Otosclerosis.
1. Definition: Otosclerosis (otospongiosis) is a primary localized bone dyscrasia of the otic capsule characterized by:
- Replacement of normal dense endochondral bone with vascular spongy bone (otospongiosis) → later sclerotic bone
- Most commonly involves the fissula ante fenestram → stapes footplate fixation → conductive hearing loss
- May also affect the cochlea → sensorineural component (cochlear otosclerosis)
Etiopathogenesis
- Genetic Predisposition: It is a familial disorder in about 50% of cases, autosomal dominant inheritance pattern with variable expression. Several linked loci: OTSC1–OTSC10
- Viral Infection: Measles virus may act as a trigger in genetically predisposed individuals.
- Hormonal Factors: more common and often progresses faster in women, often worsening during pregnancy.
- Race: More common in Caucasians than in other ethnic groups.
- Autoimmune: Evidence of immune-mediated bone remodelling abnormalities
- Bone remodeling disorder: Abnormal osteoclast and osteoblast activity
Pathogenesis
- 1. Spongiosis (Active Phase): Areas of abnormal bone resorption create a vascular, spongy, soft, demineralized bone.
- 2. Sclerosis (Inactive Phase): The spongy bone is replaced by dense, mature, but disorganized sclerotic bone.
When the focus of otosclerosis involves the stapes footplate, it restricts the normal piston-like movement required for sound transmission, leading to a Conductive Hearing Loss (CHL). If the focus spreads to the cochlea (cochlear otosclerosis), it can also cause Sensorineural Hearing Loss (SNHL) due to damage or biochemical changes within the inner ear.