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Audiology Review: Preparing for the Praxis and Comprehensive Examinations
https://t.me/medicina_free
72
APPENDIX 2–B. continued
ETIOLOGY AND
DISORDER
PATHOLOGY SYMPTOMS DIFFERENTIAL DIAGNOSIS
Temporal bone trauma (otic capsule sparing)
Result of blunt force, penetrating, compressing, or barotrauma
Otic capsule (i.e., cochlea and SCC) remains intact
Tympanosclerosis White calcified plaques
on TM
Otalgia
Bloody otorrhea
Loss of consciousness
Possible facial nerve issues
Otoscopy: Hematoma, debris or blood in EAC, hemotympanum or CSF behind TM
Tymps: Depend on damage (Ad, As, B)
Audio:
Unilateral CHL
OAEs: Absent unilateral
VNG: Possible comorbid BPPV
Primarily asymptomatic Otoscopy: Abnormal; white
areas of TM
Tymps: Type A or As
Audio: Typically normal hearing; can have CHL
OAEs: Consistent with audio
CHAPTER 2 Anatomy, Physiology, and Relevant Pathologies
https://t.me/medicina_free
Appendix 2–C
Inner Ear Disorders
ETIOLOGY AND
DISORDER
PATHOLOGY SYMPTOMS DIFFERENTIAL DIAGNOSIS
73
Autoimmune inner ear disorder
Diabetes mellitus
Enlarged vestibular aqueduct syndrome
Caused by body’s immune response directed at inner ear
Elevated blood glucose levels and altered lipids and proteins cause vascular changes that impact the stria vascularis and other cochlear anatomy and auditory nervous system
Vestibular aqueduct within the temporal bone is abnormally large
Age of onset: Typically diagnosed around age 3–4
Progressive
Aural fullness
Fluctuating tinnitus
Gradual vertigo
Typically treated as ear infection
Common in middle-aged women
Gradual progressive symptoms
Postural instability due to neuropathy
Hearing loss
Possible history of head trauma
Delayed motor milestones
General imbalance
Poor coordination
Head tilting with vomiting
Otoscopy:
Tymps: Type A
MEMR: Consistent with audio
Audio: progressive SNHL
OAEs:
Dx by exclusion
Otoscopy: WNL
Tymps: Type A
MEMR: Consistent with audio
Audio: Bilateral high­frequency SNHL
OAEs:
Otoscopy:
Tymps:
MEMR: Consistent with audio
Audio: Fluctuating and progressive HL with low-frequency air-bone gap
OAEs: Absent
WNL
Fluctuating or
Consistent with audio
Consistent with audio
WNL
Type A
Hidden hearing loss
Abnormalities in the inner hair cells or ribbon synapses of the cochlea due to exposure to noise, but present with normal hearing
History of noise exposure
Hearing loss
Difficulty with speech in noise and background noise
VNG: See Chapter 7
Otoscopy: WNL
Tymps: Type A
MEMR: Elevated to absent
Audio: Normal hearing
OAEs: Absent
ABR: Missing/reduced wave I
ECochG: Abnormal SP/AP
continues
Audiology Review: Preparing for the Praxis and Comprehensive Examinations
https://t.me/medicina_free
74
APPENDIX 2–C. continued
ETIOLOGY AND
DISORDER
PATHOLOGY SYMPTOMS DIFFERENTIAL DIAGNOSIS
Meniere’s disease
Excess endolymph fluid pressure, overproduction, or underabsorption
Meningitis Inflammation of the
meninges of the brain and spinal cord (due to primarily bacterial or viral infection) enter the inner ear
Roaring tinnitus
Aural fullness
Episodes of vertigo
Fluctuating hearing loss that worsens during episodes
Fever
Stiff neck
Persistent headache
Nausea and vomiting
Symptoms occur 3–7 days following exposure
Otoscopy: WNL
Tymps:
Type A
MEMR: Consistent with audio
Audio:
Unilateral low-frequency SNHL (progresses to bilateral) with poor word recognition
OAEs: Consistent with audio
VNG: See Chapter 7
Otoscopy:
WNL
Tymps: Type A
MEMR: Consistent with audio
Audio: Bilateral SNHL
Ossificans
Possibly abnormal
VNG:
Noise-induced hearing loss
Excessive stimulation to the inner ear causes damage to hair cells and auditory nerve fibers
Related to exposure to impulse noise (acoustic trauma) or gradual noise, causing temporary or permanent threshold shifts
Ototoxicity Toxic effect of the inner
ear — degeneration of cochlear hair cells and auditory nerve and possible alteration to fluid in the organ of Corti
History of noise exposure
Hearing loss
High-frequency constant tinnitus
Possible aural fullness with threshold shift
History of ototoxic medications or exposure to ototoxic chemical
Hearing loss
Tinnitus
General dizziness
Oscillopsia
Atypical gate
Otoscopy: WNL
Tymps: Type A
MEMR: Consistent with audio
Audio: SNHL with poorer thresholds 3–6 kHz (noise notch)
OAEs: Consistent with audio
Otoscopy: WNL
Tymps: Type A
MEMR: Consistent with audio
Audio: Bilateral progressive high-frequency SNHL; poor word recognition
VNG: See Chapter 7
CHAPTER 2 Anatomy, Physiology, and Relevant Pathologies
https://t.me/medicina_free
ETIOLOGY AND
DISORDER
PATHOLOGY SYMPTOMS DIFFERENTIAL DIAGNOSIS
75
Perilymphatic fistula
Perilymph leaks from the oval or round window, which alters the pressure differences between the scalae in the cochlea
Causes are related to head trauma, barotrauma, and activities that produce straining
Presbycusis Decrease of auditory
function (in the peripheral and central auditory system) due to aging
Episodic vertigo (during strain)
Aural fullness
Tinnitus
Nausea
Possible history of head trauma
Ocular tilt
Gradual symptoms
Hearing loss
Tinnitus
Poor speech understanding (especially in noise)
Older age
Otoscopy: WNL
Tymps:
Type A with reports of dizziness or presence of nystagmus
MEMR:
Consistent with
audio
Audio:
Fluctuating flat or
sloping SNHL
OAEs:
Consistent with audio
Fistula test: Nystagmus during pressure changes (Hennebert’s sign)
VNG: See Chapter 7
Otoscopy:
WNL
Tymps: Type A
MEMR: Consistent with audio
Audio: Bilateral high­frequency SNHL with decreased word recognition, poor speech in noise performance
Sudden SNHL Acute onset, idiopathic
hearing loss
Sudden decrease in hearing
Unilateral tinnitus
Aural fullness
Trouble in background noise
Possible dizziness and imbalance
Hyperacusis
Otoscopy: WNL
Tymps:
Type A
MEMR: Consistent with audio
Audio: SNHL with poor word recognition especially in noise
OAEs: Consistent with audio
ABR: WNL (rule out tumor)
VNG: Possible unilateral peripheral findings
continues
Audiology Review: Preparing for the Praxis and Comprehensive Examinations
https://t.me/medicina_free
76
APPENDIX 2–C. continued
ETIOLOGY AND
DISORDER
PATHOLOGY SYMPTOMS DIFFERENTIAL DIAGNOSIS
Superior semicircular canal dehiscence
Temporal bone fracture (otic capsule disrupting)
Bony covering of the superior semicircular canal thins or falls open, creating a fistula at times
Fracture of the otic capsule extends into the cochlea and/or vestibule, which can disrupt the membranous labyrinth and damage auditory nerve fibers
Autophony
Tinnitus
Hyperacusis
Aural fullness
Distorted sensation of sounds
Chronic imbalance
Possible history of recent head trauma
Tullio’s (vertigo and/ or nystagmus to loud sounds)
Raccoon eyes
Hearing loss
Vertigo
Facial nerve paralysis
Possible history of trauma
Otoscopy:
WNL
Tymps: Type A
MEMR: WNL
Audio:
Enhanced bone
thresholds (esp. at 250 Hz)
OAEs:
WNL
VNG: See Chapter 7
Otoscopy: Hemotympanum
Tymps:
Dependent on fracture
MEMR:
Consistent with
audio
Audio: SNHL
OAEs: Consistent with audio
CHAPTER 2 Anatomy, Physiology, and Relevant Pathologies
https://t.me/medicina_free
Appendix 2–D
Retrocochlear Disorders (Auditory Nerve and Central Auditory Nervous System)
DISORDER ETIOLOGY SYMPTOMS DIFFERENTIAL DIAGNOSIS
77
Auditory neuropathy spectrum disorder
Labyrinthitis Viral inflammation of
Multiple sclerosis
Impaired function of the auditory nerve
CN VIII
Autoimmune disease that causes demyelination
Adult:
Hearing loss
Difficulty with speech in noise
Child:
Abnormal birth history
Delay of speech milestones
Possible recent viral infection
Vertigo
Unilateral hearing loss
Tinnitus
Imbalance
Nausea
Numbness of extremities
Neural shock during neck movement
Tremors
Otoscopy: WNL
Tymps: Type A
MEMR: Absent
Audio: Varies from normal hearing to profound SNHL
OAEs: to absent)
ABR: abnormal with CM only
Otoscopy:
Tymps: Type A
MEMR: Consistent with audio
Audio: Unilateral SNHL
OAEs: Consistent with audio
VNG:
Otoscopy: WNL
Tymps: Type A
MEMR: Consistent with audio
Present (can progress
Absent or highly
WNL
See Chapter 7
Loss of vision/double vision
Speech and swallowing difficulties
Vertigo
Ataxia
Audio: Asymmetric high­frequency SNHL
OAEs: Consistent with audio
ABR: Possibly abnormal; abnormal morphological breakdown/latency prolongations with high rates
VNG: See Chapter 7
continues
Audiology Review: Preparing for the Praxis and Comprehensive Examinations
https://t.me/medicina_free
78
APPENDIX 2–D. continued
DISORDER ETIOLOGY SYMPTOMS DIFFERENTIAL DIAGNOSIS
Vestibular schwannoma
Benign tumor of the vestibular branch (primarily) of CN VIII originating from Schwann cells (wrap around the neurons that conduct signal)
Commonly resulting from Neurofibromatosis Type 2
Gradual changes
Unilateral tinnitus
Progressive unsteadiness and vertigo
Nausea
Possible facial weakness
Possible headaches
Aural fullness
Difficulty on the phone
Otoscopy:
WNL
Tymps: Type A
MEMR: Retrocochlear pattern
Reflex Decay:
Positive
Audio: Unilateral SNHL with positive rollover
OAEs:
Consistent with audio
ABR: Prolonged wave V
VNG: See Chapter 7
continues
https://t.me/medicina_free
ASSOCIATED
SYMPTOMS LESION SITE
AUDITORY
SYMPTOMS
Central
canal/s
instability/swaying
None Nausea; vomiting Semicircular
(posterior most
often)
Semicircular
canal/s
photophobia; nausea;
vomiting; changes in
None Aura, phono, or
Vestibular
labyrinth;
vision; anxiety
difficulties; peripheral
cerebellum;
peripheral
nervous system
neuropathy
Likely diffuse
central
Headache; cognitive
changes; mood
disorders; sleep
disorders
Aural fullness,
otalgia; tinnitus
NUMBER
VESTIBULAR
Stress None Agoraphobia;
OF ATTACKS PROVOCATION
Multiple or
Constant or
SYMPTOMS DURATION
constant
Multiple Moving head in
episodic
30–60
vague
Sudden onset
certain directions;
lying down;
rolling over in bed
seconds
vertigo
motion
intolerance
Multiple Stress, fatigue,
hours
Episodic vertigo Minutes to
None None Swallowing
Multiple or
constant
Constant
with standing
and walking
Unsteadiness/
imbalance with
standing and
walking
Position changes;
visual stimuli
Multiple or
constant
Constant or
episodic
Unsteadiness;
dizziness;
rotational/
rocking vertigo
Appendix 2–E
Vestibular Pathologies
DIAGNOSIS
Anxiety Nondescriptive;
Benign
paroxysmal
positional vertigo
(BPPV)
Benign
paroxysmal
vertigo of
childhood
(BPVC)
Cerebellar ataxia
with neuropathy
and bilateral
vestibular
areflexia
syndrome
(CANVAS)
Concussion/
traumatic brain
injury
79
ASSOCIATED
https://t.me/medicina_free
SYMPTOMS LESION SITE
AUDITORY
SYMPTOMS
Labyrinth
Delayed motor
development;
difficulty climbing
stairs, riding a bike,
moving in the dark;
Hearing loss
(sensorineural or
mixed; usually
bilateral) around
3–4 years old;
often seen as clumsy
false conductive
component in
low frequencies;
hearing loss can
fluctuate, be
stable, or progress
CN VIII and
VII; CPA
Preceded by intense
ear pain and blisters
in and around the
tongue, face, mouth,
and ear; can be
accompanied by facial
unilateral hearing
loss; hyperacusis
vestibular
labyrinth
paralysis; nausea;
vomiting
Possible oscillopsia Auditory and
Sudden unilateral
sensorineural
hearing loss;
unilateral tinnitus
None None Unknown
NUMBER
VESTIBULAR
trauma; pressure
OF ATTACKS PROVOCATION
Multiple Positional; head
Minutes to
hours
SYMPTOMS DURATION
True vertigo;
feelings of
change; loud
sounds
unsteadiness/
imbalance
True vertigo Unilateral tinnitus;
preceded by
illness
Single Sometimes
to hours
(possibly
Constant Preceded by
longer)
Constant
Persistent
prolonged travel
(over days
to months;
relieved when
in motion)
rocking (no
imbalance
or rotational
vertigo)
APPENDIX 2–E. continued
DIAGNOSIS
Enlarged
vestibular
aqueduct (EVA)/
large vestibular
aqueduct
syndrome
(LVAS)
Herpes zoster
oticus (Ramsay
Hunt syndrome)
80
Labyrinthitis True vertigo 30 minutes
Mal de
debarquement
syndrome
continues
https://t.me/medicina_free
ASSOCIATED
SYMPTOMS LESION SITE
AUDITORY
SYMPTOMS
Labyrinth
Burnout after several
years (no more
dizziness; hearing loss
remains); drop attacks
Low-frequency
fluctuating hearing
loss; low pitch/
roaring tinnitus;
aural fullness
Thought to
be labyrinth
and vestibular
nuclei; include
other areas of
Aura, phono, or
photophobia;
sensitivity to smells;
head pain does not
necessarily accompany
Many report ear
pain, fullness
the midbrain
and brainstem
dizziness
Central
fatigue; nausea;
palpitations;
headache; weakness
None Blurred vision;
NUMBER
VESTIBULAR
Changes in
OF ATTACKS PROVOCATION
Multiple
Minutes to
SYMPTOMS DURATION
atmospheric
pressure or
weather; diet;
not necessarily
(must have
at least two
episodes for
diagnosis)
hours
occurring before
hearing loss
provocation, but
accompanied
by an aura/
knowing attack is
imminent
necessarily), light,
Multiple Headaches (not
several days
Episodic vertigo 1 minute to
sound, certain
positions; visual
stimuli
Variable Variable Variable Variable Variable None
Vague case
history;
inconsistent
quickly; changes
A few seconds Multiple Standing up
timeline and
description
True vertigo,
lightheadedness,
in body posture;
may be taking
antihypertensive
medication
unsteadiness
DIAGNOSIS
Ménière’s disease Episodic vertigo
Migraine/
vestibular
migraine
81
Nonorganic/
physiologically
inconsistent
Orthostatic
hypotension/
dysautonomia