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Basic Definition, Classification,
andCharacteristics ofHearing Loss
AteşMehmetAkşit andFerdaAkdaş
9.1 Introduction
The role of auditory perception in the neural development of the brain is crucial.
Disorders of auditory function, from infancy to old age, signicantly affect the cognitive and social activities of individuals. Advances in technology have increasingly
enabled the correction of auditory function disorders. However, the success of the
treatment process relies on accurately and timely identifying the level of hearing
loss and potential pathological causes.
The goal of audiologic diagnosis is to determine the extent, cause, and impact of
hearing and balance problems using a variety of testing tools, considering the age of
the individual. Both objective and subjective tests of various types are used in audiological evaluations. The integral aspect of diagnosis lies in the combined evaluation
of these tests. In clinical practice, a unique test battery can be tailored to each case.
The basic criterion is to ensure that each step of the auditory process, from the external ear canal to the cerebral cortex, is examined in each case. Modern objective tests
in widespread use today provide reliable results in assessing various aspects of the
auditory process. Although not universally applicable, structures such as the outer
and middle ear, the outer hair cells of the cochlea, the auditory nerve, and the auditory pathways can be assessed by isolating them from other auditory processes. A
careful evaluation of the auditory process during the preparation of the audiological
report will highlight potential pathologies. Conrmation or exclusion of potential
pathologies highlighted in the audiologic report by additional medical tests ensures
a faster and more reliable denitive diagnosis.
9
A. M. Akşit (*)
Faculty of Health Sciences, Department of Audiology, Near East University,
Nicosia, Cyprus
F. Akdaş
Akademic Hospital, Audiology Clinic, Istanbul, Turkey
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2024
M. T. Kalcioglu et al. (eds.), Otology Updates, Comprehensive ENT,
https://doi.org/10.1007/978-3-031-76173-7_9
171
172
A. M. Akşit and F. Akdaş
9.2 Definition andClassification ofHearing Loss
Hearing loss is dened by its degree, type, and conguration. Pure-tone audiometry,
recognized as the gold standard for identication, efciently evaluates the entire
auditory process from the external ear to the cortex on a frequency basis. The type,
degree, and conguration of hearing loss are determined by pure-tone audiometric
thresholds at 250, 500, 1000, 2000, 4000, and 8000 Hz, with some clinics also
assessing thresholds between 3000 and 6000Hz.
9.2.1 Degree ofHearing Loss
The degree of hearing loss is typically classied by averaging thresholds at three
frequencies (500, 1000, and 2000Hz) or four frequencies (500, 1000, 2000, and
4000Hz) for pure-tone air conduction tests. Although the pure-tone average (PTA)
itself has no diagnostic value, it remains the most widely used criterion for categorizing the degree of hearing loss.
Figure 9.1 shows three audiograms. From left to right, the rst is from a patient
with a vestibular schwannoma in the left ear, the second is from a patient with auditory neuropathy spectrum disorder (ANSD), and the third is from a patient with
cochlear pathology. Despite the consistent PTA of 50dB in the left ear in all three
audiograms, the nature of the pathology causing the hearing loss, the conguration
of the hearing loss, and its impact on social communication vary from patient to
patient.
Various authors have classied the degree of hearing loss. The World Health
Organization (WHO) describes hearing below 25dB as normal but notes that individuals with hearing levels between 15 and 20dB may have some difculty hearing
[1]. Some researchers consider the range of normal hearing to be between −10 and
25dB [2]. The American Speech-Language-Hearing Association (ASHA) considers normal hearing to be between −10 and 15dB [3]. Table9.1 shows the ASHA
classication of hearing loss.
9.2.2 Types ofHearing Loss
Classication of hearing loss involves identifying the region responsible for the
hearing loss based on pure-tone threshold observations of air and bone conduction.
Hearing loss is typically classied into three types: conductive hearing loss (CHL),
sensorineural hearing loss (SNHL), and mixed hearing loss (MHL).
9.2.3 Conductive Hearing Loss
CHL is likely to be treated medically or surgically. The main factors that cause
CHL include
9 Basic Denition, Classication, andCharacteristics ofHearing Loss
173
Fig. 9.1 Auditory congurations across various pathologies
Table 9.1 American
Speech-Language-Hearing
Association’s classication of
hearing loss (Adapted from
the Ref. [3])
Hearing degree Hearing range (dB HL)
Normal hearing
Slight hearing loss 16–25
Mild hearing loss 26–40
Moderate hearing loss 41–55
Moderately severe hearing loss 56–70
Severe hearing loss 71–90
Profound hearing loss 91+
−10–15
• Blockage of the external ear canal with cerumen or a foreign object
• Infection of the external ear canal
• Eustachian tube dysfunction
• Infection of the middle ear
174
A. M. Akşit and F. Akdaş
• Perforation of the eardrum
• Rupture/fracture of middle ear ossicles due to trauma
• Congenital malformations of the external and middle ear
Audiological tests reveal cochlear reserve through bone conduction thresholds.
Bone conduction thresholds within normal limits indicate an intact cochlea. If bone
conduction thresholds are within normal limits but differ from air conduction
thresholds by more than 10dB, the condition is diagnosed as CHL (Fig.9.2).
9.2.4 Sensorineural Hearing Loss
SNHL is a type of hearing loss that results from pathology of the inner ear and/or
auditory nerve. While some sources classify pathologies occurring in the auditory
pathways extending to the cortex within SNHL [4], others categorize pathologies in
the auditory pathways and cortex as central pathology [5].
The hallmark of SNHL is that hearing thresholds are above normal limits, and
there is no signicant difference between air and bone conduction thresholds
(Fig.9.3).
Sensorineural hearing loss can be congenital or acquired. Factors causing congenital SNHL are generally genetic and syndromic. Other common factors include
illness during pregnancy, birth trauma, hyperbilirubinemia, and inner ear malformations. Factors that cause acquired SNHL include
• Viral diseases (mumps, meningitis, measles, etc.)
• Ototoxic drug use.
• Acoustic trauma.
• Head trauma.
• Age-related factors (presbycusis).
• Autoimmune diseases.
• Internal acoustic canal tumors.
As diagnostic tests have evolved, the classication of SNHL has proven inadequate in dening the pathology. Conditions previously considered within SNHL,
Fig. 9.2 Conductive hearing loss due to trauma
(ossicular chain dislocation)
9 Basic Denition, Classication, andCharacteristics ofHearing Loss
Fig. 9.3 Sensorineural hearing lossThe rst audiogram is an example of presbycusis, which
occurs with age and has progressive characteristics. The middle audiogram belongs to a patient
with Meniere’s disease. The last audiogram is an example of noise-induced hearing loss due to the
use of rearms.
175
such as internal acoustic canal tumors, ANSD, semicircular canal dehiscence
(SSCD), large vestibular aqueduct syndrome (LVA), and dead region, are now mentioned in audiologic evaluation reports.
9.2.4.1 Internal Acoustic Canal Tumors
While an accurate diagnosis of internal auditory canal tumors is often conrmed by
radiologic imaging techniques such as MRI and CT, audiologic test results may
provide compelling evidence of pathology [6, 7].
Unilateral or asymmetric hearing loss, unexpected declines in speech scores,
abnormal acoustic reex and auditory brainstem response (ABR) test results serve
as indicators of retrocochlear pathology (Fig. 9.4). The location and size of the
tumor can affect hearing and speech scores, as well as acoustic reex and ABR
results, in different ways.
9.2.4.2 Auditory Neuropathy Spectrum Disorder
ANSD is usually caused by genetic and anatomical factors and hyperbilirubinemia
[8]. It causes damage to the inner hair cells of the cochlea and/or the auditory nerve.
There may also be damage to the synaptic connections between the auditory nerve
and inner hair cells [9]. There is no typical pattern of degree and conguration of
hearing loss in ANSD (Fig.9.5).
Common test results observed in ANSB include
• Inconsistent test–retest results, resulting in an identiable air–bone gap.
• Lower-than-expected performance in speech tests.
• Normal tympanogram ndings if no other pathology is present, although acous-
tic reexes are absent.
• Obtainable otoacoustic emission (OAE) recordings.
• The presence of cochlear microphonics in ABR testing, but the absence of waves
I, III, and V (Fig.9.6).
176
Fig. 9.4 Some audiological ndings indicating unilateral or asymmetric hearing loss in internal
auditory canal tumors
A. M. Akşit and F. Akdaş
Fig. 9.5 Hearing threshold and speech scores in a child with auditory neuropathy, age 6–9
9 Basic Denition, Classication, andCharacteristics ofHearing Loss
Fig. 9.6 Auditory neuropathy spectrum disorderRefraction and condensation polarity recordings
at the top, alternate polarity recordings at the bottom, with cochlear microphonic recordings
enclosed within the circle
177
9.2.4.3 Third Window Syndrome
Third window syndrome (TWS) is a pathological condition resulting from dehiscence of the semicircular canals (SCD) or dilatation of the vestibular canal (LVA).
SCD and LVA have different characteristics in terms of the type, degree, and conguration of hearing loss. SCD is characterized by a pronounced air–bone gap,
especially at low frequencies, whereas LVA has a progressive, asymmetric conguration of hearing loss that is most prominent at high frequencies [10]. In TWS,
acoustic reexes can be obtained depending on the level of hearing thresholds.
Examples of hearing loss associated with SCD and LVA are shown in Fig.9.7. The
air–bone gap is particularly pronounced at 250Hz in both pathologies. In SCD,
bone conduction thresholds may be better than 0dB.
9.2.4.4 Dead Region
Hearing loss often results from damage to the hair cells in the cochlea. In certain
cases, there can be a complete lack of inner hair cell function in a particular region
of the cochlea, known as a “dead region” [11]. Accordingly, a threshold difference
of more than 20dB between successive frequencies indicates the possibility of a
dead region. Investigation of the effect of dead regions on audiograms and on speech
and tone perception reveals some notable ndings, some of which are (a) Dead
regions may be common in moderate-to-severe sensorineural hearing loss; (b)
Audiograms alone are unreliable for diagnosing dead regions; (c) Measuring thresholds in threshold equalizing noise (TEN) test provide a simple clinical diagnostic
method for dead regions; (d) Pure tones in dead regions may not consistently evoke
clear pitch sensations, and pitch clarity ratings are not reliable indicators of a dead
region [11].
178
ab
Fig. 9.7 Cases of (a) Superior Semicircular Canal Dehiscence (SCD) and (b) Large Vestibular
Aqueduct Syndrome (LVA)
A. M. Akşit and F. Akdaş
Fig. 9.8 Mixed type hearing loss
9.2.5 Mixed Hearing Loss
A hearing loss is considered mixed if it has both conductive and sensorineural characteristics. In mixed hearing loss, at least one of the bone conduction thresholds
must be higher than 15dB, and there should be at least 15dB difference between
the air and bone conduction thresholds. An example of mixed hearing loss (otosclerosis) is shown in Fig.9.8.