Sensorineural Disorders flashcards |

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many sensorineural disorders are

genetic (endogenous)

____ of congential hearing losses are genetic


____ of genetic hearing loss is part of syndrome


types of inheritance

-autosomar dominant inheritance
-autosomal recessive inheritcane
-x-linked inhertitance (passed from mother to son)

autosomal dominant inheritance accounts for

20% of all genetic hearing loss

autosomal recessive accounts for

80$ of all genetic hearing loss
-the majority

x-linked inheritance accounts for

2-3% of genetic hearing loss

autosomal dominant inhertitance

gene exists on one off 22 pairs of non-sex determining chromosomes
-hearing loss bc of heritence for EITHER parent
-50% chance of any child inheriting hearing loss
punett sq of Dd with dd

waardenburg's syndrome

autosomal dominant inheritance; absence of organ of corti and atrophy of spiral ganglion; accompanied by:
-white forelock of hair
-iris bicolor or heterochromia

autosomal recessive inheritance

gene present in both parents
-25% change of any child inheriting hearing loss
-children can be carriers

examples of autosomal recessive inheritance

1. usher's syndrome
2. pendred's syndrome
3. enlarged vestibular aqueduct syndrome

usher's syndrome

congenital deafness and progressive loss of vision (retinitis pigmentosa) leading to eventual blindness

pendred's syndrome

goiter and profound deafness

enlarged vestibular aqueduct syndrome

symptom of Pendred syndrome, associated with mondini cochlear dysplasia
-'reflux' of endolymph through abnormally large endolymphatic duct and vestibular aqueduct (bony canal leading from inner ear to cranial cavity) causes damage to cochlear structures in scala media (akin to meniere's mechanism), endolymphatic sac thought to regulate fluid pressureโ€”increase in CSF pressure may cause reflux ('blowing a trumpet', weight lifting, diving, air plane flight) (someitme progressive, sometimes suddenly)
-initially in high frequencies

enlarged vestibular aqueduct syndrom is commonly identificied

children with sneorineural hearing loss of unknonw etiology
-often bilatreal
-sensorineural hearing loss at birth or onset in childhood

female to male ratio for Enlarged Vestibular Aqueduct Syndrome


non-genetic causes of hearing loss

-Congenital Infections: TRCHs
-hyperbilirubinemia with kernicterus
-trauma at birth
-medication during birht
-low birth weight bc of byperbilirubinemia
-postnatal infections: bacterial mningitis, mumps, measles (rubeola)
-ototoxic medications
-meniere's disease
-noise exposure
-genetic causes
-otitis media

congenital infection



caused by parasite transmitted to child via placenta (not viral, it's a paraiste)


maternal-contracted, passed to child through placenta prenatally; typically bilateral, severe to profound loss, with worst hearing in the mid frequencies (cookie bite configuration)

-earlier infection increases the chance of hearing loss
-possibly accompanied by heart/kidney defects, visual impairment (german measles)

cytomegalovirus (CMV)

passed from the mother to the child via the bloodstream and/or contact with the birth canal; most common viral disease known to cause hearing loss
-90% of symptomatic children who survive will exhibit complications, the majority will have hearing loss
-HL is usually progressive, less often unilateral than bilateral, most often severe to profound

herpes simplex virus

sexually transmitted, passed to fetus in utero


transmitted to child in eurtero
-may manifest itself up to 60 years later, if syphilis shows < 10 yrs, profound hearing loss & bilateral with sudden onset


too much bilirubin in the blood
-hearing loss occurs in sever cases with kernicterus


bile deposits in CNS at cochlear nuclei

bacterial meningities

postnatal infection; leading cause of acquired childhood severe sensorineural hearing loss
-can affect cochlear, audiotry nerve, CANS (inflammation causes ossification inside cochlear)


postnatal infections; common causes of unilateral sever-profund sensorineural


postnatal infections; rubeola

incidence of severe sensorineural hearing loss


postnatal infections are associated with

vestibular impaiment

other acquired causes of sensorinerual hearing loss

-ototoxic medications
-meniere's desease
-noise exposure
-genetic causes
-otitis media

leading cause of sensorineural hearing loss in adults

noise exposure and presbycusis

leading cause of sensorineural hearing loss in children

genetic causes and otitits media

ototoxic medications

attack inner ear through bloodstream---high frequencies affected first
-aminoglycoside antibiotics (streptomycin[vestubulo toxin], neomycin, gentamicin, kanamycin, tobramycin [cochlea toxin)
-diuretics (especially furosemide)
-chemotherapeutic agents (platinum based agents, e.g., cisplatinin, carboplatin)

accumulation of drug in blood affects

base of cochlea (high frequencies)

apirin affects

low frequencies

meniere's disease

-excess fluid in the inner ear
-progressive or fluctuating, low-frequency HL
-bilateral or unilateral (thought to be more often unilateral)

symptoms of meniere's disease

episodic vertigo **
tinnitus, feeling of fullness in the ear

treatment for meneire's disease

-Low-sodium diets
-Sedatives, tranquilizers
-Vestibular suppressants (e.g., Antivert); drugs that suppress the vestiulus system
-Endolymphatic shunt surgery
-Destruction of the labyrinth/cutting the auditory nerve

noise induced hearing loss

hair cell and other cochlera structures dmaged or destroyed
-dependent on intensity, spectrum of noise, duration of exposure & individual resistance (not necessarily related to loudness of noise)

characteristics of nose induced hearing loss

-Almost always bilateral (unless very mild), but can be asymmetrical
-Affects frequencies between 3000 & 6000 Hz first (doesn't affect outer/middle ear, it affects cochlear
-Notched configuration


age-related hearing loss (50+)

characteristics of presbycusis

-affects cochlea, but can also affect auditory nerve and CANS
-high frequencies affected first
-bilateral, symmetrical, typically mild to moderate hearing loss

retrocochlear disorders

-acoustic neuroma/vestibular schawnnoma
-multiple sclerosis
-cerebovascular accident/stroke

acoustic neuroma/vestibular schawnnoma

benign, slow-growing tumor compressing the nerve [compresses brain stem functions]
-(must be removed surgically)
-most common tumor of temporal bone

characteristics of acoustic neuroma/vestibular schawnnoma

-almost always unilateral
-affects high frequencies first

symptoms of neuroma/vestibular schawnnoma

tinnitus & dizziness common symptoms

characteristics of multiple sclerosis
and cerebovascular accident/stroke

hearing loss and difficutly w/ speech recognition in noisy conditions

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