Otospongioosi

Otospongioosi is a rare, inherited bone disorder that affects the otic capsule, the dense bone surrounding the inner ear. It is characterized by abnormal bone development within this region, leading to progressive hearing loss. The condition is typically bilateral, meaning it affects both ears, and is most commonly caused by mutations in the OPG gene, which plays a role in bone remodeling.

The primary symptom of otospongioosi is conductive hearing loss, which occurs when sound waves are blocked from reaching the inner ear. This is often accompanied by a characteristic symptom called conductive or sensorineural hearing loss, which describes a mixed pattern of hearing impairment. Tinnitus, a ringing or buzzing in the ears, is also a common complaint. In some cases, dizziness or vertigo may occur.

Diagnosis of otospongioosi usually involves a combination of audiological testing, such as audiometry and tympanometry, and imaging studies, like computed tomography (CT) scans of the temporal bone. CT scans can reveal the characteristic areas of abnormal bone growth within the otic capsule. Genetic testing may also be performed to identify mutations in the OPG gene.

There is no cure for otospongioosi, but treatment focuses on managing hearing loss and improving quality of life. Hearing aids are often effective in restoring hearing to a functional level. In some instances, surgical interventions, such as stapedectomy, may be considered to improve sound transmission. However, the success of surgery can be variable due to the widespread nature of the bone involvement. Regular monitoring by an otolaryngologist is important to track disease progression and adjust treatment as needed.