Otosclerosis is a progressive and dysplastic metabolic bone disease known to affect only human beings and can affect both the conductive and sensorineural pathways through changes in the middle and inner ear. It is a common cause of acquired hearing loss, characterized by resorption of perivascular bone that results in enlargement of the perivascular space, followed by deposition of immature and collagen deficient bone. These occur simultaneously and continuously within a focus.1 Under light microscopy, the active stage where bone structure is being replaced by new bone can be distinguished from the inactive stage in which the bone becomes highly mineralized and thickened. Endolymphatic hydrops, a histopathological finding related but not exclusive to Meniere's disease, refers to an increased volume of endolymph within the cochlear duct. It can be caused by trauma, infection, obstruction, or have an unknown cause. It can acutely alter hearing and balance through ruptures of Reissner’s membrane and changes in endolymphatic electrolyte concentration, producing the triad of complications (fluctuating hearing loss, vertigo and tinnitus) of Meniere's disease.1 There have been many studies assessing the relationship of these two diseases since 1955, when Cawthorne described the recurrence of vertiginous attacks in 24% of patients with otosclerosis, and it is now known that severe otosclerotic lesions involving the vestibular duct obstruct endolymphatic absorption, resulting in hydrops.2,3