John M. Graham

Glenis K. Scadding

Peter D. Bull

Editors

Pediatric ENT

#John M. Graham Glenis K. Scadding Peter D. BullEditors

Pediatric ENTWith 355 Figures and 79 Tables

123

Mr. John M. GrahamRoyal National Throat, Nose and Ear Hospital330 Gray’s Inn RoadLondon WC1X 8DAUnited Kingdom

Dr. Glenis K. ScaddingRoyal National Throat, Nose and Ear Hospital330 Gray’s Inn RoadLondon WC1X 8DAUnited Kingdom

Mr. Peter D. BullSheffield Children’s HospitalWestern BankSheffield S10 2THUnited Kingdom

Library of Congress Control Number: 2007923510

ISBN 978-3-540-33038-7 Springer Berlin Heidelberg New York

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Children are special! This book is special!Hospitals for sick children were founded in the first

decade of the 20th century, and even before. Dedicated nurses and doctors with an interest in children were pio-neers in those child-friendly hospitals, although diagnos-tic and therapeutic techniques were modest.

Currently, medical and surgical disciplines cooper-ate with paramedical professionals and specially trained nurses in the treatment of children. The contribution of professionals with an expertise in biochemistry and bio-physics has also become essential, since routine proce-dures and research are dependent on advanced and ad-vancing technology.

The scope of pathologists has broadened to the cel-lular and subcellular level. Molecular medicine is mak-ing progress. Information technology has contributed to the development of disciplines like epidemiology, clinical epidemiology and genetics. Modern imaging techniques produce data pertinent to the anatomy and metabolism of pathological processes.

Pediatric otorhinolaryngology has largely benefited from these new technologies and data produced by re-cent research. Moreover, interdisciplinary teamwork in audiology, vestibular pathology, neuro-otology, speech and hearing disorders, and allergy and genetics, together

with the contributions of intensive care specialists, pedia-tricians and anesthetists have all contributed to raise the quality of patient care in pediatric otorhinolaryngology.

The biannual international congresses of the European Society of Pediatric Otorhinolaryngology (ESPO) have provided a prominent worldwide forum, presenting the latest research and evaluation of new and current prac-tice, and acting as a meeting point for all colleagues with an interest in treating children with otorhinolaryngol-ogy-related pathology.

It is my great pleasure to welcome and recommend this book. We should be most grateful to John Graham, former president of the ESPO, Glenis Scadding, eminent in the field of allergy, and Peter Bull a distinguished pe-diatric laryngologist, and appreciate their great efforts to present a state of the art account of our specialty. They have brought together a group of eminent authors, pre-senting recent advances in all aspects of pediatric oto-rhinolaryngology. You will certainly appreciate their up-to-date information and critical evaluation of the field of pediatric otorhinolaryngology.

Carel VerwoerdSecretary General

European Society of Pediatric Otorhinolaryngology (ESPO)

Foreword

This book has been written to fill a need for a basic but comprehensive text dealing with all aspects of paediat-ric ENT. It is aimed at trainees in our specialties who are developing an interest in the paediatric aspects of their work, at established ENT surgeons and audiologists who have decided to specialise in this most enjoyable subspe-cialty, or who wish to know more about paediatric ENT, and as a work of reference for those who encounter par-ticular problems in their paediatric patients.

The scope of paediatric ENT has grown over recent years to the extent that doctors may now subspecialise in paediatric otology, laryngology or rhinology rather than dealing with all aspects of the field. It was therefore neces-sary to cover a large number of different topics in consid-erable depth.

We have been truly fortunate in our authors, who come from many parts of Europe, the USA and South Africa. All share our great enthusiasm for our specialty, as we hope the reader will recognise. We are grateful to them all for working to a short timescale. This has allowed us to put together a fairly large multi-author book in a relatively short time, with up to date contributions. Our authors are active in international academic work and meet each other regularly at the international gatherings of the European Society of Pediatric Otorhinolaryngol-ogy, American Society of Pediatric Otolaryngology and Society for Ear, Nose and Throat Advances in Children, as well as at the many national paediatric ENT societies that have been formed in the last 25 years.

Paediatric ENT has developed, during the careers of the editors, from being considered a relatively simple and minor part of our professional life into a broad, complex and often difficult subject. It no longer deals primarily with tonsillectomy, adenoidectomy and the insertion of ventilation tubes. However, these common procedures remain important and this book contains detailed analy-ses of these well-known aspects of paediatric ENT life, whose rôles are still being defined.

There are some variations in the depth of cover among the 47 chapters of this book. Some surgical topics are dealt with clearly but fairly briefly. In contrast, fields such as immunology, allergy and genetics, in which there con-tinues to be quite rapid scientific progress, needed more detail.

It is a commonplace understanding that children are not small adults. Similarly, much of paediatric ENT is significantly different from the practice of ENT in adults. Children benefit from being looked after in dedicated

clinics, able to accommodate the activities that children usually indulge in, and not threatening to them. Wards staffed by nurses trained in childcare as well as in ENT nursing are now considered essential, and need to include play areas and accommodation for parents. Many aspects of the care of children with ENT problems involve several different disciplines: a multidisciplinary team. The rôles of team members need both defining and acknowledging. The ENT doctor needs to accept and to encourage this. The book includes some general chapters dealing with these aspects of the specialty.

With contributors from many different countries, the use and the spelling of medical terms inevitably vary. In general we have accepted both standard US and standard British spellings, trying to keep to one or the other within the confines of separate chapters. Some medical terms appear to be interchangeable: tracheotomy and tracheos-tomy are an example; some authors hold strong views on this subject and we have encouraged them to use the term they prefer.

We end by acknowledging the support we have re-ceived from our publishers and the staff at Springer Ver-lag. We have also received considerable help from friends and colleagues, including those authors of chapters who have taken the time and trouble to review other chapters and offer advice. Kevin Gibbin and Will Hellier in partic-ular have given much time and sound advice. Emily Gra-ham has contributed great skill and some hours of work in manipulating tables, figures and all kinds of illustration to fit the requirements of several chapters.

Every text of this kind inevitably includes a sincere vote of heartfelt thanks to the spouses and family of the editors. We now understand why. Sharing one’s marriage with a book is not necessarily something to be recom-mended. We, too, would like to thank Sandy Graham, Hilary Bull and Adam Pearson for their patience, forbear-ance, much sound advice and constructive criticism.

Letting a book loose on the public could be thought an irresponsible act. We hope this is not the case and that you, our readers, find its contents informative, useful and absorbing.

It is inevitable in a book of this nature that errors will have crept in. For these we apologise and crave your in-dulgence.

John M. GrahamGlenis K. Scadding

Peter D. Bull

Preface

Each patient is a part of a society: successful care given to that patient adds value to that society. Pediatric oto-laryngology encompasses the traditional purposes of medicine in the prevention and curing of illness. It also focuses especially on hearing, speech, gustation and the sense of smell, swallowing and respiration. Children able to express themselves will say “Thank you, I can hear bet-ter, smell and taste better, breathe better, sleep better, look better…”. Pediatric otolaryngology has particular impor-tance because it deals with communication by language, through the vehicles of hearing, voice and speech.

This is critically important in two ways. The first relates to the economic basis of our society – the way in which we make our livelihoods; this underwent fundamental change during the last half of the 20th century (Ruben 2000). In earlier periods we depended largely on manual labor. Today we depend upon communication skills, me-diated through hearing, voice, speech and language, and directly linked to literacy. Pediatric otolaryngology con-tributes to the economic basis of society by facilitating the development of the skill of communication in individual children. Comparing three different countries (Ruben 2003): one very highly dependent upon communica-tion skills (the Netherlands), one highly dependent upon communication skills (the USA), one a developing nation less dependent upon communication skills (the Philip-pines), reveals that all three nations are adversely affected economically and socially by communication disorders. It is estimated that the USA loses between 2.5 and 3% of

its gross domestic product from the economic sequelae of communication disorders.

A second substantial impact related to pediatric otolar-yngology is in the relationship between communication disorders and juvenile crime. The prevalence of commu-nication disorders is many times greater in populations of juvenile delinquents than in the general population. Communication disorders appear to act synergistically with diminished economic and social resources in lead-ing to violent behavior and crime.

The pediatric otolaryngologist’s role in the prevention, cure and care of communication disorders is therefore one of the most important fields of medical care for the 21st century – the age of communication.

Robert J. Ruben, MD, FACS, FAAPDistinguished University Professor

Albert Einstein College of MedicineDepartment of Otolaryngology

Montefiore Medical Center

Ruben RJ (2000) Redefining the survival of the fittest: com-munication disorders in the 21st century. Laryngoscope 110:241–245Ruben RJ (2003) Valedictory – why pediatric otorhinolar-yngology is important. Int J Pediatr Otorhinolaryngol 67:S53–61

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Introduction

1 A Paediatric Overview of Children Seen in the ENT Outpatient Department 1Helen Bantock

Introduction: Paediatric Consultation . . . . 1 Development in the First Five Years of Life 2 Children with Special Needs . . . . . . . . . . . . . . 2

Speech and Language Delay or Disorder 2 Autistic Spectrum . . . . . . . . . . . . . . . . . . . . . . 2 Global Delay . . . . . . . . . . . . . . . . . . . . . . . . . . . 3 Specific Learning Difficulties . . . . . . . . . . . 3

Syndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3 Down Syndrome . . . . . . . . . . . . . . . . . . . . . . 3

Behaviour Difficulties . . . . . . . . . . . . . . . . . . 3 Attention Deficit Hyperactivity Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

Social Difficulties . . . . . . . . . . . . . . . . . . . . . . 3 Types of Abuse . . . . . . . . . . . . . . . . . . . . . . . 3 Child Abuse in the Context of an ENT Clinic . . . . . . . . . . . . . . . . . . . . . 4 Alerting Signs of Non-Accidental Injury and Abuse in a Clinic Setting . . . 4

What to do if Abuse is Suspected . . . . . . . 4 Links with Local Services . . . . . . . . . . . . . . . . . 4

Primary Care Team . . . . . . . . . . . . . . . . . . . . 4 The Child Development Team . . . . . . . . . . 4 The Education Service . . . . . . . . . . . . . . . . . 4 Child and Adolescent Mental Health Services . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 Social Services . . . . . . . . . . . . . . . . . . . . . . . . . 5 Non-Statutory Organisations . . . . . . . . . . . 5

2 Nursing Aspects of Paediatric ENT . . . . . . 7Rosalind Wilson and Judith Barton

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7 Communication . . . . . . . . . . . . . . . . . . . . . . . . . 7 Consent for Surgery . . . . . . . . . . . . . . . . . . . . . . 8 Staffing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8 The Physical Environment . . . . . . . . . . . . . . . . 8 Tracheostomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8 Parents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9 Day Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9 Pain Control . . . . . . . . . . . . . . . . . . . . . . . . . . . 10

3 Anaesthesia for Paediatric ENT Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11Ian Barker

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . 11 General Considerations . . . . . . . . . . . . . . . . 12

Anaesthesia for Children . . . . . . . . . . . . . 12 Anaesthesia for the Newborn . . . . . . . . . 12

Hypoxia . . . . . . . . . . . . . . . . . . . . . . . . . . . 12 Hypothermia . . . . . . . . . . . . . . . . . . . . . . . 12 Hypoglycaemia . . . . . . . . . . . . . . . . . . . . . 12

Intravenous Fluid Therapy for Children . . . . . . . . . . . . . . . . . . . . . . . . . 12

Specific Conditions . . . . . . . . . . . . . . . . . . . . 13 Post-Tonsillectomy Bleeding . . . . . . . . . 13

Assessment . . . . . . . . . . . . . . . . . . . . . . . . . 13 Resuscitation . . . . . . . . . . . . . . . . . . . . . . . 13 Anaesthesia . . . . . . . . . . . . . . . . . . . . . . . . 13

Airway Endoscopy . . . . . . . . . . . . . . . . . . . 13 Indications . . . . . . . . . . . . . . . . . . . . . . . . . 13 Induction . . . . . . . . . . . . . . . . . . . . . . . . . . 14

Maintenance . . . . . . . . . . . . . . . . . . . . . . . . . 14 Laser Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . 14 Obstructive Sleep Apnoea . . . . . . . . . . . . . . 15 Anaesthetic Equipment . . . . . . . . . . . . . . . . . 15

Endotracheal Tubes . . . . . . . . . . . . . . . . . . 15 Laryngeal Mask Airway . . . . . . . . . . . . . . 16 Pulse Oximeter . . . . . . . . . . . . . . . . . . . . . . 16

Limitations . . . . . . . . . . . . . . . . . . . . . . . . 16 Carbon Dioxide Monitor . . . . . . . . . . . . . 16

4 The Evolution of Speech and Language . . . . . . . . . . . . . . . . . . . . . . . . . . 19John F. Stein

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . 19 Is Language Innate or Learnt? . . . . . . . . . . 20 Human Evolution . . . . . . . . . . . . . . . . . . . . . . 20

Left-Sided Specialisation . . . . . . . . . . . . . 20 Emotional Vocalisations . . . . . . . . . . . . . . 21 Mirror Neurones . . . . . . . . . . . . . . . . . . . . . 21 Bipedalism and the Aquatic Ape Hypothesis . . . . . . . . . . . . . . . . . . . . . . . . . . . 22 Larynx Descent . . . . . . . . . . . . . . . . . . . . . . 22

Contents

Brain Enlargement . . . . . . . . . . . . . . . . . . . 23 Right-Handedness . . . . . . . . . . . . . . . . . . . 23 Selective Advantages of Speech . . . . . . . 23 Gesture and Speech . . . . . . . . . . . . . . . . . . 24 Writing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24

Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24

5 Evidence-Based Management of Speech and Language Delays . . . . . . . 27Amy McConkey Robbins

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 Milestones for Communication Development . . . . . . . . . . . . . . . . . . . . . . . . . . 28

Normal Variation in Language Learning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28 Normal Variation versus Communication Delay . . . . . . . . . . . . . . . 28 Communication Milestones in the First Year of Life . . . . . . . . . . . . . . . 29 Clinical Use of Table 5.1 by Physicians 31

Speech and Language Intervention is Correlated with Young Age at Identification . . . . . . . . . . . . . . . . . . . . . . . . 32 Hearing Loss can be Identified and Treated as Early as the Newborn Period . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32 Differential Diagnosis of Several Communication Disorders . . . . . . . . . . . . . 33 Association Between Some Speech/Language Characteristics and Reading Disability . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34 Evidence-Based Approach to the Management of Children’s Speech and Language Development . . . . . . . . . . . . 34

6 Paediatric Voice . . . . . . . . . . . . . . . . . . . . . . . . . 37Mark E. Boseley and Christopher J. Hartnick

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . 37 Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38

Gross . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38 Histology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38

Diagnostic Techniques . . . . . . . . . . . . . . . . . 39 Physical Examination . . . . . . . . . . . . . . . . 39 Voice Outcome Surveys . . . . . . . . . . . . . . 39

Common Voice Pathologies . . . . . . . . . . . . 39 Vocal Fold Nodules . . . . . . . . . . . . . . . . . . 39 Voice Results Following Laryngotracheal Reconstruction . . . . . . 42 Recurrent Respiratory Papillomatosis 43 Vocal Fold Paralysis . . . . . . . . . . . . . . . . . . 43

7 Genetics of Non-Syndromic Deafness 47Maria Bitner-Glindzicz

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . 47 Investigation of the Aetiology of Hearing Loss . . . . . . . . . . . . . . . . . . . . . . . . 48

Reasons . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48 Protocol . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48 History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48 Examination . . . . . . . . . . . . . . . . . . . . . . . . . 48 Investigation . . . . . . . . . . . . . . . . . . . . . . . . . 48 Genetic Hearing Loss . . . . . . . . . . . . . . . . 49 Recessive Genes: Common/Recognizable Types of Non-Syndromic Genetic Hearing Loss 50

GJB2 (Connexin26) . . . . . . . . . . . . . . . . . 50 SLC26A4 (Pendred and Non-Syndromic Enlarged Vestibular Aqueduct) . . . . . . . . . . . . . . . . . . . . . . . . . 52 OTOF (Auditory Neuropathy) . . . . . . . 53

Dominant Genes: Common/Recognizable Types of Non-Syndromic Genetic Hearing Loss . . . . . 53

WFS1 (Dominant Low-Frequency Sensorineural Hearing Loss) . . . . . . . . 53 COCH (Menière-Like Symptoms) . . . 54

8 ENT-Related Syndromes . . . . . . . . . . . . . . . . 57David Albert and Fiona Connell

Definition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . 58 Resources . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59 Syndromes of Particular Relevance to the ENT Clinician . . . . . . . . . . . . . . . . . . . 59

Down Syndrome . . . . . . . . . . . . . . . . . . . . . 59 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 59 ENT Features . . . . . . . . . . . . . . . . . . . . . . 59 General Features . . . . . . . . . . . . . . . . . . . 59

Pierre Robin Sequence . . . . . . . . . . . . . . . 59 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 59 ENT Features . . . . . . . . . . . . . . . . . . . . . . 60

Treacher Collins Syndrome . . . . . . . . . . . 60 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 60 ENT Features . . . . . . . . . . . . . . . . . . . . . . 60 General Features . . . . . . . . . . . . . . . . . . . 60

Goldenhar Syndrome . . . . . . . . . . . . . . . . 60 CHARGE Syndrome . . . . . . . . . . . . . . . . . 60

Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 60 ENT Features . . . . . . . . . . . . . . . . . . . . . . 60 General Features . . . . . . . . . . . . . . . . . . . 61 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 61 ENT Features . . . . . . . . . . . . . . . . . . . . . . 61 General Features . . . . . . . . . . . . . . . . . . . 61

XII Contents

Branchio-oto-renal Syndrome . . . . . . . . 62 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 62 ENT Features . . . . . . . . . . . . . . . . . . . . . . 62 General Features . . . . . . . . . . . . . . . . . . . 62

22q11.2 Deletion Syndrome . . . . . . . . . . 62 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 62 ENT Features . . . . . . . . . . . . . . . . . . . . . . 62 General Features . . . . . . . . . . . . . . . . . . . 62

Craniosynostosis Syndromes . . . . . . . . . 63 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 63 ENT Features . . . . . . . . . . . . . . . . . . . . . . 63 General Features . . . . . . . . . . . . . . . . . . . 63

Alport Syndrome . . . . . . . . . . . . . . . . . . . . 63 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 63 ENT Features . . . . . . . . . . . . . . . . . . . . . . 63 General Features . . . . . . . . . . . . . . . . . . . 63

Pendred Syndrome . . . . . . . . . . . . . . . . . . . 63 Usher Syndrome . . . . . . . . . . . . . . . . . . . . . 63

Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 63 Alstrom Syndrome . . . . . . . . . . . . . . . . . . . 65

Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 65 ENT Features . . . . . . . . . . . . . . . . . . . . . . 65 General Features . . . . . . . . . . . . . . . . . . . 65

Syndromes Less Commonly Seen by ENT Surgeons . . . . . . . . . . . . . . . . . . . . . . . 65

Achondroplasia . . . . . . . . . . . . . . . . . . . . . . 65 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 65 ENT Features . . . . . . . . . . . . . . . . . . . . . . 65 General Features . . . . . . . . . . . . . . . . . . . 65

Beckwith-Wiedemann Syndrome . . . . . 65 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 65 ENT Features . . . . . . . . . . . . . . . . . . . . . . 65 General Features . . . . . . . . . . . . . . . . . . . 65

Neurofibromatosis Type 2 . . . . . . . . . . . . 66 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 66 ENT Features . . . . . . . . . . . . . . . . . . . . . . 66 General Features . . . . . . . . . . . . . . . . . . . 66

Noonan Syndrome . . . . . . . . . . . . . . . . . . . 67 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 67 ENT Features . . . . . . . . . . . . . . . . . . . . . . 67 General Features . . . . . . . . . . . . . . . . . . . 67

Osteogenesis Imperfecta . . . . . . . . . . . . . 67 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 67 ENT Features (Type 1) . . . . . . . . . . . . . . 67General Features (Type 1) . . . . . . . . . . . 67

Prader-Willi Syndrome . . . . . . . . . . . . . . . 67 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 67 ENT Features . . . . . . . . . . . . . . . . . . . . . . 67 General Features . . . . . . . . . . . . . . . . . . . 67

Stickler Syndrome . . . . . . . . . . . . . . . . . . . . 68 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 68 ENT Features . . . . . . . . . . . . . . . . . . . . . . 68 General Features . . . . . . . . . . . . . . . . . . . 68

Turner Syndrome . . . . . . . . . . . . . . . . . . . . 68 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 68

ENT Features . . . . . . . . . . . . . . . . . . . . . . 68 General Features . . . . . . . . . . . . . . . . . . . 68

Waardenburg Syndrome . . . . . . . . . . . . . . 68 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 68 ENT Features . . . . . . . . . . . . . . . . . . . . . . 68 General Features . . . . . . . . . . . . . . . . . . . 68

Jervell and Lange-Nielsen Syndrome . . 68 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 68 ENT Features . . . . . . . . . . . . . . . . . . . . . . 69 General Features . . . . . . . . . . . . . . . . . . . 69

Gorlin Syndrome . . . . . . . . . . . . . . . . . . . . 69 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 69 ENT Features . . . . . . . . . . . . . . . . . . . . . . 69 General Features . . . . . . . . . . . . . . . . . . . 69

Holoprosencephaly . . . . . . . . . . . . . . . . . . 69 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 69 ENT Features . . . . . . . . . . . . . . . . . . . . . . 69General Features . . . . . . . . . . . . . . . . . . . 69

Mucopolysaccharidoses . . . . . . . . . . . . . . 69 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 69 ENT Features . . . . . . . . . . . . . . . . . . . . . . 69 General Features . . . . . . . . . . . . . . . . . . . 69

Foetal Alcohol Syndrome . . . . . . . . . . . . . 70 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 70 ENT Features . . . . . . . . . . . . . . . . . . . . . . 70 General Features . . . . . . . . . . . . . . . . . . . 70

Foetal Cytomegalovirus Syndrome . . . . 70 Features . . . . . . . . . . . . . . . . . . . . . . . . . . . 70

Congenital rubella syndrome . . . . . . . . . 70 Features . . . . . . . . . . . . . . . . . . . . . . . . . . . 70

Acknowledgements . . . . . . . . . . . . . . . . . . 70

9 EXIT – Antenatal (Pre-natal) Diagnoses and Management . . . . . . . . . . . 73Gavin Morrison

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . 73 Pre-natal Diagnosis . . . . . . . . . . . . . . . . . . . . 74

Diagnostic Ultrasound Features . . . . . . 74 Congenital High Airway Obstruction Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75 Foetal MRI Scanning . . . . . . . . . . . . . . . . . 75

The EXIT Procedure . . . . . . . . . . . . . . . . . . . 76 Indications for the EXIT Procedure . . . 76 Techniques of the EXIT Procedure . . . . 76 Tips and Pitfalls of EXIT . . . . . . . . . . . . . 77

Spontaneous Onset of Labour . . . . . . . 77 Cystic Hygroma . . . . . . . . . . . . . . . . . . . . 78 Maintaining Placento-Foetal Circulation . . . . . . . . . . . . . . . . . . . . . . . . . 78 Loss of Placento-Foetal Circulation . . 78 Risks to Mother . . . . . . . . . . . . . . . . . . . . 78

Outcomes of EXIT . . . . . . . . . . . . . . . . . . . 78 Counselling and Ethical Issues . . . . . . . . . . 78

Contents XIII

Termination of Pregnancy . . . . . . . . . . . . 79 Foetal Surgery . . . . . . . . . . . . . . . . . . . . . . . . . 79

Intrauterine Treatment of CHD and EXIT . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79 Newer Applications and the Future . . . 80

10 The Immunocompromised Child . . . . . . . 83Mich Lajeunesse and Adam Finn

Scope of Chapter . . . . . . . . . . . . . . . . . . . . . . . 83 Primary Immunodeficiency . . . . . . . . . . . . 83

Natural History if Untreated . . . . . . . . . . 84 Prevalence and Incidence . . . . . . . . . . . . 85 Aetiology and Genetics . . . . . . . . . . . . . . . 85 Assessment and Diagnosis . . . . . . . . . . . . 85

Severe . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 85 Prolonged . . . . . . . . . . . . . . . . . . . . . . . . . . 85 Unusual . . . . . . . . . . . . . . . . . . . . . . . . . . . 86 Recurrent . . . . . . . . . . . . . . . . . . . . . . . . . . 86 Examination . . . . . . . . . . . . . . . . . . . . . . . 86

Classification . . . . . . . . . . . . . . . . . . . . . . . . 86 Antibody Deficiencies . . . . . . . . . . . . . . . 87 Complement Deficiencies . . . . . . . . . . . 87 Mannose-Binding Lectin Deficiency 87 Neutrophil Disorders . . . . . . . . . . . . . . . 88

Laboratory Tests . . . . . . . . . . . . . . . . . . . . . 88 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . 88

Prophylactic Antibiotics . . . . . . . . . . . . . 88 Immunoglobulin Replacement Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 88 Immunisation . . . . . . . . . . . . . . . . . . . . . . 88 Bone Marrow Transplantation . . . . . . 89 Gene Therapy . . . . . . . . . . . . . . . . . . . . . . 89

Secondary Immunodeficiency . . . . . . . . . . 89 Human Immunodeficiency Virus . . . . . 89 Description . . . . . . . . . . . . . . . . . . . . . . . . . . 90 Natural History if Untreated and Complications . . . . . . . . . . . . . . . . . . . 90 Prevalence . . . . . . . . . . . . . . . . . . . . . . . . . . . 90 Classification . . . . . . . . . . . . . . . . . . . . . . . . 90 Clinical Assessment . . . . . . . . . . . . . . . . . 91 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . 91

11 Cystic Fibrosis and Primary Ciliary Dyskinesia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 95Andrew Bush and Jonny Harcourt

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . 96 Cystic Fibrosis – The Disease . . . . . . . . . . . 96

Molecular Defect . . . . . . . . . . . . . . . . . . . . . 96 Pathophysiology . . . . . . . . . . . . . . . . . . . . . 96

Lung Disease . . . . . . . . . . . . . . . . . . . . . . . 96

Gastrointestinal and Other Disease Manifestations . . . . . . . . . . . . . . . . . . . . . 97

Prevalence . . . . . . . . . . . . . . . . . . . . . . . . . . . 97 Diagnostic Tests . . . . . . . . . . . . . . . . . . . . . 97

Clinical Suspicion . . . . . . . . . . . . . . . . . . 97 The Sweat Test . . . . . . . . . . . . . . . . . . . . . 98 Genetic Testing . . . . . . . . . . . . . . . . . . . . . 98 Ancillary Testing . . . . . . . . . . . . . . . . . . . 98

Features of the Disease . . . . . . . . . . . . . . . 98 Upper Airway Disease . . . . . . . . . . . . . . 98 Lower Airway Disease . . . . . . . . . . . . . . 99 Other Organ Disease . . . . . . . . . . . . . . . 99

Medical Treatment . . . . . . . . . . . . . . . . . . . 99 Upper Airway Disease . . . . . . . . . . . . . . 99 Lower Airway Disease . . . . . . . . . . . . . . 99 Other Systems . . . . . . . . . . . . . . . . . . . . . 100

When Should an Apparently Healthy Child be Suspected of Having CF? . . . 101

PCD – The Disease . . . . . . . . . . . . . . . . . . . . 102 Molecular Defect . . . . . . . . . . . . . . . . . . . . 102 Pathophysiology . . . . . . . . . . . . . . . . . . . . 102 Prevalence . . . . . . . . . . . . . . . . . . . . . . . . . . 102 Diagnostic Tests . . . . . . . . . . . . . . . . . . . . 102

In Vivo Tests of Ciliary Function . . . 102 Nitric Oxide Studies . . . . . . . . . . . . . . . 102 Measurement of Ciliary Beat Frequency and Pattern . . . . . . . . . . . . . 103 Electron Microscopy . . . . . . . . . . . . . . . 103 Culture of Cilia . . . . . . . . . . . . . . . . . . . . 104

Features of the Disease . . . . . . . . . . . . . . 104 Upper Airway Disease . . . . . . . . . . . . . 104 Lower Airway Disease . . . . . . . . . . . . . 104 Other Organ Disease . . . . . . . . . . . . . . 104

Medical Treatment . . . . . . . . . . . . . . . . . . 105 Upper Airway Disease . . . . . . . . . . . . . 105 Lower Airway Disease . . . . . . . . . . . . . 105 Other Organ Disease . . . . . . . . . . . . . . 105

When Should an Apparently Healthy Child be Suspected of Having PCD? 105

The Child Referred for Surgery for an Unrelated Condition . . . . . . . . . . . . . . . . . . 105 ENT Surgery for CF . . . . . . . . . . . . . . . . . . . 106 ENT Surgery for PCD . . . . . . . . . . . . . . . . . 108

12 Head and Neck Masses . . . . . . . . . . . . . . . . 111Ben Hartley

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 112 Part 1. Clinical Assessment of Neck Swelling in Children . . . . . . . . . . 112

History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 112 Examination of the Neck . . . . . . . . . . . . 112

Site of the Swelling . . . . . . . . . . . . . . . . . 112 Nature of the Swelling . . . . . . . . . . . . . 113

XIV Contents

Head and Neck Examination . . . . . . . 113 General Examination . . . . . . . . . . . . . . 113

Investigation . . . . . . . . . . . . . . . . . . . . . . . . 113 Laboratory Tests . . . . . . . . . . . . . . . . . . . 113 Radiology . . . . . . . . . . . . . . . . . . . . . . . . . 113 Biopsy Sampling versus Fine-Needle Aspiration for Cytology . . . . . . . . . . . . 114

Part 2. Inflammatory Disorders of the Neck and Lymphadenopathy . . . . 114

Viral Infections . . . . . . . . . . . . . . . . . . . . . 114 Viral Upper Respiratory Tract Infections . . . . . . . . . . . . . . . . . . . . . . . . . 114 Infectious Mononucleosis . . . . . . . . . . 115 Human Immunodeficiency Virus . . . 115

Bacterial Infections . . . . . . . . . . . . . . . . . 115 Acute Lymphadenopathy with Suppuration . . . . . . . . . . . . . . . . . . 115 Mycobacterial Neck Infections . . . . . . 115

Non-Infectious Inflammatory Lymphadenopathy . . . . . . . . . . . . . . . . . . 116

Kawasaki Syndrome . . . . . . . . . . . . . . . 116 Sinus Histiocytosis (Rosai Dorfman Disease) . . . . . . . . . . 116 Kikuchi-Fujimoto Disease . . . . . . . . . . 116

Part 3. Haemangiomas and Vascular Malformations . . . . . . . . . . . 116

Terminology . . . . . . . . . . . . . . . . . . . . . . . . 116 Haemangiomas . . . . . . . . . . . . . . . . . . . . 117 Vascular Malformations . . . . . . . . . . . 117 Capillary Malformations . . . . . . . . . . . 117 Venous Malformations . . . . . . . . . . . . . 118 Arteriovenous Malformations . . . . . . 119

Part 4. Head and Neck Malignancy in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . 119

Hodgkin’s Disease . . . . . . . . . . . . . . . . . . . 119 Non-Hodgkin Lymphoma . . . . . . . . . . . 119 Rhabdomyosarcoma . . . . . . . . . . . . . . . . 120 Non-Rhabdomyosarcoma Sarcomas 120 Thyroid Carcinoma in Children . . . . . 120 Salivary Gland Malignancy . . . . . . . . . . 120 Neuroblastoma . . . . . . . . . . . . . . . . . . . . . . 120 Nasopharyngeal Carcinoma . . . . . . . . . 120 Teratoma . . . . . . . . . . . . . . . . . . . . . . . . . . . 121 Juvenile Nasopharyngeal Angiofibroma . . . . . . . . . . . . . . . . . . . . . . . 121

13 Paediatric ENT in Developing Countries . . . . . . . . . . . . . . 123Neville P. Shine and Christopher A. J. Prescott

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 123 Ear Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . 124

Chronic Suppurative Otitis Media . . . 125

Tympanomastoid Surgery . . . . . . . . . . . 125 Hearing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 126 Adenotonsillar Surgery . . . . . . . . . . . . . . . . 127 HIV/AIDS and Tuberculosis . . . . . . . . . . . 127 Tracheotomy . . . . . . . . . . . . . . . . . . . . . . . . . . 128 ENT Aspects of Accidents and Trauma 128 Paediatric Head and Neck Tumours . . . . 128

14 Tonsils and Adenoids . . . . . . . . . . . . . . . . . . 131Anne Pitkäranta and Pekka Karma

Waldeyer’s Ring . . . . . . . . . . . . . . . . . . . . . . . 131 Tonsillitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132

Acute Tonsillitis . . . . . . . . . . . . . . . . . . . . . 132 Viral Tonsillitis . . . . . . . . . . . . . . . . . . . . . 132 Bacterial Tonsillitis . . . . . . . . . . . . . . . . . 133 Chronic Tonsillitis . . . . . . . . . . . . . . . . . . 134 Complications of Tonsillitis . . . . . . . . . 134

Peritonsillar Abscess (Quinsy) . . . . . . 134 Adenotonsillar Hyperplasia . . . . . . . . 134 Mouth Breathing . . . . . . . . . . . . . . . . . . 134

Tonsillectomy . . . . . . . . . . . . . . . . . . . . . . . . . 135 Indications . . . . . . . . . . . . . . . . . . . . . . . . . 135 Techniques . . . . . . . . . . . . . . . . . . . . . . . . . 135 Complications . . . . . . . . . . . . . . . . . . . . . . 136

Evaluation of the Adenoids . . . . . . . . . . . . 137 Adenoidectomy . . . . . . . . . . . . . . . . . . . . . . . 137

Indications . . . . . . . . . . . . . . . . . . . . . . . . . 137 Techniques . . . . . . . . . . . . . . . . . . . . . . . . . 138 Complications . . . . . . . . . . . . . . . . . . . . . . 138

Neoplasms of the Tonsils and the Adenoids . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 138

15 The Causes and Effects of Obstructive Sleep Apnoea in Children . . . . . . . . . . . . . 141Ray W. Clarke

Pathophysiology of Childhood Obstructive Sleep Apnoea . . . . . . . . . . . . . 142

Introduction . . . . . . . . . . . . . . . . . . . . . . . . 142 Prevalence . . . . . . . . . . . . . . . . . . . . . . . . . . 142 Aetiology . . . . . . . . . . . . . . . . . . . . . . . . . . . 142 Mechanisms . . . . . . . . . . . . . . . . . . . . . . . . 143

Diagnosis of OSA . . . . . . . . . . . . . . . . . . . . . 143 The Spectrum of Airway Obstruction 143 Diagnostic Criteria . . . . . . . . . . . . . . . . . . 144 Presentation . . . . . . . . . . . . . . . . . . . . . . . . 144

Clinical Features . . . . . . . . . . . . . . . . . . 144 Differential Diagnosis . . . . . . . . . . . . . . . 144 Effects of OSA . . . . . . . . . . . . . . . . . . . . . . 144

Management . . . . . . . . . . . . . . . . . . . . . . . . . . 145 Investigations . . . . . . . . . . . . . . . . . . . . . . . 145 Sleep Studies (Polysomnography) . . . . 145

Contents XV

Pre-Operative Investigations . . . . . . . 146 Endoscopy (Sleep Nasendoscopy) . . . 146 Paediatric Evaluation . . . . . . . . . . . . . . 147

Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . 147 General Principles . . . . . . . . . . . . . . . . . 147 Pharmacological Treatment . . . . . . . . 147 Dental Devices . . . . . . . . . . . . . . . . . . . . 147 Continuous Positive Airway Pressure 147 Postural Therapy . . . . . . . . . . . . . . . . . . 147 Nasopharyngeal Airway . . . . . . . . . . . 147 Adenotonsillectomy . . . . . . . . . . . . . . . . 147 Nasal Surgery . . . . . . . . . . . . . . . . . . . . . 148 Maxillomandibular Surgery . . . . . . . . 148 Palatal Surgery . . . . . . . . . . . . . . . . . . . . 148 Tongue-Base Surgery . . . . . . . . . . . . . . 149 Tracheotomy . . . . . . . . . . . . . . . . . . . . . . 149

Challenges in OSA Management . . . . . . . 149 OSA and Obesity . . . . . . . . . . . . . . . . . . . . 149 OSA in Special Needs Children . . . . . . 149

16 Cleft Lip and Palate . . . . . . . . . . . . . . . . . . . . 153John Boorman

Embryology . . . . . . . . . . . . . . . . . . . . . . . . . . . 153 Aetiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153 Prevalence . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153 Presentation . . . . . . . . . . . . . . . . . . . . . . . . . . 154 Robin Sequence . . . . . . . . . . . . . . . . . . . . . . . 154 Feeding . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 155 Surgery for Cleft Lip and Palate . . . . . . . . 155 Velopharyngeal Insufficiency . . . . . . . . . . 156

Sub-mucous Cleft Palate and Bifid Uvula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156 Assessment of VPI . . . . . . . . . . . . . . . . . . 157 Treatment of VPI . . . . . . . . . . . . . . . . . . . 157 Velocardiofacial Syndrome . . . . . . . . . . 157

17 Salivary Gland Disease in Childhood 159Peter D. Bull

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 159 History, Investigations . . . . . . . . . . . . . . . . 159

History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 159 Examination . . . . . . . . . . . . . . . . . . . . . . . . 160 Investigation . . . . . . . . . . . . . . . . . . . . . . . . 160

Congenital Disorders . . . . . . . . . . . . . . . . . . 160 Congenital Cysts and Fistulae . . . . . . . 160

Inflammatory Disorders of the Salivary Glands . . . . . . . . . . . . . . . . . 160

Viral Infections . . . . . . . . . . . . . . . . . . . . . 160 Bacterial Disease . . . . . . . . . . . . . . . . . . . . 160 Atypical Mycobacterial Disease of Salivary the Glands . . . . . . . . . . . . . . . 160

Features . . . . . . . . . . . . . . . . . . . . . . . . . . 160 Investigations . . . . . . . . . . . . . . . . . . . . . 161 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 161

Recurrent Parotitis of Childhood . . . . 161 Sjogren’s Syndrome . . . . . . . . . . . . . . . . . 161 Human Immunodeficiency Virus/Acquired Immune Deficiency Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 Sarcoidosis . . . . . . . . . . . . . . . . . . . . . . . . . 162

Obstructive Disorders . . . . . . . . . . . . . . . . . 162 Salivary Gland Calculi . . . . . . . . . . . . . . 162

Presentation . . . . . . . . . . . . . . . . . . . . . . 162 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 162 Ranula . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 162

Salivary Gland Tumours in Childhood 162 Benign . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 Malignant . . . . . . . . . . . . . . . . . . . . . . . . . . 163

Presentation . . . . . . . . . . . . . . . . . . . . . . 163 Pathology . . . . . . . . . . . . . . . . . . . . . . . . . 163 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 163 Complications of Parotid Surgery . . . 163

18 Drooling – Salivary Incontinence (Sialorrhoea) . . . . . . . . . . . . . . . . . . . . . . . . . . . 165Peter D. Bull

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 165 Innervation . . . . . . . . . . . . . . . . . . . . . . . . . . . 165 Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . 165

Non-Surgical Management . . . . . . . . . . 166 Posture Control . . . . . . . . . . . . . . . . . . . 166 Drug Treatment . . . . . . . . . . . . . . . . . . . 166

Surgical Management . . . . . . . . . . . . . . . 167

19 Congenital Cysts, Sinuses and Fistulae . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169Fiona B. MacGregor

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 170 Developmental Anatomy . . . . . . . . . . . . . . 170 Classification of Congenital Branchial Abnormalities in the Neck . . . . . . . . . . . . . 170

Pre-Auricular Sinuses . . . . . . . . . . . . . . . 170 Presentation . . . . . . . . . . . . . . . . . . . . . . 171 Investigations . . . . . . . . . . . . . . . . . . . . . 171 Management . . . . . . . . . . . . . . . . . . . . . . 171 Complications . . . . . . . . . . . . . . . . . . . . . 171

First Branchial Arch Anomalies . . . . . 171 Presentation . . . . . . . . . . . . . . . . . . . . . . 171 Investigations . . . . . . . . . . . . . . . . . . . . . 172 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 172 Complications . . . . . . . . . . . . . . . . . . . . . 172

XVI Contents

Second Branchial Arch Anomalies . . . 172 Presentation . . . . . . . . . . . . . . . . . . . . . . 172 Investigations . . . . . . . . . . . . . . . . . . . . . 172 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 172

Third and Fourth Arch Anomalies . . . 172 Presentation . . . . . . . . . . . . . . . . . . . . . . 172 Investigations . . . . . . . . . . . . . . . . . . . . . 173 Management . . . . . . . . . . . . . . . . . . . . . . 173 Complications . . . . . . . . . . . . . . . . . . . . . 173

Branchio-Oto-Renal Syndrome . . . . . . 173 Thyroglossal Duct Cyst . . . . . . . . . . . . . 173

Presentation . . . . . . . . . . . . . . . . . . . . . . 173 Investigations . . . . . . . . . . . . . . . . . . . . . 174 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 174 Complications . . . . . . . . . . . . . . . . . . . . . 174

Lingual Thyroid . . . . . . . . . . . . . . . . . . . . . 174 Presentation . . . . . . . . . . . . . . . . . . . . . . 174 Investigations . . . . . . . . . . . . . . . . . . . . . 174 Management . . . . . . . . . . . . . . . . . . . . . . 174 Complications . . . . . . . . . . . . . . . . . . . . . 174

Dermoid Cyst . . . . . . . . . . . . . . . . . . . . . . . 174 Presentation . . . . . . . . . . . . . . . . . . . . . . 175 Investigations . . . . . . . . . . . . . . . . . . . . . 175 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 175

Nasal Dermoid Cysts . . . . . . . . . . . . . . . . 175 Presentation . . . . . . . . . . . . . . . . . . . . . . 175 Investigations . . . . . . . . . . . . . . . . . . . . . 175 Management . . . . . . . . . . . . . . . . . . . . . . 175

20 Airway Endoscopy and Assessment in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 177Sonia Ayari-Khalfallah and Patrick Froehlich

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 177 Rigid Endoscopy . . . . . . . . . . . . . . . . . . . . . . 178 Supplementary Examinations . . . . . . . . . . 181

21 Emergency Management of the Paediatric Airway . . . . . . . . . . . . . . . 183Michael Kuo and Michael Rothera

Anatomical and Physiological Considerations . . . . . . . . . . . . . . . . . . . . . . . . 183 Clinical and Radiological Assessment of the Acutely Compromised Upper Airway . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183 Resuscitation and Pre-Operating-Room Management . . . . . . . . . . . . . . . . . . . . . . . . . . 186 Endoscopy Under General Anaesthesia 186 Surgical Management of the Acutely Obstructed Paediatric Airway . . . . . . . . . 187

22 Congenital Disorders of the Larynx, Trachea and Bronchi . . . . . . . . . . . . . . . . . . . 189Martin Bailey

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 189 Larynx . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 189 Supraglottis . . . . . . . . . . . . . . . . . . . . . . . . . . . 190

Laryngomalacia . . . . . . . . . . . . . . . . . . . . . 190 Saccular Cysts and Laryngocoeles . . . 190 Lymphangioma . . . . . . . . . . . . . . . . . . . . . 191 Bifid Epiglottis . . . . . . . . . . . . . . . . . . . . . . 191

Glottis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 191 Laryngeal Webs . . . . . . . . . . . . . . . . . . . . . 191 Laryngeal Atresia . . . . . . . . . . . . . . . . . . . 191 Cri-du-Chat Syndrome . . . . . . . . . . . . . . 191 Vocal Cord Paralysis . . . . . . . . . . . . . . . . 191

Subglottis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 192 Congenital Subglottic Stenosis . . . . . . . 192 Subglottic Haemangioma . . . . . . . . . . . . 193 Laryngeal and Laryngotracheo-Oesophageal Cleft . . . . . . . . . . . . . . . . . . . 193

Trachea and Bronchi . . . . . . . . . . . . . . . . . . 194 Agenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194 Stenosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194 Tracheomalacia and Bronchomalacia 194 Tracheo-Oesophageal Fistula . . . . . . . . 194 Vascular Compression . . . . . . . . . . . . . . 194

Vascular Ring . . . . . . . . . . . . . . . . . . . . . 195 Vascular Sling . . . . . . . . . . . . . . . . . . . . . 195

Anomalous Bifurcations . . . . . . . . . . . . . 195 Congenital Cysts and Tumours . . . . . . 195

23 Acquired Disorders of the Larynx in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 197Neville P. Shine and Christopher Prescott

Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 197 Laryngitis . . . . . . . . . . . . . . . . . . . . . . . . . . . 197 Laryngotracheobronchitis (Croup) . . 198 Bacterial Laryngitis/Tracheitis . . . . . . . 199 Supraglottitis (Epiglottitis) . . . . . . . . . . 200 Unusual Laryngeal Infections . . . . . . . 200

Herpetic Laryngitis . . . . . . . . . . . . . . . . 200 Candidiasis . . . . . . . . . . . . . . . . . . . . . . . 201 Tuberculosis . . . . . . . . . . . . . . . . . . . . . . . 201 Diphtheria . . . . . . . . . . . . . . . . . . . . . . . . 202

Gastro-Oesphageal Reflux Disease – Laryngeal Manifestations . . . . . . . . . . . . . . 202 Laryngeal Trauma . . . . . . . . . . . . . . . . . . . . . 203

External Trauma . . . . . . . . . . . . . . . . . . . . 203 Internal Trauma . . . . . . . . . . . . . . . . . . . . 204

Inhalation Burns . . . . . . . . . . . . . . . . . . 204 Corrosive Ingestion . . . . . . . . . . . . . . . . 205 Intubation Injury . . . . . . . . . . . . . . . . . . 205

Contents XVII

Vocal Abuse . . . . . . . . . . . . . . . . . . . . . . . 207 Foreign Bodies . . . . . . . . . . . . . . . . . . . . . . . . 207 Neurological Conditions . . . . . . . . . . . . . . 208

Recurrent Laryngeal Nerve Palsy . . . . 208

24 Laryngeal Webs and Subglottic Hemangiomas . . . . . . . . . . . . . . . . . . . . . . . . . 211Michael J. Rutter

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 211 Congenital Anterior Glottic Webs . . . . . 211

Diagnostic Guidelines . . . . . . . . . . . . . . . 212 Management Strategies . . . . . . . . . . . . . . 213

Acquired Anterior Glottic Webs . . . . . . . 214 Management Strategies . . . . . . . . . . . . . . 215

Hemangiomas of Infancy . . . . . . . . . . . . . . 216 Natural History . . . . . . . . . . . . . . . . . . . . . 216

Subglottic Hemangiomas . . . . . . . . . . . . . . 217 Diagnostic Guidelines . . . . . . . . . . . . . . . 218 Management Strategies . . . . . . . . . . . . . . 220 Observation . . . . . . . . . . . . . . . . . . . . . . . . 220 Pharmacotherapy . . . . . . . . . . . . . . . . . . . 220 Endoscopic Surgical Management . . . 220 Tracheotomy . . . . . . . . . . . . . . . . . . . . . . . . 221 Open Surgical Excision . . . . . . . . . . . . . . 221

25 Post-Intubation Laryngotracheal Stenosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 223David Albert

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 224 Definition . . . . . . . . . . . . . . . . . . . . . . . . . . 224 Pathology and Aetiology . . . . . . . . . . . . 224 Incidence . . . . . . . . . . . . . . . . . . . . . . . . . . . 224 Prevention . . . . . . . . . . . . . . . . . . . . . . . . . . 224

Assessment of LTS . . . . . . . . . . . . . . . . . . . . 224 History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 224 Examination . . . . . . . . . . . . . . . . . . . . . . . . 224 Pre-Endoscopy Investigations . . . . . . . 224 Endoscopy . . . . . . . . . . . . . . . . . . . . . . . . . . 224 Grading . . . . . . . . . . . . . . . . . . . . . . . . . . . . 225

Scenarios . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 225 Failed Extubation . . . . . . . . . . . . . . . . . . . 225 Existing Tracheostomy . . . . . . . . . . . . . . 226 Recurrent Croup/Progressive Stridor 226

Management of Early Soft Stenosis . . . . 226 Laryngeal Rest . . . . . . . . . . . . . . . . . . . . . . 226 Removal of Granulations . . . . . . . . . . . . 226 Avoidance of the Laser . . . . . . . . . . . . . . 226 Endoscopic Procedures . . . . . . . . . . . . . . 226 Radial Dilatation . . . . . . . . . . . . . . . . . . . . 226 Endoscopic Decompression/Cricoid Split . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 226 Mitomycin . . . . . . . . . . . . . . . . . . . . . . . . . . 227

Conventional External Cricoid Split 227 Management of Firm Established Stenosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 227

Expectant Waiting/Natural Resolution 227 Augmentation Grafting . . . . . . . . . . . . . 227 Resection . . . . . . . . . . . . . . . . . . . . . . . . . . . 227 Prevention of Post-Operative Restenosis – Salvage Techniques . . . . . 227

26 Congenital and Acquired Tracheal Stenosis in Children . . . . . . . . . . . . . . . . . . . 229Emmanuel Le Bret and E. Noel Garabédian

Congenital Stenosis . . . . . . . . . . . . . . . . . . . 230 Assessment of the Lesion . . . . . . . . . . . . 230

Presenting Symptoms and Management . . . . . . . . . . . . . . . . . . 230 Investigation, Imaging . . . . . . . . . . . . . 230

Description of the Lesions . . . . . . . . . . . 230 The Narrowness of the Trachea . . . . . 231 The Extent of Tracheal Involvement 231 The Anatomy of the Bronchi . . . . . . . 232 Associated Lesions . . . . . . . . . . . . . . . . . 232

Surgical Treatment . . . . . . . . . . . . . . . . . . 232 Gas Exchange During Reconstruction . . . . . . . . . . . . . . . . . . . . 232 Very-Short-Segment Tracheal Stenosis . . . . . . . . . . . . . . . . . . . . . . . . . . . 233 Medium-Length Stenoses (up to Two-Thirds of Tracheal Length) . . . . . . . . . . . . . . . . . . . . . . . . . . . 233 Long and Very Long Tracheal Stenoses . . . . . . . . . . . . . . . . . . . . . . . . . . . 234 Post-Operative Complications and Their Management . . . . . . . . . . . . 234

Acquired Stenosis . . . . . . . . . . . . . . . . . . . . . 235 Aetiologies and Localisation of Acquired Tracheal Stenosis in Children . . . . . . . . . . . . . . . . . . . . . . . . . 235 Patient Assessment . . . . . . . . . . . . . . . . . . 235 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . 236

Endoscopic Procedures . . . . . . . . . . . . . 236 Open Surgical Procedure . . . . . . . . . . . 236

27 Tracheomalacia in Children . . . . . . . . . . . 241Jean-Michel Triglia, Richard Nicollas and Stephane Roman

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 241 Clinical Manifestations . . . . . . . . . . . . . . . . 242 Investigations . . . . . . . . . . . . . . . . . . . . . . . . . 242 Aetiology of Tracheomalacia . . . . . . . . . . 243

Primary Tracheomalacia . . . . . . . . . . . . 243

XVIII Contents

Secondary Tracheomalacia . . . . . . . . . . 243 Oesophageal Atresia and TOF . . . . . . . 243 Tracheal Compression due to Cardiovascular Malformations . . . . 243 Tracheal Compression due to Cervical and Mediastinal Tumours . . 244

28 Tracheostomy: an Ancient Life Saver Due for Retirement, or Vital Aid in Modern Airway Surgery? . . . . . . . . . . . 247Hans L. J. Hoeve

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 247 Definitions . . . . . . . . . . . . . . . . . . . . . . . . . . . 247 Indications . . . . . . . . . . . . . . . . . . . . . . . . . . . . 247 Alternatives to Tracheotomy . . . . . . . . . . . 248 The Technique of Tracheotomy . . . . . . . . 248 Complications . . . . . . . . . . . . . . . . . . . . . . . . 249

Complications of a Tracheotomy . . . . 249 Complications of a Tracheostomy . . . 250

Types and Sizes of Tracheostomy Tube 250 Tracheostomy Care in the Hospital, an Institution or at Home . . . . . . . . . . . . . 251 Decannulation . . . . . . . . . . . . . . . . . . . . . . . . 252

29 Recurrent Respiratory Papillomatosis 255Brian J. Wiatrak

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 255 Historical Perspective . . . . . . . . . . . . . . . . . 256 Pathogenesis of RRP in Children . . . . . . 256 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . 258 Diagnosis and Staging of RRP in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . 258 Disease Progression and Complications 259 Managing RRP in Children . . . . . . . . . . . . 260 Principles of Surgical Management . . . . 260

Anesthesia/Airway Management Techniques . . . . . . . . . . . . . . . . . . . . . . . . . 261

Spontaneous Ventilation Technique 261 Intermittent Apnea Technique . . . . . . 261 Jet Ventilation Technique . . . . . . . . . . 261

Endoscopy Techniques . . . . . . . . . . . . . . 261 Laser Techniques . . . . . . . . . . . . . . . . . . . 261 Microdebridement . . . . . . . . . . . . . . . . . . 261

Adjuvant Medical Therapy . . . . . . . . . . . . 262 Indol-3 Carbinol . . . . . . . . . . . . . . . . . . . . 262 Alpha Interferon . . . . . . . . . . . . . . . . . . . . 262 Cidofovir . . . . . . . . . . . . . . . . . . . . . . . . . . . 262 Photodynamic Therapy . . . . . . . . . . . . . 263 Other Agents . . . . . . . . . . . . . . . . . . . . . . . 263 Investigational Agents . . . . . . . . . . . . . . . 263

Voice Outcome . . . . . . . . . . . . . . . . . . . . . . . . 263

30 Pharynx and Oesophagus . . . . . . . . . . . . . 267David Rawat and Mike Thomson

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 267 Ontogeny of Oesophageal Motor Function . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 269 Swallowing in the Normal Infant and Child . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 269

The Pharyngeal Phase . . . . . . . . . . . . . . . 269 The Oesophageal Phase . . . . . . . . . . . . . 270

Oesophageal Sphincter Physiology . . 271 Sphincter Dysfunction . . . . . . . . . . . . . . 271

Mucosal Immunology and Inflammation . . . . . . . . . . . . . . . . . . . . . . . . . 271 Gastro-Oesophageal Reflux . . . . . . . . . . . . 271

Pathophysiology of GOR . . . . . . . . . . . . 272 GOR-Related Apparent Life-Threatening Events and Sudden Infant Death Syndrome . . . . . . . . . . . . . 273 GOR and Asthma . . . . . . . . . . . . . . . . . . . 273 Recurrent Pneumonia . . . . . . . . . . . . . . . 273 Upper Airway Symptoms . . . . . . . . . . . . 273 Chronic Tubotympanic Disorders . . . 273 Diagnostic Options . . . . . . . . . . . . . . . . . 274 Intraluminal Impedance . . . . . . . . . . . . . 274 Endoscopy and Biopsy Sampling . . . . 274

Oesophageal Dysmotility, Extrinsic Compression and Mechanical Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . 274

31 Paediatric Thyroid Disease . . . . . . . . . . . . 277Michael Kuo and Tim Barrett

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 277 Embryology, Physiology and Anatomy 277

Embryology . . . . . . . . . . . . . . . . . . . . . . . . 277 Physiology . . . . . . . . . . . . . . . . . . . . . . . . . . 278 Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 278

Investigation of the Thyroid Gland . . . . 279 Thyroid Function Tests . . . . . . . . . . . . . . 279 Thyroid Immunology . . . . . . . . . . . . . . . 279 Genetic Testing . . . . . . . . . . . . . . . . . . . . . 279 Ultrasonography/Computed Tomography/Magnetic Resonance Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . 280 Radionucleotide Scan . . . . . . . . . . . . . . . 280 Fine-Needle Aspiration . . . . . . . . . . . . . 280

Congenital Abnormalities . . . . . . . . . . . . . 280 Thyroglossal Duct Cysts and Fistulae, and Ectopic Thyroid . . . . . . . . . . . . . . . . 280 Congenital Hypothyroidism . . . . . . . . . 280 Syndromes Associated with Thyroid Abnormalities . . . . . . . . . 281

Functional Abnormalities of the Thyroid Gland . . . . . . . . . . . . . . . . . . 281

Contents XIX

Hypothyroidism . . . . . . . . . . . . . . . . . . . . 281 Hyperthyroidism . . . . . . . . . . . . . . . . . . . . 281

Neoplasms of the Thyroid Gland . . . . . . 282 Approach to the Solitary Thyroid Nodule in Children . . . . . . . . . . . . . . . . . 282 Sporadic Differentiated Thyroid Cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 282 MTC and the Management of the Thyroid in MEN-2 . . . . . . . . . . . . 283

32 Nasal Foreign Bodies, Epistaxis and Nasal Trauma . . . . . . . . . . . . . . . . . . . . . . 285Sean Carrie

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 285 Foreign Bodies . . . . . . . . . . . . . . . . . . . . . . . . 285

Types . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 285 Presentation . . . . . . . . . . . . . . . . . . . . . . . . 285 Signs and Symptoms . . . . . . . . . . . . . . . . 286 Management . . . . . . . . . . . . . . . . . . . . . . . . 286 Rhinolith . . . . . . . . . . . . . . . . . . . . . . . . . . . 286

Epistaxis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 286 Prevalence . . . . . . . . . . . . . . . . . . . . . . . . . . 286 Blood Supply to the Nose . . . . . . . . . . . . 286

Aetiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 287 Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 287 Assessment and Management . . . . . . . . 287

Nasal Trauma . . . . . . . . . . . . . . . . . . . . . . . . . 288 Assessment . . . . . . . . . . . . . . . . . . . . . . . . . 288 Management . . . . . . . . . . . . . . . . . . . . . . . . 288

33 Management of Choanal Atresia . . . . . . 291Patrick Froehlich and Sonia Ayari-Khalfallah

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 291 Anatomical and Physiological Background . . . . . . . . . . . . . . . . . . . . . . . . . . . 291 Clinical Presentation . . . . . . . . . . . . . . . . . . 292 Management is by Surgery . . . . . . . . . . . . . 292

Intranasal Approach . . . . . . . . . . . . . . . . 292 Transpalatine Approach . . . . . . . . . . . . . 293

Indications and Timing of Surgery . . . . . 293 Contraindications . . . . . . . . . . . . . . . . . . . . . 293 Risks and Side Effects . . . . . . . . . . . . . . . . . 293

34 Allergic Rhinitis . . . . . . . . . . . . . . . . . . . . . . . . 295Alexander N. Greiner

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 295 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . 295 Etiology of AR . . . . . . . . . . . . . . . . . . . . . . . . 297 AR and its Associated Conditions and Comorbidities . . . . . . . . . . . . . . . . . . . . 297

Quality of Life . . . . . . . . . . . . . . . . . . . . . . . . 298 Classification . . . . . . . . . . . . . . . . . . . . . . . . . 298 Treatment Modalities . . . . . . . . . . . . . . . . . 300

Environmental Control Measures . . . . 300 Overview of Pharmacological Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . 300

H1-Antihistamines . . . . . . . . . . . . . . . . 300 Intranasal Corticosteroids . . . . . . . . . 302 Decongestants . . . . . . . . . . . . . . . . . . . . . 302 Cromolyn . . . . . . . . . . . . . . . . . . . . . . . . . 303 Antileukotrienes . . . . . . . . . . . . . . . . . . . 303 Omalizumab (Anti-IgE) . . . . . . . . . . . 304

Allergen Immunotherapy . . . . . . . . . . . . 304 Other Treatment Modalities . . . . . . . . . 304

35 Rhinosinusitis in Children . . . . . . . . . . . . . 307Peter A. R. Clement

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 307 Morphogenesis, Epidemiology and Pathophysiology . . . . . . . . . . . . . . . . . . 308 Classification and Definitions . . . . . . . . . 310

Acute Rhinosinusitis . . . . . . . . . . . . . . . . 310 Chronic Rhinosinusitis . . . . . . . . . . . . . . 311

Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 312 Presenting Symptoms of Rhinosinusitis in Children . . . . . . . . 312 Clinical Examination . . . . . . . . . . . . . . . . 312 Microbiology . . . . . . . . . . . . . . . . . . . . . . . 312 Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . 312 Additional Investigations . . . . . . . . . . . . 312

Respiratory Allergy . . . . . . . . . . . . . . . . 313 Immune Deficiency . . . . . . . . . . . . . . . . 313 Cystic Fibrosis . . . . . . . . . . . . . . . . . . . . . 313 Primary Ciliary Dyskinesia . . . . . . . . 313 Gastro-Oesophageal Reflux . . . . . . . . 313 Mechanical Obstruction . . . . . . . . . . . 313 Miscellaneous . . . . . . . . . . . . . . . . . . . . . 313

Therapeutic Management . . . . . . . . . . . . . 314 Medical Treatment . . . . . . . . . . . . . . . . . . 314

Antimicrobial Therapy . . . . . . . . . . . . . 314 Additional Medical Therapy . . . . . . . 316

Surgical Treatment . . . . . . . . . . . . . . . . . . . . 317 Adenoidectomy . . . . . . . . . . . . . . . . . . . . . 317 Antral Lavage, Nasal Antral Window 317 Endoscopic Sinus Surgery . . . . . . . . . . . 317

Absolute Indications . . . . . . . . . . . . . . . 318 Possible Indications . . . . . . . . . . . . . . . . 318

Complications of Rhinosinusitis . . . . . . . 318 Osteomyelitis of the Maxilla . . . . . . . . . 318 Odontogenic Sinusitis . . . . . . . . . . . . . . . 318 Orbital and Intracranial Complications . . . . . . . . . . . . . . . . . . . . . . 318

Pott’s Puffy Tumour . . . . . . . . . . . . . . . 318 Osteomyelitis of the Posterior Wall 319 Orbital Complications . . . . . . . . . . . . . 320

XX Contents

Mucocoeles . . . . . . . . . . . . . . . . . . . . . . . . . 323

36 Management of the Deaf Child . . . . . . . 327Kevin Patrick Gibbin

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 327 Aetiology and Pathology of Childhood Deafness . . . . . . . . . . . . . . . . 328

Pre-natal Causes . . . . . . . . . . . . . . . . . . . . 328 Perinatal Causes . . . . . . . . . . . . . . . . . . . . 328 Post-natal Causes . . . . . . . . . . . . . . . . . . . 329

Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . 329 Risk Factors for Hearing Loss in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 330 Identification of Hearing Loss . . . . . . . . . 330

Screening – Targeted versus Universal 330 Early Childhood/Pre-school Programmes . . . . . . . . . . . . . . . . . . . . . . . . 330 Monitoring of Special Groups . . . . . . . 331

Post-Meningitis Screening . . . . . . . . . . 331 Reactive Testing . . . . . . . . . . . . . . . . . . . . . 331 Investigation . . . . . . . . . . . . . . . . . . . . . . . . 331

Clinical History Taking . . . . . . . . . . . . 331 Clinical Examination . . . . . . . . . . . . . . 332 Special Investigations . . . . . . . . . . . . . . 332

Audiological Assessment . . . . . . . . . . . . 333 Treatment of Children with Hearing Loss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 333

37 Audiometric Testing of Children . . . . . . 337John Graham, Milanka Drenovak and William Hellier

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 338 Neonatal Tests Used From Soon After Birth Until Six Months . . . . . . . . . . . . . . . . 339

Transient Otoacoustic Emission Testing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 339

Benefits . . . . . . . . . . . . . . . . . . . . . . . . . . . 339 Limitations . . . . . . . . . . . . . . . . . . . . . . . 339 Use . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 339

Distortion Product Otoacoustic Emissions . . . . . . . . . . . . . . . . . . . . . . . . . . . 339 Auditory Brainstem Response . . . . . . . 339

Benefits . . . . . . . . . . . . . . . . . . . . . . . . . . . 341 Limitations . . . . . . . . . . . . . . . . . . . . . . . 341 Use . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 341

Electrocochleography . . . . . . . . . . . . . . . 342 Benefits . . . . . . . . . . . . . . . . . . . . . . . . . . . 342 Limitation . . . . . . . . . . . . . . . . . . . . . . . . 342

Behavioural Testing: Ages Six Months to Three Years . . . . . . . . . . . . . . . . . . . . . . . . 342

Distraction Testing: Age Six to Eighteen Months . . . . . . . . . 342

Benefits . . . . . . . . . . . . . . . . . . . . . . . . . . . 343

Limitations . . . . . . . . . . . . . . . . . . . . . . . 343 Use . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 343

Visual Reinforcement Audiometry: Age Six Months to Three Years . . . . . . 344

Benefits . . . . . . . . . . . . . . . . . . . . . . . . . . . 344 Limitations . . . . . . . . . . . . . . . . . . . . . . . 344 Use . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 344

Conditioned Audiometry: Two to Three Years and Upwards . . . . . . 344

Play Audiometry: Age Two to Three Years and Upwards 344

Benefits . . . . . . . . . . . . . . . . . . . . . . . . . . . 345 Limitations . . . . . . . . . . . . . . . . . . . . . . . 345

Speech Audiometry: Age Two to Six Years and Upwards . . 345

Benefits . . . . . . . . . . . . . . . . . . . . . . . . . . . 346 Limitations . . . . . . . . . . . . . . . . . . . . . . . 346

Cortical Electric Response Audiometry 346 Audiometric Steady-State Response . . . . 347

Benefits . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 348 Limitations . . . . . . . . . . . . . . . . . . . . . . . . . 348 Use . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 348

Tympanometry and Acoustic Reflexes 348 Special Situations . . . . . . . . . . . . . . . . . . . . . 349

38 Otoplasty and Common Auricular Deformities . . . . . . . . . . . . . . . . . . . . . . . . . . . . 351Werner J. Heppt

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 351 Basics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 352

Development of the Auricle . . . . . . . . . 352 Clinical Anatomy . . . . . . . . . . . . . . . . . . . 352

Classification of Congenital Deformities 353 Protruding Ears (Prominent Ears, Bat Ears) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 353

Causes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 353 Conservative Treatment Options . . . . 353 Surgical Correction (Pinnaplasty) . . . 354

Goals . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 354 Choice of Appropriate Method . . . . . 354 Antihelix . . . . . . . . . . . . . . . . . . . . . . . . . . 354 Conchal Hyperplasia, Increased Conchomastoid Angle . . . . . . . . . . . . . 356 Prominent Lobule . . . . . . . . . . . . . . . . . 357 Severe Deformities, Revision Surgery 357

Operative Management . . . . . . . . . . . . . 357 Timing . . . . . . . . . . . . . . . . . . . . . . . . . . . . 357 Medical Consultation . . . . . . . . . . . . . . 357 Peri- and Post-operative Care . . . . . . 357 Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . 357

Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 358 Abrasion . . . . . . . . . . . . . . . . . . . . . . . . . . . . 358 Haematoma . . . . . . . . . . . . . . . . . . . . . . . . . 358 Laceration . . . . . . . . . . . . . . . . . . . . . . . . . . 358 Thermal Injuries, Frostbite . . . . . . . . . . 359

Contents XXI

39 Diagnosis and Management Strategies in Congenital Middle and External Ear Anomalies . . . . . . . . . . . 361Frank Declau, Paul Van De Heyning and Cor Cremers

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 362 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . 362 Classification . . . . . . . . . . . . . . . . . . . . . . . . . 363

Ossicular Malformation . . . . . . . . . . . . . 363 Atresia Classification . . . . . . . . . . . . . . . . 363 Microtia Classification . . . . . . . . . . . . . . 364

Patient Evaluation . . . . . . . . . . . . . . . . . . . . . 364 History and Physical Examination . . . 364 Audiometric Evaluation . . . . . . . . . . . . . 365 Radiological Assessment . . . . . . . . . . . . 365

Medical Management . . . . . . . . . . . . . . . . . 365 Unilateral Atresia . . . . . . . . . . . . . . . . . . . 366 Bilateral Atresia . . . . . . . . . . . . . . . . . . . . . 366

Surgical Management . . . . . . . . . . . . . . . . . 366 Ossicular Malformation Surgery . . . . . 366 Atresia Repair Surgery . . . . . . . . . . . . . . 366

Surgical Planning . . . . . . . . . . . . . . . . . . 367 Timing of Surgery . . . . . . . . . . . . . . . . . 368 Surgical Techniques . . . . . . . . . . . . . . . . 368 Surgical Results . . . . . . . . . . . . . . . . . . . 369 Complications . . . . . . . . . . . . . . . . . . . . . 370

Bone Conduction Implant Surgery . . 370 Total Auricular Reconstruction Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 370

Surgical Planning . . . . . . . . . . . . . . . . . . 370 Surgical Techniques . . . . . . . . . . . . . . . . 370 Comparison of the Two Techniques 372 Other Techniques . . . . . . . . . . . . . . . . . . 372 Results in Total Auricular Reconstruction . . . . . . . . . . . . . . . . . . . . 372 Complications in Total Auricular Reconstruction . . . . . . . . . . . . . . . . . . . . 372 Complications at the Donor Site . . . . 372 Complications at the Graft Site . . . . . 372

Prosthetic Auricular Reconstruction . 373 Indications . . . . . . . . . . . . . . . . . . . . . . . . 373 Results and Complications of Prosthetic Reconstruction . . . . . . . . 373

40 Imaging of the Deaf Child . . . . . . . . . . . . . 377Timothy J. Beale and Gitta Madani

Part 1. A Radiological Overview of the Imaging of Children with Hearing Loss . . . . . . . . . . . . . . . . . . . . . . . . . . 378

Key Points of CT Imaging . . . . . . . . . . . 378 CT Imaging Protocols in Children . . 378

Key Points of MRI . . . . . . . . . . . . . . . . . . 378 MRI Protocols in Children . . . . . . . . . 378

Sedation . . . . . . . . . . . . . . . . . . . . . . . . . . . . 379

CT: Normal Cross-Sectional Anatomy 379 Systematic Review of CT Images . . . . 379

Normal MRI Anatomy . . . . . . . . . . . . . . 381 Systematic Review of MRI Images . . 381

When is Early Imaging Essential? . . . . 382 Imaging Algorithms for Conductive, Sensorineural and Mixed Hearing . . . 382

Conductive Hearing Loss . . . . . . . . . . . 382 Sensorineural Hearing Loss . . . . . . . . 382 Mixed Hearing Loss . . . . . . . . . . . . . . . 382

Part 2. Radiological Abnormalities Found in Children with Hearing Loss . . 382

Embryology of the Inner and Middle Ear and the EAC . . . . . . . . . . . . . . . . . . . . 382

Inner Ear . . . . . . . . . . . . . . . . . . . . . . . . . 382 Middle Ear . . . . . . . . . . . . . . . . . . . . . . . . 382 External Ear . . . . . . . . . . . . . . . . . . . . . . 382

Conductive Hearing Loss . . . . . . . . . . . . 383 Radiological Features of Congenital Aural Dysplasia . . . . . . . 383 Congenital Middle Ear Anomalies . . . 383 Syndromes Associated with Congenital CHL . . . . . . . . . . . . . . . . . . . . 384

Otocraniofacial Syndromes . . . . . . . . . 384 Craniosynostoses . . . . . . . . . . . . . . . . . . 386 Otocervical Syndromes . . . . . . . . . . . . 386

Acquired CHL . . . . . . . . . . . . . . . . . . . . . . 386 Trauma: Ossicular Disruption . . . . . . 386 Tympanosclerosis . . . . . . . . . . . . . . . . . . 388

Sensorineural Hearing Loss . . . . . . . . . 388 Congenital SNHL . . . . . . . . . . . . . . . . . . . 388

Abnormal Development of the Inner Ear . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 388 Syndromes Associated with Congenital SNHL . . . . . . . . . . . . . 390 The Otoskeletal Syndromes (Bone dysplasias) . . . . . . . . . . . . . . . . . . 392

Acquired SNHL . . . . . . . . . . . . . . . . . . . . . 395 Post-Meningitic Labyrinthitis Obliterans . . . . . . . . . . . . . . . . . . . . . . . . 395

The Future . . . . . . . . . . . . . . . . . . . . . . . . . . . . 395

41 Acute Otitis Media in Children . . . . . . . . 399Ingeborg Dhooge

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 400 Definitions . . . . . . . . . . . . . . . . . . . . . . . . . 400

Otitis Media . . . . . . . . . . . . . . . . . . . . . . 400 Acute Otitis Media . . . . . . . . . . . . . . . . 400 AOM Without Effusion (Myringitis) 400 Recurrent Otitis Media . . . . . . . . . . . . 400 Otorrhoea . . . . . . . . . . . . . . . . . . . . . . . . . 400

Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . 400 Predisposing Factors and Environmental Pressure . . . . . . . . . . . 400

Host-Related Parameters . . . . . . . . . . . . 400

XXII Contents

Age . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 400 Gender . . . . . . . . . . . . . . . . . . . . . . . . . . . 401 Race . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 401 Altered Host Defences and Underlying Diseases . . . . . . . . . . . 401 Genetic Predisposition . . . . . . . . . . . . . 401 Prenatal and Perinatal Factors . . . . . 401

Environmental Factors . . . . . . . . . . . . . . 401 Climate and Seasons . . . . . . . . . . . . . . . 401 Day Care/Home Care, Exposure to Other Children . . . . . . . . 401 Passive Smoking and Environmental Pollution . . . . . . . . . . . . . . . . . . . . . . . . . . 401 Socio-Economic Status/Poverty . . . . . 401 Use of Dummies (Pacifiers) . . . . . . . . 401

Pathogenesis of otitis media . . . . . . . . . . . 402 Eustachian Tube Dysfunction . . . . . . . 402 Immature Immune Response . . . . . . . . 402 Microbial Infection . . . . . . . . . . . . . . . . . 403

Natural History and Complications . . . . 403 Acute Perforation . . . . . . . . . . . . . . . . . . . 403 Suppurative Complications . . . . . . . . . . 403 Intratemporal (Extracranial) Complications of Otitis Media . . . . . . . 404

Subperiosteal Abscess . . . . . . . . . . . . . . 404 Facial Palsy . . . . . . . . . . . . . . . . . . . . . . . 404 Labyrinthitis . . . . . . . . . . . . . . . . . . . . . . 404

Intracranial Complications of Otitis Media . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 404

Assessment and Diagnosis . . . . . . . . . . . . . 405 Symptoms and Signs . . . . . . . . . . . . . . . . 405 Otoscopy . . . . . . . . . . . . . . . . . . . . . . . . . . . 405 Conventional Tympanometry . . . . . . . 405 Conventional Audiometry . . . . . . . . . . . 405 Other Diagnostic Procedures . . . . . . . . 405 Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . 406

Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 406 Medical . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 406

Symptomatic Treatment . . . . . . . . . . . 406 Antibacterial Treatment . . . . . . . . . . . 406 “Alternative” Medical Treatments . . . 408

Surgical Therapy . . . . . . . . . . . . . . . . . . . . 408 Myringotomy . . . . . . . . . . . . . . . . . . . . . . 408 Tympanostomy Tube Insertion . . . . . 408 Adenoidectomy . . . . . . . . . . . . . . . . . . . . 408

Follow-Up . . . . . . . . . . . . . . . . . . . . . . . . . . . . 409 Prevention of AOM . . . . . . . . . . . . . . . . . . . 409 Sequelae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 409

42 Otitis Media With Effusion . . . . . . . . . . . . 413Peter J. Robb

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 413 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . 414

Birth Order . . . . . . . . . . . . . . . . . . . . . . . . . 414 Gender . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 414

Breast-Feeding . . . . . . . . . . . . . . . . . . . . . . 414 Day-Care . . . . . . . . . . . . . . . . . . . . . . . . . . . 414 Parental Smoking . . . . . . . . . . . . . . . . . . . 414 Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . 414 Immune Deficiency and Allergy . . . . . 415 Reflux of Gastric Acid . . . . . . . . . . . . . . . 415

Symptoms and Signs of OME . . . . . . . . . . 415 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 415

Audiology . . . . . . . . . . . . . . . . . . . . . . . . . . 416 Natural History . . . . . . . . . . . . . . . . . . . . . . . 416 Complications . . . . . . . . . . . . . . . . . . . . . . . . 417 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 417

Medical . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 417 Hearing Aids . . . . . . . . . . . . . . . . . . . . . . . 417 Surgical . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 417

Grommet Surgery (Ventilation Tubes) . . . . . . . . . . . . . . . . . . . . . . . . . . . . 417 Adenoidectomy . . . . . . . . . . . . . . . . . . . . 418

Complications of Surgical Management 418 Premature Extrusion of the Grommets and Perforation . . . . . . . . . . 418 Otorrhoea . . . . . . . . . . . . . . . . . . . . . . . . . . 418 Myringosclerosis (Tympanosclerosis) 419 Atelectasis . . . . . . . . . . . . . . . . . . . . . . . . . . 419 Post-adenoidectomy Haemorrhage . . 419

43 Chronic Otitis Media . . . . . . . . . . . . . . . . . . . 421John Hamilton

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 422 Histology of Chronic Inflammation of the Middle Ear Mucosa . . . . . . . . . . . . . 423 Perforation of the Tympanic Membrane and Chronic Suppurative Otitis Media 423

Epidemiology . . . . . . . . . . . . . . . . . . . . . . . 423 Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . 424 Signs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 425 Investigation . . . . . . . . . . . . . . . . . . . . . . . . 425 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . 425

Prophylaxis . . . . . . . . . . . . . . . . . . . . . . . 425 Medical Treatment . . . . . . . . . . . . . . . . 426 Surgical Closure of Tympanic Membrane Perforations in Children with CSOM (Tympanoplasty) . . . . . . 426

Tympanic Membrane Atrophy and Retraction Pockets: Pars Tensa . . . . 427

Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . 427 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . 427 Classification . . . . . . . . . . . . . . . . . . . . . . . 428 Natural History . . . . . . . . . . . . . . . . . . . . . 429 Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . 429 Principles of Surgical Intervention for Tympanic Membrane Atrophy and Retraction Pockets . . . . . . . . . . . . . . 430 Surgical Treatment of Tympanic Membrane Atrophy . . . . . . . . . . . . . . . . . 430

Contents XXIII

Grommet Insertion . . . . . . . . . . . . . . . . 430 Reinforcement Tympanoplasty . . . . . 430 Retraction Pocket Excision . . . . . . . . . 430

Attic Retraction Pockets . . . . . . . . . . . . . . . 430 Cholesteatoma . . . . . . . . . . . . . . . . . . . . . . . . 432

Pathogenesis and Classification . . . . . . 432 Congenital Cholesteatoma . . . . . . . . . 432 Acquired Cholesteatoma . . . . . . . . . . . 433

Epidemiology . . . . . . . . . . . . . . . . . . . . . . . 433 Anatomy of Paediatric Cholesteatoma 433 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . 434

Medical . . . . . . . . . . . . . . . . . . . . . . . . . . . 434 Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . 434 Surgical . . . . . . . . . . . . . . . . . . . . . . . . . . . 435 Surgical Approaches for the Removal of Cholesteatoma and Restoration of a Stable, Dry Ear . . . . . . . . . . . . . . . 435

Residual and Recurrent Cholesteatoma . . . . . . . . . . . . . . . . . . . . . . 436

Residual Cholesteatoma . . . . . . . . . . . 436 Recurrent Cholesteatoma . . . . . . . . . . 437

Changes in the Ossicles Secondary to Chronic Middle Ear Inflammation . . 437

Epidemiology . . . . . . . . . . . . . . . . . . . . . . . 437 Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . 437 Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . 437 Signs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 437 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . 438 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . 438 Outcome Measures . . . . . . . . . . . . . . . . . . 438

Factors that Influence the Success of Ossicular Reconstruction . . . . . . . . 438

Complications of CSOM in Children . . 439

44 Bone-Anchored Hearing Aid (BAHA) and Softband . . . . . . . . . . . . . . . . . . . . . . . . . . 441David W. Proops

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 441 Conductive Hearing Loss in Children . . 442 Management of Congenital Conductive Hearing Loss . . . . . . . . . . . . . . . . . . . . . . . . . . 442 Classification of Congenital Ear Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 442 Investigations of Congenital Middle and External Ear Malformations . . . . . . . 442 Aiding Options for Congenital Conductive Hearing Loss . . . . . . . . . . . . . . 443 Surgical Management of Congenital Conductive Hearing Loss . . . . . . . . . . . . . . 443

Reconstructive Surgery of the External and Middle Ear . . . . . . 443 The Bone-Anchored Hearing Aid . . . . 443

BAHA Surgical Procedure . . . . . . . . . . 443 Results in Bilateral Congenital Conductive Hearing Loss . . . . . . . . . . . 444

Unilateral Congenital Conductive Hearing Loss . . . . . . . . . . . . . . . . . . . . . . 444 Fixture Survival and Skin Care . . . . . 444

Providing a Service for Children with Congenital Conductive Deafness 444 Softband . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 445

45 Cochlear Implantation in Children . . . . 447Mary Beth Brinson and John Graham

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 448 Team Approach . . . . . . . . . . . . . . . . . . . . . . . 449 Referral Guidelines . . . . . . . . . . . . . . . . . . . . 449

Group I: Congenital Profound Hearing Loss . . . . . . . . . . . . . . . . . . . . . . . . 450 Group II: Progressive Hearing Loss . . 450 Group III: Severe-to-Profound Bilateral Hearing Loss . . . . . . . . . . . . . . . 450 Group IV: Sudden Hearing Loss . . . . . 451

Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . 451 Audiological Scientist . . . . . . . . . . . . . . . 452 Speech and Language Therapist . . . . . 452 Teacher of the Deaf . . . . . . . . . . . . . . . . . 452 Psychologist . . . . . . . . . . . . . . . . . . . . . . . . 452 Medical Team Members . . . . . . . . . . . . . 453 Parents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 453

Implantation Criteria . . . . . . . . . . . . . . . . . . 453 The Child . . . . . . . . . . . . . . . . . . . . . . . . . . . 453 The Child’s Environment . . . . . . . . . . . . 453

Factors Affecting the Outcome of Cochlear Implantation . . . . . . . . . . . . . . 453 Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 454

Approach . . . . . . . . . . . . . . . . . . . . . . . . . . . 454 Preparation of the Bed for the Receiver–Stimulator Package 454 Insertion of the Electrode Array . . . . . 454

Special Medical and Surgical Situations 455 Jervell and Lange-Nielsen Syndrome 455 Meningitis . . . . . . . . . . . . . . . . . . . . . . . . . . 455 Prematurity . . . . . . . . . . . . . . . . . . . . . . . . . 455 Otitis Media . . . . . . . . . . . . . . . . . . . . . . . . 455 Ossified Cochleae . . . . . . . . . . . . . . . . . . . 455 Congenital Malformation of the Cochlea . . . . . . . . . . . . . . . . . . . . . . 456 Surgical “Gusher” . . . . . . . . . . . . . . . . . . . 456 Chronic Suppurative Otitis Media . . . 456

Complications After Implantation . . . . . 456 Device Failure . . . . . . . . . . . . . . . . . . . . . . 456 Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . 457 Skin Breakdown . . . . . . . . . . . . . . . . . . . . 457 Facial Nerve Stimulation . . . . . . . . . . . . 457

Further Considerations . . . . . . . . . . . . . . . . 457 Bilateral Cochlear Implantation in Children . . . . . . . . . . . . . . . . . . . . . . . . . 457

Future Prospects . . . . . . . . . . . . . . . . . . . . . . 457

XXIV Contents

46 The Dizzy Child . . . . . . . . . . . . . . . . . . . . . . . . 459Linda Luxon and Waheeda Pagarkar

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . 460 Clinical Evaluation . . . . . . . . . . . . . . . . . . . . 461

History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 461 General Examination . . . . . . . . . . . . . . . . 462 Eye Movement Examination . . . . . . . . . 463

Dix-Hallpike Positional Test . . . . . . . 463 Halmagyi Head Thrust Sign . . . . . . . 464 Head-Shaking Nystagmus . . . . . . . . . . 464 Dynamic Visual Acuity . . . . . . . . . . . . 465

Tests for Posture Control and Gait . . . 465 Neurological Examination . . . . . . . . . . . 466

Investigations for the Dizzy Child . . 466 Other Tests . . . . . . . . . . . . . . . . . . . . . . . . . 467

Causes of Vertigo/Dizziness/Imbalance in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . 469

Congenital Disorders . . . . . . . . . . . . . . . 469 Syndromic Hearing Loss and Vestibular Dysfunction . . . . . . . . . . . . 469 Nonsyndromic Hearing Loss and Vestibular Dysfunction . . . . . . . . 470

Otological . . . . . . . . . . . . . . . . . . . . . . . . . . 470 Inner Ear Infection or Inflammation 470

Traumatic . . . . . . . . . . . . . . . . . . . . . . . . . . . 471 Head Injury . . . . . . . . . . . . . . . . . . . . . . . 471 Benign Paroxysmal Positional Vertigo . . . . . . . . . . . . . . . . . . . . . . . . . . . . 471 Perilymphatic Fistula . . . . . . . . . . . . . . 472 Cochlear Implant Surgery . . . . . . . . . . 472

Idiopathic . . . . . . . . . . . . . . . . . . . . . . . . . . 472 Menière’s Disease . . . . . . . . . . . . . . . . . . 472

Ototoxicity . . . . . . . . . . . . . . . . . . . . . . . . . 472 Neurological . . . . . . . . . . . . . . . . . . . . . . . . 472

Migraine and Migraine Precursors 472 Episodic Ataxia Type 2 . . . . . . . . . . . . . . 474 Neoplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . 475 Cardiovascular Causes . . . . . . . . . . . . . . 475

Orthostatic Hypotension . . . . . . . . . . . 475 Miscellaneous Causes . . . . . . . . . . . . . . . 475

Ocular Disorders . . . . . . . . . . . . . . . . . . 475 Psychological Causes . . . . . . . . . . . . . . . . 475

Vertigo of Psychogenic Origin . . . . . . 475 Management of Dizziness . . . . . . . . . . . . . 475

Treatment of Paediatric Migraine . . . . 476 Vestibular Management . . . . . . . . . . . . . 476

47 The Facial Nerve . . . . . . . . . . . . . . . . . . . . . . . 479Chris Rittey

Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 479 Upper Motor Neurone Input . . . . . . . . 479

Clinical Assessment . . . . . . . . . . . . . . . . . . . 480 Isolated Facial Weakness . . . . . . . . . . . . . . 481

Bell’s Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . 481 Rare Causes of Isolated Unilateral Facial Palsy . . . . . . . . . . . . . . . . . . . . . . . . . 481 Congenital Facial Palsy . . . . . . . . . . . . . . 482

Facial Palsy as Part of Other Cranial Nerve Palsies . . . . . . . . . . . . . . . . . . . . . . . . . . 482

Ramsay Hunt Syndrome . . . . . . . . . . . . . 482 Hemifacial Spasms . . . . . . . . . . . . . . . . . . . . 483Developmental Data . . . . . . . . . . . . . . . . . . 485

Airway . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 485

Appendix . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 485Robert J. Ruben

Sinuses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 486The Adenoid . . . . . . . . . . . . . . . . . . . . . . . . 486Language and Speech . . . . . . . . . . . . . . . 486

Subject Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 489

Contents XXV

David Albert FRCSConsultant OtolaryngologistDepartment of Paediatric OtolaryngologyGreat Ormond Street Hospital for ChildrenGreat Ormond StreetLondon WC1N 3JHUKEmail: albert@easynet.co.uk

Sonia Ayari-Khalfallah MDDepartment of Otorhinolaryngology and Cervico-Facial SurgeryEdouard Herriot Hospital5 place d’Arsonval69437 Lyon cedex 03FranceEmail: sonia.khalfallah@chu-lyon.fr

C. Martin Bailey BSc, FRCSConsultant OtolaryngologistDepartment of Paediatric OtolaryngologyGreat Ormond Street Hospital for ChildrenGreat Ormond StreetLondon WC1N 3JHUKEmail: bailem@gosh.nhs.uk

Helen Bantock BSc, MB BS, FRCP, FRCPCHSenior Lecturer in Paediatrics and Consultant Physician (Retired)University College LondonGower StreetLondon WC1E 6BT

Ian Barker MB ChB, FRCAConsultant AnesthetistSheffield Children’s HospitalDepartment of AnaesthesiaWestern BankSheffield S10 2THUKEmail: i.barker@sheffield.ac.uk

Timothy G. Barrett PhD, MB, BS, MRCP, MRCPCH, DCHProfessor of Paediatric EndocrinologyPaediatric Thyroid ServiceBirmingham Children’s HospitalSteelhouse LaneBirmingham B4 6NHUKEmail: thyroid@bch.nhs.uk

Judith Barton BA (Hons) RGN, RSCNHead of NursingOperating TheatresSheffield Children’s HospitalWestern BankSheffield S10 2THUK

Timothy Beale MB, BS, FRCS, FRCRDepartment of RadiologyRoyal National Throat Nose & Ear Hospital330 Grays Inn RoadLondon WC1X 8DAUKEmail: timothy.beale@royalfree.nhs.uk

Maria Bitner-Glindzicz BSc, MBBS, DCH, PhD, FRCPReader in Clinical and Molecular GeneticsClinical and Molecular Genetics UnitInstitute of Child Health30 Guilford St.London WC1N 1EHUKEmail: mbitnerg@ich.ucl.ac.uk

John Boorman BSc (Hons), MB, ChB, FRCS (Plast)Queen Victoria HospitalHoltye RoadEast GrinsteadWest Sussex RH19 3DZUKEmail: john.boorman@virgin.net

Mark Boseley MD, MSPediatric OtolaryngologistMadigan Army Medical Center9040 A Fitzsimmons DriveTacoma, WA 98431USAEmail: mboseley@comcast.net

List of Contributors

Mary Beth Brinson Au.D, FAAA CCC-CRoyal National Throat Nose & Ear Hospital330 Grays Inn RoadLondon WC1X 8DAUKEmail: mbbrinson@aol.com

Peter D. Bull FRCSSheffield Children’s HospitalWestern BankSheffield S10 2THUKEmail: peter.bull@sheffield.ac.uk

Andrew Bush MB, BS (Hons), MA, MD, FRCP, FRCPCHProfessor of Paediatric RespirologyDepartment of Paediatric Respiratory MedicineRoyal Brompton HospitalSydney StreetLondon SW3 6NPUKEmail: a.bush@rbh.nthames.nhs.uk

Sean Carrie MB, ChB, FRCS (ORL)Consultant OtolaryngologistDepartment of OtolaryngologyFreeman HospitalNewcastle upon Tyne NE7 7DNUKEmail: sean.carrie@nuth.nhs.uk

Ray W. Clarke FRCSAlder Hey Children’s HospitalENT DepartmentEaton RoadLiverpool L12 2APUKEmail: rayclarke@aol.com or raymond.clarke@rlc.nhs.uk

Peter A.R. Clement MD, PhDProfessor of OtorhinolaryngologyA.Z.-V.U.B. (Free University Brussels)ENT-DepartmentLaarbeeklaan 1011090 Brussels BELGIUMEmail: knoctp@az.vub.ac.be or cknonsk@az.vub.ac.be

Fiona Connell MB, ChB, MRCPCHSpecialist Registrar in Clinical GeneticsNE Thames Regional Genetics ServiceGreat Ormond Street Hospital NHS TrustGreat Ormond StreetLondon WC1N 3JHUKfconnell@sgul.ac.uk

C.W.R.J. Cremers MD, PhDProfessor in OtologyUniversity Medical Centre Nijmegen St. RadboudP.O. Box 91016500 HB NijmegenThe Netherlands

Frank Declau MD, PhDResearch AssociateUniversity Hospital of AntwerpENT DepartmentWilrijkstraat 10B-2650 EedegemBelgiumEmail: frank.declau@pandora.be

Ingeborg Dhooge MD, PhDDepartment of Otorhinolaryngology, Ghent University BelgiumDeputy Head, ENT-Clinic, Ghent University Hospital, BelgiumDe Pintelaan 185B-9000 GhentBelgiumEmail: ingeborg.dhooge@ugent.be

Milanka Drenovak MSc, Au.DAudiological ScientistNuffield Centre for Speech and HearingRoyal National Throat, Nose and Ear Hospital330 Gray’s Inn RoadLondon WC1X 8DAUKEmail: milanka@compuserve.com

Adam Finn MA, PhD, FRCP, FRCPCH, DBPPDavid Baum Professor of PaediatricsUniversity of BristolInstitute of Child Life & HealthDepartment Clinical Sciences at South BristolLevel 6, UBHT Education CentreUpper Maudlin StreetBristol BS2 8AEUK Email: adam.finn@bristol.ac.uk

Patrick Froehlich MD, PhDDepartment of Otorhinolaryngology and Cervico-Facial SurgeryHôpital Edouard HerriotPavilion U5 Place d’Arsonval69437 Lyon cedex 03FranceEmail: patrick.froehlich@chu-lyon.fr

XXVIII List of Contributors

Noel Garabédian MDService d’ORL Pédiatrique Hôpital d’Enfants Armand-Trousseau26 Ave du Dr Arnold Netter75571 Paris Cedex 12FranceEmail: noel.garabedian@trs.ap-hop-paris.fr

Kevin Patrick Gibbin FRCSConsultant Paediatric OtolaryngologistDepartment of Otorhinolaryngology, Head & Neck SurgeryQueen’s Medical CentreNottingham NG7 2 UHUKEmail: kevin.gibbin@qmc.nhs.uk

John M. Graham FRCSRoyal National Throat, Nose and Ear Hospital330 Gray’s Inn RoadLondon WC1X 8DAUKEmail: john.graham10@virgin.net

Alexander N. Greiner MDAssistant Clinical ProfessorUniversity of CaliforniaSan Diego School of MedicineAllergy & Asthma Medical Group & Research Center A P.C.9610 Granite Ridge Dr., Suite BSan Diego, CA 92123Email: greineran@yahoo.com

John Hamilton FRCS Consultant ENT Surgeon/OtologistGloucestershire Hospitals NHS TrustGreat Western RoadGloucesterGL1 3EE. UKEmail: ymg34@dial.pipex.com

Jonny Harcourt MBBS (Hons) MA (Oxon) FRCSConsultant ENT SurgeonDepartment of Paediatric Respiratory MedicineRoyal Brompton HospitalSydney StreetLondon SW3 6NPUKEmail: jh@harco.demon.co.uk

Benjamin Hartley MB BS, BSc, FRCS (ORL-HNS)Consultant Paediatric OtolaryngologistGreat Ormond Street Hospital for ChildrenLondon WC1N 3JHUKEmail: hartlb1@gosh.nhs.uk

Christopher Hartnick MDDepartment of OtolaryngologyMassachusetts Eye and Ear Infirmary243 Charles Street Boston, MA 02114USAEmail: christopher_hartnick@meei.harvard.edu

William P.L. Hellier FRCS (ORL-HNS)Consultant ENT SurgeonRoyal South Hants HospitalSouthamptonHampshireEmail: whellier@hotmail.com

Werner J. Heppt MDProfessor and DirectorDepartment of Otorhinolaryngology Head and Neck SurgeryKlinikum KarlsruheMoltkestr. 9076133 KarlsruheGermanyEmail: hnoklinik@klinikum-karlsruhe.com

Hans L.J. Hoeve MD, PhDDepartment of OtorhinolaryngologySophia Children’s HospitalDr. Molewaterplein 603015 GJ RotterdamThe NetherlandsEmail: l.j.hoeve@erasmusmc.nl

Pekka Karma MD, PhDHelsinki University Central Hospital (HUCH)Eye and Ear Hospital P.O. Box 220 FIN – 00029 HUSFinlandEmail: pekka.karma@hus.fi

Michael Kuo PhD, FRCS (ORL-HNS) DCHConsultant Paediatric OtolaryngologistHead and Neck SurgeonBirmingham Children’s HospitalSteelhouse LaneBirmingham B4 6NHUnited KingdomEmail: Michael.Kuo@mac.com

List of Contributors XXIX

Mich Lajeunesse BSc, DM MRCPCHLecturer in Paediatrics and Infectious DiseasesUniversity of BristolInstitute of Child Life & HealthDepartment Clinical Sciences at South BristolLevel 6, UBHT Education CentreUpper Maudlin StreetBristol BS2 8AEUK Email: mich.lajeunesse@bristol.ac.uk

Emmanuel Le Bret MD, PhDPaediatric Cardiothoracic DepartmentCentre Cirurgical Marie Lannelongue133 Avenue de la Résistance92350 Le Plessis RobinsonFranceEmail: e.lebret@ccml.fr

Linda Luxon BSc, MB, BS, FRCPAcademic Unit of Audiological MedicineGreat Ormond Street HospitalLondon WC1N 3JHUKEmail: l.luxon@ich.ucl.ac.uk

Fiona B. MacGregor MB, ChB, FRCS, FRCS (ORL)Consultant OtolaryngologistRoyal Hospital for Sick ChildrenYorkhillGlasgow G3 8SJEmail: fiona.macgregor@northglasgow.scot.nhs.uk

Gitta Madani MBBS, FDSRCS, MRCS, FRCRSpecialist Registrar in RadiologyRoyal National Throat Nose and Ear and University College Hospitals London330 Grays Inn RoadLondon WC1X 8DAUK

Amy McConkey Robbins MS, CCC-SPSpeech-Language PathologistCommunication Consulting Services8512 Spring Mill RoadIndianapolis, IN 46260USAEmail: amcrobbins@aol.com

Gavin Morrison MA, FRCSConsultant OtolaryngologistGuy’s, & St Thomas’ and The Evelina Children’s HospitalsLambeth WingSt Thomas’ HospitalLambeth Palace RoadLondon SE1 7EHEmail: gajm@gavinmorrison.com

Richard Nicollas MDLa Timone University Children’s HospitalDepartment of Pediatric Otolaryngology Head and Neck SurgeryBd. Jean Moulin13385 Marseille Cedex 5FranceEmail: richard.nicollas@ap-hm.fr

Waheeda Pagarkar MRCPCHConsultant in Audiovestibular MedicineDonald Winnicott CentreCoate StreetLondon E2 9AGH and, Royal National Throat, Nose and Ear Hospital330 Gray’s Inn RoadLondon WC1X 8DAUKEmail: apargarkar@nhs.net

Anne Pitkäranta MDDocent, Head of SectionHelsinki University Central Hospital (HUCH)Eye and Ear Hospital P.O. Box 220 00290 Helsinki FinlandEmail: anne.pitkaranta@hus.fi

Chris Prescott MB, ChB FRCS (Eng)ENT DepartmentRed Cross Children’s HospitalKlipfontein RoadRondebosch, 7700Cape TownSouth AfricaEmail: Christopher.Prescott@uct.ac.za

David Proops BDS (Hons), MB ChB, FRCS, FRCS (Ed) HonConsultant ENT Surgeon, Hon. Senior LecturerDepartment of Paediatrics, University of BirminghamDepartment of Paediatrics and ENTThe Birmingham Children’s HospitalSteelhouse LaneBirmingham B4 6NHEmail: david.proops@talk21.com

David Rawat MB, BAO, ChB, LRCP & SI (Irl), MRCP ( Irl), MScConsultant Paediatric GastroenterologistChelsea and Westminster Hospital369 Fulham RoadLondon SW10 9NHUKEmail: rawatdavid@hotmail.com

XXX List of Contributors

Chris Rittey MB, ChB, FRCP, FRCPCHConsultant Paediatric NeurologistSheffield Children’s HospitalWestern BankSheffield S10 2THUKEmail: chris.rittey@sch.nhs.uk

Peter J. Robb BSc (Hons) FRCS FRCSEdHonorary Senior Lecturer, Middlesex UniversityConsultant ENT SurgeonEpsom and St. Helier University Hospitals NHS TrustEpsom KT18 7EGSurrey UKEmail: peter.robb@epsom-sthelier.nhs.uk

Stéphane Roman MD, PhDLa Timone University Children’s HospitalDepartment of Pediatric Otolaryngology Head and Neck SurgeryBd. Jean Moulin13385 Marseille Cedex 5FranceEmail: stephane.roman@mail.ap-hm.fr

Michael Rothera FRCSManchester Children’s HospitalDepartment of ENT SurgeryHospital RoadPendleburyManchester, M27 4HAUKEmail: mprothera@aol.com

Robert J. Ruben MD, FAAP, FACSDistinguished University Professor Departments of OtorhinolaryngologyHead and Neck Surgery and PaediatricsAlbert Einstein College of MedicineMontefiore Medical CenterMedical Arts Pavilion3400 Bainbridge Avenue - 3rd FloorBronx, New York 10467-2490USAEmail: ruben@aecomyu.edu

Michael J. Rutter FRACSCincinnati Children’s Hospital Medical CenterDivision of Pediatric Otolaryngology/ Head & Neck Surgery3333 Burnet AvenueCincinnati, OH 45229-3039USAEmail: mike.rutter@cchmc.org

Glenis K. Scadding MA, MD, FRCPRoyal National Throat, Nose and Ear Hospital330 Gray’s Inn RoadLondon WC1X 8DAUKEmail: g.scadding@ucl.ac.uk

Neville P. Shine MB, FRCS (ORL-HNS)ENT FellowRoyal Perth HospitalPerthWestern AustraliaEmail: shiner1@gmail.com

John Stein FRCPLecturer in NeuroscienceUniversity Laboratory of PhysiologyParks Rd.Oxford OX1 3PTUKEmail: jfs@physiol.ox.ac.uk

Michael Thomson MB, ChB, DCH, FRCP, FRCPCH, MDPaediatric GastroenterologistCentre for Paediatric Gastroenterology Sheffield Children’s HospitalWestern BankSheffield S10 2THUKEmail: mike.thomson@sheffch-tr.trent.nhs.uk

Jean-Michel Triglia, MDLa Timone University Children’s HospitalDepartment of Pediatric Otolaryngology Head and Neck SurgeryBd. Jean Moulin13385 Marseille Cedex 5FranceEmail: jean-michel.triglia@ap-hm.fr

Paul H. Van de Heyning MD, PhDProfessor and ChairmanUniversity Department of Otorhinolaryngology and Head and Neck SurgeryAntwerp University HospitalUniversity of AntwerpWilrijkstr 102650 AntwerpBelgium Email: paul.van.de.heyning@uza.be

Carel D.A. Verwoerd MD, PhDEmeritus Professor Otorhinolaryngology and Head -Neck SurgeryErasmus University Medical Center RotterdamSecretary-general European Society of Pediatric Otorhinolaryngology ESPOEmail: kroeskarper@hotmail.com

List of Contributors XXXI

Brian J. Wiatrak MDPediatric ENT AssociatesThe Children’s Hospital of Alabama1940 Elmer J. Bissell Road Birmingham, AL 35243USAEmail: brian.wiatrak@chsys.org

Rosalind Wilson RGN, RSCNWard SisterDundas Grant WardRoyal National Throat, Nose and Ear Hospital330 Gray’s Inn Rd.London WC1X 8DAUKEmail: rosalind.wilson@royalfree.nhs.uk

XXXII List of Contributors

Introduction: Paediatric Consultation

Children are not small adults and their developmental status and metabolic needs must be considered care-fully by those entrusted with their medical care. In a paediatric consultation, the history (anamnesis) is es-pecially important, as it is essential to have a clear idea of the nature of the problem before examining the child. This is because children are often reluctant to be exam-ined by strangers.

1. After discussing the reason for the referral with par-ents or carers, go over the child’s developmental his-tory and especially their communicative abilities.

2. Enquire about the family history, especially first-de-gree relatives: siblings and parents.

3. Observe how the child interacts with others and uses play materials.

4. Look for dysmorphic features, also physical signs, such as mouth breathing and dribbling.

Core Messages

■ Special techniques are needed for a successful pae-diatric consultation.

■ It is important for the clinician to be aware of the normal stages of a child’s development, in order to identify delay in an individual child. In the field of paediatric ENT, this particularly applies to delays in speech and language.

■ One child in five will have special needs, either mi-nor or major, at some time during childhood.

■ The paediatric ENT doctor needs to be aware of dis-orders such as autism, global delay, specific learning difficulty, Down and other syndromes, behavioural, social and cultural difficulties and the possibility of abuse.

■ Liaison with other agencies is an integral part of the management of the child in an ENT clinic.

A Paediatric Overview of Children Seen in the ENT Outpatient DepartmentHelen Bantock

Social Difficulties . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

Types of Abuse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

Child Abuse in the Context of an ENT Clinic . . . . . 4

Alerting Signs of Non-Accidental Injury and Abuse in a Clinic Setting . . . . . . . . . . . . . . . . . . . 4

What to do if Abuse is Suspected . . . . . . . . . . . . . . . . . 4

Links with Local Services . . . . . . . . . . . . . . . . . . . . . . . . . . 4

Primary Care Team . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4

The Child Development Team . . . . . . . . . . . . . . . . . . . 4

The Education Service . . . . . . . . . . . . . . . . . . . . . . . . . . 4

Child and Adolescent Mental Health Services . . . . . . 5

Social Services . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5

Non-Statutory Organisations . . . . . . . . . . . . . . . . . . . . 5

1

Contents

Introduction: Paediatric Consultation . . . . . . . . . . . . . . . 1

Development in the First Five Years of Life . . . . . . . . . . . 2

Children with Special Needs . . . . . . . . . . . . . . . . . . . . . . . 2

Speech and Language Delay or Disorder . . . . . . . . . . . 2

Autistic Spectrum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

Global Delay . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

Specific Learning Difficulties . . . . . . . . . . . . . . . . . . . . 3

Syndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

Down Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

Behaviour Difficulties . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

Attention Deficit Hyperactivity Disorder . . . . . . . . . 3

Chapter 1

5. Try to engage children in simple play by following their lead in the choice of play materials.

6. Only examine the children physically once you have gained their confidence, and leave possibly stressful things, such as tympanoscopy, till last.

7. Do not forget to ask older children about their own concerns.

8. At the end of the consultation explain what you feel the problem is and how you plan to proceed. Ask the accompanying adults or older children if they have any questions.

Development in the First Five Years of Life

In assessing a child’s development, it is usual to look at gross and fine motor development, speech and language development, including social interaction, and visual and hearing capabilities. Older children are able to have a more formal assessment of their cognitive abilities, both verbal and non-verbal.

In terms of infant development, huge gains are made in terms of visual skills in the first few months. A key stage is the development of reciprocal social interaction at about 6 weeks of age. Not only do babies smile back at their care-givers at this stage and vocalise, they have begun to read facial expressions and will look distressed if people frown at them. Parents of children later diagnosed with autism often notice the lack of these early socialisation skills, es-pecially if they have other children who are not autistic.

Motor development is a very obvious developmental parameter, and by the age of 7 months, children are sit-ting securely and able to transfer objects from one hand to another. Soon afterwards, they begin to crawl, al-though it is worth remembering that 10% of children are bottom shufflers, who may not walk till around 2 years of age, especially if they are lax jointed. In this situation there is often a family history of bottom shuffling. Most children are walking by 18 months, but it is worth em-phasising that most children who walk later than this will have no long-term difficulties. On the other hand, children with global delay often have slow motor devel-opment as part of the picture. However, they may not and it is essential to look at the whole child, and espe-cially at how he or she attempts to communicate (Hall et al. 1999).

Children with Special Needs

One child in five has special needs at some point in child-hood. These range from major health and developmental problems to temporary and more minor ones, and include medical conditions such as epilepsy and diabetes melli-tus, speech and language difficulties, conductive deafness (“glue ear”), visual acuity problems that require spectacle prescription, and mental health problems.

Speech and Language Delay or Disorder

Speech and language difficulties are very common, with children in the lowest 10th centile being more than 1 year behind the average with their expressive language, al-though their comprehension is age appropriate. If 100 children with the same birthday are considered, 10 of them will fall on or below the 10th centile in terms of speech and language development. These children do catch up, but even a temporary hearing impairment may slow down their development further. If children also have a delay in the understanding of language, the dif-ficulties are greater and if they have both a receptive and expressive delay greater than 1 year, they have a speech and language disorder. About 0.5–1% of children have a language disorder.

Before they begin to talk, children develop their pre-verbal skills. For example, if you give a cup, especially a familiar one, to an 8-month-old child, he or she will look inside it. By 18 months definition by use has progressed so that when shown simple items, such as a cup, spoon, brush, ball or doll, a child should be able to play appropri-ately with them all, showing an understanding of what they are for. Many children at this age will hand over the items on request, even if they cannot name them, thus show-ing that they are beginning to understand verbal labels.

As a rule of thumb, by the age of 2 years, children will have a single word vocabulary of about 200 words and by then are beginning to link two words together. At 3 years, simple three-word sentences with subject, object and verb are achieved. For more details of developmental milestones in speech and language see Chapter 5.

Autistic Spectrum

As mentioned above, children on the autistic spectrum have difficulties with social communication. As a conse-quence, most have delayed speech and language develop-ment, although children with Asperger’s syndrome, who often present later, in the early school years, may speak at the normal time but have difficulties with the use (prag-matics) and meaning (semantics) of words, as well as more subtle difficulties with social interaction. Because the more severely affected children appear to live “in a world of their own”, a hearing loss is often suspected. Be-cause they do not enjoy turn taking with another person, it is difficult to test an autistic child using behavioural testing, such as play audiometry.

The introduction of newborn hearing screening pro-grammes means that these children have usually been shown to have the potential for normal hearing and there is less need to resort to auditory brainstem response test-ing. Of course, they may suffer from glue ear and conduc-tive hearing difficulties, so a history of mouth breathing and snoring needs to be taken seriously. All children with developmental social and communication difficulties are

2 Helen Bantock

1

at risk of incurring further delay in their development if they suffer from conductive hearing loss (see Table 5.2 for warning signs that assist in the differential diagnosis of communication disorders).

Global Delay

As with children with social and communication diffi-culties, children with global delay are disadvantaged by hearing loss. The causes of generalised delay include some syndromes (see below and Chapter 8), but in about one-third of children it is still not possible to find a cause.

Specific Learning Difficulties

Specific learning difficulties are common and can lead to educational failure if they are not recognised. Problems may occur with reading and spelling (often called dys-lexia) and with coordination and fine motor difficulties (developmental coordination disorder or dyspraxia).

Syndromes

A syndrome is defined as a group of symptoms that col-lectively indicate or characterise a disease, a psychologi-cal disorder or other abnormal condition. There are many paediatric syndromes, several of which are associated with deafness (see Chapter 8). Many syndromes are associated with learning difficulties and because of this, conductive deafness, which affects as many as one in five under fives at some time, needs to be treated if it occurs.

Down Syndrome

Down syndrome, or trisomy 21, is the commonest syn-drome encountered in paediatrics and affected individ-uals have a 30% risk of “glue ear” and a 30% risk of later sensorineural deafness. Regular hearing assessment is needed, and many children benefit from temporary hear-ing aids if the conductive deafness persists. Children with trisomy 21 have other physical difficulties. They are often very floppy at birth and may have abnormali-ties of the cardiovascular and digestive systems. For this reason they benefit from a multidisciplinary approach (see below under The Child Development Team).

Behaviour Difficulties

Child mental health problems are common and some, such as attention deficit hyperactivity disorder (ADHD), affect a child’s educational progress, especially if specific learning difficulties are also present.

Attention Deficit Hyperactivity Disorder

Conservative estimates suggest that between 1 and 5% of children, mostly males, are affected by ADHD. The behav-ioural features are inattention, hyperactivity and impul-sivity. The DSM-IV definition (Diagnostic and Statistical Manual of Mental Disorders, 1994) is the one most often used to classify the condition. Affected children are unable to concentrate on activities other than self-chosen ones, such as computer games. They fidget and are constantly on the move, interfering and butting in on the activities of others. Affected girls are more likely to have attention deficit disorder (ADD) and are not hyperactive. They are more placid and tend to daydream, and their difficul-ties are not easily apparent to their carers and teachers. Children with ADHD and ADD frequently fail screening hearing tests at school entry and form one of a group of children with non-organic hearing loss. Children who have been abused and neglected in early childhood de-velop behaviour that is very similar to ADHD, and care needs to be taken in separating these conditions. How-ever, especially in children with associated learning diffi-culties, medication such as methylphenidate (Ritalin) can dramatically improve school progress (Guevera and Stein 2001). The exact mode of action is not fully understood, but Ritalin appears to stimulate inhibitory pathways, lead-ing to improved concentration and short-term memory (UK National Institute for Clinical Excellence 2006).

Social Difficulties

Children who have been abused and neglected are fre-quently encountered in paediatric practice. Child Protec-tion services are coordinated by the local Social Services in the United Kingdom and there are regular interagency meetings in all areas to discuss how to improve local services. Within each local authority area, a designated doctor and nurse take the strategic lead in all aspects of child protection, ensuring that the necessary policies and procedures are in place and that training and super-vision are available for everyone who works in the lo-cal health services, including reception staff, managers, doctors and nurses, and members of all the professions allied to medicine. In addition, in the UK, local health services for children have both a named doctor and nurse who are responsible for ensuring that child protection strategies for the hospital or community are in place. Comparable systems exist in other developed countries.

Types of Abuse

Neglect is the commonest form of abuse, but is often accompanied by physical, emotional or sexual abuse. A more detailed description of the types and presentation of abuse can be found in Barker and Hodes (2004).

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