title: Cochlear neuronal loss are determined by use of a neurofilament protein antibody in cases of bilateral profound deafness.
abstract: The temporal bones of two patients with profound bilateral deafness from infancy were studied immunohistochemically, using a neurofilament protein antibody to detect the cochlear neuronal elements. One patient exhibited Mondini dysplasia of the inner ear, with the organ of Corti almost completely deteriorated. The other patient is the first reported case involving complete aplasia of the organ of Corti in all turns. In both cases, the immunohistochemical staining clearly revealed a severe reduction in the number of afferent neurons, such as dendrites, spiral ganglion cells and cochlear axons. The number of efferent spiral bundles in the osseous spiral lamina and intraganglionic portion also decreased in parallel with the reduction in the number of cochlear afferent neurons. Our results are inconsistent with previously reported cases of presbycusis and acquired deafness induced by the measles virus, in which efferent neurons were preserved while afferent neurons degenerated. The loss of both the efferent and afferent neurons might be characteristics of congenital deafness.
authors.affiliation: Department of Otolaryngology, Teikyo University School of Medicine, Tokyo. Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Iino
authors.forename: T
authors.initials: T
authors.lastname: Komiya
authors.forename: T
authors.initials: T
authors.lastname: Fujii
authors.forename: Y
authors.initials: Y
authors.lastname: Nakamoto
authors.forename: S
authors.initials: S
authors.lastname: Koyama
authors.forename: J I
authors.initials: JI
authors.lastname: Suzuki
authors.forename: M
authors.initials: M
authors.lastname: Toriyama
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000906:Antibodies; D003056:Cochlear Nerve / Q000473:pathology*; D003638:Deafness / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D016900:Neurofilament Proteins / Q000276:immunology*; D009474:Neurons / Q000473:pathology*; D013701:Temporal Bone / Q000473:pathology
chemical_list: D000906:Antibodies; D016900:Neurofilament Proteins