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A new phenomenon of cochlear otosclerosis: an acquired or congenital disease? - A clinical report of cochlear otosclerosis
- Source :
- Acta oto-laryngologica. 141(6)
- Publication Year :
- 2021
-
Abstract
- No cochlear otosclerosis in infants with congenital bilateral SNHL has been reported.We report an infant male with bilateral cochlear otosclerosis, suggesting that cochlear otosclerosis may be a congenital disease and to further analyze the etiology of and genetic expression in congenital bilateral cochlear otosclerosis. We also describe the clinical characteristics and experience of patients with bilateral cochlear otosclerosis treated with cochlear implants (CIs).Seven patients, including an infant, who were diagnosed with cochlear otosclerosis underwent CI surgery. Their medical records, audiological and radiological results, surgical procedures, and CI outcomes were collected and reviewed.The median age at hearing loss was 38 years, ranging from 0 to 47 years. The child had bilateral hearing loss at birth and received a CI at 1 year of age. He also had growth retardation and was diagnosed with 3q+/3p- syndrome. All patients (8 ears) had better postoperative auditory performance than that preoperatively.Although cochlear otosclerosis often starts at middle age and progresses slowly, it may be a congenital disease that is related to chromosome abnormality. This disease presents with SNHL or MHL, and treatment with a CI is beneficial.
- Subjects :
- Adult
Male
medicine.medical_specialty
Hearing loss
Cochlear Diseases
medicine.medical_treatment
Hearing Loss, Sensorineural
Audiology
Hearing Loss, Bilateral
Clinical report
Cochlear implant
otorhinolaryngologic diseases
medicine
Humans
Age of Onset
Retrospective Studies
business.industry
Infant
General Medicine
Middle Aged
medicine.disease
Cochlear Implantation
Otosclerosis
Otorhinolaryngology
Cochlear otosclerosis
Female
sense organs
medicine.symptom
Congenital disease
business
Tomography, X-Ray Computed
Subjects
Details
- ISSN :
- 16512251
- Volume :
- 141
- Issue :
- 6
- Database :
- OpenAIRE
- Journal :
- Acta oto-laryngologica
- Accession number :
- edsair.doi.dedup.....2a27b8d106dfa6fe02d935d668663985