Cochlear implants can neither cure nor stop otosclerosis - but they can help!

In what is known as otosclerosis, a swarm-like mass, known as fibrosis, forms - usually at the transition from the auditory ossicles to the inner ear - which spreads from there and stiffens in osteogenesis. As a result, those affected lose their hearing, including functional or complete deafness.

Surgical replacement of affected auditory ossicles with a so-called stapesplasty can often fully or partially restore hearing, while conventional hearing aids can also help in the initial stages. However, as soon as the hearing loss in the respective ear reaches the criteria for a cochlear implant, or CI for short, this becomes the appropriate choice of treatment.

People with otosclerosis have good hearing success with CI!^{1 2}
As soon as the extent of the hearing loss makes a cochlear implant sensible, people with otosclerosis are more satisfied with it than with a stapes reconstruction - this is proven by scientific studies³!

As with all CI users, subsequent hearing success in otosclerosis also depends largely on how long the affected ear was deaf. Although the CI cannot stop the progression of the disease, it restores the ability to hear and communicate, avoids the consequential problems associated with hearing loss and thus maintains the functionality of the auditory nerve^{4 5 6} and the cognitive allocation of auditory areas.

Three strong reasons to consider cochlear implantation at an early stage if you have advanced deafness due to otosclerosis!

Prevalence of otosclerosis and possible triggers

Otosclerosis affects more women than men. It is usually diagnosed for the first time when patients are between 15 and 45 years old: This is no coincidence, as changes in hormone levels are one of the typical triggers of the disease. However, other triggers can also be infectious diseases or excessive noise, and genetic causes are also suspected.

Progression and effects of otosclerosis

The tissue changes caused by otosclerosis do not necessarily always lead to hearing loss. If it does, then it usually begins in the low frequency range. Dizziness and ringing in the ears also often occur. It is striking that those affected sometimes understand speech better in a loud environment than in a quiet one.

In around 30 percent of otosclerosis sufferers, the ossifications also affect the inner ear over the years (cochlear ossification). Even if the structures in the middle ear remain mobile, harmful substances can develop that damage the inner ear (inner ear otosclerosis). Even if both ears are not equally affected, the hearing impairment makes communication more difficult and can lead to numerous subsequent impairments. It is therefore essential to hear well again!

Literature

[1] Skarzynski PH et al. Outcomes of Cochlear Implantation in Patients with Far-Advanced Otosclerosis Who Had Previously Undergone Stapes Surgery. J Int Adv Otol. 2024; 20(2): 101-107. doi: 10.5152/iao.2024.231332.

[2] Assiri M et al. Cochlear implantation among patients with otosclerosis: a systematic review of clinical characteristics and outcomes. Eur Arch Otorhinolaryngol. 2022; 279(7): 3327-3339. doi: 10.1007/s00405-021-07036-5.

[3] Kondo M et al. Cochlear Implantation in Far Advanced Otosclerosis: A Systematic Review and Meta-Analysis. Laryngoscope. 2023; 133(6): 1288-1296. doi: 10.1002/lary.30386.

[4] Kopelovich JC et al. Intracochlear electrical stimulation suppresses apoptotic signaling in rat spiral ganglion neurons after deafening in vivo. Otolaryngol Head Neck Surg. 2013 Nov; 149(5): 745-752. doi: 10.1177/0194599813498702.

[5] Chen I et al. The effect of cochlear-implant-mediated electrical stimulation on spiral ganglion cells in congenitally deaf white cats. J Assoc Res Otolaryngol. 2010 Dec; 11(4): 587-603. doi: 10.1007/s10162-010-0234-3.

[6] Fallon JB et al. Effects of chronic cochlear electrical stimulation after an extended period of profound deafness on primary auditory cortex organization in cats. Eur J Neurosci. 2014 Mar; 39(5): 811-820. doi: 10.1111/ejn.12445.

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