Hearing Disorder PDF

Hearing disorder
Han Young Hoon, M.D.

Department of Otorhinolaryngology, head & neck surgery, MNUSC
Hearing Disorders
• The most common hearing disorders are those that
affect hearing sensitivity. When a sound is presented to
a listener with a hearing sensitivity disorder, one of two
things may occur:
1.The listener with a HS disorder may be unable to
detect the sound.
2.The sound will not be as loud to that listener as it
would be to a listener with normal hearing.
Hearing Disorders
• Note:
Vision is different. The most common vision disorders
affect acuity, not sensitivity to light. Acuity is the ability
to resolve differences.
• Eyeglasses and contact lenses improve acuity, not
sensitivity to light.
• Hearing aids are just the opposite
– they improve sensitivity to sound by amplifying it
– but do not improve acuity.
Hearing Disorders
• Important:
This sensitivity vs. acuity distinction is not quite so simple
with hearing:
hearing loss of any significance nearly always involves
problems of both sensitivity and acuity
– sounds are harder to hear (sensitivity) and they are
nearly always distorted (acuity) more later.
Pure Tone Average (PTA)
• Most common way to measure hearing sensitivity is to
measure pure-tone (sinusoid) thresholds (PTA).
• Threshold: Intensity required to barely detect a sound.
• Rt side : red, round
• Lt. side : blue(black), X
• Average thresholds at 500, 1000, 2000 Hz – the frequencies
most important for speech understanding.
PTA
• Normal Hearing: PTAs < 25 dB
• Hearing Impairment: PTAs 25-92 dB
• Deaf: PTAs > 92 dB
• The term deafness is reserved for cases in which “ … the
handicap for hearing everyday speech … [is] … total” (Davis &
Silverman, 1979).
• Despite these conventions:
(1) there is no sharp dividing line between hearing I
mpairment and deafness, and
(2) degrees of deafness are meaningful; e.g., there is an
important difference between PTAs of 110 and 95.
PTA- normal
Audiogram of a Listener with
Thresholds in the Normal Range
125 250 500 1000 2000 4000 8000
-10
0
10
20
Intensity (dB HL)

30
40
50
60
70
80
90
100
110
Frequency (Hz)
Mild high-frequency loss
Audiogram of a Listener with a
Mild High-Frequency Hearing Loss
125 250 500 1000 2000 4000 8000
-10
0
10
20
Intensity (dB HL)
30
40
50
60
70
80
90
100
110
Frequency (Hz)
Moderate-to-profound bilateral loss
Severe Loss Left, Ear Moderate Loss Right Ear
125 250 500 1000 2000 4000 8000
-10
Pure Tone Average (PTA) 10
20
From the audiogram above: 30
Intensity (dB HL)

40
Pure-tone Average, Left Ear: 93 dB 50
Pure-tone Average, Right Ear: 50 dB 60
70
80
90
100
110
Frequency (Hz)
Hearing disorders
• Many ways to classify hearing disorders
1. Nature of the loss:
• Sensitivity vs. Acuity
 Dysacusia – Deficit in discrimination or interpretation of sound:
“Don’t shout, I can hear you just fine. I just can’t understand
what you’re saying.” Disacusia is a good term that isn’t in very
common use.
 Acuity deficits sometimes due to disorders of the central
auditory system.
 Disorders of sensitivity and acuity are not mutually exclusive.
Hearing disorders
2. Functional Classification
Conductive – Disorders involving the conduction of sound to
the cochlea.
Sensori-neural – Disorders involving the cochlea (usually the
hair cells) or 8th N.
Central – Disorders affecting the brain stem or auditory cortex.
• Two related terms:
Peripheral – Not central; i.e., conductive or sensorineural.
Retrocochlear – Disorders involving anatomical structures
beyond the cochlea; i.e., 8th N, brain stem,
auditory cortex.
Hearing disorders
3. Cause or Etiology of the Disorder
 Ototoxic drugs
 Noise exposure
 Old age (presbycusis)
 Otitis media
 8th N tumors
 Meniere’s Disease
Conductive hearing disorders
External Ear
 Congenital malformations. There are many of these. Most serious
is congenital atresia – collapse or closure of the EAM (ear canal).
May occur in isolation, but typically associated congenital
malformations of the middle ear as well.
 Impacted wax (cerumen) – results in mild hearing loss; easily
treated by removal of the wax.
Middle Ear
a. Otitis Media
 By far the most common cause of conductive hearing loss.
 By far the most common health problem in children.
 Otitis media means inflammation of the middle ear.
Middle Ear
a. Otitis Media
Cf) Disease Process – OM involves a very specific chain of events
revolving around the abnormal functioning of the Eustachian tube.
 Begins with an ordinary upper respiratory infection (cold) involving,
among other things, the nasopharynx.
 Nasal secretions infect the Eustachian tube.
 The Eustachian tube, which is normally closed, becomes inflamed
and can no longer open upon swallowing, yawning, etc. This means
that pressure can no longer be equalized between the middle ear and
the ambient air. **** This is the key to the whole deal ****
Middle Ear
a. Otitis Media
 Oxygen in the ME cavity is consumed by ordinary metabolic
processes. Ordinarily, this oxygen is re-supplied through the
Eustachian tube. With a plugged ET, this cannot occur.
 The absorption of oxygen without re-supply results in a partial
vacuum (i.e., lower than normal pressure) in the ME.
 The pressure drop sucks the TM inward into the ossicular chain,
reducing its mobility. Consequences: hearing loss and pain – often
quite intense.
Middle Ear
a. Otitis Media
 Partial vacuum creates another problem: Recall that the entire ME
cavity is lined with mucous membrane. The pressure drop causes
clear fluid to be sucked out of the mucosal lining of the ME. This
accumulation of fluid contributes to the conductive hearing loss.
Condition is called serous otitis media or nonsuppurative otitis
media. Defining features: (1) clear, thin, watery fluid, (2) fluid is
sterile (not infected).
 Suppurative or Purulent Otitis Media: As the disease progresses,
the fluid can become infected and thickens into (eck) pus.
Middle Ear
a. Otitis Media
< Consequences >
• The major consequences of OM are hearing loss and pain.
• The hearing loss is typically mild (usually 20-30 dB) and often
fluctuating.
• The pain varies quite a bit but is often quite severe. It is not
unusual for the pressure drop in the ME to become severe enough
to cause the TM to rupture.
Middle Ear
a. Otitis Media
• Acute vs. Chronic OM: A specific bout of OM with pain,
accumulation of fluid, etc., is called acute otitis media. If OM lasts
more than 2-3 months, the condition is known as chronic otitis
media, which has mostly membrane rupture.
• Recurrent OM: Frequent bouts of OM: OM is treated successfully,
then returns, then treated, then returns … This gets old after a
while but is pretty common.
Middle Ear
a. Otitis Media
• Treatment of Otitis Media
Most common treatment by far
: P.O. Antibiotics (amoxicillin, 1st~2nd cephalosporines)
antibiotics drop : aminoglycosides(gentamycin), Quinolones
anti-inflammatory drugs : NSAID, steroids
antihistamine
anti-fungal drugs
Middle Ear
a. Otitis Media
• Treatment of Otitis Media
Common treatment for recurrent or
chronic OM: PE Tubes (PE = pressure
equalization).
This is a small plastic tube inserted
into the TM. Why would such a tube
be expected to treat OM?
Middle Ear
a. Otitis Media
• A Few Additional Terms
Otitis Media with Effusion (OME): Otitis media characterized by the
accumulation of fluid. This term refers to any kind of fluid -- sterile,
infected, it doesn't matter.
Mucoid or Mucous Otitis Media (“Glue Ear”): Fluid in middle ear is
thick and gooey rather than thin and watery. Hearing loss is often more
severe than serous OM. Seen in some cases of recurrent OM – purulent
OM is treated, killing the infection, but fluid does not drain.
Middle Ear
• Speech and Language Delay??
• Evidence is mixed and controversial, but there is some
research suggesting that frequent bouts of OM *may*
result in delays in acquiring speech and language.
• Surprising to some since hearing loss is usually relatively
mild and fluctuating, with significant periods of normal
hearing in between bouts of OM for most kids.
• But, there is also clear evidence from other sources
indicating that kids need higher sound levels to perceive
speech with the same accuracy as adults. [Elliott et al. (1979). Children’s
understanding of monosyllabic nouns in quiet and in noise. J. Acoust. Soc. Am. 66, 12-21.]
Middle Ear
b. Otosclerosis
(note: topic here is still conductive HL, sorted by cause)
 Begins as a soft, spongy growth of new bone – may appear
anywhere in the ME, but most often near oval window.
 Later hardens (i.e., becomes sclerotic)
 In 90% of cases: No symptoms
Middle Ear
b. Otosclerosis
• In unlucky 10% : Growth reduces mobility of stapes, causing a
conductive HL.
• Progressive. Beginning in childhood. For that unlucky 10%, HL
typically begins in late teens, early 20s.
 Maximum HL seldom worse than ~50-60 dB.
 Treatment: Stapedectomy (removal of stapes and replacement
with an artificial stapes)
Stapedectomy
incus prosthetic stapes

Middle Ear
c. Cholesteatoma
 Skin Cyst that invades the ME; usually grows rapidly
• Can:
(1) destroy the ossicular chain,
(2) invade the cochlea, or
(3) break through the thin shelf of bone that forms the
superior surface of the ME cavity, invading the meninges. This
is extremely not good.
 HL is usually mild and not really the major concern.
Middle Ear
c. Cholesteatoma
c. Cholesteatoma
• The only treatment available to cure : surgery.
1) The surgery that is typically performed is tympanoplasty with
or without mastoidectomy.
• Complications of Cholesteatomas
: cause serious and sometimes life threatening health problems,
such as meningitis or brain abscess.
• Cholesteatomas can develop in both children and adults.
• Surgery is usually done as day surgery (outpatient).
Sensoryneural hearing disorders
• General
: By far the most common underlying cause of SNHL
- damage to the hair cell transducers.
: In these most common cases,
the auditory nerve & central auditory pathway
are intact, but stimulation of the auditory nerve
is abnormal due to damaged hair cells.
: There are many possible reasons for hair cell damage.
: The various etiologies of SN HL consist mainly of a
catalog of different causes of HC damage.
a. Presbycusis
• Hearing loss associated with aging
• Most common cause of SN HL – and most common cause of HL
overall
• Presbycusis begins in adolescence. Sad but true.
Men • This figure shows data for men (top)
and women (bottom) at different ages.
Not good.
Note that high frequencies are more strongly
affected than lows. (We’ll see this again when
we talk about noise-induced HL.) Any guesses
about why high-freqs are more vulnerable?
(Answer: The traveling wave starts at the base [high freq] and
heads to the apex [low freq]. So, low freq sound stimulates
HCs at the low freq end and the high freq end, while high freq
sound stimulates HCs at the high freq end only. And if you look
back to the Rose et al. FRC data from the physiology lecture,
the spread of excitation is especially large on the low freq side.
So, for low freq sound HCs at both ends of the BM are
vulnerable, but for high freq sound only HCs at the basal end
are affected, preserving low freq HCs. Moral: If you’re a hair cell,
you’re better off at the apex.)
Women
a. Presbycusis
• Moral: We all have a long, slow slide ahead of us. Don’t squander the
hearing you have by needlessly exposing yourself to long periods of loud
sound.
• Wear ear plugs or muffs when mowing the grass, snow-blowing, etc., and
use some sense in listening to music. Once hair cells are damaged, they’re
gone for good.
• One last point : Presbycusis is listed here under the SN category since it is
clear that this is the dominant component. However:
(1) The SN component may not be due exclusively to hair cell loss.
- Changes in the elasticity of the basilar membrane
- metabolic changes in the stria vascularis may also play a role
(Davis, H. and Silverman, S., 1978, Hearing and Deafness, New York: Holt,
Rinehart & Winston ).
a. Presbycusis
(2) There may also be a conductive component
- due to age-related changes in the mobility of tissues in the m. ear.
(3) There is sometimes a central component
- due to the loss of neurons in the CNS(related primarily to
arteriosclerosis). - The result of this CNS damage is a reduction in
acuity and speech perception abilities. The resulting deficit in speech
perception ability is sometimes referred to as phonemic regression. In
some cases it is this problem rather than a loss of hearing sensitivity
that is the patient’s primary complaint.
b. Noise-Induced Hearing Loss
• Exposure to high levels of noise can damage HCs and cause SN HL.
• Two types:
• Acoustic trauma :
 Injury due to brief exposure to very intense sounds such as gun shots,
artillery fire, explosions, etc.
 HL may be severe and permanent, but substantial recovery is common.
• Long-term noise exposure (more common):
 Damage results from long-term exposure to high levels of noise.
 Common in some occupational settings – heavy manufacturing and
agriculture being the most common.
 Amount of inner-ear damage depends on the combination of:
 Intensity of the noise
 Length of exposure
• Pretty simple: High levels x long exposures=Bad news
Low levels x brief exposures=Not so bad news
b. Noise-Induced Hearing Loss
• Audiometric Pattern is distinctive (audiogram on right shows more advanced progression than left)
• Note :
(1) Dip or “notch” at 3-6 kHz
(2) Typical progression shows the notch broadening (especially on the high frequency side) and
deepening
(3) High frequencies more affected than lows
c. Ototoxic Drugs
• Certain drugs can cause SN HL. Toxicity effects vary from mild and
temporary to severe and permanent.
• Some very common drugs such as aspirin (especially in large doses)
can cause hearing loss (and/or tinnitus), but not in most people, and
the loss is typically mild and temporary.
• An especially important group of antibiotics are notoriously ototoxic.
Examples include neomycin, streptomycin, kanamycin.
• Since this is well known, why might these drugs ever be
administered? (Answer: They’re used when death is the likely
alternative.)
ANTIBIOTICS WITH GOOD EVIDENCE FOR OTOTOXICITY
Drug Vestibulotoxicity Hearing Toxicity Toxic Level
Erythromycin yes High IV doses only

Gentamicin 8.6% minor Usually 2 weeks
Streptomycin very toxic minor
dihydrostreptomicin minor toxic very toxic
Tobramycin Yes minor in 6% Less toxic than Gentamicin
Netilmicin 2.4%
Amikacin not toxic 13.9%
Neomycin minor very toxic In topical ear drops
Kanamycin minor very toxic
Etiomycin moderate
Vancomycin nontoxic none to moderate synergistic with gentamicin
Metronidizole toxic (rarely) unknown

Capreomycin yes
Table from: http://www.tchain.com/otoneurology/disorders/bilat/ototoxins.html See other classes of ototoxic drugs on the same web site.
For your reference. The list below is from: www.lhh.org/hrq/22-2/ototoxic.htm
A. Salicylates
1. aspirin and aspirin-containing products 2. salicylates & methyl-salicylates (linaments)
B. Non-Steroidal Anti-Inflammatory Drugs (NSAIDS)
1. diclofenac (Voltaren) 2. etocolac (Lodine)
3. fenprofen (Nalfon) 4. ibuprofen (Motrin, Advil, Nuprin, etc.)
5. indomethacin (Indocin) 6. naproxen (Naprosyn, Anaprox, Alleve)
7. piroxicam (Feldene) 8. sulindac (Clinoril)
(Toxic effects are dose related and are almost always reversible once medications are discontinued).
C. Antibiotics
1. Aminoglycosides
a. amikacin (Amakin) b. gentamycin (Garamycin) c. kanamycin (Kantrex)
d. neomycin (Found in many over-the-counter antibiotic ointments) e. netilmicin (Netromycin)
f. streptomycin g. tobramycin (Nebcin)
(Of particular interest is that topical ear drop medications containing gentamycin or neomycin do not appear to be
ototoxic in humans unless the tympanic membrane (ear drum) is perforated. When a solution of an aminoglycoside
antibiotic is used on the skin together with an aminoglycoside antibiotic used intravenously, there is a risk of an
increase of the ototoxic effect, especially if the solution is used on a wound that is open or raw, or if the patient has
underlying kidney damage. Neomycin is the drug that is most toxic to the structure involved in hearing, the cochlea,
so it is recommended for topical use only.
But even topical therapy has resulted in hearing loss when large areas were treated which allowed for large
amounts of the drug to be absorbed into the body. Hearing loss caused by this class of antibiotics is usually
permanent).
2. erythromycin
a. EES b. E-mycin c. Ilosone d. Eryc
e. Pediazole f. Biaxin g. Zithromax
(Usually ototoxic when given in intravenous doses of 2-4 grams per 24 hours, especially if there is underlying
kidney failure).
3. vancomycin (Vancocin) (Similar to aminoglycosides in that it may be ototoxic when used intravenously in life-
threatening infections. To further exaggerate the problem is the fact that aminoglycosides and
vancomycin are often used together intravenously when treating life-threatening infections).
4. minocycline (Minocin) (Similar to erythromycin). 5. polymixin B & amphotericin B (Antifungal preparations)
6. capreomycin (Capestat) (Anti-tuberculosis medication).
D. Diuretics
1. bendroflumethazide (Corzide) 2. bumetadine (Bumex) 3. chlor-thalidone (Tenoretic)
4. ethacrynic acid (Edecrin) 5. furosemide (Lasix)
(These are usually ototoxic when given intravenously for acute kidney failure, acute hypertensive crisis, or acute
pulmonary edema/congestive heart failure. Rare cases of ototoxicity have been found when these medications
are taken orally in high doses by people with chronic kidney disease).
E. Chemotherapeutic Agents
1. bleomycine (Blenoxane) 2. bromocriptine (Parlodel)
3. carboplatinum (Carboplatin) 4. cisplatin (Platinol)
5. methotrexate (Rheumatrex) 6. nitrogen mustard (Mustargen)
7. vinblastin (Velban) 8. vincristine (Oncovin)
(The ototoxic effects can be minimized by carefully monitoring blood levels).
F. Quinine
1. chloroquine phosphate (Aralen) 2. quinacrine hydrochloride (Atabrine)
3. quinine sulfate (Quinam)
(The ototoxic effects are very similar to those of aspirin).
G. Mucosal Protectant
1. misoprostol (Cytotec)
d. Meniere’s Disease
• Serious, often debilitating disease of hearing and balance of
uncertain cause.
• MD affects a single ear in about 75% of cases.
• Four major symptoms:
(1) Periodic episodes of rotary vertigo (the sensation of spinning)
or dizziness (the “Meniere’s attack”)
(2) Fluctuating, progressive, low-frequency SN hearing loss
(3) Roaring or ringing tinnitus
(4) A sensation of "fullness" or pressure in the ear
(1) Rotary Vertigo
• the most disruptive and debilitating symptom of Meniere’s Ds.
• Similar to the mild vertigo you get from too many beers,
• similar to vertigo you may remember from spinning around on a
playground.
• Some major differences:
• Dramatically more severe
• Often accompanied by nausea, vomiting, sweating
• Onset is usually sudden
• Typically persists for hours or even days
• Patient has little or no ability to control it
• Condition often leaves the patient confined to a bed and as stationary
as possible for long periods of time, until the symptoms subside.
• Head movements can exacerbate the Sx.
(2) SN Hearing Loss
 Fluctuating
 Initially affects low-frequencies more than highs, but may
spread to highs as the disease progresses
 Progressive (i.e., gets worse with time)
 Hearing may be completely lost in the affected ear
 Usually unilateral
 Sounds may appear “tinny” (due to low-freq loss) and
distorted
 Loudness intolerance is common (abnormal sensitivity to
intense sounds)
(3) Tinnitus
 Ringing, roaring, or buzzing sensation
 Fluctuates in intensity but does not abate
 Pretty annoying
(4) Sensation of “fullness”

• Like the weird sensation you get on an airplane or elevator before
your ears pop – except it can’t be cleared.
• Cause of fullness sensation is unrelated to M.E. function
• Cause of Meniere’s
 The proximate (i.e., immediate) cause of MD is thought by some to
be excessive and fluctuating pressure in the endolymphatic fluid
that courses through the membranous labyrinth of the cochlea and
vestibular systems. This causes the membranous labyrinth to
balloon or dilate.
 Condition is known as endolymphatic hydrops.
 Result is progressive damage to the hair cells responsible for both
hearing and balance.
 Underlying cause of the fluid imbalance (if that actually is what’s
going on) is not known. Likely suspects – viral infection or
autoimmune disorder affecting production or absorption of
endolymph (duh).
Normal Ear Ear w/ Endolymphatic
Hydrops
Note bulging of membranous labyrinth

(www.tchain.com/otoneurology/disorders/menieres/men_eti.html)
Age and Sex Distribution:
MD is an Equal-Opportunity Disease
Moral: The disease strikes all ages and both sexes.

• Incidence (number of new cases diagnosed per year)
: Estimates vary, but probably somewhere between 100 and 200 new
cases per year per million (see citations below).
TJ Wilmot. Ménière's disorder. Clinical Otolaryngology 1979 4: 131-43.
J Stahle, C Stahle, K Arenberg. Incidence of Ménière's disease. Archives of Otolaryngology 1978 104:
99-102.
I Watanabe. Incidence of Ménière's disease, including some other epidemiological data.
Ménière's Disease: A Comprehensive Appraisal, ed WJ Oosterveld. 1983 J Wiley & Sons.
P Wladislavosky-Waserman, GW Facer, B Mokri, LT Kurland. Ménière's disease: a 30-year
epidemiological and clinical study in Rochester MN, 1951-1980. Laryngoscope 1984 94: 1098-1102.
D Celestino, G Ralli. Incidence of Ménière's disease in Italy. American Journal of Otology 1991 12:
135-8.
• Prevalence (number of cases present at any given time)

: 2,182 per million
P Wladislavosky-Waserman, GW Facer, B Mokri, LT Kurland. Ménière's disease: a 30-year
epidemiological and clinical study in Rochester MN, 1951-1980. Laryngoscope 1984 94: 1098-1102.
• Treatment
• Numerous: Everything from diet to medications (aimed at treating
nausea and vertigo) to surgery.
• Considerable debate about the effectiveness of various treatments.
• Medical treatment
: maintenance treatment, acute attack treatment
• Steroids, Diuretics, antihistamine, anticholinergics, antiemetics
• Treatment
clinical pathway
• Treatment
• A few surgical treatments deserve mention:
(1) Endolymphatic shunt
: Plastic tube installed to drain excessive fluid and reduce pressure
: There is controversy about effectiveness
– some have called it a “placebo” surgery.
(2) Vestibular Nerve Resection
• Vestibular branch of 8th N is cut, leaving cochlear branch intact
(therefore preserving residual hearing).
• Vertigo abates, but balance problems may persist.
• Treatment
(3) Destruction of the Affected Ear (pray for no mix-ups)
• In some severe (always or nearly always unilateral) cases of MD
that are unresponsive to other treatment, complete ablation of
the labyrinth is recommended.
• (Surgeon goes in through the EAM, removes the incus and
stapes, pokes a hole in the promontory, and removes the
sensory tissue in the labyrinth. In some cases the 8th N is cut as
well.)
• This surgery is pretty extreme – good indication of how
debilitating MD can be.
• To my knowledge, there have been no randomized clinical trials
of any surgical treatment for MD.
For your reference – Drug Therapy: Betahistine
There have been several well designed randomized clinical trials testing
the effectiveness of a synthetic histamine call betahistine hydrochloride in
treating vertigo/nausea. Results have been encouraging.
Dark bars=No. of attacks/month
Light bars=Duration of attacks
TJ Wilmot, GE Menon. Betahistine in Ménière's disease. Journal

of Laryngology and Otology 1976 90: 83340
IJC Frew, GE Menon. Betahistine hydrochloride in Ménière's
disease. Postgraduate Medical Journal 1976 52: 501-3.
A Fischer, L van Elfren. Betahistine in the treatment of
paroxysmal attacks of vertigo. A double blind trial (transl.). TGO
tijdschrift voor Therapie, Geneesmiddel en Onderzoek. 1985 10:
Fischer & van Elferen (1985) 933-7.
• Prognosis
• According to one source, vestibular symptoms can be controlled
(not prevented) in about 70% of patients, meaning that attacks of
vertigo will be prevented or will be reduced in severity/frequency.
• Tinnitus seldom disappears.
• If treatment is started while a patient's hearing is still fluctuating, it
is sometimes improved by medical management. In some patients,
however, hearing loss will continue to worsen.
e. Infections
 Bacterial or viral infections that invade the inner ear can cause SN HL
and disruptions of vestibular function.
 Generic term for infections that invade the inner ear: labyrinthitis.
Meningitis can sometimes spread to the inner ear and result in
labyrinthitis.
 Other infectious diseases: Mumps, measles, meningitis, encephalitis,
chicken pox, influenza, and syphilis can also invade the inner ear and
cause SN HL and/or vestibular symptoms
f. 8th N Tumors (acoustic neuroma)
• Benign (i.e., nonmalignant) tumor that exerts pressure on 8th N
• Almost always slow growing
• Most common symptom: hearing loss (mild initially), often
accompanied by tinnitus
• Vestibular problems may also occur
• Cause is unknown
• Continued tumor growth can be life threatening
• Treatment: Surgical removal or radiation
• Early detection is really important: Small tumors can be removed with
less risk of destroying the 8 N (and sometimes the 7th N as well).
th
• Early diagnosis is tough – early-stage symptoms are un-dramatic

• Acoustic neuromas sometimes run in families
g. Congenital Causes
• Congenital: Present at (or before) birth
• This is to be distinguished from acquired or adventitious hearing loss.
• Congenital etiologies may be hereditary or non-hereditary.
(1) Non-Hereditary Causes
(a) Maternal rubella (German measles)
When an expectant mother is exposed to rubella, the mother is not
in any great danger, but the fetus is – especially in the 1st trimester.
Effects can include:
Heart defects, brain damage, various visual impairments
SN HL, often profound
(a) Maternal rubella (German measles)
• Less common now since the development of a rubella vaccine
• A rubella vaccine was not available when the last rubella
epidemic occurred in 1964. A large proportion of the current
population of congenitally deaf adults lost their hearing as a
result of this epidemic. These folks are now in their mid-40s.
• The incidence of congenital deafness has been greatly reduced
in recent years since maternal rubella has come under better
control.
(b) Anoxia (asphyxia)
Insufficient oxygen during birth/delivery can cause all sorts of
problems for the newborn.
There’s hardly anything that’s not on the list of anoxia
consequences. SN HL is on the list.
(c) Many other etiologies that are less common
(2) Hereditary Causes
Genetic factors are thought to cause more than 50% of all incidents
of congenital hearing loss in children (NIDCD, 1989).
Two patterns:
(a) autosomal dominant (autosome = not a sex chromosome)
 One parent has a dominant gene for SN HL (and typically has a
hearing loss).
 There is at least a 50% probability that the child will also have a
hearing loss.
 Probability is higher if both parents have the dominant gene.
(2) Hereditary Causes
(b) autosomal recessive
 Both parents (typically with normal hearing) carry a recessive gene for
SN HL.
 Each child will have a 1 in 4 chance of inheriting the bum gene.
Approximately 80% of inherited hearing loss is autosomal recessive.
This makes early detection tough since, with both parents hearing normally,
the children are not considered at risk.
cf) Syndromes:
Inherited hearing loss can also be associated with a complex of inter-
related symptoms in the form of a syndrome. A few examples include:
 Waardenburg Syndrome
 Treacher-Collins Syndrome
 Klippel-Feil Syndrome
Treacher-Collins
Syndrome
Waardenburg
Syndrome

Hearing Disorder PDF

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Hearing Disorder PDF

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Hearing disorder

Han Young Hoon, M.D.

Intensity (dB HL)

Pure Tone Average (PTA) 10

From the audiogram above: 30

Intensity (dB HL)

incus prosthetic stapes

Drug Vestibulotoxicity Hearing Toxicity Toxic Level

Erythromycin yes High IV doses only

Metronidizole toxic (rarely) unknown

(4) Sensation of “fullness”

Note bulging of membranous labyrinth

Moral: The disease strikes all ages and both sexes.

• Prevalence (number of cases present at any given time)

Dark bars=No. of attacks/month

Light bars=Duration of attacks

TJ Wilmot, GE Menon. Betahistine in Ménière's disease. Journal

• Early diagnosis is tough – early-stage symptoms are un-dramatic

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