Cochlear Otosclerosis: An Undiagnosed Cause of Progressive Sensorineural Hearing Loss

Main Article Content

Asish Kumar Lahiri
Anandita Gupta
Rakesh Vohra
Shalabh Sharma
Satinder Singh

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.

Article Details

How to Cite
1.
Lahiri AK, Gupta A, Vohra R, Sharma S, Singh S. Cochlear Otosclerosis: An Undiagnosed Cause of Progressive Sensorineural Hearing Loss. BJOHNS [Internet]. 2020Aug.31 [cited 2024Dec.28];28(2):127-3. Available from: https://bjohns.in/journal3/index.php/bjohns/article/view/329
Section
Main article

References

MN Wali. Otosclerosis. Can Fam Physician. 1987; 33: 1491-3

Guild SR. Histologic otosclerosis. Annal Otol Rhinol Laryngol. 1944; 53: 46

Declaua F, Spaendonck MV, Timmermans JP, Michaels L, Liang J, Qiu JP, et al. Prevalence of otosclerosis in an unselected series of temporal bones. Otol Neurotol. 2001; 22: 596-602

Schuknecht HF. Cochlear otoslerosis- A continuing fantasy. Arch Otolaryngol Head Neck Surg. 1979; 222: 79-84

Schuknecht HF, Kirschner JC. Cochlear otosclerosis: fact or fantasy. Laryngoscope 1974; 84: 766-82

Shambaugh G, Jr. The therapy of cochlear otosclerosis. Ann Otol Rhinol Laryngol. 1966; 75: 579-83

Linthicum FH, Balle V. Histologically proven cochlear otosclerosis with pure SNHL. Annal Otol Rhinol Laryngol. 1984; 93: 105-11

Freeman J. Progressive Sensorineural hearing loss and cochlear otosclerosis: A prospective study. Laryngoscope 1979; 89: 1487-521

Franceschini SS, Ravecca F, De Vito A, Berrettini S. Progressive sensorineural hearing loss in cochlear otosclerosis. Acta Otorhinolaryngol Ital. 1998; 18: 59-65

Beales PH. Otosclerosis – past and present. J. R. Soc. Med. 1979; 72: 553-61

Schuknecht HF, Barber W. Histologic variants in otosclerosis. Laryngoscope 1985; 95: 1307-17

Causse JR, Chevence LG. Sensorineural hearing loss due to cochlear otospongiosis: etiology. Otolaryngol Clin North Am. 1978; 11: 125-34

Parahy C, Linthicum FH Jr. Otosclerosis: relationship of spiral ligament hyalinization to sensorineural hearing loss. Laryngoscope 1983; 93: 717-20

Petrovic AG, Stuzmann JJ, Shambaugh GE Jr. Experimental studies on pathology and therapy of otospongiosis. Am J Otol. 1985; 6: 43-50

Valvassori GE. Radiologic diagnosis of cochlear otosclerosis. Laryngoscope. 1965; 75: 1563-71

Veillon F, Riehm S, Emachescu B, Haba D, Roedlich MN, Greget M, et al. Imaging of the windows of the temporal bone. Semin Ultrasound CT MR. 2001; 22: 271-80

Mafee MF, Henrikson GC, Deitch RL, Norouzi P, Kumar A, Kriz R, et al. Use of CT in stapedial otosclerosis. Radiology 1985; 156: 709-14

Guneri EA, Ceryan K, Ada E, Guneri A. High resolution computed tomographic evaluation of cochlear capsule in otosclerosis: relationship between densitometry and sensorineural hearing loss. Ann Otol Rhinol Laryngol. 1996; 105: 659-64

Youssef O, Rosen A, Chandrashekhar S, Lee HS. Cochlear otosclerosis: The current understanding. Ann Otolol Rhinol Laryngol. 1998; 107: 1076-9

Rotteveel LJ, Proops DW, Ramsden RT, Saeed SR, van Olphen AF, Mylanus EA. Cochlear implantation in 53 patients with otosclerosis: Demographics, Computed tomographic scanning, surgery and complications. Otol Neurotol. 2004; 25: 943-52

Merkus P, van Loon MC, Smit CF, Smit C, de Cock Adrianus FC, Hensen EF. Decision making in advanced otosclerosis: An evidence based strategy. Laryngoscope. 2011; 121: 1935-41

Lagleyre S, Sorrentino T, Calmels MN. Shin YJ, Escude B, Deguine O, et al. Reliability of High Resolution CT Scan in Diagnosis of Otosclerosis. Otol Neurotol. 2009; 30: 1152-9

Causse JR, Causse JB, Uriel J, Berges J, Shambaugh GE Jr, Bretlau P. Sodium fluoride therapy. Am J Otol. 1993; 14: 482-90

Kennedy DW, Hoffer ME, Holliday M. The effects of etidronate disodium on progressive hearing loss from otosclerosis. Otolaryngol Head Neck Surg. 1993; 109: 461-7

Brookler K. Medical treatment of otosclerosis: rationale for use of bisphosphonates. Int Tinnitus J. 2008; 14: 92-6

Hentschel MA, Huizinga P, van der Velden DL, Bittermann AJN, van der Heijden GJM, Grolman W. Limited evidence for the effect of sodium fluoride on deterioration of hearing loss in patients with otosclerosis: A systematic review of literature. Otol Neurotol. 2014; 35: 1052-7

Deka RC, Kacker SK, Shambaugh GE Jr. Intestinal absorption of fluoride preparations. Laryngoscope 1978; 88:1918-21

Cruise AS, Singh A, Quiney RE. Sodium fluoride in otosclerosis treatment: review. J Laryngol Otol. 2010; 124: 583-6

Bretlau P, Salomon G, Johnsen NJ. Otospongiosis and sodium fluoride. A clinical double-blind, placebo-controlled study on sodium fluoride treatment in otospongiosis. Am J Otol. 1989; 10: 20-2

Derks W, De Groot JA, Raymakers JA, Veldman JE. Fluoride therapy for cochlear otosclerosis? An audiometric and computerized tomography evaluation. Acta Otolaryngol. 2001; 121: 174-7

Forquer BD, Linthicum FH, Bennett C. Sodium fluoride: Effectiveness of treatment for cochlear otosclerosis. Am J Otol. 1986; 7: 121-5.

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