Human Cochlear Histopathology Reflects Clinical Signatures of Primary Neural Degeneration

Abstract Auditory neuropathy is a significant and understudied cause of human hearing loss, diagnosed in patients who demonstrate abnormal function of the cochlear nerve despite typical function of sensory cells. Because the human inner ear cannot be visualized during life, histopathological analysi...

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Autores principales:	Jessica E. Sagers, Lukas D. Landegger, Steven Worthington, Joseph B. Nadol, Konstantina M. Stankovic
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2017
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/8b86ca2222324a1083758744abd7e600
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spelling	oai:doaj.org-article:8b86ca2222324a1083758744abd7e6002021-12-02T12:32:54ZHuman Cochlear Histopathology Reflects Clinical Signatures of Primary Neural Degeneration10.1038/s41598-017-04899-92045-2322https://doaj.org/article/8b86ca2222324a1083758744abd7e6002017-07-01T00:00:00Zhttps://doi.org/10.1038/s41598-017-04899-9https://doaj.org/toc/2045-2322Abstract Auditory neuropathy is a significant and understudied cause of human hearing loss, diagnosed in patients who demonstrate abnormal function of the cochlear nerve despite typical function of sensory cells. Because the human inner ear cannot be visualized during life, histopathological analysis of autopsy specimens is critical to understanding the cellular mechanisms underlying this pathology. Here we present statistical models of severe primary neuronal degeneration and its relationship to pure tone audiometric thresholds and word recognition scores in comparison to age-matched control patients, spanning every decade of life. Analysis of 30 ears from 23 patients shows that severe neuronal loss correlates with elevated audiometric thresholds and poor word recognition. For each ten percent increase in total neuronal loss, average thresholds across patients at each audiometric test frequency increase by 6.0 dB hearing level (HL). As neuronal loss increases, threshold elevation proceeds more rapidly in low audiometric test frequencies than in high frequencies. Pure tone average closely agrees with word recognition scores in the case of severe neural pathology. Histopathologic study of the human inner ear continues to emphasize the need for non- or minimally invasive clinical tools capable of establishing cellular-level diagnoses.Jessica E. SagersLukas D. LandeggerSteven WorthingtonJoseph B. NadolKonstantina M. StankovicNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 7, Iss 1, Pp 1-10 (2017)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Jessica E. Sagers Lukas D. Landegger Steven Worthington Joseph B. Nadol Konstantina M. Stankovic Human Cochlear Histopathology Reflects Clinical Signatures of Primary Neural Degeneration
description	Abstract Auditory neuropathy is a significant and understudied cause of human hearing loss, diagnosed in patients who demonstrate abnormal function of the cochlear nerve despite typical function of sensory cells. Because the human inner ear cannot be visualized during life, histopathological analysis of autopsy specimens is critical to understanding the cellular mechanisms underlying this pathology. Here we present statistical models of severe primary neuronal degeneration and its relationship to pure tone audiometric thresholds and word recognition scores in comparison to age-matched control patients, spanning every decade of life. Analysis of 30 ears from 23 patients shows that severe neuronal loss correlates with elevated audiometric thresholds and poor word recognition. For each ten percent increase in total neuronal loss, average thresholds across patients at each audiometric test frequency increase by 6.0 dB hearing level (HL). As neuronal loss increases, threshold elevation proceeds more rapidly in low audiometric test frequencies than in high frequencies. Pure tone average closely agrees with word recognition scores in the case of severe neural pathology. Histopathologic study of the human inner ear continues to emphasize the need for non- or minimally invasive clinical tools capable of establishing cellular-level diagnoses.
format	article
author	Jessica E. Sagers Lukas D. Landegger Steven Worthington Joseph B. Nadol Konstantina M. Stankovic
author_facet	Jessica E. Sagers Lukas D. Landegger Steven Worthington Joseph B. Nadol Konstantina M. Stankovic
author_sort	Jessica E. Sagers
title	Human Cochlear Histopathology Reflects Clinical Signatures of Primary Neural Degeneration
title_short	Human Cochlear Histopathology Reflects Clinical Signatures of Primary Neural Degeneration
title_full	Human Cochlear Histopathology Reflects Clinical Signatures of Primary Neural Degeneration
title_fullStr	Human Cochlear Histopathology Reflects Clinical Signatures of Primary Neural Degeneration
title_full_unstemmed	Human Cochlear Histopathology Reflects Clinical Signatures of Primary Neural Degeneration
title_sort	human cochlear histopathology reflects clinical signatures of primary neural degeneration
publisher	Nature Portfolio
publishDate	2017
url	https://doaj.org/article/8b86ca2222324a1083758744abd7e600
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_version_	1718393931895406592

Human Cochlear Histopathology Reflects Clinical Signatures of Primary Neural Degeneration

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