Questioning the Relationship between Cochlear Otosclerosis and Sensorineural Hearing Loss: A Quantitative Evaluation of Cochlear Structures in Cases of Otosclerosis and Review of the Literature

Nelson, Erik G.; Hinojosa, Raúl

doi:10.1097/00005537-200407000-00016

Cited by 28 publications

(22 citation statements)

References 54 publications

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“…True cochlear otosclerosis is an issue that has been widely debated (13). Nevertheless, recent studies question the relationship between cochlear otosclerosis and SNHL (14). Audiological studies have shown mostly stable BC thresholds in otosclerosis (15,16), whereas some studies have also reported the progression of BC thresholds (17,18).…”

mentioning

confidence: 99%

Audiometric Analyses Confirm a Cochlear Component, Disproportional to Age, in Stapedial Otosclerosis

Topsakal¹,

Fransén²,

Schmerber³

et al. 2006

Otology &Amp; Neurotology

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The preoperative audiological profile of otosclerosis is described. There is a significant sensorineural component in patients with otosclerosis planned for stapedotomy, which is worse than age-related hearing loss by itself. Deterioration rates of AC and BC thresholds have been reported, which can be helpful in clinical practice and might also guide the characterization of allegedly different phenotypes for familial and sporadic otosclerosis.

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confidence: 99%

Audiometric Analyses Confirm a Cochlear Component, Disproportional to Age, in Stapedial Otosclerosis

Topsakal¹,

Fransén²,

Schmerber³

et al. 2006

Otology &Amp; Neurotology

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“…1 To investigate the plausibility of this concept, we have evaluated the composition of these hyalinized soft tissues using immunostaining techniques.…”

Section: Resultsmentioning

confidence: 99%

“…Histopathologic findings and morphometric data from these cases have been reported previously. 1 The images presented in this report are from the right temporal bone of patient 1 (the fifth case in the earlier study 1 ), a woman in her 80s with a history of gradual progressive hearing loss resulting in deafness. In this case, extensive otosclerosis involved the anterior otic capsule, posterior otic capsule, and oval window, with endosteal involvement in the basal, middle, and apical turns of the cochlea.…”

Section: Methodsmentioning

confidence: 97%

“…Stapes fixation was present, and the cochlear elements were severely degenerated (Table). Patient 2 (the second case from the earlier study 1 ) was also a woman in her 80s, with a bone conduction threshold pure tone average (PTA) of 43 dB at 500, 1000, 2000 Hz and otosclerosis involving the anterior and superior oval window of the right temporal bone. She also had endosteal involvement of the basal turn.…”

Section: Methodsmentioning

confidence: 99%

“…Patient 3 (the first from the earlier study 1 ) was a woman in her 50s with a bone conduction threshold PTA of 18 dB and otosclerosis involving the anterior otic capsule of the left temporal bone. She also had extensive endosteal involvement of the second and apical cochlear turns.…”

Section: Methodsmentioning

confidence: 99%

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Analysis of the Hyalinization Reaction in Otosclerosis

Nelson

Hinojosa

2014

JAMA Otolaryngol Head Neck Surg

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O tosclerosis is a disease of abnormal bone remodeling, which is unique in that it occurs only in the otic capsule of the human temporal bone. Large foci of otosclerosis may penetrate the endosteal layer of the cochlea, resulting in direct contact of otosclerotic bone with the softtissue structures of the inner ear. We have previously reported variable amounts of cochlear element degeneration with otosclerotic endosteal penetration, 1 and in that earlier study, the cochlear elements, including the hair cells, ganglion cells, and stria vascularis, were evaluated quantitatively. A reduction in the population of cochlear elements was not related to the extent and location of otosclerotic endosteal involvement, and a reduction in sensorineural thresholds was observed in some, but not all cases. These observations suggest that factors that limit the effect of otosclerotic endosteal involvement on the cochlear elements may be present. Reports on the association between endosteal involvement of the otic capsule with otosclerosis and sensorineural hearing loss have been summarized in a review of the literature. 1As early as 1899, Siebenmann 2 speculated that the diffusion of toxic substances from otosclerotic bone into the inner ear fluids resulted in hearing loss. Evidence now supports this concept. Hydrolytic enzymes produced by otosclerotic bone have been identified in perilymph and appear to be a factor leading to the degeneration of cochlear sensorineural elements and sensorineural hearing loss. 3,4 In addition, cytokines released by otosclerotic bone have been suggested as a potential source of spiral ligament dysfunction resulting in a loss of cochlear fluid and ion homeostasis. 5Histologic changes occurring in the cochlear soft tissues at the site of otosclerotic endosteal penetration have been descriptively referred to as a hyalinization reaction. [6][7][8] In hematoxylineosin stained temporal bone sections, the hyalinized tissue appears pink in color and is characterized by decreased cellularity IMPORTANCE Otosclerotic bone has been observed to penetrate the endosteal layer of the cochlea, resulting in direct contact with the soft-tissue structures of the inner ear. Sensorineural hearing loss has been observed in some, but not all, of these cases. The development of histologic changes occurring in the cochlear soft tissues at the site of otosclerotic endosteal penetration has been descriptively referred to as a hyalinization reaction. The role of the hyalinization reaction in the development of hearing loss is unknown.OBJECTIVE To evaluate the composition of these hyalinized soft tissues using immunostaining techniques. DESIGN, SETTING, AND PARTICIPANTSRetrospective review in a human temporal bone histopathology research laboratory of 3 specimens from patients with endosteal otosclerotic involvement.INTERVENTIONS Evaluation of human temporal bone pathology findings. MAIN OUTCOMES AND MEASURESHuman temporal bone sections with endosteal otosclerotic involvement were studied using immunostaining techniques to ide...

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Lyme neuroborreliosis as a cause of sudden sensorineural hearing loss and facial palsy

Nitro

Martino

Fuccillo

et al. 2022

Clinical Case Reports

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We present a case of sudden sensorineural hearing loss and rapidly progressive facial palsy in a female patient in her 40s with no, apparently, notable past medical or surgical history. Investigations revealed a positive serology for B. burgdoferi and the MRI allowed us to identify suggestive signs of Lyme meningitis with multiple cranial nerve involvement. After diagnosis, the patient was treated with intravenous ceftriaxone with a full recovery of sensorineural deafness and facial palsy. This case report highlights the importance of collecting a complete medical history in all cases of facial palsy and sudden hearing loss while presenting an infrequent clinical presentation of early disseminated Lyme disease with neuroborreliosis.

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Questioning the Relationship between Cochlear Otosclerosis and Sensorineural Hearing Loss: A Quantitative Evaluation of Cochlear Structures in Cases of Otosclerosis and Review of the Literature

Cited by 28 publications

References 54 publications

Audiometric Analyses Confirm a Cochlear Component, Disproportional to Age, in Stapedial Otosclerosis

Audiometric Analyses Confirm a Cochlear Component, Disproportional to Age, in Stapedial Otosclerosis

Analysis of the Hyalinization Reaction in Otosclerosis

Lyme neuroborreliosis as a cause of sudden sensorineural hearing loss and facial palsy

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