Audiology

Audiology

Mary Anne Maltby and Pamela Knight

Mary Anne Maltby and Pamela Knight

A David Fulton Book

6.69 x 9.61 .27 6.69 x 9.61

 Audiology
An Introduction for Teachers and Other Professionals

MARYANNE TATE MALTBY

AND PAMELA KNIGHT

I� ��o����n���up
LONDON AND NEW YORK
First published 2000 by David Fulton Publishers

This edition published 2015 by Routledge

2 Park Square, Milton Park, Abingdon, Oxon OX14 4RN
711 Third Avenue, New York, NY 10017 USA

Routledge is an imprint of the Taylor & Francis Group, an informa business

Note: T he right of Maryanne Tate Maltby and Pamela Knight to be identified as

the authors of this work has been asserted by them in accordance with the
Copyright, Designs and Patents Act 1988.

Copyright ©Maryanne Tate Maltby and Pamela Knight 2000

British Library Cataloguing in Publication Data

A catalogue record for this book is available from the British Library.

ISBN 978-1-853-46665-6
ISBN 978-0-203-46232-4 (eiSBN)

All rights reserved. No part of this publication may be reproduced, stored in a

retrieval system or transmitted in any form, or by any means, electronic,
mechanical, photocopying, recording or otherwise, without the prior
permission of the publishers.
Contents

Foreword v
Acknowledgements vi
Introduction vii
Glossary IX

1 The Ear and How it Works 1

Anatomy and physiology of the ear 1
Aetiology: types and causes of deafness 5
Conductive hearing loss 6
Sensori-neural or nerve hearing loss 8
Implications of unilateral hearing loss 10
Non-organic hearing loss 11
2 The Physics of Hearing 13
An introduction to sound 13
Frequency (pitch) 14
Intensity (loudness) 15
Phase and phase cancellation 17
Hearing threshold 19
3 Assessment of Hearing Loss 24
Hearing tests 24
Behavioural tests 29
Objective hearing tests 36
Assessment of children with complex needs 37
Implications for teachers 38
4 Amplification Systems 38
Hearing aids 38
How the hearing aid works 38
Types of hearing aid 43
1v Audiology

5 The Acoustic Environment 54

Factors within the acoustic environment affecting speech
intelligibility 54
Controlling the acoustic environment 57
6 Systems in the Classroom 61
The problem in the classroom 61
Alternative forms of amplification 61
7 Cochlear Implants 70
Components of a cochlear implant system 71
The paediatric cochlear implant team 72
Guidelines and criteria 73
Stages of implantation 74
The role of the teacher of the deaf 75
Issues relating to cochlear implants 76
8 Management and Maintenance of Hearing Aids 79
Personal hearing aids 79
Radio hearing aids 82
Cochlear implants 84
The hearing aid test box 85

9 Assessing the Benefits of Hearing Aids 88

Tests used in the assessment of hearing aid benefit 88
10 Teachers' Roles and Responsibilities 95
Audiological services 95
The educational audiologist 96
The role of the teacher of the deaf 96
11 Developing Spoken Language 102
Developing listening skills 102
Facilitating language development 104
The role of the teacher of the deaf 105
The role of the speech and language therapist 106
Appendix 1: Audiological descriptors 109
Appendix 2: Checking hearing aids 110
Appendix 3: Checking radio hearing aids 112
Appendix 4: General instructions for using a test box 113
References 114
Index 116
Foreword

The primary aim of this book is to meet the audiological needs of those
studying to become teachers of the deaf, as well as other teachers and
professionals working with deaf students and their families. It will also
be of interest to anyone new to the field of deafness and deaf education.
This book is intended as an introduction to audiology for those who
have little or no previous knowledge. It offers a basic knowledge of
audiology to a depth required by teachers of the deaf on training courses
and also gives enough information to allow the reader access to the more
specialised journal articles and textbooks. The book addresses the
practical implications of audiological support for deaf pupils both in the
home and in the school setting.
Acknowledgements

The authors wish to express their gratitude to the following people for
their help: Sue Archbold (Nottingham Cochlear Implant Team) and
Louisa Booth (Advanced Bionics) for their most helpful comments with
regard to cochlear implants; Nicola Knight for the freehand diagrams
and her innovative ideas; David Gaszczyk for the computer drawn
diagrams and for helpful comments and suggestions.
 Introduction

A firm knowledge of audiology is essential for all those working with

deaf and hearing impaired pupils. Audiology is a scientific subject,
which is continually developing and changing. It is important that those
who are involved with deaf pupils are responsible for their own
continuing professional development in this area. This means
personally updating their audiological knowledge through courses,
current literature, the internet and journal articles, as well as working
closely with other professionals, such as educational audiologists and
members of the cochlear implant teams. This book is intended to
provide a basic knowledge of audiology, as required by teachers of the
deaf, speech and language therapists and others, and to give them
sufficient knowledge to access more specialised literature.
Teachers of the deaf are specialist teachers, whose firm base in
audiological knowledge is a fundamental part of their specialism. They
also need to be competent in the practical areas of assessment and the
management of amplification systems. The teacher's knowledge of
audiology has many implications for their work.
Firstly, they will be a rich source of information about all aspects of
deafness for parents, mainstream teachers and others involved in
working with deaf children, as well as for deaf children themselves.
When working with the parents of young deaf children, teachers need to
understand the effect that the initial diagnosis of deafness can have,
upon both deaf and hearing families, and be able to discuss all aspects of
deafness from an informed and sensitive standpoint.
Secondly they need to be conversant with all audiological assessment
procedures and be able to interpret and discuss the implications of test
results with parents and subsequently with other teachers. They need to
have a clear understanding of the implications of different types and
degrees of deafness in social and educational environments.
Thirdly, they need to be conversant with the most appropriate
amplification system for the individual child and be able to work in
partnership with families and schools on the effective management of
hearing aids and the listening environment. They have a role to play in
assessing the effectiveness of hearing aids in the 'real world', at home
viii Audiology

and school, and are in a unique position to report back to the clinic
setting on the effectiveness of prescribed hearing aids.
Teachers of the deaf also need to be conversant with the implications
of deafness for language development and the curriculum. They have to
be able to contribute to individual education programmes (IEP) which
are tailored to the needs of each hearing impaired child, whatever the
educational setting. The implementation of audiological knowledge and
skill with deaf pupils and their families, in the educational and in the
home setting, is a fundamental role of the teacher of the deaf.
Where pupils are developing sign language as a first language, the
ultimate aim is one of competent sign bilingualism. This means English
(or the language of the country) will become their second language. All
deaf children have the right to develop competent literacy skills and
spoken language skills in their second language. To this end,
audiological support will focus on the accurate assessment of deafness,
appropriate amplification and acoustic conditions. Input to the
development of listening and spoken language skills should be equal
with those deaf children with whom an oral approach is being used.
In the context of this book, the authors have not taken a rigid
standpoint on the use of the such terms as 'deaf', 'hearing impaired' and
'hearing loss'. Instead they have reflected terminology that is in
common use and sometimes the terms are used interchangeably.
Generally, the term 'deaf' has been used in relation to losses of a more
profound nature and 'hearing impaired' for those of a mild or moderate
nature, but no hard and fast rules have been followed.
This book is especially for all those interested in deaf children who do
not yet have a theoretical base in audiology. It does not require prior
knowledge of audiology but will furnish the basics and allow access to
other books and material for those who wish to extend their knowledge
further.
 Glossary

acoustic conditioning - training a child to respond to a sound (stimulus)

by means of a reward.
acoustic energy - sound.
acoustic feedback - unwanted sound which leaks from an amplification
system (usually in the form of a high pitched whistle).
acquired deafness - forms of deafness that have been acquired, e.g.
through trauma or infection such as otitis media.
audible frequency range- frequencies the human ear is capable of hearing.
audiogram - a graph showing a person's hearing threshold levels.
audiological descriptors - standard terminology used to describe degrees
of deafness and the shape of the audiogram.
audiology- the study of all aspects of hearing and hearing loss.
auditory feedback- perception of the speaker's own voice.
auditory information - information gained through hearing.
auditory pathway - channel through which sound travels (outer, middle
and inner ear).
auditory processing- interpretation of acoustic information.
bilingual- describes an individual who uses two or more languages in
their everyday lives.
binaural hearing - hearing in both ears.
monaural hearing- hearing in one ear only.
unilateral hearing- hearing in one ear only.
British Sign Language (BSL) - the natural language of the adult deaf
community in the UK.
cochlear implant - a device implanted into the cochlea, which aims to
stimulate the auditory nerve directly to give a sensation of hearing.
component frequencies - tones which make up a specific sound.
conductive hearing loss - deafness associated with problems in the outer
and/ or middle ear.
congenital hearing loss - this refers to deafness which may be in-
herited, or due to environmental factors, such as illness during
the mother's pregnancy.
first language - a child's first language is normally the language of their
home environment and of the wider society in which they live. For
x Audiology

deaf children it is the language they prefer to use, regardless of the

home language.
glue ear- a common condition in young children related to infections in
the middle ear.
hearing status- this term is used when considering a person or family in
terms of whether they are a hearing family, a deaf family or a family
with both deaf and hearing members.
mild hearing loss -a hearing loss of between 20 dBHL- 40 dBHL.
moderate hearing loss - a hearing loss of between 41 dBHL- 70 dBHL.
oral method - the method of developing language in deaf children which
precludes the use of any form of formalised signs.
oral/aural communication - the use of the vocal and auditory tract
respectively for expressive and receptive communication.
phonology- the sound system of language.
preferred language - this term is used to describe the language a child
would most easily acquire and develop to a level most appropriate to
their age and development.
prelingual deafness - deafness that has been apparent before a person has
begun to develop a language.
postlingual deafness- deafness that has been acquired after the person has
partially or fully developed their spoken language.
profound hearing loss - a hearing loss greater than 90 dBHL.
recruitment - the sensation of abnormal loudness growth in relation to
input often associated with sensori-neural hearing loss.
semi-circular canals - part of the structure of the inner ear which is
concerned with balance.
severe hearing loss - a hearing loss of between 71 dBHL- 95dBHL.
sign language - the visual I gestural language of the deaf community. In
the UK this language is British Sign Language; in the USA it is
American Sign Language. It is a naturally evolved visual gestural
language with its own grammatical structure and lexicon.
sign bilingual - describes an individual who engages with and uses sign
language and the language of the hearing community.
transduce - change from one state to another.
 Chapter 1
The Ear and How it Works

Anatomy and physiology of the ear

The ear can be divided into three parts. The outer ear, the middle ear and
inner ear (Figure 1.1).

The outer ear

The outer ear comprises:
• the pinna or auricle;
• the ear canal or external auditory meatus;
• the ear drum or tympanic membrane.

ear canal brain

ossicles semi-
circular
eardrum
canals

Eustachian
tube

From ---+-'1:---1~
outer
ear

Outer ear Middle ear Inner Ear

Conductive pathway Sensori-neural pathway

Figure 1.1 The complete hearing system (after Tate 1994)

2 Audiology

The pinna or auricle is a structure of pliable cartilage with a tight

covering of skin. It is a complex shape designed to collect sound waves,
particularly from a forward direction, and funnel them into the ear canal
and to the ear drum. The pinna enhances sound reception by about SdB
and assists with the localisation of sound.
The ear canal, or external auditory meatus, is a tube approximately 2.5
em long which is closed at the inner end by the ear drum or tympanic
membrane. The outer third of the ear canal is composed of cartilage,
which is continuous from the pinna. This part of the ear canal contains
ceruminous glands. These secrete cerumen or wax which helps to
protect the ear canal and to moisturise the air in the canal. Wax, which is
a mixture of the secretions of sebaceous and sweat glands, mixes with
the skin debris which collects in the ear, and migrates naturally
outwards, eventually coming out of the ear as a brown moist secretion.
Wax is water resistant and inhibits bacterial growth thus protecting the
ear from infection. Wax is a natural substance and should not normally
be artificially cleaned out of the ear canal.
The inner two thirds of the canal are composed of bone lined with
skin, which becomes very thin in the deeper parts of the canal.
The ear drum or tympanic membrane is the membrane found at the
end of the canal, separating the outer ear from the middle ear. It is
composed of three layers:
• the outer layer is a skin or epithelial layer;
• the middle layer is a fibrous layer;
• the inner layer is a mucosal layer.
The ear drum itself is divided into an upper and a lower section. The
upper section is the smaller. It has no fibrous layer and is called the pars
flaccida. The lower and larger portion of the membrane is called the pars
tensa.
The ear drum vibrates in response to sound waves (acoustic energy)
funnelled down the ear canal. These vibrations pass from the ear drum to
the bones in the middle ear. The ear drum changes, or transduces, the
acoustic energy into mechanical energy in the middle ear.

The middle ear

The middle ear, Figure 1.1, is an air filled cavity beyond the ear drum. A
chain of three bones, called ossicles, is supported by ligaments and
muscles and bridges the middle ear cavity. The three bones in the
ossicular chain serve to link the outer ear to the inner ear. They have a
vital function in the transference of sound energy from the outer ear to
the inner ear and can increase the intensity of the sound by about 28 dB.
 The Ear and How it Works 3

The chain consists of the malleus (hammer) which has a handle and a
head. The handle is attached to the ear drum and can be seen though the
ear drum by means of an otoscope. The head of the malleus is attached
to the incus (anvil) by a joint. The anvil makes contact with the head of
the stapes (stirrup). The footplate of the stapes is attached to the oval
window, which is the entrance to the inner ear.
Sound passes most effectively through the middle ear when the air
pressure in the middle ear is equal to the atmospheric pressure. This
pressure equalisation is maintained by the Eustachian tube.
The Eustachian tube is about 3.5 em long and runs in an inward and
downward direction, from the middle ear, opening into the
nasopharynx or throat. The function of the Eustachian tube is to balance
the air pressure in the middle ear and also to drain the middle ear of any
gathering fluid or mucus.
The middle ear also serves to protect the delicate inner ear structures
from any potentially damaging noises. In response to loud sounds,
especially low frequency (see Chapter 2), the middle ear muscles
contract, causing the ossicular chain to stiffen and so transfer sound less
effectively.
The middle ear ends at the oval and round windows, which separate
the middle ear from the inner ear. These windows are membrane-
covered holes in the bony wall of the cochlea.

The inner ear

The inner ear consists of all those parts of the auditory system beyond
the middle ear. Sound waves pass through the oval window to the
cochlea where they are converted into electrical signals, which travel
along the nerve or neural pathways to the auditory centre of brain. The
inner ear is concerned with both hearing and with balance. The cochlea
is concerned with hearing while the three semi-circular canals are
concerned with balance and are part of the vestibular system. The
cochlea and the semi-circular canals make up a fluid filled cavity, which
is set in the petrous portion of the skull.

The cochlea
The cochlea looks rather like a snail shell, has two and a half coils and is
about the size of a pea. A cross-section of the cochlea (see Figure 1.2),
allows us to see that the cochlea has three fluid-filled 'galleries'. These
are:
• the scala media,
• the scala vestibuli and
• the scala tympani.
4 Audiology

Reissner's membrane

Scala media

Nerve

Tectorial membrane

Corti's arch

Tunnel of Corti

Nerve fibres

Figure 1.2 Cross-section of the cochlea (after Tate 1994)

The Organ of Corti lies in the central gallery of the cochlea. It consists of
rows of hair cells served by nerve fibres. The hair cells are responsible for
the transduction of mechanical vibrations received from the middle ear,
into electrical impulses. The sound waves travel along the basilar
membrane and 'deform' it. Because of the movement, nerve impulses are
sent along the nerve fibres. The nerve fibres join to form the auditory nerve,
which is part of the eighth cranial nerve. The cochlea is the site where
sounds are first analysed into their component frequencies. Different parts
of the basilar membrane are sensitive to different frequencies of the sound
spectrum (like a piano keyboard). The base of the cochlea is sensitive to
high frequency sound waves and the apex of the cochlea is sensitive to low
frequency sound waves. All sound waves must travel across the base of the
cochlea and maximum 'wear and tear' occurs in this area. Therefore high
frequency hearing losses are the most common.
 The Ear and How it Works 5

The auditory nerve and brain

The auditory nerve carries the signal to the hearing centres of the brain.
The brain interprets frequency according to the part of the cochlea from
which the nerve impulses are sent and the intensity according to the
number of nerve impulses received.
The area of the brain that is primarily involved in decoding sensory
information is the cerebral cortex. Signals from each ear are sent to both
left and right hemispheres of the brain but most information is projected
to the opposite side of the body. Thus neurons from the left cochlea
project predominantly to the right cortex and vice versa. At a functional
level, the cortical auditory regions in the left and right hemispheres have
different specialisations. The left hemisphere is specialised for language
processing (Binder et al. 1996) and the right hemisphere for processing
music (Penhune et al. 1999).

Aetiology: types and causes of deafness

Types of deafness
Audiologically the ear can be divided into conductive and sensori-
neural pathways in relation to the transmission of sound energy. The
type of deafness is largely related to the place in the hearing system
where the cause of deafness is situated.
• Conductive hearing loss is deafness resulting from any malfunction or
abnormality which prevents or reduces the conduction of sound
waves through the outer or middle ear to the oval window of the inner
ear.
• Sensori-neural hearing loss or 'nerve' deafness is hearing loss that
occurs in the inner ear, i.e. the cochlea, the auditory nerve, the
auditory pathway or the hearing centres of the brain. This prevents,
reduces and distorts the sounds reaching the auditory cortex.
• Mixed hearing loss describes the presence of sensori-neural deafness
with some conductive deafness in addition.
• Unilateral or monaural hearing loss is deafness that is apparent in one
ear only. It may be conductive or sensori-neural or mixed. The child
can hear well in one ear but may have difficulties in noisy
surroundings and with locating the direction of sound (see later in this
chapter).

Causes of deafness
Most causes of deafness in children fall into three categories:
• hereditary (congenital);
• peri-natal (that which occurs around the birth);
• acquired (occurring during a child's lifetime).
6 Audiology

These can affect the conductive pathway, the sensori-neural pathway, or

both.

Conductive hearing loss

Causes of conductive hearing loss
Hereditary conductive hearing loss is largely caused by:
• Anatomical abnormalities - these arise in the womb as the ear is
developing and can include an absence of the outer ear, atresia
(occlusion or closure of the ear canal) and the absence of ossicles.
Peri-natal conductive hearing loss is largely caused by:
• Fluid and debris in the ear canal, which is usually treatable.
Acquired conductive hearing loss is largely caused by:
• Otitis media or inflammation of the middle ear. This is one of the most
common disorders in children. The most usual cause is the loss of
eustachian tube function, through upper respiratory tract infections
(throat and nose infections) impairing the function of the middle ear.
This condition, also called glue ear, is discussed further in the
following section.
• Foreign bodies- children sometimes push small items, for example
beads, into their ears. If these block the ear canal, they will cause some
degree of deafness.
• Otitis externa or inflammation of the outer ear. If the ear canal swells
or if there is discharge, this may also cause hearing loss. The whole
canal can be affected or a localised area. In either case the ear canal is
very tender.
• Impacted wax. Wax may accumulate and turn brown and hard in the
ear canal . It will cause hearing loss if it blocks the ear canal. This
deafness may be accompanied by a 'buzzing' tinnitus.
• Otosclerosis is a condition in which the movement of the stapes is
restricted by an abnormal growth of bone in the ossicular chain.
(Otosclerosis is a hereditary condition which is not present at birth but
which develops later in life).
• Stenosis is a closure or narrowing of the ear canal. (Atresia is similar
but present at birth).

Glue ear (Otitis Media)

The most common cause of conductive hearing loss is otitis media,
commonly known as 'glue ear'. This is a prevalent condition in children
under eight years old. About 15-20 per cent of children in the two to five
year age range will have 'glue ear' at any one time. Glue ear gives rise to
 The Ear and How it Works 7

fluctuating hearing loss, which is largely a passing condition but it is

important to realise that when it is present it can significantly affect
children's hearing at a crucial time in their linguistic development.
Otitis media occurs when the Eustachian tube is unable to keep the
middle ear ventilated, often due to ear or throat infections. Fluid in the
middle ear is then also unable to drain away. This problem is most
common in young children because the Eustachian tube is narrow and
situated horizontally, which impedes ventilation and drainage. As
children mature, the Eustachian tube widens and becomes angled
downwards towards the throat thus facilitating the drainage process
(see Figure 1.3).

(i) young child Iii) adult

Figure 1.3 The Eustachian tube in child and adult

During ear and throat infections the oxygen in the air in the middle
ear is gradually used up and this creates negative pressure (a vacuum) in
the middle ear. The ear cannot allow this to happen and to compensate,
a watery fluid leaks from the walls of the middle ear, preventing a
vacuum from forming. Although at first the fluid is thin and watery, as
time passes, it can become thicker and glue-like'. Hence the term glue
1
I

ear'. In the medical profession it is also called otitis media with effusion,
secretory otitis media, and serous otitis media. These terms are
descriptive of the condition along a continuum from the early
accumulation of the fluid to the development of glue ear. As the fluid
accumulates, it impedes the function of the ossicular chain in the middle
ear to transmit sound energy in the form of vibrations. This causes a
dullness of hearing, or some degree of deafness, particularly in the low
frequencies. The fluid should drain away via the Eustachian tube when
the infection is resolved and the tube is no longer blocked. However, if
the fluid has reached glue-like thickness the secretion may not be able to
drain away even when the infection is resolved. Glue ear may therefore
become a long-standing condition. There are implications for children
who have frequent bouts of otitis media and glue ear including:
8 Audiology

• levels of deafness;
• auditory processing difficulties;
• lower educational attainments;
• restricted language development.
Children with fluid in the ear frequently have hearing loss that fluctuates
from day to day and even between the two ears. They have problems
associated with the deafness, but also have particular problems due to its
fluctuating nature. Fluctuating hearing loss may affect children's ability to
localise sound, thus making it difficult for them to identify the person
speaking and so they may miss parts of the spoken message. As words may
not sound the same from one time to the next, the child's ability to process
sound and subsequently develop language may also be impaired. This in
turn may affect their educational progress.
An inability to follow conversations easily can lead to issues of
behaviour management both at home and in the classroom. Poor
acoustic conditions compound the problems for children with glue ear.
Fluid in the middle ear is commonly diagnosed using tympanometry
(see Chapter 3). Treatment includes the use of antibiotics and
antihistamines for the infection. When the problem is long standing,
surgery is often undertaken to insert grommets (see Figure 1.4). This
minor operation involves making a small hole in the ear drum through
which the fluid is removed. The hole is then kept open for a period of
time by inserting a ventilation tube (grommet). There are several types
of grommet including a T tube grommet commonly used for long term
ventilation of the middle ear. The hole in the eardrum gradually heals
and the grommet then naturally comes out via the ear canal.

(i) A 'Shah' grommet (ii) grommet in place (iii) cross-section of grommet in place

Figure 1.4 Grommets (after Tate 1994 and Ballantyne 1993)

 The Ear and How it Works 9
Effects of conductive hearing loss on sound perception
In conductive hearing loss, sound is prevented, to a greater or lesser
degree, from reaching the inner ear. All sounds are available to the inner
ear but the loudness (intensity) will be reduced or not perceived at all.
Although with a conductive hearing loss the sounds reaching the inner ear
may be reduced in intensity, they are not distorted. Generally conductive
deafness can be addressed medically and at least responds well to the use
of hearing aids.

Sensori-neural or nerve hearing loss

Causes of sensori-neural hearing loss
The most common cause of sensori-neural hearing loss is maturity (age-
related hearing loss is called 'presbyacusis'). The majority of adults over
the age of 50 years will begin to experience some loss of hearing acuity,
generally in the high frequency range of hearing. Most sensori-neural
hearing loss both in adults and children primarily affects the high
frequencies of the sound spectrum. Other causes of sensori-neural
hearing loss in children include:

Congenital causes:
• Genetic: Inherited deafness is related to the chromosomatic makeup of
the parents. Chromosomes determine all our physical functions
including hearing. There are dominant (or active) and recessive (or
weaker) chromosomes. The dominant one governs the hearing status
of the child. It is the match of chromosomes between parents that
affects the hearing status of the child and it is possible for one parent
to have a dominant chromosome which is a carrier of sensori-neural
hearing loss even though they themselves are hearing.
• Infection in expectant mothers, e.g. maternal rubella, cytomegalovirus
(CMV) and syphilis, can cause sensori-neural hearing loss and other
developmental problems in babies. (Deafness through maternal
rubella has been largely addressed by early inoculation). The effect
upon the developing embryo depends to a great extent upon the stage
of the pregnancy. Infection in early pregnancy is the most likely cause
of damage to the unborn child.

Peri-natal causes (around birth):

• prematurity- some sensori-neural hearing loss is known to occur in
cases of severe prematurity, although improved medical intervention
can address many of the issues arising.
• anoxia -lack of oxygen to the baby immediately after birth is thought
10 Audiology

to be linked to sensori-neural hearing loss, although identification of

'at risk' babies has ameliorated this problem to a large extent.
• jaundice - is related to high levels of bilirubin in the blood which
result in jaundice. This damages the nerve of hearing at the brain stem.
• rhesus incompatibility - this is related to the blood grouping which
can be either rhesus positive or negative. If a mother with a positive
rhesus factor is carrying a baby with a negative rhesus factor then anti-
bodies form in the blood of the mother and can be passed onto the
baby with possible harmful effects to the baby, and their hearing
mechanism. This situation is well documented and largely addressed
by medical intervention.

Acquired causes (post-natal):

• bacterial and viral infections, e.g. mumps, measles and meningitis,
which may affect one or both ears. These are the most common causes
of unilateral hearing loss;
• head injuries;
• ototoxic drugs e.g. streptomycin, quinine etc.
• over-exposure to loud noise;
• tumours on the acoustic nerve.

Effects of sensori-neural hearing loss on sound perception

Sensori-neural hearing loss is a result of damage to the inner ear and is
generally permanent. It is likely to be the result of local damage to a
particular site in the cochlea or group of nerve fibres. Therefore this form
of deafness can produce both distortions in sound perception, where
specific frequencies of sound may be affected, and also reduced
responses to sound. An example of this is in high tone hearing loss
where response to high frequency tones is seriously affected, while that
for low tones may be near to normal. This causes a distortion in the
auditory information arriving at the auditory cortex.

Implications of unilateral hearing loss

Children with unilateral hearing loss are often considered by the general
public as having 'normal hearing', because they tend to manage well in
most situations. Unilaterally deaf children may produce good free-field
speech test results in quiet conditions, have no speech production
problems, use the telephone, hear the television and have no noticeable
difficulty in understanding. Clearly the degree of deafness is a factor in
the potential difficulties caused by unilateral hearing loss, but teachers
should not underestimate those difficulties. These include:
The Ear and How it Works
Maltby, M. (2001) Chapter 2 'Anatomy and physiology of the ear' and Chapter 3 'Medical aspects of hearing
loss', in Principles of Hearing Aid Audiology , 2nd edn. London: Whurr Publishers.

The Physics of Hearing

Maltby, M. (2001) Chapter 1: 'Acoustics', in Principles of Hearing Aid Audiology , 2nd edn. London: Whurr
Publishers.
Northern, J.L. and Downs, M.P. (latest edition) Chapter 1: 'What is hearing loss?', in Hearing in Children .
Baltimore: Williams and Wilkins.

Assessment of Hearing Loss

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Amplification Systems
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The Acoustic Environment

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Systems in the Classroom
Berg, F. (1993) Chapter 5, 'Individual amplification systems' and Chapter 6, 'Sound field devices in
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Cochlear Implants
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Management and Maintenance of Hearing Aids

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Assessing the Benefits of Hearing Aids

Lewis, D. E. (1997) Chapter 9, 'Selection and assessment of classroom amplification', in McCracken, W.
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Teachers' Roles and Responsibilities

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Developing Spoken Language

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Carr, G. (1997) 'Development of listening skills', in McCracken, W. and Laoide-Kemp, S. (eds) Audiology in
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Dyar, D. (1994) 'Monitoring progress - the role of the speech and language therapist', in McCormick, B. et al.
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