Spiral ganglion degeneration patterns in endolymphatic hydrops. 2008

Paul J Bixenstine, and Mauricio P Maniglia, and Amit Vasanji, and Kumar N Alagramam, and Cliff A Megerian
Department of Otolaryngology-Head and Neck Surgery, Case Western Reserve University, University Hospitals of Cleveland, Cleveland, Ohio 44106, USA.

OBJECTIVE The mechanistic association between endolymphatic hydrops (ELH) and hearing loss (HL) is unclear. Although ELH severity has been shown to correlate in some studies with HL, injury of vital structures, including hair cells and the cochlear nerve, have failed to demonstrate correlation with ELH severity. The goal of this study is to evaluate the hypothesis that spiral ganglion cell degeneration is the principle pathologic site of ELH-related cochlear injury, correlates with ELH severity, and is most profound in the apical region. METHODS Surgical induction of ELH in the guinea pig model was followed by histologic confirmation of ELH and subsequent correlation with segmental spiral ganglion cell densities. METHODS Guinea pigs (N = 14) were subjected to unilateral ELH induction and killed after 4 to 6 months. ELH severity and spiral ganglion densities were obtained using computer-aided morphometric analysis. Densities were normalized by calculating a spiral ganglion degeneration index (DI) for each animal. RESULTS The apical spiral ganglion demonstrated significantly greater degeneration than that noted in the basal spiral ganglion (DI: 1.93 vs. 1.13; P = .004). The degree of spiral ganglion degeneration in the apex correlates well with a total hydrops index (P = .006) and an apical hydrops index (P = .003). Basal spiral ganglion degeneration however, does not correlate well with hydrops severity (total hydrops index: P > .05; basilar hydrops index: P > .05). CONCLUSIONS ELH-related pathology appears to focus initially on the apical spiral ganglion and the degree of deterioration correlates well with the severity of ELH. These findings mirror some reports in the human condition, and imply that the mechanism of cochlear injury in ELH and secondary dysfunction appears to be a neural toxicity that begins in the apex of the cochlea.

UI MeSH Term Description Entries
D008575 Meniere Disease A disease of the inner ear (LABYRINTH) that is characterized by fluctuating SENSORINEURAL HEARING LOSS; TINNITUS; episodic VERTIGO; and aural fullness. It is the most common form of endolymphatic hydrops. Meniere's Disease,Meniere's Syndrome,Vertigo, Aural,Auditory Vertigo,Aural Vertigo,Ménière Disease,Ménière's Disease,Ménière's Vertigo,Otogenic Vertigo,Auditory Vertigos,Disease, Meniere,Disease, Meniere's,Disease, Ménière,Disease, Ménière's,Diseases, Ménière,Diseases, Ménière's,Meniere Syndrome,Menieres Disease,Menieres Syndrome,Ménière Diseases,Ménière Vertigo,Ménière's Diseases,Ménière's Vertigos,Ménières Disease,Ménières Vertigo,Otogenic Vertigos,Syndrome, Meniere's,Vertigo, Auditory,Vertigo, Ménière's,Vertigo, Otogenic,Vertigos, Auditory,Vertigos, Ménière's,Vertigos, Otogenic
D009410 Nerve Degeneration Loss of functional activity and trophic degeneration of nerve axons and their terminal arborizations following the destruction of their cells of origin or interruption of their continuity with these cells. The pathology is characteristic of neurodegenerative diseases. Often the process of nerve degeneration is studied in research on neuroanatomical localization and correlation of the neurophysiology of neural pathways. Neuron Degeneration,Degeneration, Nerve,Degeneration, Neuron,Degenerations, Nerve,Degenerations, Neuron,Nerve Degenerations,Neuron Degenerations
D009474 Neurons The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the NERVOUS SYSTEM. Nerve Cells,Cell, Nerve,Cells, Nerve,Nerve Cell,Neuron
D003053 Cochlear Duct A spiral tube that is firmly suspended in the bony shell-shaped part of the cochlea. This ENDOLYMPH-filled cochlear duct begins at the vestibule and makes 2.5 turns around a core of spongy bone (the modiolus) thus dividing the PERILYMPH-filled spiral canal into two channels, the SCALA VESTIBULI and the SCALA TYMPANI. Ductus Cochlearis,Scala Media,Cochlear Ducts,Cochlearis, Ductus,Duct, Cochlear,Ducts, Cochlear,Media, Scala,Medias, Scala,Scala Medias
D003056 Cochlear Nerve The cochlear part of the 8th cranial nerve (VESTIBULOCOCHLEAR NERVE). The cochlear nerve fibers originate from neurons of the SPIRAL GANGLION and project peripherally to cochlear hair cells and centrally to the cochlear nuclei (COCHLEAR NUCLEUS) of the BRAIN STEM. They mediate the sense of hearing. Acoustic Nerve,Auditory Nerve,Acoustic Nerves,Auditory Nerves,Cochlear Nerves,Nerve, Acoustic,Nerve, Auditory,Nerve, Cochlear,Nerves, Acoustic,Nerves, Auditory,Nerves, Cochlear
D004195 Disease Models, Animal Naturally-occurring or experimentally-induced animal diseases with pathological processes analogous to human diseases. Animal Disease Model,Animal Disease Models,Disease Model, Animal
D005260 Female Females
D006168 Guinea Pigs A common name used for the genus Cavia. The most common species is Cavia porcellus which is the domesticated guinea pig used for pets and biomedical research. Cavia,Cavia porcellus,Guinea Pig,Pig, Guinea,Pigs, Guinea
D006319 Hearing Loss, Sensorineural Hearing loss resulting from damage to the COCHLEA and the sensorineural elements which lie internally beyond the oval and round windows. These elements include the AUDITORY NERVE and its connections in the BRAINSTEM. Deafness Neurosensory,Deafness, Neurosensory,Deafness, Sensoryneural,Neurosensory Deafness,Sensorineural Hearing Loss,Sensoryneural Deafness,Cochlear Hearing Loss,Hearing Loss, Cochlear,Deafnesses, Neurosensory,Deafnesses, Sensoryneural,Neurosensory Deafnesses,Sensoryneural Deafness,Sensoryneural Deafnesses
D000818 Animals Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA. Animal,Metazoa,Animalia

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