Jensen syndrome
Overview
A rare genetic disorder characterized by degeneration of the optic nerve (causing impaired vision), deafness due to nerve damage and dementia due to calcification of the central nervous system
Symptoms
* Nerve deafness * Impaired vision * Optic nerve atrophy * Progressive dementia
Causes
1. Conductivehearing loss 1. Externalauditory canal disorders 1. Atresia of external auditory canal 2. Impacted cerumen 3. Otitis externa 4. Exostosis 5. Masses 2. Middle ear disorders 1. Acuteand chronic otitis media 2. Otitis media with effusion 3. Tympanic membrane perforation 4. Hemotympanum 5. Tympanosclerosis 6. Ossicular chain defect, disruption,or fixation 7. Cholesteatoma and other middle earmasses 2. Sensorineural hearing loss 1. Sensorineuralhearing loss without associated abnormalities 2. Sensorineural hearing loss with associatedabnormalities 3. Chromosomal disorders 4. Inner ear malformations 1. Labyrinthineaplasia 2. Common cavity malformation 3. Cochlear malformations 4. Large vestibular aqueduct 5. Prematurity 6. Hypoxic-ischemic encephalopathy 7. Bilirubin encephalopathy (kernicterus) 8. Infection 9. Trauma 10. Drugs 11. Perilymph fistula 12. Neoplasm 13. Ménière disease 14. Unknown 3. Mixed hearing loss