 61-year-old man, had a submandibular gland sample showing IgG4-positive plasma cell infiltration. Case 2, a 61-year-old man, was diagnosed with IgG4-related Mikulicz's disease confirmed by a sublingual gland sample. Case 3, a 57-year-old woman, had a diagnosis of IgG4-related Mikulicz's disease confirmed based on labial and sublingual gland samples. All reported oral dryness and bilateral submandibular swelling. Cases 1 and 2 recovered following Predonine administration tapered from 30 or 20 mg. DISCUSSION: IgG4-related Mikulicz's disease must be recognized as a clinical entity, together with its diagnostic criteria and treatment. Sublingual gland biopsy should be done to confirm its diagnosis following sublingual gland swelling.
