pituitrin and Deafness

The etiology of Ménière's disease (MD) is still not completely clear, but it is believed to be associated with endolymphatic hydrops (EH), which is characterized by auditory functional disorders. Vasopressin injection in C57BL/6J mice can induce EH and serve as a model for MD. Optical Coherence Tomography (OCT) has shown its advantages as a non-invasive imaging method for observing EH.. C57BL/6J mice received 50 μg/100g/day vasopressin injections to induce EH. Auditory function was assessed using auditory brainstem response (ABR) and distortion product otoacoustic emissions (DPOAE). OCT was used to visualize the cochlea.. OCT observed accumulation of fluid within the scala media in the cochlear apex. ABR showed significant hearing loss after 4 weeks. DPOAE revealed low-frequency hearing loss at 2 weeks and widespread damage across frequencies at 4 weeks.. The development of hearing loss in mouse models of MD is consistent with EH manifestations.

Topics: Animals; Deafness; Endolymphatic Hydrops; Hearing Loss; Magnetic Resonance Imaging; Meniere Disease; Mice; Mice, Inbred C57BL; Tomography, Optical Coherence; Vasopressins

The p-ADH level in cases of juvenile unilateral profound deafness (JUPD) and the timecourse of the level were examined to investigate whether or not an increase of p-ADH is involved in the development of delayed endolymphatic hydrops (DEH) in JUPD.. In 90 consecutive patients with unilateral profound or total sensorineural deafness with the onset in early childhood, pure-tone audiometric examination and the measurement of p-ADH and plasma osmolality (p-OSM) were followed up once or twice a year as far as possible. At every testing, we performed careful history-taking about episodic vertigo/dizziness, fluctuant hearing loss, and tinnitus in order to find out whether patients had experienced these clinical signs of the development of DEH.. Means and standard deviation (S.D.) of p-ADH level and osmolality in all samples tested (n=368) were 7.3+/-7.0 pg/mL (0.7-52.0 pg/mL), and 288.6+/-4.4 mOsm/L (273-306 mOsm/L), respectively. The mean of p-ADH level was much higher than those previously reported in children and adolescents. High levels of p-ADH (over 5.0 pg/mL) were often observed in subjects between 6 and 19 years of age, but not so frequently in subjects of 20 years of age or older. Long-term follow-up of p-ADH levels revealed that DEH frequently developed in cases with persistent elevation of p-ADH.. The elevation of p-ADH is likely to promote the development of DEH in cases of JUPD, although the underlying mechanism remains to be elucidated.

Topics: Adolescent; Adult; Age Factors; Aged; Audiometry, Pure-Tone; Child; Child, Preschool; Deafness; Endolymphatic Hydrops; Female; Humans; Male; Meniere Disease; Middle Aged; Neurophysins; Osmolar Concentration; Protein Precursors; Reference Values; Vasopressins

Plasma antidiuretic hormone (p-ADH) concentrations were determined with a radioimmunoassay, using a reversed-phase C18 silica column, in 300 patients with vertigo, dizziness and/or deafness; 119 of them had a diagnosis of Menière's disease. The p-ADH level was significantly elevated in patients with Meniere's disease and others with endolymphatic hydrops, e.g. cochlear Menière's disease or delayed hydrops. By contrast, the p-ADH level was not so high in cases without the endolymphatic hydrops. The increase in the p-ADH level was closely linked to vertigo attacks, the glycerol test results and an enhanced negative summating potential (-SP) in electrocochleogram (ECochG). These results lead to the assumption that disorders of ADH-dependent hormonal control in the inner ear may constitute the possible mechanism underlying vertiginous attacks and deafness in patients with endolymphatic hydrops.

Topics: Cochlea; Deafness; Dizziness; Edema; Electrophysiology; Endolymphatic Hydrops; Glycerol; Heating; Humans; Meniere Disease; Vasopressins; Vertigo

Three children with diabetes insipidus, diabetes mellitus, optic atrophy, and high-tone deafness were shown to lack vasopressin, indicative of degeneration of the cells of the hypothalamic supraoptic nuclei. The syndrome being due to a single gene defect, inherited as an autosomal recessive, is therefore likely to be the result of an inborn error of metabolism with variable periods of latency in those affected.

Topics: Adolescent; Child; Deafness; Diabetes Insipidus; Diabetes Mellitus, Type 1; Humans; Male; Optic Atrophy; Syndrome; Vasopressins

A few rare syndromes have been delineated in which diabetes mellitus is inherited in association with other conditions. This paper describes five patients, including four siblings in one family, who have diabetes insipidus, diabetes mellitus, optic atrophy and deafness (the DIDMOAD syndrome). The parents of both families are normal but are first cousins. All the patients have insulin-dependent diabetes mellitus with a typical juvenile-onset. The onset of diabetes insipidus was insidious and the symptoms could easily have been ascribed to poor control of diabetes mellitus. The importance of diagnosing diabetes insipidus is that all these patients had dilatation of the urinary tract varying from mild hydroureter to severe hydronephrosis and this improved with treatment of the diabetes insipidus. It is suggested that patients with diabetes mellitus and optic atrophy should have regular screening tests for diabetes insipidus since it is likely that they represent cases of the full syndrome with incomplete clinical expression. The occurrence of this rare syndrome in four siblings of unaffected parents indicates that the syndrome is due to a recessive gene, but the pathogenesis is unknown.

Topics: Adolescent; Adult; Deafness; Diabetes Insipidus; Diabetes Mellitus; Female; Genes, Recessive; Humans; Hydronephrosis; Male; Optic Atrophy; Osmolar Concentration; Pedigree; Syndrome; Urinary Bladder; Urine; Vasopressins; Water Deprivation