Type:
Educational Exhibit
Keywords:
Ear / Nose / Throat, Head and neck, Neuroradiology brain, CT, CT-High Resolution, MR, Complications, Diagnostic procedure, Structured reporting, Demineralisation-Bone, Prostheses
Authors:
F. J. Barba Tamargo1, N. Perez Iglesias1, L. calvo apraiz1, J. Elejondos1, A. Aguado1, I. Goicoechea1, M. Gredilla2, M. LETURIA ETXEBERRIA3, A. Serdio4; 1San Sebastian/ES, 2DONOSTIA/ES, 3San Sebastian, Gipuzkoa/ES, 4Donostia - San Sebastián/ES
DOI:
10.26044/ecr2019/C-1660
Background
Otosclerosis is an otodystrophy of the otic capsule that is characterized by the replacement of the normal ivory-like enchondral bone by spongy vascular bone.
In its evolution the disease will subsequently present abnormal bone deposits with sclerosis.
Otosclerosis is more prevalent in women than in men (♀>♂ 2:1).
Tipically,
patients present during their 4th and 5th decades.
Caucasians are more frequently affected than other racial group.
The pathogenesis of otosclerosis is likely multifactorial and is not completely understood,
with viral,
inflammatory,
genetic an autoimmune components.
CLINICAL PRESENTATION:
- The typical clinical presentation of otosclerosis is conductive hearing loss,
frequently bilateral.
In some cases affected patients may have sensorineural or mixed hearing loss.
- The disease typically presents a fluctuating course with periods of remission.
- Pregnancy often produces an exacerbation of symptoms.
- Otoscopy is often normal.
However,
in severe cases,
the Schwartze sign may be observed during an otoscopic examination.
The Schwartze sign is a characteristic reddish discoloration of the cochlear promontory.
This discoloration results from the increased blood flow to the promontory.
- Despite the important role of radiology in some aspects of this disease,
the diagnosis of otosclerosis is based on clinical and audiometric studies.
Otosclerosis evolution is classically divided into two phases.
The first phase (otospongiosis or active phase) the endochondral bone of the otic capsule is replaced by disorganized cancellous bone,
less dense and with a greater vascular component.
The second phase (otosclerosis or inactive phase) the pathological cancellous bone tend to recalcify and presents a greater component of sclerosis.
The fissula ante fenestram or anterior edge of the oval window is the most frequently affected area and causes a progressive conductive hearing loss by stapedo-vestibular join ankylosis.
In its evolution it can spread throughout the labyrinth capsule,
causing sensorineural hearing loss and vestibular symptoms.
Treatment: stapedotomy,
partial or complete stapedectomy with stapes prosthesis in fenestral otosclerosis.
In some cases cochlear implant placement is required in cochlear otosclerosis