Otosclerosis: an organ-specific inflammatory disease with sensorineural hearing loss

Sziklai, István; Batta, Tamás József; Karosi, Tamás

doi:10.1007/s00405-009-0967-y

Cited by 45 publications

(40 citation statements)

References 24 publications

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“…The theory of virus etiology has been supported by many reports (4,8,(13)(14)(15)(16)(17)26). Recently, Sziklai et al showed that MV N mRNA could be detected in all of 160 otosclerotic stapes bones by using the RT-PCR method (39). Furthermore, Gantumur et al reported that MV N mRNA was detected in primary cells derived from stapes bones (8).…”

Section: Discussionmentioning

confidence: 97%

No Evidence for an Association between Persistent Measles Virus Infection and Otosclerosis among Patients with Otosclerosis in Japan

et al. 2012

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Otosclerosis, which is characterized by disordered bone remodeling, occurs exclusively in the human temporal bone. The etiology of the disease is unknown, but a popular hypothesis is that it is caused by persistent measles virus (MV) infection. Paramyxovirus-like filamentous structures were found in otosclerotic lesions of stapes footplates from patients with otosclerosis. Although MV RNAs have been detected in otosclerotic samples by using reverse transcription-PCR, no complete MV mRNA sequence has been reported, nor has infectious virus been isolated from clinical samples. Furthermore, one study failed to obtain evidence of MV infection in otosclerotic bone samples. In this study, we tested, by three different protocols, for the presence of MV in clinical samples from patients with otosclerosis in Japan. We used a highly sensitive reverse transcription-quantitative PCR method which is able to detect viral mRNA in cells infected with MV at around one infectious unit per well. We obtained no evidence of MV infection in bone samples, primary cell cultures derived from stapes bones, or MV-susceptible cell lines (Vero/ hSLAM and II-18 cells) cocultured with bone samples or primary cell cultures derived from them. Thus, our results do not support the hypothesis that persistent MV infection is involved in the pathoetiology of otosclerosis.

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Section: Discussionmentioning

confidence: 97%

No Evidence for an Association between Persistent Measles Virus Infection and Otosclerosis among Patients with Otosclerosis in Japan

et al. 2012

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“…2 They are similar to the bone resorption defects described in human otosclerosis, 8 a complex disease attributed to persistent measles virus infection as well as genetic factors. 29 Otosclerosis is morphologically characterized by proliferation of spongiotic bone with lacunae centered on capillaries and filled with osteoblasts, osteoclasts, and macrophages. 8 In MPS VII dogs, temporal bone lesions are similar and characterized by increased bone deposition (thickening), with the presence of confluent perivascular osteolytic spaces filled with osteoclasts and numerous vacuolated macrophages.…”

Section: Discussionmentioning

confidence: 99%

Histopathologic Changes of the Ear in Canine Models of Mucopolysaccharidosis Types I and VII

Hordeaux

Deniaud²,

Bemelmans

et al. 2010

Vet Pathol

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Mucopolysaccharidosis (MPS) types I and VII are inborn errors of metabolism caused by mutation of enzymes involved in glycosaminoglycan catabolism, which leads to intralysosomal accumulation of glycosaminoglycans. In children, severe forms of MPS I and VII are characterized by somatic and neurologic manifestations, including a poorly understood hearing loss. The purpose of this study is to describe the age-related histopathologic changes of the ear in spontaneous canine models of MPS I and VII. Pathologic changes in the ear were assessed in MPS I and VII dogs ranging from 1.6 to 9.3 months of age. Paraffinembedded sections of the whole ear and Epon-embedded semithin sections of the cochlea were examined. The following lesions were blindly scored in the middle and inner ear: inflammation, cells vacuolization, thickening of osseous and membranous structures, perivascular vacuolated macrophages infiltration, and bone resorption. All dogs had lysosomal storage within cells of tympanic membrane, ossicles, tympanic bone and mucosa, cochlear bone, spiral ligament, limbus, and stria vascularis. The MPS I dogs mainly had progressive cochlear lesions. The MPS VII dogs had severe and early middle ear lesions, including chronic otitis media and bone resorption. The MPS I dog only partially recapitulates the pathology seen in humans; specifically, the dog model lacks inflammatory middle ear disease. In contrast, the MPS VII dog has severe inflammatory middle ear disease similar to that reported in the human. In conclusion, the canine MPS VII model appears to be a good model to study MPS VII-related deafness.

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“…The pathologic process of otosclerosis is characterized by an inflammatory lytic phase followed by abnormal bone remodeling, which leads to sclerosis at very specific sites of predilection [Arnold and Friedmann, 1988;Sziklai et al, 2009]. Histologically, the oval window is the most frequently affected area of this process, causing partial or total fixation of the stapes footplate.…”

Section: Introductionmentioning

confidence: 99%

“…This loss in BC hearing is worse than age-related hearing loss by itself [Topsakal et al, 2006]. Proteolytic enzymes (hydrolases) from bursting lysosomes in histiocytes as well as TNF-␣ from otospongiotic microfoci have been suspected of causing sensorineural hearing loss in otosclerosis when released into the perilymph [Bretlau et al, 1982;Causse et al, 1989;Causse and Chevance, 1978;Sziklai et al, 2009]. In rare cases, sensorineural hearing loss is induced by otosclerotic foci that penetrate the cochlear endosteum to grow into the scala tympani or vestibuli [Schuknecht, 1983].…”

Section: Introductionmentioning

confidence: 99%

Influence of a Totally Open Oval Window on Bone Conduction in Otosclerosis

et al. 2010

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Objective: The aim of this study was to evaluate changes in bone conduction thresholds before, during and after total stapedectomy. Study Design: Prospective clinical study. Methods: In 27 ears of 26 patients undergoing stapedectomy under local anesthesia, bone conduction was measured before surgery, during surgery under open oval window conditions, and after the insertion of a steel wire connective tissue prosthesis. Statistical data analysis was performed on the audiometric results. Results: Under open oval window conditions, bone conduction hearing was found to be improved between 500 and 2000 Hz, but not at 4000 Hz. After insertion of the prosthesis, an additional improvement was evident at 500 and 1000 Hz, but a loss was seen at 2000 and 4000 Hz. Conclusion: This is the first investigation reported in which audiometry was performed under open oval window conditions during stapes surgery. Our results demonstrate that at least part of the preoperative bone conduction hearing loss in otosclerosis must be of mechanical, but not of sensorineural origin, as already suspected by Carhart. The fixed footplate suppresses cochlear micromechanics mainly at frequencies between 500 to 2000 Hz. Furthermore, the loss in bone conduction hearing at 2000 and 4000 Hz after insertion of the prosthesis indicates that rather than the surgical procedure of total removal of the footplate, other factors such as the handling of the prosthesis or its mechanical properties after insertion cause high-frequency hearing loss after stapes surgery.

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Otosclerosis: an organ-specific inflammatory disease with sensorineural hearing loss

Cited by 45 publications

References 24 publications

No Evidence for an Association between Persistent Measles Virus Infection and Otosclerosis among Patients with Otosclerosis in Japan

No Evidence for an Association between Persistent Measles Virus Infection and Otosclerosis among Patients with Otosclerosis in Japan

Histopathologic Changes of the Ear in Canine Models of Mucopolysaccharidosis Types I and VII

Influence of a Totally Open Oval Window on Bone Conduction in Otosclerosis

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