Progressive hearing loss stapes fixation
Overview
A rare condition characterized by hearing loss, fixation of stapes (ear bone which causes conductive deafness if it becomes fixed and immovable) and reduced vestibular response
Symptoms
Symptomes includes:
- Progressive mixed hearing loss in males
- Fixed stapes
- Slight hearing loss in females
- Reduced vestibular response
- progressive
- Hearing loss-Inner ear defect - stapes fixation
- Vestibular problems
- Delay in learning to walk
- Perilymphatic gusher resulting from stapes surgery
- Dizzines due to perilymphatic gusher Increased rate of hearing loss - due to perilymphatic gusher
- Balance problems
Causes
- Conductivehearing loss
- Externalauditory canal disorders
- Atresia of external auditory canal
- Impacted cerumen
- Otitis externa
- Exostosis
- Masses
- Middle ear disorders
- Acuteand chronic
- otitis media
- Otitis media with effusion
- Tympanic membrane perforation
- Hemotympanum
- Tympanosclerosis
- Ossicular chain defect, disruption,or fixation
- Cholesteatoma and other middle earmasses
- Sensorineural hearing loss
- Sensorineuralhearing loss without associated abnormalities
- Sensorineural hearing loss with associatedabnormalities
- Chromosomal disorders
- Inner ear malformations
- Labyrinthineaplasia
- Common cavity malformation
- Cochlear malformations
- Large vestibular aqueduct
- Prematurity
- Hypoxic-ischemic encephalopathy
- Bilirubin encephalopathy (kernicterus)
- Infection
- Trauma
- Drugs
- Perilymph fistula
- Neoplasm
- Ménière disease
- Unknown
- Mixed hearing loss
Diagnosis
Audiometry and caloric testing or electronystagmography:
Should be done in almost all cases in which the ear examination is normal. It is probably wise to consult an otolaryngologist at this point.
Tympanography:
will be helpful in diagnosing subtle cases of serous otitis media.
X-rays:
of the mastoids, petrous bones, and internal auditory canal should be done for chronic otitis media, cholesteatoma, and acoustic neuroma. If an acoustic neuroma is suspected.
MRI of the brain and auditory canals:
must be done. If basilar artery insufficiency is suspected, four-vessel cerebral angiography should be done. Magnetic resonance angiography is an excellent noninvasive alternative for diagnosing vertebral-basilar artery disease.
If multiple sclerosis is suspected, MRI of the brain, BSEP and VEP studies, and a spinal tap for spinal fluid analysis may be done. Rather than perform these tests, the most cost-effective approach would be to refer the patient to a neurologist if other focal neurologic findings are evident.