Open Access Medical Books

HEARING LOSS

HEARING LOSS
Edited by Sadaf Naz .

Hearing loss affects 278 million people world wide and is one of the most significant 
health problems. Deafness can be acquired due to environmental insults including 
injuries and infections. Additionally, about 50 % of hearing loss is genetic and 
scientists have identified nearly 100 genes implicated in deafness, while many more 
remain to be identified. This book brings together different topics on our current 
understanding of hearing loss.
The first section on “Hearing” presents a brief overview about normal hearing as well 
as the technology and instruments involved in its measurement including audiometry 
and auditory brainstem response as well as methods to record otoacoustic emissions.
Scientists have also investigated the listening scores of audio signals and the 
psychological impressions related to speech audibility. In the last chapter in this 
section, the suitability of the Mongolian gerbil is discussed, particularly as a model of 
structural and functional aspects of age-dependent hearing loss in humans.
The section on “Childhood Hearing loss” provides an overview of parental practices 
and behaviours related to children with and without hearing loss. The results 
indicated that the interactions established between mothers and children favour the 
acquisition and maintenance of social skills. Additionally, early detection of hearing 
loss and prompt intervention gives children with deafness a chance to develop spoken 
language comparable to those with normal hearing.
“Injuries & traumas” as cause of hearing loss are the focus of the third chapter in this 
book. This section includes a report on head injuries in India and the ensuing hearing 
loss. Symptoms of hearing loss were found in 10% of the patients with head trauma.
Sensorineural hearing loss was the most common finding in the affected individuals.
Additionally, perforation of the tympanic membrane is reported as a common finding 
in Nigeria due to trauma to the ear. The next two chapters explore the role of ototoxic 
agents such as solvents, pesticides and metals, and their interaction with noise in 
causing hearing loss. Research conducted in human subjects exposed to these 
chemicals indicates that these agents cause auditory dysfunction. The combined 
influence of noise and chemical pollutants on hearing loss confirmed the existence of 
an interaction between physical and chemical factors which influence the alteration of 
auditory function.
The “Genetics” of hearing loss is discussed in a series of review articles in the next 
section. An overview is provided of different genes involved in nonsyndromic and 
syndromic hearing loss and reflects the unparalleled heterogeneity of deafness genes.
Different modes of inheritance of deafness are also discussed. The genetics of 
recessively inherited, moderate to severe and progressive hearing loss in humans is 
covered in detail. Strategies are described about identification of modifiers of deafness 
genes. Common craniofacial abnormalities inherited together with hearing loss are 
discussed in the next chapter. The genes involved in causing Usher syndrome as well 
as different mouse models for each of these genes are also comprehensively reviewed.
The last section of the book presents the “Treatment” of hearing loss for both genetic 
and acquired cases of deafness. Cochlear implants are now accepted as the standard of 
care for children with severe to profound hearing loss. They have allowed many 
children to attend regular schools, and to develop their language, social and academic 
skills to levels that exceed those for their peers with severe to profound hearing loss 
using hearing aids. The efficacy of corticosteroid treatment through the eardrum and 
into the middle ear to treat various otologic disorders, such as Meniere’s disease and 
sudden sensorineural hearing loss is also reviewed. The last chapter discusses an 
ancient Chinese technique of acupuncture for treatment of sudden sensorineural 
hearing loss. The authors’ experimentally demonstrate improvement of the hearing 
loss in more than 20% of the individuals who underwent the treatment. Acupuncture 
has gained scientific ground after it was experimentally demonstrated that during this 
process adenosine is released and improves different symptoms in patients. Exactly 
how acupuncture may help in treating hearing loss remains to be determined.
It is hoped that the research and the reviews on various aspects of hearing loss 
described in this book will be of benefit to different students and the researchers 
working actively in this field. The coming years will see accelerated discoveries in 
many research areas discussed in this book, particularly in genetics of deafness due to 
the use of massively parallel sequencing technologies to identify disease genes.
Therapies based on genetic findings for treatment of hearing loss may also become 
possible in future.
Ms. Martina Blecic, the publishing process manager, Ms Ana Pantar, editor relations 
consultant and Ms Nina Dundovic, the technical editor contributed to editing of this 
book.

Sadaf Naz
School of Biological Sciences,
University of the Punjab, Lahore 54590,
Pakistan

CONTENTS :

Part 1 Hearing 1


Chapter 1 Technology for Hearing Evaluation 3
Josefina Gutierrez

Chapter 2 Contralateral Suppression of Otoacoustic Emissions: 
Working Towards a Simple Objective Frequency Specific Test for Hearing Screening 25
Nikolaus E. Wolter, Robert V. Harrison and Adrian L. James

Chapter 3 A Prediction Method for Speech Audibility 
Taking Account of Hearing Loss Due to
Aging Under Meaningless Noise 51
Takahiro Tamesue

Chapter 4 The Mongolian Gerbil as a Model for the Analysis of 
Peripheral and Central Age-Dependent Hearing Loss 67
Gleich Otto and Strutz Jürgen

Part 2 Childhood Hearing Loss 93


Chapter 5 Families of Children with Hearing Loss 
and Parental Educational Practices 95
Bolsoni-Silva Alessandra Turini and
Rodrigues Olga Maria Piazentin Rolim

Chapter 6 Early Intervention with Children Who Have a Hearing Loss: 
Role of the Professional and Parent Participation 117
Zerrin Turan

Part 3 Injuries & Traumas 133


Chapter 7 Hearing Loss in Minor Head Injury 135
Lingamdenne Paul Emerson

Chapter 8 Conductive Hearing Loss Due to Trauma 157
Olushola A. Afolabi, Biodun S. Alabi, Segun Segun-Busari and Shuaib Kayode Aremu

Chapter 9 Occupational Chemical-Induced Hearing Loss 171
Adrian Fuente and Bradley McPherson

Chapter 10 Exploration Databases on Occupational Hearing Loss 191
Juan Carlos Conte, Ana Isabel García, Emilio Rubio and Ana Isabel Domínguez

Part 4 Genetics 209


Chapter 11 Genetics of Hearing Loss 211
Nejat Mahdieh, Bahareh Rabbani and Ituro Inoue

Chapter 12 Genetics of Nonsyndromic Recessively Inherited Moderate to 
Severe and Progressive Deafness in Humans 247
Sadaf Naz

Chapter 13 Genetic Hearing Loss Associated with 
Craniofacial Abnormalities 275
S. Lunardi, F. Forli, A. Michelucci, A. Liumbruno, F. Baldinotti, A. Fogli, V. Bertini, A. Valetto, B. Toschi, P. Simi, A. Boldrini, S. Berrettini and P. Ghirri

Chapter 14 Usher Syndrome: Genes, Proteins, Models, Molecular 
Mechanisms, and Therapies 293
Jun Yang

Part 5 Treatment 329


Chapter 15 Cochlear Implants in Children: A Review 331
Julia Sarant

Chapter 16 Intratympanic Corticosteroid 
for Neurosensorial Hearing Loss Treatment 383
Malek Mnejja, Bouthaina Hammami, Amine Chakroun, Adel Chakroun, Ilheme Charfeddine and Abdelmonem Ghorbel

Chapter 17 Effects and Prognostic Factors of Acupuncture Treatment for 
Idiopathic Sudden Sensorineural Hearing Loss 393
Kyu Seok Kim and Hae Jeong Nam .


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