Cochlear Otosclerosis: An Undiagnosed Cause of Progressive Sensorineural Hearing Loss

Abstract

Introduction
 Otosclerosis presents as conductive or mixed hearing loss depending upon the stage of the disease. Isolated sensorineural hearing loss though known to occur has been rarely reported. Pure cochlear otosclerosis presenting as sensorineural hearing loss should be considered as a differential diagnosis in young patients presenting with progressive hearing loss. The aim of this article is to note the prevalence of cochlear otosclerosis in young patients presenting with progressive sensorineural hearing loss. 
 Materials and Methods
 Retrospective chart analysis of 19 patients who presented with progressive SNHL with unknown etiology was done.
 Results
 Otosclerosis involving the pericochlear region was diagnosed in 27 ears which was identified by high resolution CT scan of temporal bone. In four ears, in addition, there was demineralization just anterior to oval window without clinical evidence of stapes fixation.
 Conclusion
 In young patients with progressive SNHL, high resolution CT scan of temporal bone should be done to identify progressive cochlear otosclerosis. Medical management should be initiated in these patients to halt the progression of disease.

Diagnostic Assessment of Progressive Fluctuating Sensorineural Hearing Loss in Children

“…Both illustrative modalities are helpful in bacterial meningitis where neither imaging technique by itself is adequate in exhibiting anatomical modifications due to fibrosis or ossification [2]. In rare cases of children with PFSNHL, high resolution CT can manage to identify potential progressive cochlear otospongiosis [11]. Appropriate intervention and if needed amplification should be initiated in these young patients while monitoring the progression of the disorder.…”