Malignant external otitis.
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Biomedical subjects
Publications and source records attributed to F H Linthicum.
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PURPOSE: Although there have been numerous studies of autonomic innervation of the middle ear mucosa, and the mechanism of effusion into the middle ear cavity in animals, the autonomic innervation of the human middle ear has not received much attention. The purpose of this study is to show the presence of catecholaminergic nerve fibers in the human middle-ear mucus membrane that may play an important role in the pathogenesis of middle-ear effusion. MATERIALS AND METHODS: A total of 126 celloidin-embedded temporal bone sections from the temporal bone bank at the House Ear Institute were used for immunohistochemical study. A polyclonal antibody to tyrosine hydroxylase enzyme was used to show the presence of catecholaminergic nerve fibers. RESULTS: Tyrosine hydroxylase immunoreactive nerve fibers containing numerous fine varicosities along their course, characteristic of noradrenergic neurons, were observed throughout the middle-ear mucosa including the promontary, sinus tympani, mesotympanum, and hypotympanum. In addition, these nerve fibers were seen in close promixity to the small-caliber blood vessels. A striking variation in the intensity of staining as well as in the amount of nerve fibers was observed among the temporal bone sections. CONCLUSION: It is possible that the catecholaminergic nerve fibers, like elsewhere in the body, may exert a direct influence on the middle-ear mucosal blood vessels. We speculate that the effusion into the middle-ear space is an active, rather than a passive process. It is conceivable that cholinergic-sympathetic nerves might exist in the human middle-ear mucus membrane, and that these autonomic nerves, in conjunction with the neuropeptides, may play an active role in the pathogenesis of human middle-ear effusion.
Cochleosaccular dysplasia or degeneration (Scheibe degeneration) is considered the most common cause of profound congenital hearing impairment, and accounts for approximately 70% of cases 2 with hereditary deafness. A five-generation family with hereditary hearing impairment associated with cochleosaccular degeneration has recently been identified. The diagnosis of classical Scheibe degeneration was based on histopathological findings in the temporal bones of the proband, a 61-year-old profoundly deaf male. Auditory structures in the brainstem of the proband were also studied. Twenty-two members of the family were contacted for surveys and blood samples. Of these, 6 males and 2 females have hearing impairment. Complete audiological evaluation was done on 12 family members, and prior audiologic records of the proband and affected family members were available for study. Affected family members suffer a mild bilateral high-frequency hearing loss during childhood and adolescence, and progress to moderate-to-profound deafness in the second and third decades of life. The family is suitable for linkage analysis and does not map to previously reported loci harboring autosomal dominant, nonsyndromic hereditary hearing impairment genes. The genetic study of this family will be helpful in identifying the genes which, when mutated, result in Scheibe degeneration.
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