Inner Ear Flashcards
(13 cards)
How are disorders of the inner ear evaluated?
Disorders of the inner ear are generally identified using a combination of pure tone testing and OAEs.
OAEs refer to otoacoustic emissions, which are sounds produced by the inner ear’s hair cells in response to sound stimuli.
What type of hearing loss results from inner ear disorders?
Inner ear disorders result in sensorineural hearing loss.
Sensorineural hearing loss involves damage to the inner ear or the auditory nerve pathways.
Where do most pathologies of the inner ear occur?
Most pathologies of the inner ear occur at the level of the hair cells.
Hair cells are essential for converting sound vibrations into neural signals.
What are the impacts of hearing loss affecting the cochlea?
Hearing loss affecting the cochlea results in loss of sensitivity, speech understanding, and difficulty listening in noise.
Cochlear damage can significantly impair auditory processing and communication.
What percentage of inherited hearing loss is autosomal recessive?
80% of inherited hearing loss is autosomal recessive.
Autosomal recessive conditions require two copies of the mutated gene for the trait to manifest.
What gene is commonly associated with severe-to-profound autosomal recessive nonsyndromic hearing loss?
Mutations in the GJB2 gene are commonly associated with severe-to-profound autosomal recessive nonsyndromic hearing loss.
The GJB2 gene encodes for a protein essential for the function of hair cells in the cochlea.
What percentage of inherited hearing loss is autosomal dominant?
20% of inherited hearing loss is autosomal dominant.
Autosomal dominant conditions can manifest with just one copy of the mutated gene.
How does autosomal dominant hearing loss typically present?
Autosomal dominant hearing loss is usually post-lingual (adult-onset) and more often progressive.
This means it typically develops after language acquisition and worsens over time.
What is non-syndromic hearing loss?
Non-syndromic hearing loss is a genetically-inherited hearing problem that is not associated with any other signs or symptoms.
It constitutes the majority of genetic hearing loss cases.
What is syndromic hearing loss?
Syndromic hearing loss is a genetically-inherited hearing problem occurring in combination with other signs and symptoms affecting other parts of the body.
Examples include conditions like Usher Syndrome and Pendred Syndrome.
What are some symptoms of Waardenburg Syndrome?
Symptoms of Waardenburg Syndrome include hearing loss, white forelock, light-colored eyes, and depigmented regions of the skin.
This syndrome is associated with genetic mutations affecting pigmentation and hearing.
What are the major diagnostic criteria for CHARGE Syndrome?
The major diagnostic criteria for CHARGE Syndrome include coloboma, cranial nerve abnormalities, choanal atresia, and CHARGE ear.
CHARGE Syndrome is a complex genetic condition with multiple systemic effects.
What are ototoxic medications?
Ototoxic medications are drugs that cause damage to the ear, such as aminoglycosides, platinum-based chemotherapy agents, and loop diuretics.
These medications can lead to temporary or permanent hearing loss.
