Original Paper

Folia Phoniatr Logop 2011;63:43–48 Published online: August 10, 2010

DOI: 10.1159/000316412

Definitions of Types of Hearing

Impairment: A Discussion Paper
Katrin Neumann a Dafydd Stephens b
a
Department of Phoniatrics and Pediatric Audiology, University of Frankfurt am Main,
Frankfurt am Main, Germany; b School of Medicine, Cardiff University, Cardiff, UK

Key Words particular, the division between retrocochlear hearing

Auditory neuropathy ⴢ Central auditory disorders ⴢ Hearing disorders, central hearing disorders, central auditory
impairment processing disorders, and auditory neuropathy is not al-
ways clear and often confuses students of acoustics and
audiology or physicians who specialize in hearing prob-
Abstract lems and related fields.
Objective: To develop a clear and meaningful set of defini- Clear definitions are necessary both for aetiological
tions of types of hearing impairment. Method: A critical anal- diagnosis and for appropriate and optimal habilitative or
ysis was made of previous definitions together with a con- rehabilitative management of the individual concerned.
sideration of current knowledge of functions and dysfunc- Appropriate definitions, adapted to the current state of
tions of the auditory system. Results and Conclusions: A scientific knowledge, can also contribute to the patient’s
coherent set of definitions compatible with the contempo- acceptance of their condition. Furthermore, definitions,
rary understanding of auditory disorders and their percep- as far as they can be made, are absolutely necessary for
tual effects is proposed. Copyright © 2010 S. Karger AG, Basel educational purposes.
Within this brief overview, we shall discuss early and
more recent definitions and concentrate on those most
appropriate in the current state of knowledge. Here, we
Introduction shall focus on definitions relevant to clinical diagnosis
and shall specifically exclude both quantitative defini-
Over the years a range of definitions of different types tions and also the differentiation between impairment
of hearing impairment has been proposed, starting with and disability. In the latter context, we regard the World
a separation of conductive and sensorineural impair-
ment. Such definitions have changed and been elaborated
on with time, with increasing the number of subdivi-
sions. However, overlapping, unclear or absent defini- This article is based on a paper presented at the IAPA Summer
tions have resulted in some confusion in the past. In School, Berlin, April 2008.

© 2010 S. Karger AG, Basel Prof. Katrin Neumann, MD

1021–7762/11/0631–0043$38.00/0 Department of Phoniatrics and Pediatric Audiology
Fax +41 61 306 12 34 University of Frankfurt am Main, Theodor-Stern-Kai 7, House 7A
E-Mail karger@karger.ch Accessible online at: DE–60590 Frankfurt am Main (Germany), Tel. +49 69 6301 5775, Fax +49 69 6301 5002
www.karger.com www.karger.com/fpl E-Mail Katrin.Neumann @ em.uni-frankfurt.de
Health Organization’s ‘International classification of
functioning, disability and health’ [1] as the most useful Auditory cortex
source, albeit with certain limitations. Medial geniculate

Historical Origins Inferior colliculus

CAPD
The first separation of hearing impairment into what
Lateral lemniscus
is now known as conductive and sensorineural hearing KKS ?
impairment came with the work of Capivacci in 1603 [2]. Superior olive
He argued that if a patient could not hear the sound of a
zither connected via a metal rod held between his teeth, Cochlear nucleus
AN
he had ‘labyrinthine’ deafness. This led some two centu-
Cochlear nerve
ries later to the development of the Weber and Rinne tests
using tuning forks. Towards the end of the 19th century, Inner hair cells
the separation of cochlear and neural disorders was de-
Outer hair cells
scribed by Eitelberg [3] and Corradi [4] using measures of
abnormal adaptation to the stimulus presented. Middle/outer ear

Developments in Audiometry
Fig. 1. Recent diagnostic classifications of King Kopetzky syn-
With the development of valve audiometers, more elab- drome (KKS; also known as obscure auditory dysfunction), audi-
orate and consistent tests were developed, leading to a cat- tory neuropathy (AN), and central auditory processing disorder
egorization of conductive, cochlear, retrocochlear, and (CAPD).
central disorders. The advent of the assessment of oto-
acoustic emissions enabled a distinction between hearing
disorders affecting solely the outer hair cells from others.
The term conductive hearing impairment, which is high resolution CT and MRI imaging, and three-dimen-
still useful, encompassed disorders of both the outer and sional reconstruction of the auditory structures.
middle ears, delineated by a comparison of air-conduc- Central auditory dysfunction is generally regarded as
tion and bone-conduction thresholds with more detailed beginning in the cochlear nuclei and extending to all
information coming from ear microscopy, otoadmit- higher levels in the auditory pathway. A wide range of
tance (impedance) measures, and high resolution CT and tests of central auditory function was developed follow-
MRT scans. ing the pioneering work of Bocca and co-workers [6] and
Sensorineural hearing impairment comprised the two were concerned with the diagnosis of lesions at various
elements cochlear, or sensory, and retrocochlear, or neu- levels from the cochlear nucleus to the auditory cortex.
ral, which were considered important in the delineation Such tests purported to separate those arising from the
of vestibular Schwannomas and other conditions, leading brainstem from the more central lesions, particularly
to a differentiation of neural lesions from cochlear disor- those caused by a lesion in the auditory cortex.
ders. In addition, the terms ‘perceptive hearing impair-
ment’, and ‘nerve deafness’ were often regarded in many Recent Definitions
ENT circles, particularly in the UK, as synonymous with Over the past 20 years, a number of new terms have
sensorineural hearing impairment. However, the condi- been introduced for a number of different reasons. Such
tion was one of sensation rather than perception and gen- reasons include the development of improved diagnostic
erally originated in the cochlear end-organ rather than in techniques, the clarification of previously obscure dis-
the auditory nervous system. ease entities and the desire to integrate diagnostic con-
Retrocochlear has been defined as ‘a lesion in the audi- cepts with educational and rehabilitative approaches.
tory pathway after leaving the cochlea but before entering Terms applied include (central) auditory processing dis-
the brainstem’ [5]. Test batteries to separate cochlear and orders ((C)APD), auditory neuropathy and King Kopetz-
retrocochlear disorders comprised measures of otoacous- ky syndrome (obscure auditory dysfunction). There is
tic emissions, recruitment, intensity discrimination, ab- much overlap between these conditions, which are sum-
normal adaptation and speech recognition. Such an ap- marized in figure 1, and also between them and earlier
proach was largely discarded with the introduction of definitions.

44 Folia Phoniatr Logop 2011;63:43–48 Neumann /Stephens

Table 1. Recent definitions of (C)APD, King Kopetzky syndrome and auditory neuropathy

Type of auditory disorder Definition

(C)APD A deficit in neural processing of auditory stimuli that is not due to higher-order language,
cognitive or related factors, yet (C)APD may lead to or be associated with difficulties in
higher-order language, learning, cognitive and communication functions.
King Kopetzky syndrome Perceived auditory disability or disabilities in association with an essentially normal pure-tone
(obscure auditory dysfunction) audiogram. The condition could be regarded as primarily a psychogenic (auditory stress) disorder,
and in many cases is associated with a subclinical cochlear disorder.
Auditory neuropathy A set of auditory disorders with poor speech recognition arising in the inner hair cells, in the
cochlear nerve or in the synaptic connection between them, which share the following diagnostic
findings: combination of normal otoacoustic emissions and/or cochlear microphonics with absent
or severely abnormal click-evoked auditory brainstem responses at high stimulus levels.

Table 2. Central auditory disorders due to demonstrable brain lesions

Type Sub-categories Examples

2.1. Central deafness mostly due to extended (bilateral) temporal Auditory agnosia (cortical deafness)
lesions Perceptual form of auditory agnosia
Developmental auditory agnosia
Non-verbal agnosia (environmental sound agnosia)
Verbal agnosia
Amusia
2.2. Moderate central auditory deficits due to circumscribed Disturbed perception of contralaterally presented stimuli
lesions in the auditory cortex or in the acoustic radiation (unilateral lesions)
Hemianacusia
Audiogenic memory disturbances
2.3. Central auditory disorders due to neurodegenerative diseases Multiple sclerosis, Alzheimer’s disease
2.4. Central auditory disorders associated with aphasia or other Wernicke’s aphasia, transcortical aphasia, global aphasia,
central speech/language disorders disconnection syndrome
Amusia: Type of auditory agnosia, inability to recognize and tifying non-verbal or environmental sound. Perceptual form au-
enjoy music. Auditory agnosia: Inability to interpret (recognize) ditory agnosia: Sound cannot be interpreted although it is de-
sounds but relatively normal pure-tone hearing on audiometry. tected; mostly due to bilateral or left-hemispheric lesions of the
Developmental auditory agnosia: A congenital form of amusia temporal cortex, but also due to subcortical lesions, e.g. of cap-
with deficits in the perception of music (tone deafness, tune deaf- sula interna or insula. Verbal agnosia: Only perception of spoken
ness). Hemianacusia: Heavily disturbed or no hearing on the language is disturbed but speech production, reading, and spell-
contralateral ear due to unilateral temporal or callosal lesions. ing are normal.
Non-verbal agnosia: Selective difficulty in recognizing and iden-

Even the term (C)APD has been the subject of different is not an appropriate term to use here as a number of ele-
definitions in the USA and UK [7–9]. A recent definition ments of auditory processing have already been shown to
in the Handbook of (Central) Auditory Processing Dis- occur within the cochlea [e.g. 11]. In addition, the exist-
orders [10] is shown in table 1. ing definitions of (C)APD are somewhat restrictive. Such
Furthermore, the rationale behind the use of the term restricted definitions may be useful from the standpoint
(C)APD is not clear and would appear to be related to two of audiometric tests, but have less value from a clinical
competing schools of thought. We would argue that APD diagnostic standpoint.

Definitions of Types of Hearing Folia Phoniatr Logop 2011;63:43–48 45

Impairment
 Table 1 also gives definitions of King Kopetzky syn- memory and attention, neurodegenerative alterations,
drome [12] and of auditory neuropathy [13]. King Kopetz- demonstrable brain lesions or states of altered con-
ky syndrome has been known as obscure auditory dys- sciousness but without peripheral hearing disorders.
function [14] and auditory disability with normal hearing (c) CAPDs associated with peripheral hearing disor-
[15] as well as by a range of other designations [12]. It has ders but without cerebral deficits. (d) CAPDs associ-
been argued recently that it is one of the group of ‘medi- ated with both peripheral hearing disorders and cer-
cally unexplained diseases’ comprising difficulty in hear- ebral deficits.
ing in noise with a normal pure-tone audiogram [16]. Its (2) Central auditory disorders due to demonstrable brain
basis, while generally including minimal auditory dys- lesions. (a) Central deafness mostly due to extended
function, may be entirely psychological or linguistic, and (bilateral) temporal lesions. (b) Moderate central audi-
for that reason we prefer to use the term King-Kopetzky tory deficits due to circumscribed lesions in the audi-
syndrome rather than its various synonyms [17]. Sensi- tory cortex or in the acoustic radiation. (c) Central au-
tized tests of hearing may demonstrate minor pathology ditory disorders due to neurodegenerative diseases. (d)
in many parts of the auditory system, most commonly in Central auditory disorders associated with aphasia or
the cochlea, but sometimes in the central auditory system other central speech/language disorders.
[e.g. 17]. (3) Other abnormal central auditory activities such as cer-
Auditory neuropathy can involve dysfunction from tain forms of tinnitus and abnormal central auditory
the inner hair cells to the lower brainstem, resulting in processing during states of altered consciousness such
poor speech recognition and abnormal auditory brain- as autistic disorders, schizophrenia and coma [21–23].
stem responses to sound stimuli. Its impact and manage- This category is concerned with other abnormal cen-
ment will depend very much on whether the lesion is lo- tral auditory activities.
cated in the inner hair cells and their synapses with the
cochlear nerve, or more centrally. It has also been labelled
as ‘auditory dys-synchrony’ [e.g. 18]. The peripheral ele- Recommendations
ments of it have been named ‘auditory synaptopathy’ [e.g.
19] or ‘endocochlear hearing loss’ [e.g. 20]. At the present time, with rapid developments in our
understanding of the auditory system, it is difficult to be
precise in this respect. We would argue in favour of a split
Central Auditory Disorders in the definition of auditory neuropathy between disor-
ders arising from the inner hair cells, their synapses with
We argue here that current audiological definitions of the cochlear nerve and those disorders arising more cen-
central auditory disorders are too limited, excluding a trally. As mentioned above, the terms ‘auditory synap-
range of related conditions. They generally exclude pa- topathy’ and ‘endocochlear hearing loss’ have been pro-
tients with any associated conditions which are very like- posed for the former, particularly in the context of audi-
ly, given the nature of lesions affecting the brain. At the tory lesions related to the Otoferlin gene (DFNB9). The
same time it is, anatomically, an all-embracing condition first term is somewhat problematic as it would appear to
including lesions in most parts of the central auditory exclude a specific lesion of the inner hair cells and the fact
pathways and so has little value from a diagnostic and that there is a range of different synapses throughout the
functional standpoint. Thus, from an aetiological stand- auditory system. The second term (endocochlear hearing
point, we would argue that central auditory disorders loss) is clearly unacceptable as it could be taken to include
may be subclassified into: lesions in any part of the cochlea.
(1) Central auditory processing disorders (CAPDs) (which However, in the absence of a better term, together with
are further subdivided in a later section). (a) CAPDs the fact that the vast majority of lesions affecting the in-
without any cognitive deficit, without a peripheral ner hair cells and their synapses with the cochlear nerve
hearing disorder and without any hearing disorders dendrites arise from alleles of the Otoferlin gene, at the
due to demonstrable brain lesions or states of altered present time we would favour the term ‘auditory synap-
consciousness. This can be regarded as the (C)APDs topathy’ to refer to lesions arising in the synapses between
according to recent definitions including the defini- the inner hair cells and the dendrites of the cochlear
tion from ASHA [9]. (b) CAPDs associated with ce- nerve, and ‘cochlear sensory lesion’ for disorders arising
rebral deficits such as cognitive deficits involving specifically in the inner hair cells. This term could be

46 Folia Phoniatr Logop 2011;63:43–48 Neumann /Stephens

 With regards to CAPDs, Baran and Musiek [24] have
Locus Lesion type suggested a division, on the basis of functional effects,
into low brainstem, high brainstem, auditory cortex and
Corpus callosum Interhemispheric pathways
Auditory cortex Cortical interhemispheric pathways, which seems a reasonable
Medial geniculate Midbrain standpoint at the present juncture. These and other cur-
Inferior colliculus High brainstem rent recommendations are summarized in figure 2.
Lateral lemniscus Low brainstem The definition of central auditory disorders must take
Superior olive
Cochlear nucleus Auditory neuropathy into consideration the fact that many conditions exist
Cochlear nerve other than ‘pure’ CAPDs and that auditory disorders due
Spiral ganglion Auditory synaptopathy to demonstrable brain lesions and other abnormal central
Inner hair cells Cochlear sensory auditory activities constitute a considerable proportion of
Outer hair cells Cochlear motor
Middle/outer ear Conductive central auditory disorders [25]. Sometimes, hearing defi-
cits are the first or only manifestations of central patho-
logical processes such as ischemia. If a hearing distur-
Fig. 2. Proposed terminology of hearing disorders. Note: Disor- bance does not fit with pure-tone audiogram, differenti-
ders resulting in King Kopetzky syndrome may occur in any part ated tests of central auditory functions are recommended
of the auditory system from the cochlea to the cortex. [26].
For CAPDs we recommend not only to consider the
pure (C)APDs but also their combinations with other ce-
rebral deficits and with peripheral hearing disorders,
which should be specified. Thus, for example, we may re-
considered to differentiate such lesions from true audi- fer to an ‘auditory cortical disorder associated with amu-
tory neuropathy, which should be reserved for lesions of sia’ or ‘auditory cortical disorder associated with tinnitus’.
the somata and axons of the cochlear nerve and the co- Finally, as King Kopetzky syndrome can cover disor-
chlear nuclei. ders in any part of the auditory system from the cochlea
Outer hair cell lesions are generally related to the mo- to the auditory cortex, as well as psychological disorders,
tility of the outer hair cells, which is essential for fine fre- we propose that this be retained as a generic term for pa-
quency tuning, and we consider the term ‘cochlear motor tients with poor speech recognition but a normal audio-
lesions’ to be the most relevant in such cases. gram.

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48 Folia Phoniatr Logop 2011;63:43–48 Neumann /Stephens

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