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Pathology and pathophysiology of idiopathic sudden sensorineural hearing loss.
Otol Neurotol 2005; 26(2):151-60ON

Abstract

BACKGROUND

The cause and pathogenesis of idiopathic sudden sensorineural hearing loss remain unknown. Proposed theories include vascular occlusion, membrane breaks, and viral cochleitis.

AIMS

To describe the temporal bone histopathology in 17 ears (aged 45-94 yr) with idiopathic sudden sensorineural hearing loss in our temporal bone collection and to discuss the implications of the histopathologic findings with respect to the pathophysiology of idiopathic sudden sensorineural hearing loss.

METHODS

Standard light microscopy using hematoxylin and eosin-stained sections was used to assess the otologic abnormalities.

RESULTS

Hearing had recovered in two ears and no histologic correlates were found for the hearing loss in both ears. In the remaining 15 ears, the predominant abnormalities were as follows: 1) loss of hair cells and supporting cells of the organ of Corti (with or without atrophy of the tectorial membrane, stria vascularis, spiral limbus, and cochlear neurons) (13 ears); 2) loss of the tectorial membrane, supporting cells, and stria vascularis (1 ear); and 3) loss of cochlear neurons only (1 ear). Evidence of a possible vascular cause for the idiopathic sudden sensorineural hearing loss was observed in only one ear. No membrane breaks were observed in any ear. Only 1 of the 17 temporal bones was acquired acutely during idiopathic sudden sensorineural hearing loss, and this ear did not demonstrate any leukocytic invasion, hypervascularity, or hemorrhage within the labyrinth, as might be expected with a viral cochleitis.

DISCUSSION

The temporal bone findings do not support the concept of membrane breaks, perilymphatic fistulae, or vascular occlusion as common causes for idiopathic sudden sensorineural hearing loss. The finding in our one case acquired acutely during idiopathic sudden sensorineural hearing loss as well as other clinical and experimental observations do not strongly support the theory of viral cochleitis.

CONCLUSION

We put forth the hypothesis that idiopathic sudden sensorineural hearing loss may be the result of pathologic activation of cellular stress pathways involving nuclear factor-kappaB within the cochlea.

Authors+Show Affiliations

Otopathology Laboratory, Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts, USA. Saumil_merchant@meei.harvard.eduNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

15793397

Citation

Merchant, Saumil N., et al. "Pathology and Pathophysiology of Idiopathic Sudden Sensorineural Hearing Loss." Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, vol. 26, no. 2, 2005, pp. 151-60.
Merchant SN, Adams JC, Nadol JB. Pathology and pathophysiology of idiopathic sudden sensorineural hearing loss. Otol Neurotol. 2005;26(2):151-60.
Merchant, S. N., Adams, J. C., & Nadol, J. B. (2005). Pathology and pathophysiology of idiopathic sudden sensorineural hearing loss. Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, 26(2), pp. 151-60.
Merchant SN, Adams JC, Nadol JB. Pathology and Pathophysiology of Idiopathic Sudden Sensorineural Hearing Loss. Otol Neurotol. 2005;26(2):151-60. PubMed PMID: 15793397.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Pathology and pathophysiology of idiopathic sudden sensorineural hearing loss. AU - Merchant,Saumil N, AU - Adams,Joe C, AU - Nadol,Joseph B,Jr PY - 2005/3/29/pubmed PY - 2005/9/13/medline PY - 2005/3/29/entrez SP - 151 EP - 60 JF - Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology JO - Otol. Neurotol. VL - 26 IS - 2 N2 - BACKGROUND: The cause and pathogenesis of idiopathic sudden sensorineural hearing loss remain unknown. Proposed theories include vascular occlusion, membrane breaks, and viral cochleitis. AIMS: To describe the temporal bone histopathology in 17 ears (aged 45-94 yr) with idiopathic sudden sensorineural hearing loss in our temporal bone collection and to discuss the implications of the histopathologic findings with respect to the pathophysiology of idiopathic sudden sensorineural hearing loss. METHODS: Standard light microscopy using hematoxylin and eosin-stained sections was used to assess the otologic abnormalities. RESULTS: Hearing had recovered in two ears and no histologic correlates were found for the hearing loss in both ears. In the remaining 15 ears, the predominant abnormalities were as follows: 1) loss of hair cells and supporting cells of the organ of Corti (with or without atrophy of the tectorial membrane, stria vascularis, spiral limbus, and cochlear neurons) (13 ears); 2) loss of the tectorial membrane, supporting cells, and stria vascularis (1 ear); and 3) loss of cochlear neurons only (1 ear). Evidence of a possible vascular cause for the idiopathic sudden sensorineural hearing loss was observed in only one ear. No membrane breaks were observed in any ear. Only 1 of the 17 temporal bones was acquired acutely during idiopathic sudden sensorineural hearing loss, and this ear did not demonstrate any leukocytic invasion, hypervascularity, or hemorrhage within the labyrinth, as might be expected with a viral cochleitis. DISCUSSION: The temporal bone findings do not support the concept of membrane breaks, perilymphatic fistulae, or vascular occlusion as common causes for idiopathic sudden sensorineural hearing loss. The finding in our one case acquired acutely during idiopathic sudden sensorineural hearing loss as well as other clinical and experimental observations do not strongly support the theory of viral cochleitis. CONCLUSION: We put forth the hypothesis that idiopathic sudden sensorineural hearing loss may be the result of pathologic activation of cellular stress pathways involving nuclear factor-kappaB within the cochlea. SN - 1531-7129 UR - https://www.unboundmedicine.com/medline/citation/15793397/Pathology_and_pathophysiology_of_idiopathic_sudden_sensorineural_hearing_loss L2 - http://Insights.ovid.com/pubmed?pmid=15793397 DB - PRIME DP - Unbound Medicine ER -