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Hipoacuzia Neurosenzoriala
Hipoacuzia Neurosenzoriala
Definition
The term “hearing impairment” refers to a lessen-
ing of hearing ability in the widest possible sense,
ranging from subjectively barely appreciable
impairments to total deafness.
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TABLE 1
The WHO classification of the severity of hearing impairment, with general clinical recommendations*1
*1
The mean hearing loss is calculated separately for each ear as the mean value of hearing loss for the four frequencies 500 Hz, 1000 Hz, 2000 Hz and 4000 Hz.
Modified from WHO: Grades of hearing impairment; www.who.int/pbd/deafness/hearing_impairment_grades/en/index.html
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FIGURE 1 Topographic-
functional
classification
of hearing
impairment
according to the level
of the lesion in the
organs of hearing
No study has yet addressed the question of the Common hallmarks of hearing impairment are repeated
relative prevalence of the various types of hearing im- questioning about things that have not been properly
pairment (classified by cause). heard, inappropriate answers to misheard questions,
The most common type of hearing impairment in and an excessively loud speaking voice.
childhood is transient conductive hearing loss due to a
tympanic effusion. 10% to 30% of children suffer from The state of the evidence for treatments of hearing impairment
this problem before their third birthday, with a preva- Randomized trials have been performed on middle-ear
lence as high as 8%. Congenital, permanent, bilateral surgery and on the provision of implantable hearing
hearing loss is much rarer, with a prevalence of 1.2 per aids and cochlear implants. Poorer evidence is avail-
1000 children. In adulthood, the most common type of able from clinical trials on the pharmacotherapy of
hearing impairment is the sensorineural hearing loss of acute inner-ear disorders, in particular sudden sensori-
old age (presbycusis), which affects 40% of all persons neural hearing loss. It can now be said that nearly every
aged 65 or older. The next most common types are per- kind of permanent hearing loss is treatable.
manent conductive or combined hearing loss due to chronic
otitis media (prevalence 1.5%) and hearing impairment The classification of hearing impairment
due to acoustic trauma (prevalence 0.05%) (3–8). The main classifications that are currently in clinical
use are based on the severity of hearing impairment, as
Clinical features assessed by pure-tone audiometry (Table 1), and on the
Persons with early hearing impairment can often com- basic topographic and functional distinction between
pensate for it for a relatively long time, e.g., by turning conductive hearing loss, sensorineural hearing loss and
up the volume of the radio or television set or (in unilat- central hearing loss (Figure 1). Other types of classifi-
eral hearing impairment) by turning the healthy ear to cation are by age (e.g., hearing impairment in child-
the sound source. As hearing impairment worsens, hood vs. in old age), temporal course, severity, and the
vision is used as an additional aid to speech recogni- pattern of variation of the auditory threshold as a
tion, with an increasing reliance on lip-reading. function of frequency on audiograms (9, 10).
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Figure 3:
a b a) Small choles-
teatoma in the
upper posterior
quadrant of the
tympanic
membrane with
destruction of
the crus of the
incus
b) View after
reconstruction
with an angular
prosthesis (as
described by
Plester), bridging
the defect be-
tween the incus
and the stapes
mucoserous tympanic effusion arises, leading to con- ity can itself conduct acoustic vibrations and thus partly
ductive losses of up to 40 dB over the entire frequency compensate for a pathological deficit (12). The
range. Tubal blockage is more common in children than treatment of choice is surgery, with the twin goals of
in adults because they have narrower Eustachian tubes eradicating infection and reconstructing the ossicular
and are more susceptible to middle ear infection. 10% chain (Figure 3).
to 30% of all children have a tympanic effusion leading
to conductive hearing loss at some time before their Otosclerosis
third birthday (3). This problem is much more likely to Otosclerosis, which has a clinical prevalence of 0.3% to
occur in children with anatomical malformations of the 0.4%, consists of fixation of the stapes leading to up to
palate and Eustachian tubes, including cleft lip, 40 dB of conductive hearing loss in the lower frequen-
maxilla, and palate, and in those with Down or Turner cies (13). It is the result of remodeling processes in the
syndrome. A tympanic effusion that has been present bony cochlear wall that can ultimately affect the stapes
for three months or longer should be treated with and the membrane of the round window. Its presumed
tympanic drainage, as well as adenotomy if necessary, causes include inflammation (autoimmune processes,
in order to prevent a disturbance of speech measles virus) as well as genetic, metabolic, and hor-
development (6). monal factors (e2). It is most common between the ages
of 15 and 40 years and is twice as common in women
Middle ear as in men. Hearing impairment due to otosclerosis can
Permanent conductive hearing loss: Permanent con- now be treated successfully in 94% of cases with
ductive hearing loss is generally caused by chronic bac- microsurgical stapedoplasty (14).
teria infection of the middle ear, affecting either the
mucosa (otitis media mesotympanalis) or the bone Perceptual hearing impairment
(cholesteatoma). Hearing is impaired as a result of (synonym: sensory hearing loss)
muffling of sound by granulations or cholesteatoma as The organ of Corti is the functional unit that transduces
well as enzymatic destruction or inflammatory fixation perilymphatic vibrations into neural signals (Figure 4).
of the tympanic membrane and ossicular chain. The de- Vibration of the basilar membrane leads to mechanical
gree of hearing impairment (in the 30-to-60-dB range) deflection of the stereocilia and thereby to electrolyte
is poorly correlated with the extent of tissue destruc- influx into the hair cells, causing depolarization. Motor
tion, because inflammatory tissue in the tympanic cav- proteins in the cell walls of the outer hair cells bring
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TABLE 2
Synopsis of the causes and clinical features of hearing impairment, with differential diagnoses for each hearing impairment syndrome
Conductive hearing loss Sensory hearing loss Neural hearing loss Central hearing loss
Cause – acoustic-mechanical distur- – dysfunction of the hair cells or – cochlear nerve dysfunction – dysfunction of the audtiory
bance of sound conduction in their synaptic connections to – delayed impulse conduction pathway or auditory cortex
the external auditory canal, the cochlear nerve; if the outer – disturbed neural encoding of (processing of bilateral audito-
across the tympanic mem- hair cells are affected, loss of the acoustic signal ry stimuli, synchronization, sig-
brane, or in the ossicular chain cochlear amplification and thus nal modulation, recognition,
of recruitment of intermediate noise suppression)
intensities
– blurring of frequency resolution
– reduction of temporal resolution
Clinical features If the cause is in the external – loss of intensity and dynamics – similar to sensory hearing loss, – there may be no disturbance
auditory canal: – soft noises or speech may be but usually unilateral of tone perception
– reduced sound intensity perceived as either too soft or – speech perception worse than – impaired rapid speech proces-
(sound is perceived as soft) too loud tone perception sing
– often, distorted perception – impairment of sound localiza-
If the cause is in the tympanic tion, poor understanding of
membrane or ossicular chain: speech with superimposed
– altered sound frequency and noise, impairment of auditory
intensity (high and low tones memory
may be either softer or louder)
Differential Acute: Acute: – acoustic neuroma – infarction
diagnosis – blockage by cerumen – idiopathic sudden sensorineu- (= vestibular schwannoma) – hemorrhage
– tubular catarrh ral hearing loss – other tumors of the petrous – tumor
– tympanic effusion – acute noise-induced trauma bone or cerebellopontine angle – multiple sclerosis
– traumatic eardrum perforation – blast trauma (meningioma, chordoma, chon- – auditory processing disorder
– acute otitis media or externa – explosion trauma drosarcoma)
– bacterial/viral labyrinthitis – compression syndrome
Permanent:
– canal stenosis/atresia Hereditary/permanent::
– defect of eardrum or ossicular – hereditary hearing impairment
chain due to chronic purulent – presbycusis
infection of the mucosa – noise-induced hearing impair-
– cholesteatoma ment
– malformation – toxic (incl, drug-induced)
– otosclerosis hearing impairment
– tympanosclerosis – idiopathic chronic progressive
hearing impairment
– drug side effects
– lasting sequelae of infections
and sudden hearing loss
Audiological – tuning-fork test – tuning-fork test – pure-tone audiogram – test of hearing at a distance
testing – whispering test – whispering test – speech audiogram – pure-tone audiogram
– test of hearing at a distance – test of hearing at a distance – supraliminal tests – speech audiogram
– pure-tone audiogram – pure-tone audiogram – auditory fatigue tests – supraliminal tests
– impedance audiometry – speech audiogram – electric response audiometry – auditory fatigue tests
– otoacoustic emissions – electric response audiometry
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dB) in which the rise of pressure takes place over a very BOX 2
short time (<1.5 ms). In contrast, explosion trauma in-
volves a slower rise of pressure (> 2 ms) and leads to
rupture of the tympanic membrane. Both of these types Ototoxic causes of sensory hearing loss (after e4)
of acoustic trauma cause acute hearing loss with tinni-
tus that usually lasts for several hours. In Germany, 28
● Medications
– aminoglycoside antibiotics (gentamicin, streptomycin, tobramycin, amikacin)
to 107 per 100,000 persons sustain a blast trauma each
– cytostatic agents (cisplatin, cyclophosphamide)
year on New Year’s Eve (19). Another type of acoustic
– diuretics (furosemide, ethacrynic acid)
trauma is acute noise trauma, which results from expo-
– other (salicylates, quinine)
sure to relatively loud noise over a longer period of
time (seconds to hours), e.g., at rock concerts. In such ● Industrial substances
situations, hearing impairment is mainly due, not to – heavy metals (mercury, lead, arsenic)
structural injury of the sensory cells, but rather to a – solvents (aminobenzenes, nitrobenzenes)
severe metabolic disturbance (oxidative stress), which – other (carbon monoxide, fluorocarbons, organosulfur compounds, carbon tetra-
can be either reversible or irreversible depending on its chloride)
duration and can manifest itself symptomatically as ● Abused substances
loss of hearing in the high-frequency range (ca. 4 kHz), – cocaine, heroin, tobacco, alcohol
and tinnitus in the same range. Potential treatments in-
clude the intravenous infusion of rheological agents ● Viral and bacterial toxins
and cortisone. – bacterial toxins (pneumococci, staphylococci, streptococci, Haemophilus)
in: otitis media, meningitis, scarlet fever, sepsis, syphilis
Hearing impairment due to acute toxic – viral infections: mumps, measles, rubella, influenza, HIV, cytomegalovirus
damage to the inner ear ● Metabolic causes
Medications and bacterial and viral toxins can reach the – vitamin B12 deficiency, hyperlipidemia, folic acid deficiency
inner ear by way of the membrane of the round
window, the cerebrospinal fluid, or the bloodstream and
irreversibly damage the hair cells. Medications that are
well known to be ototoxic include the aminoglycosides,
cytostatic agents, loop diuretics, salicylates, and
quinine. The risk of hair-cell damage from medications ● trauma, 4.2% (e.g., blast trauma, skull-base frac-
can be reduced by careful monitoring of serum concen- ture),
trations (Box 2). ● cardiovascular disease, 2.8%, and
Bacterial toxins and inflammatory mediators that are ● paraneoplastic involvement of the inner ear, 2.2%.
called forth by viral infection can also have a toxic ef- In 71% of cases, however, the diagnostic algorithm
fect on the inner ear (labyrinthitis). Toxic labyrinthitis fails to reveal any cause (idiopathic sudden sensorineu-
can occur, for example, in influenza otitis, purulent ral hearing loss). Models of the etiology and patho-
meningitis, and chronic otitis media. Systemic viral in- genesis of this disorder generally proceed from the
fections (mumps, measles, rubella, cytomegalovirus, assumption of an unknown viral, vascular, or immuno-
HIV) reach the labyrinth by way of the bloodstream logical cause that leads to a disturbance of homeostasis
(e4). in the inner ear (e5).
Hearing impairment due to acute toxic Ototoxic causes of sensory hearing loss
damage to the inner ear • Medications, industrial chemicals
Medications that are well known to be ototoxic in- • Substances of abuse
clude the aminoglycosides, cytostatic agents, loop • Viral and bacterial toxins
diuretics, salicylates, and quinine. • Metabolic abnormalities
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processes, particularly as they affect the microvascular mors can be treated either by surgical removal or by
blood supply of the hair cells (sensory type), with re- stereotactic radiation procedures (“radiosurgery”); for
sulting ischemia, hypoxia, and oxidative stress. Such some patients, close observation is a further option
processes can also affect the ganglion cells (neural (including serial MRI scans and tests of hearing and
type) or the stria vascularis (metabolic type). About vestibular function).
40% of persons over age 65 suffer from presbycusis Auditory synaptopathy/neuropathy is a special type
(7). There is no known way to prevent it; it can be of neural hearing loss affecting the synapses of the
treated symptomatically with the provision of a hearing inner hair cells that can arise either in isolation or as a
aid if the auditory threshold shift is 30 dB or more in component of a generalized neuropathy. There is a dis-
the speech-frequency range. Severe hearing impair- turbance of temporal processing that may lead to total
ment in old age with loss of speech comprehension can blockage of impulse conduction (22).
be treated with a cochlear implant.
Central hearing loss
Occupationally induced hearing impairment The auditory signal, originally consisting of mechan-
Noise-related hearing loss accounts for 40% of all cases ical pressure waves, is processed and encoded in vari-
of recognized occupational disease and is thus the most ous forms at multiple levels of the auditory pathway.
common of all occupational diseases (e6). The main The higher the neural dysfunction is located, the more
causative factor is the quantity of energy transferred complex the hearing disturbance; thus, the patient may
into the inner ear, i.e., the intensity of noise combined have difficulty in recognizing certain signals amid
with its duration. Hearing impairment generally arises acoustic noise, in disentangling simultaneous speech
after years of exposure to noise above 85 dB for the signals, or in recognizing timbre.
entire working day. Damage to the outer hair cells Adults can suffer from central hearing impairment as
typically begins in the 4 kHz range (revealed as a C5 a result of trauma, inflammatory/infectious processes,
drop on pure-tone audiometry) (21). infarction, and space-occupying lesions (tumors,
Another type of hearing impairment due to long- hemorrhage) that affect the auditory pathway and the
term exposure is chronic, toxic occupationally induced auditory centers. Bulbopontine hearing impairment is
hearing impairment. It is caused by years of exposure usually associated with central dizziness, ataxia, and
to substances such as heavy metals, benzenes, and vari- other neurological abnormalities pointing to brainstem
ous other carbon compounds. A typical feature of this involvement. Its diagnosis is confirmed by brainstem
kind of hearing impairment is a symmetric rise of the audiometry with measurement of the intermediate po-
auditory threshold (Box 2). tentials. Midbrain hearing impairment (e.g., in multiple
sclerosis) is accompanied by clinically evident neur-
Neural hearing loss ological abnormalities (motor and sensory deficits);
This category includes all types of hearing impairment specific questioning is required to elicit the history of
due to diseases affecting the cochlear nerve (including paracusis (false acoustic perception) and diplacusis
its synapses). The common causes of neural hearing (hearing of double tones) (23). Special hearing tests can
loss are tumors (meningioma, acoustic neuroma, chor- also reveal abnormalities of directional hearing and of
doma, chondrosarcoma) and inflammatory destruction rapid speech recognition.
of the petrous bone (cholesterol granuloma, cholestea- A special type of central hearing loss affecting
toma). school-age children is known as “central auditory pro-
Acoustic neuroma (more properly, “vestibular cessing disorder” (CAPD). This is a dysfunction of cen-
schwannoma”) is the most common cause of neural tral, modality-specific auditory processing. It is de-
hearing loss, with an incidence of 1.74 per 100 000 per- bated whether CAPD truly constitutes an independent
sons per year (e7). Any patient with unilateral loss of disease entity, as some claim that it is seen only as a
bone conduction on pure-tone audiometry, without any component finding in the setting of developmental dis-
immediately recognizable cause, should undergo brain- orders, intellectual impairment, multimodal perceptual
stem potential recording (BERA) and an MRI scan with disorders, and activity and attention disorders (24). Its
contrast medium. The management of acoustic treatment is interdisciplinary and involves specific
neuroma is now an interdisciplinary matter, as these tu- training of the deficient auditory functions.
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Conflict of interest statement 20. Chau JK, Lin JR, Atashband S, Irvine RA, Westerberg BD: Systematic
Prof. Zahnert has received reimbursement of travel fees for lectures and work- review of the evidence for the etiology of adult sudden sensorineural
shops as well as scientific lecture honoraria from the following companies: hearing loss. Laryngoscope. 2010; 120: 1011–21.
Med-El Deutschland, Cochlear GmbH, ATOS, MIP Pharma GmbH, Merck Phar-
ma GmbH, and HNO-Update GmbH. He is active as a consultant and is en- 21. Brusis T: Berufliche Lärmschwerhörigkeit. Diagnose, Differenzialdiag-
gaged in scientific collaboration with the following companies, with financial nose und Begutachtung. Trauma Berufskrankh 2006; 8: 65–72.
support of research projects: Kurz-Medizintechnik GmbH, Med-El, Cochlear
22. Moser T, Strenzke N; Meyer A, et al.: Diagnostik und Therapie der
GmbH, and Omega Consulting GmbH. Prof. Zahnert has 11 patents pending for
middle-ear implants and implantable hearing systems. auditorischen Synaptopathie/Neuropathie. HNO 2006; 54: 833–9.
23. Schorn K, Differenzialdiagnose der Hörstörung. In: Nauman H: Diffe-
Manuscript submitted on 26 January 2011; revised version accepted on 17 rentialdiagnostik der Schwerhörigkeit, Stuttgart: Thieme 1990;
May 2011. 56–94.
Translated from the original German by Ethan Taub, M.D. 24. Nickisch A, et al.: Auditive Verarbeitungs- und Wahrnehmungsleis-
tungen. Laryngo-Rhino-Otol 2009; 88: 469–76.
REFERENCES
Corresponding author
1. Mathers C, Smith A, Concha M: Global burden of hearing loss in the Prof. Dr. med. habil. Dr. h. c. Thomas Zahnert
year 2000. Global Burden of Disease. Geneva: World Health Organi- Universitäts-HNO-Klinik Dresden
zation, 2000: 130. Fetscherstr. 74
2. Sohn W: Schwerhörigkeit in Deutschland, Repräsentative Hörscreen- 01307 Dresden, Germany
Thomas.Zahnert@uniklinikum-dresden.de
ing-Untersuchung bei 2000 Probanden in 11 Allgemeinpraxen. Z Allg
Med 2001: 77; 143–7.
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4. Gross M, Finckh-Krämer U, Spormann-Lagodzinski M: Angeborene
@ For eReferences please refer to:
www.aerzteblatt-international.de/ref2511
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Please answer the following questions to participate in our certified Continuing Medical Education
program. Only one answer is possible per question. Please select the answer that is most appropriate.
Question 1 Question 6
Defects of the tympanic membrane cause what kind of From what age onward is there a 40% prevalence of presbycusis?
hearing impairment? a) From age 25 onward
a) Conductive hearing loss b) From age 35 onward
b) Perceptual hearing loss c) From age 45 onward
c) Sensory hearing loss d) From age 55 onward
d) Central hearing loss e) From age 65 onward
e) Auditory processing disorder
Question 7
Question 2 Which of the following diagnoses is associated with central hearing loss?
What is the most common cause of transient hearing a) Menière’s disease
impairment in childhood? b) Otosclerosis
a) Acute purulent otitis media c) Auditory processing disorder
b) Congenital malformation of the middle ear d) Acoustic neuroma
c) Otosclerosis e) Presbycusis
d) Tympanic effusion
e) Chronic otits media
Question 8
A patient complains of difficulty hearing, stating that he cannot understand or
Question 3 carry on a conversation from a distance of more than 1 meter. His pure-tone
Which of the following classes of medications is not audiogram shows an intermediate degree of hearing loss (30 dB).
oto-toxic? According to the WHO classification, how severe is this man’s hearing
a) Loop diuretics impairment?
b) Cytostatic agents a) Grade 0, no impairment
c) Salicylates b) Grade 1, slight impairment
d) Corticosteroids c) Grade 2, moderate impairment
e) Aminoglycosides d) Grade 3, severe impairment
e) Grade 4, profound impairment including deafness
Question 4 Question 9
What is most commonly found to be the cause of sudden During what period of time should neonatal auditory screening for the
sensorineural hearing loss? detection of congenital hearing loss be performed?
a) A local disease of the ear a) When the baby is no more than 30 days old
b) A viral infection b) When the baby is no more than 3 months old
c) A cardiovascular disease c) When the baby is no more than 7 days old
d) An acoustic neuroma d) On the day the baby is born
e) No determinable cause e) When the baby is no more than 2 weeks old
Question 5 Question 10
What frequency is most severely affected in incipient During what period of time does congenital hearing loss tend to
noise-induced hearing impairment? progress?
a) 500 Hz a) In the first six months
b) 1 kHz b) In the second half of the first year
c) 8 kHz c) Before the third birthday
d) 4 kHz d) From the first to the third birthday
e) 15 kHz e) From age 2 to age 5
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eReferences
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