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. 2023 Apr-Jun;64(2):189-197.
doi: 10.47162/RJME.64.2.09.

Otosclerosis under the magnifying glass

Claudia Raluca Bălaşa Vîrzob  1 , Raluca Maria Cloşca, Mărioara Poenaru, Raluca Morar, Nicolae Constantin Balica, Cristian Andrei Sarău, Ioana Ioniţă, Flavia Baderca
Affiliations

Otosclerosis under the magnifying glass

Claudia Raluca Bălaşa Vîrzob et al. Rom J Morphol Embryol. 2023 Apr-Jun.

Abstract

Otosclerosis is a bone condition affecting the stapes bone within the otic capsule, and its exact cause is still unknown. It is characterized by a lack of proper remodeling of newly formed vascular and woven bone, leading to the development of abnormal osteons and the formation of sclerotic bone. Bilateral otosclerosis is seen in 80% of patients and 60% of otosclerosis patients have a family history of the condition. The etiology of this disease is still unknown, there are lots of theories to explain it. The histopathological (HP) studies of otosclerosis showed that osteoblasts, osteoclasts, vascular proliferation, fibroblasts, and histiocytes were observed in the stapes footplate. The onset of the symptoms occurs by the early third decade of life, usually it doesn't start later. In otosclerosis, the energy exerted by sound at the level of the tympanic membrane is reduced in the inner ear due to the fixation and rigidity of the ossicular chain, leading to hearing loss, especially for low frequencies. The primary clinical symptom of otosclerosis is conductive hearing loss but it is important to note that sensorineural hearing loss and mixed hearing loss can also occur as secondary symptoms of the condition. Another symptom present in patients with otosclerosis is tinnitus. The paper carried out a retrospective study of 70 patients diagnosed with otosclerosis in the Department of Otorhinolaryngology of Emergency City Hospital, Timişoara, Romania, between January 2021 to December 2022. Tissue fragments were processed at Service of Pathology by standard Hematoxylin-Eosin staining. The HP diagnosis was completed using Masson's trichrome staining, Giemsa histochemical staining, and immunohistochemical (IHC) reactions with anti-cluster of differentiation (CD)20, anti-CD3, anti-CD4, anti-CD8, anti-CD34, and anti-CD31 antibodies. The microscopic examination showed a chronic diffuse inflammatory infiltrate that consisted predominantly of mature T-lymphocytes, immunohistochemically positive for CD3, CD4 and CD8. There were also present rare CD20-positive B-lymphocytes. Among the lymphocytes, relatively numerous mast cells were identified, highlighted histochemically by the Giemsa staining. They had numerous purple-violet intracytoplasmic granules. In the connective tissue support, a relatively rich vascular network was identified, consisting of hyperemic capillaries, highlighted immunohistochemically with anti-CD31 and anti-CD34 antibodies. Bone tissues trabeculae showed extensive areas of fibrosis. The collagen fibers were highlighted by Masson's trichrome staining, being stained in green, blue, or bluish green.

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Conflict of interest statement

The authors declare that they have no conflict of interests.

Figures

Figure 1
Figure 1
Distribution of patients according to age, gender, and place of living.
Figure 2
Figure 2
Distribution of patients diagnosed with unilateral otosclerosis according to gender and place of living
Figure 3
Figure 3
Distribution of patients diagnosed with bilateral otosclerosis according to gender and place of living.
Figure 4
Figure 4
Distribution of right ear hypoacusis according to subtypes and intensity.
Figure 5
Figure 5
Distribution of left ear hypoacusis according to subtypes and intensity.
Figure 6
Figure 6
The genealogy of patient’s family. Orange symbols denote individuals with clinical features of otosclerosis.
Figure 7
Figure 7
Incomplete shaped osteons, surrounded by connective tissue with interstitial edema and lymphocytes. HE staining, ×50. HE: Hematoxylin–Eosin.
Figure 8
Figure 8
Newly-formed osteoid lined by osteoblasts and osteoprogenitor cells in a connective tissue with diffuse moderate lymphocytic infiltrate. HE staining, ×50.
Figure 9
Figure 9
Newly-formed osteoid with osseous lamella arranged in osteons, and lacune with osteocytes, surrounded by connective tissue with lymphocytes, plasma cells, macrophages, and multinucleated giant cells. HE staining, ×200
Figure 10
Figure 10
Osseous trabeculae formed intertrabecular spaces with bone marrow. Masson’s trichrome staining, ×100.
Figure 11
Figure 11
Newly-formed osteoid. Masson’s trichrome staining, ×200
Figure 12
Figure 12
Dense sclerotic bone with prominent cement lines. Masson’s trichrome staining, ×200
Figure 13
Figure 13
Diffuse mild lymphocytic infiltrate around the newly-formed osteoid. HE staining, ×100
Figure 14
Figure 14
Heavy diffuse inflammatory infiltrate and with formation of small follicles around osteoid trabeculae. HE staining, ×50
Figure 15
Figure 15
Small orthochromatic mast cells in perivascular space. Giemsa staining, ×100
Figure 16
Figure 16
Mast cells filled with orthochromatic granules in the connective tissue around osseous trabeculae. Giemsa staining, ×50.
Figure 17
Figure 17
Diffuse inflammatory infiltrate composed of many CD3-positive T-cells. Anti-CD3 antibody immunomarking, ×200. CD: Cluster of differentiation
Figure 18
Figure 18
The inflammatory infiltrate consisted of many CD4-positive T-helper cells. Anti-CD4 antibody immunomarking, ×200
Figure 19
Figure 19
Only a few cytotoxic CD8-positive T-cells were identified in the inflammatory infiltrate. Anti-CD8 antibody immunomarking, ×200
Figure 20
Figure 20
Inflammatory infiltrate also contained a small number of CD20-positive B-cells. Anti-CD20 antibody immunomarking, ×200
Figure 21
Figure 21
Rich vascular network highlighted by CD31 reaction. Anti-CD31 antibody immunomarking, ×200
Figure 22
Figure 22
Newly-formed small blood vessels surrounding osseous trabeculae. Anti-CD34 antibody immunomarking, ×200.
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