Otosclerosis is an otologic disease characterized by disordered resorption and deposition of the otic capsule bone. It can lead to progressive conductive, mixed or sensorineural Hearing Loss (HL). In rare cases, it manifests itself with a tendency for massive bone subsequent formation cavities (“cavitating otosclerosis”). Cavities sometimes realize communication between Cerebrospinal Fluid (CSF...

Otosclerosis is an otodystrophy of the otic capsule and is a cause of conductive, mixed or sensorineural hearing loss in the 2nd to 4th decades of life. Otosclerosis is categorised into two types, fenestral and retrofenestral. Imaging plays an important role in the diagnosis and management of otosclerosis. High-resolution CT (HRCT) of the temporal bone using 1-mm (or less) thick sections is the...

Otosclerosis is a circumscribed disease of the enchondral bone of the otic capsule. It is characterized by alternating phases of bone resorption followed by replacement with dense sclerotic bone. The first identification of this disease was made by Valsalva in 1704 in a description of the bony obliteration of the oval window in a patient with an aquired deafness [64]. The term otosclerosis was ...

OBJECTIVE To have a new macroscopic classification of otosclerosis based on appearance variations of the stapes footplate due to the disease and to determine if the color variations are correlated to a minor or major incidence of footplate complications. STUDY DESIGN Retrospective study. MATERIALS AND METHODS From January 2006 to December 2006, 106 patients affected by otosclerosis underwen...

We have chosen among many temporal bones of donors deceased individuals with concomitant otosclerosis, three particular cases, one with classic otosclerosis, another with cochlear otosclerosis with concomitant oval window ankylosis and another with cochlear otosclerosis without stapes fixation. The different histopathologic features are discussed and clinical and therapeutical guidelines are pr...

PURPOSE OF REVIEW The aim of this article is to summarize and put into historical perspective current advances in research in otosclerosis, a disorder of the human temporal bone with a hereditary predisposition that is among the most common causes of acquired hearing loss. RECENT FINDINGS Genetic studies have revealed that otosclerosis is heterogeneous, with evidence for defects in at least s...

OBJECTIVE To perform chromosomal mapping and clinical analysis of hereditary otosclerosis linked to the fourth locus for otosclerosis (OTSC4) in an Israeli family. DESIGN Pedigree study. SETTING A genetics of hearing loss research laboratory, a clinical genetics laboratory, a center for speech and hearing, and an otolaryngology department at a university and medical centers in Israel. SUB...

Otosclerosis is a bony dyscrasia characterized by histopathological findings of osteoclast production. Osteoclastogenesis explains the pathogenesis of otosclerosis. Basic science research in the experimental animal otic capsule has given insight into the process of evolution of otosclerosis. The normal otic capsule is preserved with very little bone turnover as a result of the production of ost...

Otosclerosis is a frequent cause of nonsyndromic hearing loss which affects exclusively the human temporal bone. Various etiopathogenetic hypotheses have been proposed. The major hypotheses considered are genetic factors, immunologic factors and viral infection. Since the familial incidence of otosclerosis is known a recent genetic analysis has given evidence of three otosclerosis genes (OTSC1-...

Otosclerosis is defined as an overgrowth of the bone of the otic capsule just anterior to stapes footplate in between cochlea and vestibule. In such scenario, the stapes is unable to function normally as a piston and eventually becomes completely fixated, which results in a weaker conduction [1,2]. Clinical features are characterised by hearing loss, tinnitus and vertigo, of combination of thes...