Audiology Disorders

Atresia

congenital absence or closure of the ear canal, which can lead to hearing loss. Small ECV, MEMR & Audio show CHL, OAEs reduced to absent

Exostoses

surfers ear, bony growth in ear canal

Microtia

small or misshapen pinna

osteoma

benign bony tumors in the ear canal that can lead to hearing loss.

Otitis externa

inflammation of the outer ear and ear canal, commonly known as swimmer's ear, which can cause pain and discomfort.

Perichondritis

inflammation of the cartilage of the ear, often resulting from infection or trauma.

Cholesteatoma

a destructive and expanding growth of skin cells in the middle ear and/or temporal bone that can result in hearing loss and infection.

Unilateral

White mass behind TM, Type Ad or B, MEMR conductive pattern, unilateral HL, OAEs reduced to absent

disarticulation of ossicular chain

a condition where the bones of the middle ear become separated, which can lead to conductive hearing loss.

Type Ad, MEMR are absent contralaterally, CHL, OAEs absent

eustachian tube dysfunction

a condition where the Eustachian tube fails to open properly, leading to pressure imbalance and fluid accumulation in the middle ear.

Type C, MEMR- conductive pattern, low freq CHL, OAEs reduced to absent

Glomus tumor

a rare vascular tumor that arises from the glomus body, typically located in the middle ear, which can cause pulsatile tinnitus, facial weakness, and conductive hearing loss.

Red mass behind TM, unilateral CHL or MHL, OAEs absent unilaterally.

Otitis Media

inflammation or infection of the middle ear, often associated with fluid buildup, leading to pain and conductive hearing loss.

Type B, MEMR conductive, low frequency HL or flat CHL with normal speech scores, OAEs absent.

Otosclerosis

a condition characterized by abnormal bone growth in the middle ear, leading to conductive hearing loss. Stapes becomes mineralized in oval window causing stapes fixation.

hormone changes in women. TM is red, Type A or As, Carhart notch (CHL at 2k)

Perforation

Perforation in TM, Type B, conductive hearing loss, unilateral low freq CHL

Tympanosclerosis

white calcifed plaques on TM, could have normal or CHL

Autoimmune inner ear disorder

aural fullness, fluctuating HL, gradual vertigo, common in middle age women

fluctuating or progressive SNHL

Diabetes Mellitus

Causes vascular changes to stria vascularis and other structures.

postural instability due to neuropathy, bilateral HFHL

Enlarged Vestibular Aqueduct Syndrome

age onset is 3-4 years old.

possible head trauma, delayed motor milestones, general imbalance, poor coordination, head tilting with vomit

flucuating and progressive HL with low freq air-bone gap

true vertigo, minutes to hours, positional changes

Labyrinth

hidden hearing loss

abnormailites in inner hair cells due to exposure to noise, but normal audio.

MEMR: elevated to absent

OAEs absent

ABR: missing or reduced wave 1

Meniere’s Disease

excess endolymph fluid pressure, overproduction, or underabsorption

roaring tinnitus, aural fullness, episode of vertigo, fluctuating hearing loss

unilateral low freq SNHL with bad WRS

minutes to hours

labyrinth

Meningitis

Inflammation of meninges of brain and spinal cord

fever, stiff neck, persistent headache.

Type A, bilateral SNHL, ossificans

Perilymphatic fistula

perilymph leaks from oval or round window, which alters the pressure scalae in cochlea

episodic vertigo, aural fullness, tinnitus, ocular tilt

Type A with presence of dizziness or nystagmus

fluctuating flat or sloping SNHL

Superior semicircular canal dehiscene

bony covering of SCC thins or falls open, creating fistula at times.

autophony( hear own voice or blood in head), tinnitus, hyperacusis, aural fullness,

audio: enhanced bone thresholds at 250 Hz

30-60 secs

Temporal bone fracture

fracture of otic capsule that extends into cochlear and/or vestibule

raccoon eyes, HL, vertigo, facial nerve paralysis

hemotympanum and SNHL

ANSD

impaired function of the auditory nerve

difficulty with speech and speech milestones

Audio varies

ABR: absent or highly abnormal with CM present

Labyrinthitis

viral inflammation of CN VIII

vertigo, unilateral HL, tinnitus, imbalance

30 minutes to hours

auditory and vestibular labyrinth

Multiple sclerosis

autoimmune disease causes demyelination

asymmetric HF SNHL

Vestibular schwannoma

benign tumor of CN VIII (vestibular branch) from schwann cells, result from NF2

unilateral tinnitus, progress unsteadiness and vertigo, facial weakness, aural fullness

MEMR: retrocochlear pattern (weakness in muscle during contraction)

audio: unilateral SNHL with positive rollover.

prolonged wave 5

BPPV

sudden onset vertigo, 30-60 sec

move head in certain direction

SCC (posterior)

cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome

unsteadiness/ imbalance with standing and walking

vestibular labyrinth; cerebellum; peripheral nervous system

Mal de Debarquement

persistent rocking

constant over days

Migraine/ vestibular migraine

episodic vertigo

1 min to several days

Persistent postural- perceptual dizziness

dizziness, constant for days more than 3 months

Vestibular neuritis

rotational vertigo

30 min to days

neural/ vascular leading to labyrinth damage

Pendred syndrome

recessive

enlarged thyroid

bilateral HF SNHL

EVA

Sticklet syndrome

dominant

type 1- HF SNHL

type 2- severe and progressive SNHL

typ3- mild to moderate SNHL

Treacher Collins Syndrome

dominant or recessive

pinna deformities

CHL can be others

Ushers syndrome

recessive

eye disease bilateral SNHL, dizziness

type 1- age 10

type 2- early 20s

type 3- puberty

Waardenburg syndrome

autosomal dom (type 1 &2) recessive ( type 3 & 4)

white forelock

SNHL and vestibular dysfunction