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Manual of Contemporary
Otological Practice
Manual of Contemporary
Otological Practice
Chief Editor:
Dr Uma Patnaik
Professor and Head, Department of ENT
Command Hospital (SC)
Pune, Maharashtra, India
Executive Editors:
Dr Amit Sood
Assistant Professor (ENT)
Command Hospital (SC)
Dr Dilip Raghavan
Professor and Head, Department of ENT
Air Forces Medical College
Dr Sabarigirish K
Professor, Department of ENT
Armed Forces Medical College
First edition published 2021
by CRC Press
6000 Broken Sound Parkway NW, Suite 300, Boca Raton, FL 33487-2742
and by Taylor & Francis Group
2 Park Square, Milton Park, Abingdon, Oxon, OX14 4RN
© 2021 Taylor & Francis Group, LLC
CRC Press is an imprint of Taylor & Francis Group, LLC
For permission to photocopy or use material electronically from this work, access www.copyright.com
or contact the Copyright Clearance Center, Inc. (CCC), 222 Rosewood Drive, Danvers, MA 01923, 978-
750-8400. For works that are not available on CCC please contact mpkbookspermissions@tandf.co.uk
Trademark notice: Product or corporate names may be trademarks or registered trademarks
and are used only for identification and explanation without intent to infringe.
Library of Congress Cataloging‑in‑Publication Data
Names: Patnaik, Uma, editor. | Sood, Amit (Otolaryngologist), editor. | K., Sabarigirish (Kanjully),
editor. | Raghavan, Dilip, Professor, editor.
Title: Manual of contemporary otological practice / edited by Dr. Uma Patnaik, Wg Cdr Amit
Sood, Dr. Sabarigirish K., Surg Cmde Dilip Raghavan.
Description: First edition. | Boca Raton, FL : CRC Press, 2021. | Includes bibliographical references
and index. | Summary: “Practice of otology today, requires a contemporary knowledge base,
coupled with concurrent skill sets, and tempered with familiarity of the technological advances.
This manual has been designed to address these three domains, making it a ready reference to
guide specialists on the standards of care in practice. The chapters explore the current concepts,
with a background of past practices, touching upon the basics of anatomy and physiology before
dealing with clinical conditions and their management, covering specific clinical scenarios to
develop a patient-oriented approach in the readers using evidence-based guidelines”-- Provided
by publisher.
Identifiers: LCCN 2020051508 (print) | LCCN 2020051509 (ebook) | ISBN 9780367753184 (hard-
back) | ISBN 9780367489441 (paperback) | ISBN 9781003161974 (ebook)
Subjects: MESH: Ear Diseases--diagnosis | Ear Diseases--therapy | Diagnostic Techniques,
Otological
Classification: LCC RF291 (print) | LCC RF291 (ebook) | NLM WV 215 | DDC 617.8/075--dc23
LC record available at https://lccn.loc.gov/2020051508
LC ebook record available at https://lccn.loc.gov/2020051509
ISBN: 9780367753184 (hbk)

ISBN: 9780367489441 (pbk)
ISBN: 9781003161974 (ebk)
Typeset in Palatino LT Std
by KnowledgeWorks Global Ltd.
Table of Contents
List of Abbreviations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vii
Preface. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . x
Foreword . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xi
Contributors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xii
Editor Biography. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xiv
Section I: Hearing Loss: Approach to the Patient and Management. . . . . . . . . . . . . . . . . . 1
1. Hearing Loss in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

Mary John
2. Hearing Loss in Adults . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27

Amit Sood
3. Sudden Sensorineural Hearing Loss. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49

Atul Gupta
4. Single-Sided Deafness: A Long-Neglected Entity in Otology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58

Garima Upreti and Uma Patnaik
Section II: The Discharging Ear: Differential Diagnosis and Management. . . . . . . . . . . 75
5. The Discharging Ear: Differential Diagnosis and Management. . . . . . . . . . . . . . . . . . . . . . . . . . . 76

Anandita Gupta
Section III: Approach to a Patient with Otalgia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97
6. Approach to a Patient with Otalgia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98

Rohit Singh, Amit Sood and N Ramakrishnan
Section IV: C
linical Decision-Making in the Vertiginous Patient: Differential
Diagnosis and Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109
7. Vertigo in Children and Adults . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 110

Anupam Kanodia and Hitesh Verma
8. Vertigo in Elderly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 121
Gunjan Dwivedi and Uma Patnaik
Section V: Tinnitus Symptomatology: Diagnosis and Management. . . . . . . . . . . . . . . . . 137
9. Tinnitus Symptomatology: Diagnosis and Management. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 138

Abha Kumari and Uma Patnaik
Section VI: Facial Palsy in Otology: Approach to a Patient. . . . . . . . . . . . . . . . . . . . . . . . . . 153
10. Facial Palsy in Otology: Approach to a Patient . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 154

SK Singh, Sunil Goyal and Roohie Singh
v
Table of Contents
Section VII: Neoplasms in Otology: Guide to Diagnosis and Management. . . . . . . . . . 175
11. Malignant Neoplasms in Otology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176

Smriti Panda, Chirom Amit Singh and Uma Patnaik
12. Benign Neoplasms in Otology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 197

Smriti Panda, Chirom Amit Singh and Uma Patnaik
Section VIII: Otology at the Frontiers. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 213
13. Endoscopes in Otology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 214

Sasikanth CM and Manikandan
14. Anaesthesia Techniques in Otology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 221

Deepak Dwivedi and Gunjan Dwivedi
15. Imaging in Otology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 230

Virender Malik, Dhanalakshmi B and Abha Kumari
16. Tools and Technology in Otology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 245

Sabarigirish K and Uma Patnaik
Section IX: Record Keeping in Otology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 253
17. Record Keeping in Otology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 254

Amit Sood and Dilip Raghavan
Index. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 263
vi
List of Abbreviations
5 FU 5-Fluorouracil CISS Constructive interference in steady

AABR Automated auditory brainstem state
response CMV Cytomegalovirus
AAO-HNS American Association of CN Cranial nerve
Otolaryngology-Head & Neck CNS Central nervous system
Surgery CO2 Carbon dioxide
AB Gap Air-bone gap COM Chronic otitis media
ABI Auditory brainstem implant COX Cyclo-oxygenase
ABR Auditory brainstem response CP Angle Cerebellopontine angle
AC Air conduction CPA Conditioned play audiometry
ACE Angiotensin-converting enzyme CROS Contralateral routing of signal
AFB Acid fast bacilli CRP C-reactive protein
AI Artificial intelligence CS Corrective saccades
ANA Anti-nuclear antibody CSD Chronic subjective dizziness
ANCA Anti-neutrophilic cytoplasmic CSF Cerebrospinal fluid
antibody CT Computed tomography
ANN Artificial neural network CVA Cerebrovascular accident
ANSD Auditory neuropathy spectrum CW Continuous wave
disorder CWD Canal wall down
AOAE Automated oto acoustic emissions dB Decibel
AOM Acute otitis media DBN Deep belief network
AP Action potential DM Diabetes mellitus
APUD Amine precursor and uptake DNES Diffuse neuroendocrine system
decarboxylase DP Directional preponderance
ASSR Auditory steady-state response DSA Digital subtraction angiography
AVS Acute vestibular syndrome DWI Diffusion-weighted images
BAAP Bone-anchored auricular ECochG Electrocochleography
prosthesis eABR Evoked auditory brainstem
BAHA Bone-anchored hearing aid response
BC Bone conduction EAC External auditory canal
BCD Bone conduction device EBM Evidence-based medicine
BCHD Bone-conducting hearing devices ECAP Evoked compound action potential
BERA Brainstem-evoked response EEG Electroencephalogram
audiometry ELST Endolymphatic sac tumor
BOA Behavioural observation EMG Electromyography
audiometry EnoG Electroneuronography
BP Bell's palsy EPI Echo planar imaging
BPPV Benign paroxysmal positional ESR Erythrocyte sedimentation rate
vertigo ESRT Elicited stapedial reflex threshold
BPVC Benign positional vertigo of ET Eustachian tube
childhood EVA Enlarged vestibular aqueduct
BTE Behind the ear FDA Food and Drug Administration
CAD Computer-aided design FDG Fluorodeoxyglucose
CAEP Central auditory-evoked potential FM Frequency modulation
CAPD Central-auditory processing FN Facial nerve
disorder FNC Facial nerve canal
CBCT Cone beam computed tomography FNH Facial nerve haemangioma
CBT Cognitive behavioural therapy FNS Facial nerve schwannoma
CCA Congenital canal atresia GA General anaesthesia
CE European conformity GAN Greater auricular nerve
CEMRI Contrast-enhanced magnetic GBS Guillain-Barre syndrome
resonance imaging GM Granular myringitis
CERA Cortical-evoked response GORD Gastro-oesophageal reflux disorder
audiometry GSA General somatic afferent
CI Cochlear implant GSPN Greater superficial petrosal nerve
CIC Completely in canal GTR Gross total resection
vii
GVE General visceral efferent MML Minimum masking level

HA Hearing aids MOE Malignant otitis externa
HAT Hearing-assistive technology MPO Myeloperoxidase
HBOT Hyper baric oxygen therapy MRI Magnetic resonance imaging
HC Horizontal canal MRS Melkersson-Rosenthal syndrome
HINTS Head impulse, nystagmus, test of NF Neurofibromatosis
skew NIHL Noise-induced hearing loss
HIT Head impulse test NLF Nasolacrimal fold
HL Hearing loss NLP Natural language processing
HRCT High-resolution computed NO Nitrous oxide
tomography NOHL Non-organic hearing loss
HST Head shake test NRR Noise reduction rating
HSV Herpes simplex virus NSAID Non-steroidal anti-inflammatory
HTT Head thrust test drugs
IAC Internal auditory canal NTT Neuromonics tinnitus therapy
IAD Implantable auditory device OAE Oto acoustic emissions
IAM Internal acoustic meatus OCT Optical coherence tomography
ICA Internal carotid artery OE Otitis externa
ICU Intensive care unit OHC Outer hair cell
ICVD International Classification of OM Otitis media
Vestibular Disorders OMAAV ANCA-associated otitis media
ICW Intact canal wall OME Otitis media with effusion
IHC Inner hair cell OPD Outpatient department
ILD Inter-aural level difference PA Petrous apex
IPOG International Paediatric PCHI Permanent childhood hearing
Otolaryngology Group impairment
ISO International Organisation for PCR Polymerase chain reaction
Standardisation PCS Posterior circulation stroke
ISRS International Stereotactic PET Positron emission tomography
Radiosurgery Society PFNS Post-paralytic facial nerve
ISSNHL Idiopathic sudden sensorineural syndrome
hearing loss PFP Post-paralytic facial palsy
ITC In the canal PICA Posterior inferior cerebellar artery
ITD Inter-aural time difference PLA People Liberation Army
IVN Inferior vestibular nerve PONV Postoperative nausea vomiting
JTP Jugulotympanic paraganglioma PORP Partial ossicular replacement
KTP Potassium titanyl phosphate prosthesis
LA Local anaesthesia PRRT Peptide receptor radionuclide
LCH Langerhans cell histiocytoma therapy
LCN Lower cranial nerves PSCC Posterior semicircular canal
LLR Low-power laser irradiation PTA Pure tone audiometry
LMA Laryngeal mask airway PTS Permanent threshold shift
LMN Lower motor neuron QOL Quality of life
LO Labyrinthitis ossificans RBC Red blood cell
LPR Laryngopharyngeal reflux RCT Randomized control trial
LSCC Lateral semicircular canal RF Rheumatoid factor
LTBR Lateral temporal bone resection RHS Ramsay Hunt syndrome
MAC Monitored anaesthesia care RMS Rhabdomyosarcoma
MAV Migraine-associated vertigo ROS Reactive oxygen species
MCF Middle cranial fossa RSV Respiratory syncytial virus
MD Ménière's disease RTA Road traffic accident
MDCT Multi-detector computed SBO Skull base osteomyelitis
tomography SCC Semicircular canal
MEP Middle ear pressure SDS Speech discrimination score
MERI Middle ear risk index SLE Systemic lupus erythematosus
MIBG Metaiodobenzylguanidine SMF Stylomastoid foramen
ML Machine learning SNHL Sensorineural hearing loss
MLP Multilayer perception SOM Secretory otitis media
viii
SP Summating potential TORP Total ossicular replacement

SPECT Single-photon emission-computed prosthesis
tomography TQ Score Tinnitus quotient score
SR Subtotal resection TRT Tinnitus retraining therapy
SRS Stereotactic radiosurgery TTBR Total temporal bone resection
SRT Speech reception thresholds TTO Tympanostomy tube-related
SSCD Superior semicircular canal otorrhoea
dehiscence TTS Temporary threshold shift
SSD Single-sided deafness UHL Unilateral hearing loss
SSNHL Sudden sensorineural hearing loss UMN Upper motor neuron
SSRI Selective serotonin reuptake UW Unilateral weakness
inhibitor VCN Vestibulo-cochlear nerve
STBR Subtotal temporal bone resection VEMP Vestibular evoked myogenic
SVA Special visceral afferent potential
SVE Special visceral efferent VHL Von Hippel-Lindau
SVM Support vector machine VM Vestibular migraine
SVN Superior vestibular nerve VN Vestibular neuritis
TA Transcranial attenuation VNG Video Nystagmography
TBM Temporal bone malignancy VOR Vestibulo-ocular reflex
TEES Transcanal endoscopic ear surgery VP Vestibular paroxysmia
TFT Tuning fork tests VRA Visual reinforcement audiometry
TIVA Total intra-venous anaesthesia VS Vestibular schwannoma
TM Tympanic membrane VZV Varicella zoster virus
TMS Transcranial magnetic stimulation WHO World Health Organisation
TN Trigeminal nerve WRS Word recognition score
TOM Tubercular otitis media YAG Yttrium aluminium garnet
ix
Preface
“When you don’t see the book you making a paradigm shift in the way we practice.
want on the shelf, write it.” This, we believed, was an appropriate juncture
to provide an approach-based compendium in
–Beverly Cleary
otology. The book is designed to be a confidant
for the reader to refer to, in various clinical sce-
Practice of otology today requires a contem- narios where sound clinical decisions will have
porary knowledge base, coupled with concur- a positive impact on the patient outcomes.
rent skill sets, tempered with familiarity of the We express our sincere compliments to all
technological advances. This manual has been the contributors for all their efforts, which I am
designed to address these three domains, so sure, will be appreciated by the readers. I would
that it becomes a companion to guide special- also like to express my sincere gratitude to our
ists on the standards of care in otology. The seed executive editors, Amit Sood, Dilip Raghavan
for this manual was sown four years ago when and Sabarigirish K, whose meticulous work of
the abject need for such a manual was realized editing has substantially improved the quality
and it took dedicated work of the team to con- of content. We also thank Taylor & Francis India
ceptualize its focus and content. The immense for the determined support of the project, but in
task of collating the current data and compiling particular, Ms Shivangi Pramanik, the commis-
them into a reader-friendly format has indeed sioning editor and Ms Himani Dwivedi, with-
been onerous. out whose patience and persistence, this project
The art and science of otology has undergone would not have seen the light of day.
metamorphosis in the last few decades with sig- Lastly, on a personal note, this mission would
nificant changes, not only in the evaluation and not have been possible without the blessings of
management of diseases, but also in the basic my parents Saroja and R Jayaraman, unflinching
understanding of their pathology. Evolution of cooperation of my spouse, Col S K Patnaik, and
new age surgical microscopes, image-guided of course, my three-year-old angel, Ms Udisha,
systems and endoscopy-assisted surgery tech- who ensured that the maternal nightouts were
niques have been game changers in the practice gainfully utilized for this academic effort.
of otological surgery, while advances in molecu- Join us on this voyage, . . . “Read along, as the
lar biology and nanotechnology are beginning book starts when reader enters. . . . .”
to gives us new perspectives on the disease
processes. Add to this, the inroads made by Uma Patnaik
artificial intelligence and machine learning are
x
Foreword
Otology has witnessed many advances over in implantation otology. This book stands out in
the last few decades. These include many excit- detailing not only the contemporary topics, but
ing and headline grabbing advances such as also the basic concepts pertaining to middle ear
cochlear implants and brainstem implants disease which are less emphasized today. The
and also implantable hearing devices. Other experienced authorship has ensured that appro-
advances seem mundane – such as the revolu- priate lessons pertaining to middle ear disease
tion in imaging – but they nevertheless have which are slowly being castigated to history
brought about an immense change in our prac- continue to be listed and emphasized.
tice and the quality of service we provide to our My congratulations to all authors and par-
patients. ticularly to the chief editor, Dr Uma Patnaik,
The Manual of Contemporary Otological Practice for bringing this challenging task to fruition
is a focused, very well-researched and referenced and providing us a book which will serve as an
and a complete and comprehensive textbook on informed and enjoyable read, and also a quick
otology. It is a labour of love written with pas- reference guide to the rapidly evolving science
sion which brings out both the knowledge and and craft of otology.
the experience of the authors. In recent years,
the focus of otology has moved from the basic Alok Thakar, MS, FRCSEd
concepts of middle ear pathology and surgery Professor of Otolaryngology & Head-Neck Surgery
pertaining to chronic otitis media to the current All India Institute of Medical Sciences, New Delhi
hot topics relating to the inner ear and advances
xi
Contributors
Dr Manikandan A, MS (ENT) Dr Abha Kumari, MS

Assistant Professor Assistant Professor ENT
Base Hospital Command Hospital (SC)
Guwahati, India Pune, India
Dr Dhanalakshmi B, MD (Radiodiagnosis) Dr Sabarigirish K, MS

Assistant Professor, Department of Imaging Professor ENT
and Intervention Radiology Armed Forces Medical College
Army Institute of Cardiothoracic Sciences Pune, India
(AICTS)
Pune, India Dr Virender Malik, MD (Radiodiagnosis)
Assistant Professor, Department of Imaging
Dr Sasikanth CM, MS (ENT) and Intervention Radiology
Assistant Professor Army Institute of Cardiothoracic Sciences
Base Hospital (AICTS)
Guwahati, India Pune, India
Dr Gunjan Dwivedi, MS Dr Smriti Panda, MS

Associate Professor, Department of ENT-HNS Assistant Professor, Department of
Command Hospital (Southern Command) Otolaryngology and Head and Neck
Pune, India Surgery
AIIMS
Dr Deepak Dwivedi, MS (Anaes) New Delhi, India
Associate Professor, Department of Anaesthesia
and Critical Care Dr Uma Patnaik, MS, DNB
Command Hospital (Southern Command) Professor and Head of Department ENT
Pune, India Command Hospital (SC)
Pune, India
Dr Sunil Goyal, MS
Associate Professor, Department of ENT HNS Dr N Ramakrishnan, MS
AHRR Professor ENT
Delhi Cantt, India HQ 2 Corps
Ambala, India
Dr Atul Gupta, MS
Assistant Professor ENT Dr Dilip Raghavan, MS
153 General Hospital Prof & HoD (ENT)
Leh, India AFMC
Pune, India
Dr Anandita Gupta, MS
Assistant Professor ENT Dr SK Singh, MS (ENT), DNB (ENT)
Command Hospital (EC) Professor, Deputy Director General (Medical)
Kolkata, India Delhi Cantt, India
Dr Mary John, MS, DNB, PhD Dr Roohie Singh, MS

Christian Medical College Associate Professor, Department of ENT HNS
Vellore, India Command Hospital (Air Force)
Bengaluru, India
Dr Anupam Kanodia, MS
Senior Resident, Department of ENT and Dr Chirom Amit Singh, MS (ENT)
Head-Neck Surgery Additional Professor, Department of
All India Institute of Medical Sciences Otolaryngology and Head and Neck
New Delhi, India Surgery
AIIMS
New Delhi, India
xii
Contributors
Dr Rohit Singh, MS Dr Garima Upreti, MS

Senior Resident, Department of ENT Assistant Professor ENT
AFMC Christian Medical College
Pune, India Vellore, India
Dr Amit Sood, MS Dr Hitesh Verma, MS

Assistant Professor ENT Associate Professor, Department of ENT and
Command Hospital (Southern Command) Head-Neck Surgery
Pune, India All India Institute of Medical Sciences
New Delhi, India
xiii
Editor Biography
Dr Uma Patnaik studied Otorhinolaryngology Dr Dilip Raghavan, MS (ENT), is currently
from Armed Forces Medical College, Pune. She Professor & Head of the Department of
further pursued her sub-speciality training Otorhinolaryngology at Armed Forces Medical
in Neurotology under the aegis of Prof Mario College, Pune, India. He has been associated
Sana, Italy and AIIMS New Delhi. The focus of with Postgraduate Training of residents for close
her work has been on neurotology, skull base to two decades. He has a special interest in otol-
and childhood hearing loss. She has been work- ogy and paediatric hearing loss and has made
ing on minimally invasive skull base surgery numerous presentations and published many
and hearing rehabilitation surgery. articles in his field of interest.
Dr Uma’s research work includes five funded
projects. She has numerous research publications Dr Sabarigirish K is an officer in Armed Forces
to her credit in various national/international Medical Services of India, currently serving as
journals. Her research has focused on clinical and Professor in the Department of ENT as well
applied aspects of otorhinolaryngological prac- as the administrative head of graduate course
tice and has numerous awards for her research. at Armed Forces Medical College (AFMC),
Outside of ENT, Dr Uma enjoys classical Pune. In his three decades of service, he has
dance. been in the teaching faculty at various tertiary
care teaching hospitals of Armed Forces. He
Dr Amit Sood is a graduate from Armed Forces has been in the forefront for adoption of UNHS
Medical College and earned his MS (ENT) and has been credited for significant expansion
from Delhi University. He has a keen interest of Cochlear Implant services in Armed Forces.
in skull base surgery and occupational hear- With many major research projects and publica-
ing hazards. Dr Amit's research work includes tions to his credit, he is an ardent proponent of
funded research on effects of high-altitude and the practice of evidence-based medicine in oto-
occupational noise exposure on hearing. He has laryngology practice.
various research publications to his credit. Dr
Amit has a liking for adventure sports and has
been involved in activities like parajumping and
white water rafting.
xiv
SECTION I
HEARING LOSS: APPROACH

TO THE PATIENT AND
MANAGEMENT
1
SECTION I: HEARING LOSS: APPROACH TO THE PATIENT AND MANAGEMENT
1 Hearing Loss in Children
Mary John
CONTENTS
Introduction........................................................................................................................................................2
Review of Literature..........................................................................................................................................2
Paediatric Hearing Loss...............................................................................................................................2
Workup of a Child with Hearing Loss.......................................................................................................2
Audiological Evaluation of Children with Hearing Loss.......................................................................3
Subjective Tests........................................................................................................................................3
Objective Measurement..........................................................................................................................6
Functional Auditory Assessment...............................................................................................................6
Neonatal Hearing Screening.......................................................................................................................7
Radiological Evaluation of Children with Hearing Loss........................................................................7
Differential Diagnosis of Hearing Loss in Children......................................................................................8
Congenital Hearing Loss.............................................................................................................................8
Congenital Abnormalities of External and Middle Ear........................................................................12
Acquired Abnormalities of External and Middle Ear...........................................................................13
Otitis Media.................................................................................................................................................13
Acute Otitis Media......................................................................................................................................13
Serous Otitis Media....................................................................................................................................14
Chronic Otitis Media..................................................................................................................................15
COM Mucosal.............................................................................................................................................16
COM Squamous..........................................................................................................................................17
Other Causes of Hearing Loss in Children.............................................................................................19
Discussion.........................................................................................................................................................19
Recent Advances..............................................................................................................................................19
Conclusion........................................................................................................................................................19
References.........................................................................................................................................................20
INTRODUCTION children with HL. The advancement in surgical

Hearing Loss (HL) in children is caused by a techniques and implantable hearing devices has
variety of conditions, of which congenital HL further improved hearing rehabilitation results.
and Otitis Media (OM) are the most common. In This chapter summarizes the approach to a
recent times, otology has witnessed tremendous child with HL.
expansion with newer options for evaluation
and management of HL. HL is one of the most
common of all childhood disabilities where REVIEW OF LITERATURE
early identification and intervention provides Paediatric Hearing Loss
better overall development and optimal perfor- Permanent Childhood Hearing Impairment
mance of the child in the society (1, 2). (PCHI) is defined as confirmed permanent bilat-
HL in newborns is a disability which can eral hearing impairment of ≥40 dB HL, aver-
affect the speech and language acquisition and aged over the frequencies of 0.5, 1, 2 and 4 kHz
communication skills of the child. The esti- in the better hearing ear. The prevalence of HL
mated prevalence of congenital HL is 1–4/1000 in children increases to 6% by the time the child
population (3, 4). OM consisting of Acute Otitis starts primary school (5). As per WHO, disabling
Media (AOM), serous OM and Chronic Otitis HL in children is termed more than 30 dB HL
Media (COM) is a common disease that causes in the better ear. The classification according to
HL in childhood. the severity of the degree of HL is discussed in
Implementation of universal (newborn) hear- the section on adult HL.
ing screening can help in early identification
and rehabilitation of this correctable disability Workup of a Child with Hearing Loss
in children. The accessibility of imaging has Since congenital HL is usually asymptomatic
revolutionized the diagnosis and follow-up of and early intervention is essential for the optimal
2
performance of the child, history and examina- it is considered as the most common cause of
tion should incorporate all details needed to acquired HL (10, 11). Semicircular canal dehis-
identify various causes of HL. The onset of HL, cence, enlarged vestibular aqueducts, X-linked
pre-lingual or post-lingual, is an important fac- gusher and dilated internal auditory meati can
tor in the management of the child. Conditions cause third window effect and mixed HL. In
like microtia and anotia cause obvious defor- countries with no rubella vaccine, congenital
mity and are associated with conductive HL. rubella syndrome is the most common cause
AOM and middle ear effusion are more com- of acquired congenital HL. CMV infection,
mon causes of HL in children 5–8 years of age. especially in the first trimester, is considered a
A detailed proforma including the relevant common cause of non-syndromic HL in western
history for evaluation of a newborn with HL countries (12–14).
is provided in Figure 1.1. There are risk factors Vestibular function test is important in chil-
which increase HL in a child; however, nearly dren with HL as 70% of children with profound
half of the times no risk factors are identified. The HL can have some vestibular dysfunction
risk factors for HL in newborns adapted from (15, 16). Testing ability to stand on one foot
the 2007 position statement of Joint Committee with eyes closed for a minimum of 4 seconds in
on infant hearing are given in Table 1.1 (6), the child with SNHL, aged 4 years and above, is a
first three being considered as major risk fac- good screening test to detect bilateral vestibular
tors (7, 8). Evaluation of a child with HL requires impairment.
multiple specialty teamwork; the evaluation
flowchart for a child with congenital HL is sum-
marized in Figure 1.2. Audiological Evaluation of
Although autosomal recessive HL is the Children with Hearing Loss
most common cause of genetic HL, HL can Assessment of hearing is crucial for manage-
present in families with no history of HL. ment and can be challenging in young children.
The prevalence of permanent Sensori Neural Hearing evaluation can be subjective or objective
Hearing Loss (SNHL) will double by the sec- with the objective measurements more reliable
ond decade of life (9). Progressive HL can be in smaller children and children with learning
associated with Pendred syndrome and con- disabilities (17). The audiological tests usually
genital Cytomegalovirus (CMV) infection. done for hearing assessment are summarized in
Prematurity, hypoxia, sepsis and hyperbiliru- Figure 1.3.
binemia increase the risk of auditory neuropa-
thy spectrum disorder. The risk of developing
significant SNHL is 10% after meningitis and
Subjective Tests
Behavioural Observation Audiometry (BOA) assesses
Table 1.1: Common Risk Factors of response of the infant less than 6 months of
Hearing Loss in Children age to auditory stimuli such as warbled pure
tones, narrowband noise and speech presented
• Neonatal ICU stay more than 48 hours (major)
through speakers. However, BOA neither mea-
• Family history of childhood hearing loss (major)
sures hearing thresholds accurately nor provides
• Craniofacial anomalies (major) ear-specific information.
• In utero infection like CMV or toxoplasmosis
• Syndromes associated with hearing loss ◾◾ Moros reflex: Sudden movements of the limbs
• Neurodegenerative disorder and extension of the head to 80–90 dB sound.
• Bacterial or viral meningitis ◾◾ Cochleo-palpebral reflex: Blink to loud sounds.
• Head trauma, especially skull base/ temporal
bone fracture ◾◾ Cessation reflex: Stops activity or starts crying
• Chemotherapy for sound at 90 dB
• Persistent otitis media for min 3 m
• Caregivers concerns regarding hearing or speech Visual Reinforcement Audiometry (VRA) (18)
is done for children between 6 months and 2
• Birth weight less than 1500 g
years of age to obtain frequency-specific test-
• Hyperbilirubinemia requiring blood transfusion
ing of each ear. The child is required to turn to
• Others: respiratory distress syndrome, asphyxia, the sound source, usually at 90°, and is coupled
meconium aspiration, chromosomal
abnormalities, drug / alcohol abuse by mother, with conditioned reinforcement like a lighted
maternal diabetes, prolonged ICU stay, APGAR toy. Ear-specific information can be obtained by
score of 0–4 at 1 m, 0–6 at 5 m. using insert earphones or headphones.
3
Proforma for Evaluation of Hearing Loss
Figure 1.1
4
Figure 1.2 Flowchart for evaluation of children with hearing loss.
Conditioned Play Audiometry (CPA) is used for taught to listen to the sound and to perform an
children from 2 years through 4–5 years. The activity like putting a block in the box or pegs
aim of the testing is to obtain frequency and in a board as response.
ear-specific information on both air conduction Pure Tone Audiometry (PTA) can be done for
and bone conduction. Insert earphones are children 4–5 years of age with average cognitive
used for air conduction, whereas bone oscilla- abilities. Ear-specific, air conduction and bone
tors are used for bone conduction. The child is conduction thresholds can be measured using
Figure 1.3 Tests of Hearing.
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Table 1.2: Examples of Speech Perception Tests Used in Assessment of Children

with Hearing Loss and Children Fitted with Cochlear Implant
In Hearing Loss Children In Cochlear Implant Children
Open-set Closed-set Open-set Closed-set
Phonetically balanced Auditory numbers test (ANT) Lexical neighbourhood Monosyllabic trochee
kindergarten (PB-K) test (LNT) spondee (MTS)
Pediatric speech intelligibility Hearing in noise test Early speech perception test
(PSI) for children (HINT-C) (ESP)
Word intelligibility by picture Pediatric-AzBio auditory screening procedure
identification (WIPI) sentence (GASP) Glendonald
pure tones with responses such as hand raising decreased to minimum intensity of sound needed
or button pushing. to generate a reliable wave V.
Speech audiometry helps to understand the Auditory Steady-State Response (ASSR) and
ability of the child to recognize and understand Cortical Evoked Response Audiometry (CERA) are
simple and complex sound stimuli. Sound field other audiological tests which can be done after
testing or testing for individual ears can be done. routine objective investigations, especially for
Speech detection threshold is the lowest inten- decision-making on surgical intervention like
sity level the child is aware that a speech signal cochlear implant (CI) or brainstem implant.
is delivered. ASSR is an auditory-evoked potential which can
Speech Reception Threshold (SRT) is the min- assess the degree of HL.
imum intensity at which the child can repeat
50% of the spondee words correctly. Pure tone Functional Auditory Assessment
averages and SRT correlates well within 6 dB. Along with accurate diagnosis of the HL in a
Speech perception threshold measures the child, it is important to assess how the child
ability of the child to discriminate speech at a functions in day-to-day life. There are various
suprathreshold level like conversational speech questionnaires designed for caregivers and
and detects the effect of HL in everyday con- teachers to obtain adequate information on the
versation. The speech perception tests are done child’s hearing and speech; some are summa-
using closed-set tests and open-set tests; a few rized in Table 1.3 (19, 20).
examples are given in Table 1.2.
Table 1.3: Commonly Used

Objective Measurement Functional Auditory
Although the behavioural responses are the Assessment Tools
gold standard, objective measurement of hear-
Age of
ing complements behavioural responses and
Tool the Child Description
can be done without the cooperation from the
child, especially in smaller children. Infant toddler Birth to Parent questionnaire
meaningful 3 years (10 Q) to assess the
Otoacoustic Emissions (OAE) are sound signals auditory meaningful use of
generated by outer hair cells of cochlea which integration the sound
are measured using a microphone kept in the ear scale (IT-MAIS)
canal. For clinical testing, two types of evoked Little Ear: From birth Parent questionnaire
OAEs are used: transient evoked OAE and distor- Auditory (35 Q) to assess the
tion product OAE. OAE will be absent in HL more questionnaire age appropriate
auditory development
than 30 dB. A positive OAE response with absent
ABR is suggestive of auditory neuropathy. Auditory 2–12 years 24-item questionnaire
behaviour in to assess the auditory
Auditory Brainstem Response (ABR) is the electri- everyday life behaviour in
cal potential recorded from a signal generated by (ABEL) everyday life
the sound as it passes through the auditory path- Meaningful 3 years Parent questionnaire
way. In children, ABR is done under sedation or auditory and to assess the
when the child is sleeping, using air conduction integration above meaningful use of
and bone conduction transducers. ABR report scale (IT-MAIS) the sound
shows graphs with seven peaks representing spe- Children’s home 2–12 years Questionnaire for both
inventory for child and parents to
cific areas of auditory pathway, with wave V being listening rate speech
the most important one. The testing is started difficulties understanding in 15
at the high intensity levels and then gradually (CHILD) difficult situations
6
Inconsistency between subjective and objective However, MRI provides better soft tissue details,
measurements can be due to Central Auditory including membranous labyrinth and central
Processing Disorder (CAPD), which is the inabil- auditory pathway along with identification of
ity of the brain to fully interpret and process the intracranial pathologies (23, 24). MRI is cost-
sound with normal peripheral hearing. lier and needs sedation in younger children.
HRCT temporal bone is recommended for
Neonatal Hearing Screening children with cholesteatoma (25). CT shows
pneumatization of the mastoid air cell – varia-
Early identification and rehabilitation pro-
tions in position of structures embedded in
vide optimal results for congenital HL and can
temporal bone. It will identify scutum erosion
be achieved by neonatal hearing screening.
– an early sign of squamous COM, erosion of
Automated ABR (AABR) and OAE (AOAE) are
air cells, bony labyrinth, facial canal and teg-
two commonly used screening tools. Both have
men tympani (26). Diffusion-weighted MRI
portable handheld screening devices, which use
(DW MRI) can differentiate cholesteatoma from
algorithms and display pass or refer to response
inflammatory tissue and brain tissue; hence, it
during testing. The test and the protocol used
is useful in detecting recurrent or residual cho-
depend on facilities available at each centre. Since
lesteatoma after surgery (27, 28).
50% of the children diagnosed with HL do not
In congenital profound HL, morphologically
have any risk factors, universal screening of all
only 20% of the malformations are bony which
the newborns is recommended. AOAE is quick
can be picked up by HRCT and the rest 80% will
and cost-effective in terms of equipment and con-
have a normal imaging. Jackler et al. in 1987 clas-
sumables compared to AABR, but it has high fail-
sified the congenital malformations of the inner
ure rate and can miss auditory neuropathy (21, 22).
ear as malformations of osseous and membra-
The Joint Committee of Infant Screening 2007
nous labyrinth and malformations limited to
Position Statement suggested all infants should
membranous labyrinth (29). With the advances
have access to hearing screening by 1 month of
in imaging, especially HRCT, Sennaroglu et al.
age, confirmation of HL if present by 3 months
added more details to the classification and
of age and intervention by 6 months of age,
surgical approach to various anomalies (30, 31),
which is termed as 1–3–6 rule (6).
which are summarized in Table 1.4.
Enlarged Vestibular Aqueduct (EVA) is the
Radiological Evaluation of most common inner ear malformation (84%)
Children with Hearing Loss detected in patients with SNHL (32). HL is often
A clear understanding of the osseous anatomy bilateral, progressive and fluctuating, though it
of the temporal bone can be obtained through can present as sudden SNHL also. The common
high-resolution CT of the temporal bones. syndromes associated with HL are described in
Table 1.4: Classification of Inner Ear Malformations (IEM) and Their

Characteristics
Type of IEM Radiological Findings Type of Implant Surgical Implications
Complete Absent labyrinth Brainstem implant CI contraindicated, facial nerve
labyrinthine aplasia anomalies may be present
Rudimentary otocyst Remnant of otic capsule Brainstem implant CI contraindicated, facial nerve
anomalies may be present
Cochlear aplasia Absent cochlea Brainstem implant CI contraindicated
Common cavity Fused cochlea and CI Transmastoid labyrinthotomy or
vestibule forming ovoid double labyrinthotomy lateral wall
cystic structure placing electrode
Cochlear hypoplasia Smaller cochlea; type1-4 CI or HA as per HL If CI, short compressed electrode
Incomplete partition Cystic cochlea CI with a stopper 50% CSF gusher, facial nerve
(IP) type 1 abnormalities may be present
Incomplete partition Basal turn normal; fused CI with a stopper CSF oozer, 10% CSF gusher
(IP) type 2 middle and apex turns
Incomplete partition Modiolus absent with CI with a stopper 100 % CSF gusher, lateral wall placing
(IP) type 3 interscala septa present electrode
Enlarged vestibular Cochlea normal enlarged CI/ HA Varying degree of HL, progressive HL
aqueduct VA
Cochlear aperture Narrow or absent CI/ ABI ABI in cochlear naplasia, CI in cochlear
abnormalities cochlear aperture N hypoplasia
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Table 1.6 (33–38). The most common cause for autosomal recessive and usually presents pre-
bilateral postnatal acquired SNHL is meningitis lingually. Autosomal dominant type is around
(39). Cochlear ossification can be picked up by 20%, which is usually progressive and post-
T2-weighted MRI, whereas fibrosis and earlier lingual, and 1% is X-linked recessive/mitochon-
cochlear changes preceding ossification can be drial inheritance (42). GJB2 mutation, the gene
seen in T1-weighted MRI (40). Post-meningitic that encodes the protein Conexin 26, accounts
scarring and labyrinthine ossification follow- for nearly 50% of non-syndromic severe to pro-
ing meningitis are concerns during cochlear found HL (43) and can be screened with Sanger
implantation. sequencing. The differential diagnosis of PCHI
is summarized in Table 1.5.
DIFFERENTIAL DIAGNOSIS OF In autosomal recessive type, the hearing-
HEARING LOSS IN CHILDREN impaired child has normal parents and rela-
tives; there is 25% chance for siblings to develop
HL in children can be congenital or acquired HL. The genes identified are enumerated by let-
and progressive or fluctuant according to its ters “DFN” followed by “A” if gene is dominant
onset and progression. It can also be classified and by “B” if the gene is recessive. The mutation
according to the aetiology and severity of the of gene OTOF encoding the protein otoferlin, a
HL. The management varies according to the protein needed for synaptic transmission, and
cause, the age of the child, pre-lingual/post-lin- expressed in inner hair cell also causes autoso-
gual and the severity of HL. mal recessive HL (auditory neuropathy).
The common syndromes causing HL are sum-
Congenital Hearing Loss marized in Table 1.6.
In nearly 45% of newborns with HL, the cause Management of Congenital Hearing Loss:
remains unknown. Among the remaining, Management of HL in children needs multi-
nearly 60%, have a genetic cause and 70% of chil- disciplinary approach, including otolaryngolo-
dren with a genetic cause are non-syndromic gist, audiologist, speech therapist, paediatrician,
(41). Nearly 80% of the non-syndromic HL is child psychiatrist and ophthalmologist. It also
Table 1.5: Aetiology and Classification of Hearing Loss in Children

Congenital Hearing Loss
Genetic Syndromic Autosomal recessive (AR)
Autosomal dominant (AD)
X-linked
Mitochondrial
Non-syndromic Autosomal recessive (AR)
Autosomal dominant (AD)
X-linked
Mitochondrial
Non-genetic Congenital rubella syndrome, CMV infection, congenital syphilis, toxoplasmosis
Drugs during pregnancy – aminoglycosides, antiepileptic drugs, cytotoxic
drugs, antimalarial drugs, diuretics
Maternal substance abuse – alcohol, drugs
Maternal endocrine disorder – thyroid dysfunction, DM
Third window effect caused by SSC dehiscence, EVA, X-linked gusher syndrome,
enlarged IAM
Acquired Hearing Loss
Perinatal Hypoxia
Hyperbilirubinaemia
Low birth weight
Birth asphyxia
Birth trauma – intracranial haemorrhage
Postnatal Infections – meningitis, measles, mumps, COM, Herpes, HIV
Ototoxic drugs
Trauma and noise exposure
Neoplastic disorder, immune system disorder, foreign body EAC
Idiopathic
Source: Adapted from Scott-Brown, 8th edition; Chapter 10: Management of the hearing impaired child.
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Table 1.6: Summary of Common Syndromes Causing Hearing Loss
Chromosomal HL-related Radiological
Syndrome Abnormality Ear Abnormality Other Abnormalities Findings
Down’s syndrome Trisomy 21 Small low set pinna, narrow EAC, Brachycephaly, upslanting palpebral fissures, flat Narrow external and internal ear
IAC stenosis, ossicular and facial profile with epicanthal folds and brushfield canals with ossicular
vestibular malformation, OME spots, upper airway obstruction with macroglossia abnormalities
Conductive or mixed hearing loss and increased risk for atlanto-occipital subluxation
during intubation, mental retardation, single
palmar crease, heart disease, wide sandal gap
Treacher Collins TCOF1 gene mutations Bilateral microtia, CCA, ossicular Micrognathia, cleft palate, mandibular hypoplasia Narrow or absent external ear
abnormalities and flat malar region, down slanting palpebral canals with ossicular
First and second arch Conductive hearing loss fissures, coloboma of the lower eyelids, sparse eye abnormalities, absent mastoid
abnormalities lashes pneumatization, a bony cleft in the
lateral aspect of the temporal bone
Goldenhar Several chromosomal Unilateral microtia, preauricular Hemifacialmicrosomia, epibulbardermoid, Unilateral smaller external ear with

abnormalities appendages, ossicular congenital heart disease and vertebral ossicular abnormalities and
U//L first and second abnormalities, abnormal facial abnormalities abnormal facial canal
arch abnormalities nerve
CHARGE CHD7 gene mutations Dysplastic (short, wide) pinnae, Coloboma, Heart defects, Atresia choanae, Hypoplasia of SCC, abnormalities
Autosomal dominant various middle and inner ear Retarded growth and development, Genital of ossicluar chain and facial nerve
abnormalities, facial nerve palsy abnormalities, Ear abnormalities
Sensorineural /mixed/conductive
hearing loss of varying degree
Branchio-oto-renal EYA1, SIX1 and SIX5 Preauricular sinuses or tags, Branchial cleft abnormalities and renal Malformed ossicles, tapered basal
gene mutations ossicular abnormalities WVA malformations turn and hypoplasia of the middle
Autosomal dominant malformed cochlea and and apical turns of cochlea –
vestibular apparatus “umwound appearance”
Alports syndrome Gene mutation that Sensorineural hearing loss Nephropathy, lenticonus and macular flecks
codes for collagen 1V – varying and progressive
Pendred syndrome Congenital sesorineural hearing Hypothyroidism and goiter. perchlorate testing Mondini malformation and EVA
loss replaced by genetic testing
Usher syndrome Autosomal recessive Hearing loss Progressive retinitis pigmentosa. Three types
depending on the degree of hearing loss and
vestibular dysfunction
Waardenburg Autosomal dominant Congenital HL Synophrys, heterochromia iridium, white forlock, Aplasia of the posterior
syndrome dystopia canthorum, heterochromicirides semicircular canal, poorly
developed vestibule and EVA
Jervell Lange- Autosomal recessive Profound hearing loss Syncopal attacks, prolonged QT interval,
Neilson syndrome ventricular tachycardia of ventricular fibrillation
Congenital rubella Congenital hearing loss Growth retardation and mental retardation, cataracts
syndrome and pigmentary retinopathy, patent ductus
9
arteriosus, peripheral pulmonary artery stenosis

requires ongoing monitoring to detect progres- the device. In younger children, regular follow-
sive or late-onset HL. Exposure to consistent up is required as the ear canal volume increases,
and meaningful sounds during the early years requiring changes in ear mould.
of the child is required for the development of Implantable Auditory Devices (IADs): IADs are
auditory neural pathways. Hence, early identi- active implants and can be acoustic or electrical
fication and management is required. Auditory- implants. Acoustic implants transduce acoustic
oral communication assisted by hearing aids sound energy to the perilymph through one of
(HAs) or CI results in highest levels of educa- the pathways – ossicular chain, skull vibration
tional achievements and job opportunities for or direct stimulation. The electrical implants,
a child. the CI and auditory brainstem implants (ABI)
Hearing Aids: HAs provide the child with audi- stimulate the cochlear nerve or brain stem nuclei
ble broad frequency range of speech at soft to electrically.
loud levels. Air conduction HAs are commonly Acoustic implants work using various prin-
used, which amplify the sound transmitted via ciples and include bone-conducting devices,
the ear canal to the tympanic membrane (TM). active middle ear implants and direct acoustic
In chronically draining ears, atresia of the ear cochlear stimulators and can be partially or
canal where HA cannot be used or in patients fully implantable (44–47). The summary of the
with severe conductive/mixed HL, bone con- acoustic implants is given in Table 1.7.
duction HAs are beneficial. Behind the ear type Bone-Anchored Hearing Aid (BAHA): BAHA
is the most common HA used in children. The works by transmitting sound through the bone
ear mould should snugly fit in the ear canal to using an osseo-integrated abutment, which
reduce acoustic feedback and help retention of can be percutaneous or transcutaneous. The
Table 1.7: Summary of the Middle Ear Implants Used in Children

Type Examples Indications and Age Features Disadvantages
Bone conduction devices (BCD)
Skin drive Baha Attract SSD, mild to moderate Osseointegration – Transcutaneous energy loss
Sophono CHL, chronic external processor MRI incompatible
discharging ear, CCA with internal magnet
>5 years of age (FDA) fixed to mastoid bone
Short surgery
Direct drive Baha Connect SSD, mild to moderate Osseointegration – Abutment visible
Ponto CHL, chronic external processor on Skin infection and reaction
discharging ear, CCA, percutaneous MRI compatible
radiated bone abutment fixed to
>5 years of age (FDA) mastoid bone
Short surgery
Direct drive Bonebridge SSD, mild to moderate External processor MRI incompatible
(active) CHL, chronic magnetically attaches Need favourable mastoid
discharging ear, CCA to the internal anatomy – pre-op CT and
>5 years of age (EU, FDA transducer fixed to planning
not approved in children) mastoid bone
Active middle ear implants
Partially Vibrant sound Moderate-severe to External processor with MRI incompatible
implantable bridge severe CHL/mixed HL, internal floating mass Need favourable mastoid
CCA, advanced transducer (FMT) anatomy – pre-op CT and
otosclerosis FMT couple to planning
5 years (EU, FDA not in ossicular chain Surgery challenging
children) CI in active ME disease,
radiation and tumour
Fully Carina Moderate-severe to Internal sensor and MRI incompatible
implantable severe CHL/mixed HL, transducer couple to Need favourable mastoid
cosmetic reasons, poor ossicular chain anatomy – pre op CT and
tolerance to HA planning
Surgery challenging
CI in active ME disease,
radiation and tumour
Reoperation for battery
change
10
common indications are when conventional HA evoked compound action potential (ECAP)
cannot be fitted and in single-sided deafness have poorer outcomes (58, 59). In case of COM,
(48). BAHA soft band can be used in children staged surgery can be performed with clearing
till there is reasonable skull thickness to fix the of the disease and making the ear dry at the first
abutment, which is usually at 5 years of age. Soft stage, followed by implantation at a later stage.
tissue complications are more common in chil- The electrode can be inserted through the
dren and include soft tissue reactions, implant round window (60) after drilling the round
infections, soft tissue overgrowth of the abut- window niche or extended round window or
ment and failure of osseointegration (49, 50). through a cochleostomy. Insertion via cochleos-
Cochlear Implants: CI has revolutionized hear- tomy provides more favourable angle and thus
ing rehabilitation in pre-lingually deaf chil- can be less traumatic (60); however, round win-
dren with the aim to restore the hearing for dow insertion decreases the risk of traumatiz-
adequate speech and language development ing basilar membrane and acoustic/mechanic
(51). It replaces the non-functional transducer trauma to the cochlea from drilling a fenestram
system of the hair cells of cochlea by convert- (61). However, studies have not shown much
ing the sound signals into electronic signals, difference in the audiological outcome (62) and
thus directly stimulating the cochlear nerve. hearing preservation (63). Atraumatic insertion
According to the latest NICE guidelines, the techniques include minimal manipulation of
candidacy for CI has been expanded with revi- the ossicles, minimizing cochlear bony drill-
sion of hearing thresholds for CI candidacy. ing, avoidance of perilymph suctioning and
Children with hearing level less than 70 dB and slow insertion of the electrode (64). Soft inser-
with poor aided speech understanding are con- tion technique minimizes cochlear reaction to
sidered for CI as per the revised criteria (73). the implant, which also includes avoidance of
Children with profound HL implanted as early bone dust and blood entering the cochlea and
as less than 1 year with bilateral CI develop topical application of the steroids just before the
better binaural skills, better language skills electrode insertion (65). A small amount of hyal-
and thus more chance of attending normal uronic acid will act as a barrier to bone dust and
school (52). blood, decreases the electrode insertion force by
Cochlear Implant in Congenital Malformations: lubrication and hence may be beneficial for hear-
The facial nerve course can be altered in ing preservation (66). Early age of implantation,
cochlear hypoplasia and common cavity defor- use of peri-operative steroids, electrode length
mities. When the basal turn of the cochlea is not and insertion depth also contribute to success of
well-formed, electrode insertion through facial hearing preservation techniques (67, 68).
recess approach may be difficult and alterna- Postoperative measurements of impedance
tive methods such as transmastoid labyrin- and neural responses along with check X-ray
thotomy, scala vestibular insertion, canal wall (modified Stenver’s view) confirm the optimal
down (CWD) with blind sac closure, trans-canal placement of the electrodes. After 2–3 weeks, the
approach are planned (53, 54). In common cav- CI is switched on, mapping is done, an optimal
ity, an electrode with complete contact rings programme for the CI made and auditory verbal
and hypoplastic cochlea, shorter electrode, will therapy is commenced. Complications are usu-
be more beneficial. Cerebrospinal fluid (CSF) ally rare with the incidence less than 3%; how-
gusher is the main complication, especially ever, increased incidence is noted in malformed
in incomplete partition type 3, which can be cochleae (69, 70). Wound infection, facial/chorda
managed by using tissue threaded through the tympani nerve (71) injury, CSF fistula, device
electrode as stopper or using electrode with a failure and cholesteatoma are the possible com-
stopper. Radiologically, when cochlear nerve plications of CI surgery.
aplasia/cochlear aperture stenosis is suspected, Multiple factors, including age of the implan-
electrically evoked ABR (eABR) is a useful tool; tation, residual hearing, prior use of HAs, con-
if present, it indicates the favourability for CI. sistent use, family support and motivation
However auditory outcome after CI in cochlear and rehabilitation, will affect the outcome of
nerve hypoplasia is poor (55). CI is contraindi- implantation. In children with complex needs,
cated in cochlear aplasia and absent cochlear the results can be obtained by quality of life
nerve and is an indication for ABI. measurement parameters (72). CI technology is
Auditory neuropathy spectrum disor- rapidly progressing with newer less traumatic
der (ANSD) patients can benefit with CI in electrode designs, research and animal model
selected cases like OTOF gene mutation (56, 57). studies looking at gene therapy and delivery
However, children with associated hypoplas- of drugs to cochlea and development of totally
tic cochlear nerve and abnormal electrically implantable CI.
11
Auditory Brainstem Implants: ABI directly Congenital Abnormalities of

stimulate the cochlear nuclei electrically. ABI External and Middle Ear
surgery was introduced in children after it was Abnormalities of the external ear canal cause
successfully done in adults (74). Along with obstruction of air conduction mechanism and
cochlear/cochlear nerve aplasia, severe cochlear thus HL. Severe external ear deformity is usu-
ossification and advanced cochlear otosclerosis ally associated with middle ear ossicular chain
are the indication for ABI (75). Labyrinthine and deformity; many of them with named syn-
retrosigmoid approach have been used for ABI dromes are summarized in Table 1.6.
placement. Cerebral oedema, CSF leak, men- Congenital Canal Atresia (CCA) can cause
ingitis, bleeding and facial nerve palsy are the conductive HL and is commonly associated
usual complications (76). Only few children will with severe degree of microtia. The prevalence
develop open-set speech discrimination after of microtia is 1/10,000. CCA and microtia are
ABI (77, 78). graded according to the increasing severity of
Assistive Listening/Alerting Devices: For opti- deformity, which is summarized in Table 1.8
mum listening in noisy environment, a signal- (79, 80).
to-noise ratio of 20 dB is advisable, which helps Management: The Management of Microtia
those with hearing impaired to listen efficiently and CCA should consider auditory rehabilita-
in the background noise, over telephone and tion and cosmesis. Nearly 90% of the patients
in auditoriums or theatres. Frequency modula- have normal cochlear function. Bone conduc-
tion (FM) devices, induction loops, amplitude tion hearing device (BCHD) is a good option
modulation system and infrared signals are for Hearing Rehabilitation in Children (81). For
used. In older children, in a daily situation like the pinna deformity, the options available will
doorbell or an alarm, an extra loud signal is include no treatment, autologous ear reconstruc-
relayed to alert the child; vibrations or flashing tion using cartilage, Bone-Anchored Auricular
lights can also be used for assistance. Devices Prosthesis (BAAP).
fitted to the telephone like a telephone amplifier Isolated middle ear abnormalities are rare
which amplifies the telephone sound or a tele- and usually are minor malformations with
phone coupler which allows the signals from the delay in diagnosis. Minor malformation was
telephone to be picked up by the HAs can also classified by Cremer and modified by Tos into
be used. four subtypes: isolated stapes ankylosis, stapes
Table 1.8: Common Grading and Description of Microtia and Congenital

Canal Atresia (CCA)
Grading System Grades Description and Significance
Microtia Weerda 1° dysplasia 2° dysplasia 3° dysplasia Anotia or
(according to surgical Most structures of some structures of none of the absence of
management) pinna present pinna present structures of pinna the ear
are clear
No additional Partial reconstruction – Total construction:
skin/cartilage for some skin/ cartilage lot of skin/ cartilage
reconstruction
Marx Grade 1 Grade 2 Grade 3 Grade 4
(According to clinical Small pinna, all Some features Rudiment of skin Absent
appearance) features present recognizable and cartilage pinna/ear
canal
CCA Weerda Type A Type B Type C
(According to clinical Marked narrowing with Partial patency lateral wall Complete atresia
appearance) intact skin layer with medial atretic plate of the ear canal
Jahrsdoefer Parameters – points Rating for surgery
(According to Stapes present – 2 10 – excellent for surgery
imaging) Open oval window – 1 9 – very good
Middle ear space – 1 8 – good
Mastoid well pneumatized – 1 7 – fair
Malleus/ incus complex normal 6 – marginal
–1 <5 – poor
Round window normal – 1
Incus /stapes connection – 1
External ear appearance – 1
12
ankylosis with other ossicular anomaly, iso- inflammation of the middle ear cleft of acute
lated ossicular anomaly and aplasia/dysplasia onset and infective origin associated with
of oval widow or round windows. Unilateral or middle ear effusion and varied signs and
bilateral conductive HL, normal TM and nor- symptoms.
mal middle ear status are the usual findings, According to the nature of the AOM, it can be
which can be misdiagnosed as OM. HRCT tem- divided into four subgroups: sporadic, resistant,
poral bone along with middle ear endoscopy persistent and recurrent. It usually starts as a
assist diagnosis (82, 83). Auditory rehabilitation viral infection, which later becomes secondarily
can be achieved by HA or BAHA, according to infected with bacteria. The predominant clinical
severity of the condition. Surgical reconstruc- features are due to presence of inflammation,
tion should be attempted only by experienced although HL is also a symptom.
surgeons due to the expertise required and the The most common route of spread of infec-
chance of SNHL. tion from upper airway is via the eustachian
tube either through the lumen or along the sub-
Acquired Abnormalities of epithelial peritubal lymphatics. The eustachian
External and Middle Ear tube is shorter, wider and more horizontal in
Local irritation of the external auditory canal children and some ethnic populations (93), thus
by trauma, inflammation or burns can cause making them more prone for OM. Infection can
acquired EAC stenosis. It can be of two types – also reach the middle ear via a perforation in
solid or membranous – with solid being more the TM or through blood (rare).
common (84) and can be complicated by canal Aetiology: The most common organisms
cholesteatoma. During the wet/discharging that cause AOM are Streptococcus pneumoniae,
phase, regular ear cleaning and topical treat- Moraxella catarrhalis and Haemophilus influenzae.
ment is required. HL can be addressed by In 60–90% of the children with AOM, respira-
bone conduction or air conduction HAs. Long- tory viruses, especially respiratory syncytial
term success after surgery requires expertise virus (RSV), have been detected (94).
(85). Exostosis are multiple bony swellings Clinical features: The child usually pres-
in the EAC with strong association with cold ents with otalgia and fever. Examination may
water exposure and are usually asymptom- reveal a mild conductive HL along with find-
atic (86). Osteomas are single bony swellings ings of a retracted/hyperaemic/red bulging
of EAC. However, if the EAC is significantly TM, which clinches the diagnosis (95). Otalgia
obstructed, it can cause conductive HL and may be relieved with onset of otorrhoea, which
will require surgical removal (87, 88). is initially bloodstained and later becomes
muco-purulent.
Treatment: In uncomplicated AOM, watch-
Otitis Media
ful waiting can be considered as two-thirds of
Otitis Media (OM), the most common cause of children will recover in 24 hours with or with-
acquired HL in children, can be classified as out treatment. Antibiotic is recommended for
Acute Otitis Media (AOM), Serous Otitis Media AOM in children less than 6 months, if more
(SOM) or Chronic Otstis Media (COM). than 6 months with severe symptoms (pyrexia
Any factor predisposing to eustachian tube [>39°C], severe otalgia), recurrent episodes
(ET) dysfunction, which includes recurrent in less than 2 years and not responding after
upper respiratory infections, exanthematous watchful waiting for 2 days. Children who
fever, nasal allergy, adenoid hypertrophy caus- are at high-risk OM also require antibiotics.
ing obstruction in the tubal end of ET, tumours Amoxicillin 80–90 mg/kg/day is the dose rec-
of the nasopharynx, palatal paralysis and anom- ommended for 10–14 days. For persistent or
alies like cleft palate causing impaired function resistant AOM, higher dose of amoxycillin will
of the tensor palati muscle, increases the chances help in treating resistant pneumococcal infec-
of OM (89). There are multiple risk factors for the tion. However, Haemophilus being beta-lactam
OM, which include genetic, ethnicity, defect in producing may require broad-spectrum anti-
the immunity and environmental factors such biotics. If allergic to penicillin, cefdinir, cefu-
as day care attendance, poor socioeconomic sta- roxime or ceftriaxone is advised (95, 178).
tus and passive smoking (90). Systemic analgesic either acetaminophen or
ibuprofen is recommended for pain manage-
Acute Otitis Media ment, though topical analgesics may provide
It is one of the commonest illness of the child- short-lived benefit (96). Decongestant nasal
hood with the highest incidence at the first drops, oral anti histamine and corticosteroids
year of life (91, 92). AOM can be defined as (97) are supportive measures for which the level
13
of evidence is debatable. Modification of the risk may be observed. Behavioural issues along with
factors (day care attendance, parental smok- inattention may be noticed by children attending
ing, absence of breastfeeding, usage of pacifier day care by the teachers (110). Delayed speech
use, supine bottle feeding) (98) and antibiotic and language development may occur second-
prophylaxis during winter months are also ary to the HL. Child may also have symptoms
recommended. Vaccination against causative of associated pathology like upper respiratory
organism like S. pneumoniae has proven benefi- infection, allergy, adenoid hypertrophy etc.
cial (99). Examination: Otoscopic examination of small
Myringotomy is indicated to release the ten- children can be challenging; however, otomi-
sion of the pus accumulation if the TM is still croscopy has an accuracy more than 90% in
bulging after a week of antibiotics or incomplete diagnosing OME (111). On examination, the TM
resolution with persistent HL or when effusion appear dull and lustreless with varying shades
persists beyond 12 weeks. Ventilation tubes of yellow, grey or blue in colour. The position
were shown to reduce the number of episodes of of the TM can be retracted or bulged out due to
AOM by 50%, though cautious interpretation of fluid with the restriction of mobility, fluid level
the study is required (100). or air bubbles.
Investigations: Tuning fork test results are
Serous Otitis Media confirmed with audiometry and shows conduc-
(OM with effusion (OME)/secretory OM/glue tive HL usually within 25–30 dB. Audiometry
ear) Serous otitis media is termed as accumula- also helps to rule out associated causes of HL,
tion of fluid in the middle ear cleft, which is usu- including SNHL. The “B”-type tympanogram
ally thick and viscid. OME is termed chronic, if it is suggestive of middle ear fluid. The combined
persists for a minimum of 3 months (101). Nearly sensitivity of type “B” tympanogram with oto-
80% of the children will have at least one episode scopic findings is 98%. When HL is confirmed,
of OME before the age of 3 years (102). Boys are active monitoring of HL for 3 months are
more affected and those with less pneumatized advised (112).
mastoids are more prone for OME. A review Management: The aim of the treatment is
of multiple studies showed that the distribu- removal of fluid and prevention of recurrence.
tion of OME showed two peaks: one at around In half the children, it resolves in 3 months, but
2 years, when the social contact of the child in 5% it can persist even after 1 year. In more
increases, and another at 5 years, the time when than 90% of the patients, the OME resolves in 9
child enters preschool (103). Though OME is not months, but there is a chance of recurrence.
considered to have infective aetiology, positive Medical: Topical decongestants in the form
bacteriological culture has been observed in the of drops or spray help to relieve the muco-
middle ear aspirate of children with OME. In sal oedema. Antihistamine and topical ste-
another study, biofilms were demonstrated from roid spray will be beneficial in allergy (113).
middle ear mucosal biopsy in 92% of patients Antibiotics have been found to be benefi-
undergoing grommet insertion. In the temper- cial in treating upper respiratory infection.
ate climates, the incidence of OME was twice in However, according to studies, none of the
winter months as compared to summer months above medications have significant effect
(104, 105). Unilateral OME is twice as common as in improving OME (114). Amoxycillin-
bilateral OME. clavulanic acid combination was found to
Eustachian tube dysfunction and increased be beneficial in short term. Auto-inflation
secretory activity of the middle ear mucosa are methods like Valsalva’s manoeuvre (115),
the two major mechanisms in the pathogen- repeated swallowing and using chewing
esis of OME (106, 107). Unresolved otitis media gum can improve the ventilation of the mid-
due to inadequate treatment and other factors dle ear cleft. Hib immunization have also
can lead to increase in mucous production and shown to have positive effect on resolution
OME. Allergy and upper respiratory viruses of OME.
can cause increased secretory activity of the Surgical: In children with documented HL
middle ear mucosa. Gastroesophageal reflux with bilateral OME for a minimum of 3 months,
disease (GORD) and craniofacial anomalies can recurrent AOM, and at-risk children with uni-
also be associated with OME (108). lateral or bilateral OME, myringotomy and
History: HL is usually mild (less than 25 dB) grommet insertion are recommended (116).
and can be the only symptom, which may pass At-risk children are prone to developmental dif-
unnoticed in nearly 80% cases (109). Difficulty in ficulties due to physical, sensory or cognitive
understanding in a noisy environment is more disorder (117). Myringotomy is done by placing
evident in children. Seasonal fluctuation of HL a radial incision in the antero-inferior quadrant
14
of the TM. The radial incision can prevent exces-

sive damage to the middle layer and hold the
grommet in place. When the mucous is thick,
saline or mucolytic agents can be used to thin out
the mucous before aspiration. Continued aera-
tion of the middle ear is obtained by grommet
insertion, which can be long stay or short stay
ones, individualized depending on the pathol-
ogy. Short-term tubes such as Shah tubes and
Paparella tubes usually extrude between 6 and
14 months, whereas long-term tubes can stay up
to 2 years (115). Titanium or gold or silver oxide
coated grommets prevent biofilm formation.
Antibiotic-coated grommet was shown to pre-
vent biofilm formation in vitro. Adenoidectomy/
tonsillectomy may be combined with grommet Figure 1.4 Left healed tympanic membrane.
insertion to treat the causative factor. (Courtesy of Scott-Brown’s Otorhinolaryngology
Sequelae: The long-standing nature of the dis- Head and Neck Surgery; Chronic Otitis Media;
ease can cause dissolution of the fibrous layer George G Browning, Justin Weir, Gerard Kelly,
of the TM leading to atrophic changes and atel- Iain R C Swan; Paediatrics, The Ear, Skull
ectasis of the TM. Ossicular necrosis though Base; Eighth Edition; Volume 2; 2018; Page
rare can also occur, mostly affecting long pro- 992, Chapter 83: Figure 83.17; reproduced with
cess of the incus causing the HL to worsen. permission.)
Tympanosclerosis, cholesterol granuloma,
retraction pockets (RPs) and cholesteatoma can
also be sequelae of OME.
Table 1.9: Summary of the Types of
Chronic Otitis Media Chronic Otitis Media (COM)
The term COM comprises varied pathology
Types Description Findings
resulting from chronic middle ear inflamma-
tion. The incidence of COM is estimated to be COM Permanent Central dry
31 million cases globally, with the highest inci- mucosal perforation in perforation
inactive the pars tensa,
dence occurring in the first year of life and the with non-
highest percentage of affected people occurring inflamed ME
geographically in Oceania (118). Environmental mucosa
factors and population characteristics play a COM Permanent Discharge –
role in the prevalence of COM, with American mucosal perforation in mucoid, profuse,
Indians, Australian Aborigines and Eskimos active the pars tensa, non-foul smelling,
with inflamed non-blood stained
having the highest incidence in the developed ME mucosa and Pale polyps,
countries, probably due to genetic predisposi- discharge granulation
tion (119, 120). uncommon
Continuous inflammation in COM causes COM Retraction of the Retraction pocket
submucosal cellular infiltration, increased vas- squamosal pars tensa or with visible
cularity and increase in goblet cell production. inactive pars flaccida fundus
with a potential
There is osteitis with bone destruction, to which to become active
the body react with healing and repair. COM can COM Retraction of the Discharge is scanty,
be active with ongoing inflammation or can be squamosal pars tensa or foul smelling and
inactive where there is no active inflammation, active pars flaccida purulent.
but there is a chance to become active. Healed with retained Inflamed polyps,
squamous granulation
COM (Figure 1.4) is resolved inflammation with epithelial debris, common
time or by medical or surgical intervention. The with
description and clinical features of mucosal and inflammation,
squamosal COM are given in Table 1.9. discharge
The prevalence of COM is more in lower socio- COM Thinning of pars Dimeric drum/
economic group of people. Nutritional and envi- healed tensa (local/ tympanosclerosis
generalized)
ronmental factors, including lack of hygiene, with or
predisposes children to COM (121). COM has thickened
much higher incidence in children with cleft opaque TM
15
palate – at 10 years follow-up, 20% developed pars antibiotics drops are more effective compared
tensa retraction (122) and 2% developed choles- to systemic antibiotics (127–129). Quinolone
teatoma (123). Biofilms are also associated more antibiotics (ciprofloxacin/ofloxacin) are the pre-
commonly with COM (124). Tympanosclerosis ferred topical agents used in active COM (130).
is found in 25% of the ears undergoing surgery. Supportive measures like treatment of the under-
Pseudomonas aeruginosa, Proteus mirabilis and lying infective and allergic focus in the upper
Staphylococcus aureus are the common organisms respiratory tract is done. The aim of the surgery
isolated from patients with COM (125). is to reduce the hearing disability and to prevent
recurrent ear infection. Most surgeons prefer to
COM Mucosal do myringoplasty at 7 years of age as ET func-
COM mucosal presents with TM changes, like tion improves at 7 years. The success rate of tym-
perforation, tympanosclerosis or atrophy, and panoplasty in expert hands is around 95% (131);
ossicular changes, including erosion or fixation. though longer follow-up showed recurrent per-
It develops as a sequela to AOM with perforation foration (132). The failure rate is higher in larger
of pars tensa. The margin of the perforation epi- perforation and anterior perforations, which can
thelializes making it permanent, thus permit- be overcome by tucking the anterior margin
ting repeated infection from the ear canal. The beneath the annulus (133). The success rate is
middle ear mucosa gets exposed to the external reduced after revision surgery (132).
environment and is sensitized to airborne aller- In patients with air-bone gap more than 35
gens causing persistent otorrhea. dB, ossicular erosion or fixation due to tympa-
Diagnosis: Ear discharge and HL are the most nosclerosis is expected and requires ossicu-
common symptoms. HL is usually conductive loplasty (126). The most common ossicle to be
type. Otoscopic examination will show a cen- affected is long process of incus. If the handle
tral perforation with sizes varying from small of the malleus is also eroded, then prosthesis
to subtotal (Figure 1.5). Ear findings are con- sitting over the stapes head (PORP – partial
firmed by examination under microscope, after ossicular replacement prosthesis) connecting
clearing the discharge if present. Audiogram is to TM can be used. Prosthesis over footplate
done to assess the type and severity of the HL. (TORP – total ossicular replacement prosthesis)
The degree of HL depends on the size of the is used, if stapes suprastructure is also absent.
perforation, erosion of the ossicles especially The choice of the prosthesis depends on the
long process of the incus and tympanosclerosis diseased ear, availability of the prosthesis and
obstructing ossicular mobility (126). surgeon’s expertise. An analysis done by Lurato
Treatment: Aural toilet is done usually using et al. showed that when malleus and stapes
microscope and suction clearance. Topical suprastructure were present, the postoperative
air-bone gap of 0–10 dB was achieved in experts
hand only in 50% of the cases (134).
Ossiculoplasty results are significantly affected
by the status of the middle ear along with prosthe-
sis design and surgical technique. The materials
used for reconstruction are autograft (incus most
commonly used), alloplastic materials (Table 1.10)
and to a less extent homograft. Meaningful analy-
sis of the results of ossiculoplasty can be done by
various classifications proposed by Austin (135,
136), Belluci (137) and Kartush (138) and Middle
Table 1.10: Materials Used for

Ossiculoplasty
Figure 1.5 Left COM mucosal inactive
type showing tympanic membrane with Material Examples
central perforation. (Courtesy of Scott-Brown’s Solid plastics Polytetrafluoroethylene,
Otorhinolaryngology Head and Neck Surgery; polyethylene
Chronic Otitis Media; George G Browning, Solid metals Stainless steel, gold,
Justin Weir, Gerard Kelly, Iain R C Swan; titanium
Paediatrics, The Ear, Skull Base; Eighth Edition; Porous sponge-like plastics Proplast®, Plasti-Pore®
Volume 2; 2018; Page 992, Chapter 83: Figure Ceramics Aluminium oxide,
83.19; reproduced with permission.) hydroxyapatite
16
Table 1.11: Various Classifications of Ossicular Chain Defects and

Middle Ear Status
Kartush Modification of
Austin Classification with Absent Incus Ossicular Status
Group 1 Malleus handle (M)+ ; Stapes suprastructure (S)+ Group O Intact ossicular chain
Group 2 M+ S− Group E Ossicular head fixation
Group 3 M− S+ Group F Stapes fixation
Group 4 M− S−
Middle Ear Risk Index (MERI) Belluci classification of otorrhoea
Otological factor Maximum score Otorrhoea Risk value
Otorrhoea 3 Dry ear 0
Perforation 1 Occasionally wet 1
Cholesteatoma 2 Persistently wet 2
Ossicular status 4 Persistently wet + cleft 3
palate
Middle ear granulations 2
Previous surgery 2
Smoking 2
Ear Risk Index (MERI) (139), which are summa-

rized in Table 1.11.
COM Squamous
“Cholesteatoma is a mass formed by keratin-
izing squamous epithelium in the middle ear/
mastoid, sub-epithelial connective tissue and
by the progressive accumulation of the keratin
debris with or without surrounding inflam-
matory reaction” (140). It can be congenital or
acquired.
Congenital cholesteatoma (Figure 1.6) is an
expanding cystic mass with keratinizing squa-
mous epithelium, located medial to the intact
TM, assumed to be present at birth, but usually
diagnosed during infancy or in early childhood
in patients with no prior history of otorrhea, per-
foration or previous ear surgery (140). The most
accepted theory of congenital cholesteatoma is Figure 1.6 Left ear congenital cholesteatoma.
persistence of epidermoid cell that rests in ante- (Courtesy of Scott-Brown’s Otorhinolaryngology
rior epitympanum (141). A four-point staging Head and Neck Surgery; Chronic Otitis Media;
system was used by Potsic (142) to describe the William P L Hellier; Paediatrics, The Ear, Skull
extent of the spread of cholesteatoma. Base; Eighth Edition; Volume 2; 2018; Page 158,
Acquired cholesteatoma can be primary or Chapter 15: Figure 15 .2; reproduced with
secondary. Primary acquired cholesteatoma permission.)
develops with in-growth of keratin epithelium
through the perforation of the TM or due to
trauma or can be iatrogenic. (Figure 1.8) and can be graded into four types, as
The pathogenesis of secondary acquired cho- is described in Table 1.12.
lesteatoma is not clear and there are four theories Paediatric cholesteatoma is more aggressive
– metaplasia theory/retraction theory, immigra- and extensive (145) with higher recurrence rate
tion theory and the basal hyperplasia theory. The (146), due to immaturity of the ET function, and
RP can develop either in pars tensa or pars flac- thus need long-term follow-up. Eustachian tube
cida; when the pocket cannot self-clean, it will dysfunction leading to negative middle ear pres-
cause slow invasion. The RPs can be present in sure and retraction of the TM is the most widely
pars tensa (143) (Figure 1.7) or pars flaccida (144) accepted theory.
17
Figure 1.7 Grade 4 retraction pocket of Figure 1.8 Retraction pocket in the
the pars tensa. (Courtesy of Scott-Brown’s pars flaccida. (Courtesy of Scott-Brown’s
Otorhinolaryngology Head and Neck Surgery; Otorhinolaryngology Head and Neck Surgery;
Chronic Otitis Media; George G Browning, Chronic Otitis Media; George G Browning,
Justin Weir, Gerard Kelly, Iain R C Swan; Justin Weir, Gerard Kelly, Iain R C Swan;
Paediatrics, The Ear, Skull Base; Eighth Edition; Paediatrics, The Ear, Skull Base; Eighth Edition;
Volume 2; 2018; Page 994, Chapter 83: Figure Volume 2; 2018; Page 995 Chapter 83: Figure 83:
83:29; reproduced with permission.) 33; reproduced with permission.)
Diagnosis: HL and ear discharge are the com- Ventilation tubes do not have long-term effect
mon symptoms and on examination the pres- on TM RPs (149, 150).
ence of RP with keratin pearls is diagnostic of Cholesteatoma: The primary treatment modal-
COM squamous active. Inactive squamous type ity is surgery with the aim of eradication of all
will present with RPs without keratin debris. cholesteatoma and any complication, thus mak-
However, in smaller children, eliciting the ing the ear dry and self-cleaning and prevent-
history and examination remain a challenge. ing further recurrence. The surgical procedure
Diagnosing unilateral disease or congenital cho- can be broadly divided into CWD surgery or
lesteatoma is even more challenging. less invasive canal wall up (CWU) procedures.
Treatment: Retraction Pockets: Self-cleaning RPs CWD procedures include inside-out surgery like
have to be closely monitored in children. Small atticotomy +/− reconstruction, modified radical
RPs can be regularly cleaned. Surgical treatment mastoidectomy and subtotal petrosectomy for
is to prevent discharge, prevent progression of extensive diseases, which can be combined with
RP and improve hearing. This also includes cavity obliteration techniques. CWU procedures
management of TM and ventilation of the mid- are mastoidectomy, combined approach tympa-
dle ear. Excision of RP and grafting of the TM noplasty and tympanoplasty. The advantages of
is a good option in most of the cases (147, 148). CWU procedure over CWD are it does not need
Table 1.12: Classification of Retraction Pockets in Pars Tensa and

Pars Flaccida
Pars Tensa Retraction Pocket Pars Flaccida Retraction Pocket
(Sade et al.) 140 (Tos et al.) 141
Grade 1 Minimal retraction of pars tensa Grade 1 Dimple in PF, not attached to malleus
Grade 2 Retraction touching the long Grade 2 RP attached to malleus neck, full
process of the incus extent visible
Grade 3 Retraction touching the medial Grade 3 RP full extent not visible, minimal
wall but can lift up erosion of bony attic wall
Grade 4 Retraction adherent to medial Grade 4 Definite erosion of bony attic wall
wall with extent of RP not visible
18
repeated cleaning of cavity and HA can be fitted burden of disease globally. For any disability,
if HL warrants; however, there is more chance prevention can be primary, secondary or tertiary.
for recurrence. Studies support better audiologi- Genetic counselling (158), immunization, avoid-
cal outcomes with CWU procedures (151, 152). ance of trauma and ototoxic medications (159)
However, the procedure is technically more constitute primary prevention of HL. Hearing
demanding and requires a second look surgery. screening (68, 160) and treatment of HL are con-
In the recent times, endoscopic ear surgery has sidered secondary prevention and early rehabili-
become adjuvant to microscopic ear surgery tation of HL (161), the tertiary prevention. Newer
(153, 154), the efficacy and safety of this tech- and improved methods are added rapidly to all
nique are validated in paediatric cholesteatoma these areas, thus making management options
(155, 156). for children with HL better every day.
In OM, if not treated appropriately, patients
can develop extracranial complications such RECENT ADVANCES
as acute mastoiditis, abscesses in relation to The advancement made in genetics in recent
pinna, labyrinthitis, facial palsy, petrositis and years, for example, whole genome sequencing,
intracranial complications such as extradural has helped to map deafness and other associ-
abscess, subdural abscess, meningitis, brain ated traits to specific chromosomes, thus help-
abscess, lateral sinus thrombosis and otitic ing to identify the causes of various syndromic
hydrocephalus (157). and non-syndromic HL. In bilateral SNHL,
genetic testing proves to be high yielding with
Other Causes of Hearing Loss in Children identification of cause in 44% cases (169, 170).
Unilateral Hearing Loss: It is one condition The introduction of smaller diameter endo-
which is under-diagnosed, but it has a preva- scopes with high definition has expanded the
lence of 0.6 per 1000 (8) among neonatal screen- field view and improved resolution of endo-
ing and 1 per 1000 among school-age children. scopic ear surgery as compared to microscopic
Children with unilateral HL showed lower oral ear surgery (171). Angled endoscopes enable
language scores, manifested behavioural issues to visualize areas which are difficult to assess
and some required academic assistance; the such as sinus tympani and epitympanum (172).
maximum effect is seen in children with pro- Incorporation of flexible fibre CO2 laser (173)
found HL (162). Preferential seating, frequency and ultrasonic bone curette (174) has broad-
modulating system, conventional HAs and ened the endoscopic ear surgery indications.
contralateral routing of signal (CROS) aids are Ear-specific 3D printed temporal bones with
found to be beneficial (163). Single-sided deaf‑ add-on details of external and middle ears will
ness (SSD) is defined as severe to profound HL be highly beneficial for skills training of pae-
with minimal benefit with hearing amplification diatric otologists. Robotic-assisted and robotic-
and normal hearing in contralateral ear (164). CI performed otological surgery that has potential
is the available option for the SSD which can to decrease the morbidity and improve the out-
provide binaural auditory input (165, 166). comes is still under development stage.
Ototoxicity: The common drugs causing oto- Medical therapy for SNHL targets inner ear
toxicity are aminoglycosides – amikacin, strep- for novel gene therapy, RNA-based therapy
tomycin and tobramycin, antitumour agents and stem cell therapy. It aims at modification
– cisplatin, antimalarials – quinine, chloroquine, of hair cells or auditory neurons at cellular
and loop diuretics – frusemide; these medica- level through gene therapy to augment protein
tions need to be used with caution with serial production that may protect or regenerate hair
audiogram to document hearing level. cells (175); RNA-based therapy to inhibit protein
Sudden sensorineural hearing loss: It is an that causes hair cell damage (176) and stem cell
otological emergency with HL of 30 dB or more therapy to replace damaged or dead hair cells or
developing in three consecutive frequencies in auditory neurons (177).
less than 72 hours (167). The possible causes can
be infectious, traumatic, metabolic, neurologic, CONCLUSION
circulatory, toxic or others; immediate manage- In the formative years of childhood, HL caused
ment mainly consists of anti-inflammatory such by a variety of reasons can go unnoticed affect-
as steroids (168) and antivirals. ing the overall development of the child and
suboptimal performance in the society. Early
DISCUSSION detection and tailor-made treatment is highly
The WHO lists HL as one of the leading causes rewarding owing to the recent advances
of disability and one of the leading causes of which are rapidly evolving and redefining the
19
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CONCLUSION
Constantine’s accession proved to be, like the coming of Alexander,

a turning-point in the history of the world. His so-called conversion
put into the hands of the Catholic Church a weapon for the
suppression of all rivalry, of which she was not slow to make use.
Already in his reign many of the heathen temples were torn
down[1217], and under the rule of his morose and gloomy successor,
Constantius, the work of demolition went on apace[1218]. The
accession of the philosophic Julian gave the worshippers of other
gods than Christ a short respite, and even allowed some of the
temples destroyed in the former reigns to be restored by or at the
expense of the Christians[1219]. Julian’s heroic death in Persia again
threw the crown into the hands of a Christian emperor, whose reign
of seven months gave him little time, as he perhaps had small
inclination, for persecution[1220]; but under his successors Valentinian
and Valens, heathen sacrifices were forbidden under severe
penalties. The end came under Gratian, when the temple estates
were confiscated, the priests and vestals deprived of the stipends
which they had hitherto received from the public treasury, and the
heathen confraternities or colleges were declared incapable of
receiving legacies[1221]. Only a few rich men like the Vettius Agorius
Praetextatus whom we have seen among the worshippers of
Mithras, or the Quintus Aurelius Symmachus, whose learned and
patriotic life has been so well described by Sir Samuel Dill[1222], could
henceforth venture to practise, even with maimed rites, the faiths
condemned by the Court and the Church.
As for the Gnostic sects, which since Hadrian’s time had striven with
such success as we have seen to combine magic and other ancient
beliefs with Christianity, they found but short shrift at the hands of the
triumphant Church. By an edict issued by Constantine before his
own reception into the Church, all their “houses of prayer” were
confiscated for the benefit of the Catholic Church, their meeting even
in private forbidden, and their books seized and burned[1223].
“Thus,” says Eusebius, “were the lurking places of the heretics

broken up by the emperor’s command, and the savage beasts
they have harboured (I mean the chief authors of their impious
doctrines) driven to flight. Of those whom they had deceived,
some, intimidated by the emperor’s threats, disguising their real
feelings, crept secretly into the Church. For since the law
directed that search should be made for their books, those of
them who practised evil and forbidden arts were detected, and
these were ready to secure their own safety by dissimulation of
every kind[1224].”
Throughout the length and breadth of the Roman Empire all but a
very few Roman nobles thus professed the faith of Christ. In the
words of the dying Julian, the Galilaean had conquered.
From this time until our own, Christianity has reigned in the West
with no serious rival. In the VIIth century, when Mahommed’s Arabs,
flushed with the enthusiasm of a new faith which owed something at
least to the relics of Gnosticism, poured in upon an Empire wearied
out alike by perpetual war against the barbarians and by its own civil
and religious dissensions, the Church was compelled to abandon to
them her conquests in Africa and the East. In Europe, however, she
continued in unchecked supremacy, gathering to herself and
assimilating the barbarians who at one time seemed likely to
extinguish all civilization; and she thus became a bond uniting many
nations and languages in one community of faith and thought. She
even succeeded in keeping alive the remains of that Greek art and
learning which still form our best and proudest intellectual
possession, and if during her reign many of the precious monuments
of antiquity perished, the fault was not entirely hers. In every respect,
her rule was supreme; and such enemies as she had in Europe were
those of her own household. The Manichaeans who, as has been
said, once bid fair to deprive her of some of her fairest provinces,
never dared to make open war upon her, and their secret defection
was punished by an unsparing use of the secular arm. The German
Reformation of the XVIth century has probably left her stronger than
before, and the few losses that she has suffered in the Old World
have been more than compensated by the number of lieges she has
succeeded in attaching to herself in the New.
In the days of her infancy, and before she thus came into her
inheritance, Christianity borrowed much from the rivals over which
she was in the long run to reign supreme. Her outward observances,
her ritual, and the organization of her hierarchy, are perhaps all due
to the associations that she finally overcame. The form of her
sacraments, the periods of her fasts and festivals, and institutions
like monachism, cannot be explained without reference to those
religions from whose rivalry she so long suffered. That, in such
matters, the Church should take what was useful to her was, as said
above, part of her consciously expressed policy, and doubtless had
much to do with her speedy triumph. To show that her dogmas also
took many things from the same source would involve an invasion
into the domain of professional theology, for which I have neither
authority nor desire. But if, at some future time, investigation should
show that in this respect also Christianity owes something to her
forerunners and rivals, the argument against her Divine origin would
not thereby be necessarily strengthened. That, in the course of her
development, she acquired characteristics which fitted her to her
environment would be in strict conformity with the laws which appear
to govern the evolution of all institutions; and if the Power ruling the
universe chooses to work by law rather than by what seems to us
like caprice, such a choice does not show Him to be lacking either in
wisdom or benevolence.
As was said at the outset, everyone must be left to place his own
interpretation on the facts here attempted to be set forth. But if, per
impossibile, we could approach the study of the origins of
Christianity with the same mental detachment and freedom from
prejudice with which we might examine the worship of the Syrian
Jupiter Dolichenus or the Scandinavian Odin, we should probably
find that the Primitive Church had no need of the miraculous powers
which were once assigned as the reason for her gradual and steady
advance to all but universal dominion. On the contrary, it may be that
Christianity would then appear as a link—although a most important
and necessary link—in a regular chain of events which began more
than three centuries before she emerged from her birthplace in
Palestine into that Roman world which in three centuries more was
to be hers of right. No sooner had Alexander’s conquests made a
world-religion possible, than there sprang up, as we have seen, in
his own city of Alexandria, a faith with a far higher and purer idea of
Divinity than any that had until then been known in the West. Then
the germs already present in small fraternities like those of the
Orphics and the Essenes blossomed forth into the fantastic and
unwholesome growths, as we must needs think them, of that
Gnosticism which marked the transition of the ancient world from
Paganism to Christianity. Lastly there came in from the countries
under the influence of Rome’s secular enemy, Persia, the heresy of
Marcion, the religion of Mithras, and the syncretistic policy of Manes
and his continuators. Against all these in turn, Christianity had to
struggle in a contest where the victory was not always on her side:
and if in time she overthrew them all, it can only be because she was
better fitted to the needs of the world than any of her predecessors
or contemporaries.
INDEX
Abel, Ophite story of, ii. 52;

and Manichaean, ii. 304
Aberamenthôu, name used in Magic Papyri and Pistis Sophia, i. 102.
See Jesus, Texts of Saviour
Abiuth, receiver of Ariel in Texts of Saviour, ii. 186
Abraham, named in Mag. Pap., i. 106 n. 6; ii. 34;
an astrologer apud Artapanus, i. 173;
inspired by Ialdabaoth, ii. 53;
Bosom of, in Marcion’s system, ii. 211
Abraxas, in system of Basilides, ii. 90, 92
Abydos, gods of, i. 33 n. 1;
excavations at, i. 36
Achaea, worship of Goddesses Twain in, i. 135;
Cilician pirates deported to, ii. 229
Achaemenides, Persian religion under, i. 122; ii. 234
Achamoth, Sophia of Ophites, ii. 45 n. 1;
called the Mother by Valentinus, ii. 112 n. 3;
the Sophia Without of Valentinus, ii. 117 n. 2;
baptism in name of, by Marcus, ii. 189 n. 1.
See Sophia (2)
Acheron, Isis shining in, i. 60
Achilles, his horror of Hades, i. 59, 150;
his flattery of Zeus, i. 95;
his purification by Ulysses, i. 121 n. 4
Achrammachamari, name of Great Propator in Texts of Saviour and
Mag. Pap., ii. 142 n. 2
Acropolis, sacred things of Eleusis lodged in, i. 39;
Serapeum built opposite, i. 52
Acrostics, use of, in Jewish, Greek and Christian literature, i. 169 n.
1;
in Valentinian epitaph, ii. 129 n. 3
Adam, the protoplast, Ophite story of, ii. 52, 58, 70;
and Manichaean, ii. 299;
and neo-Manichaean, ii. 329
Adam or Adamos, god of Samothrace, i. 139 n. 1; ii. 54 n. 6
Adamas, the Ophite, the First Man or Great Light, ii. 38;
gives birth to Second Man or Son, ibid.;
called Father-and-Son, ii. 39;
androgyne, ii. 40;
forms triad with Holy Spirit, ii. 41 nn. 2, 3;
all things except matter contained in, ii. 44 n. 2, 64;
all light returns to, ii. 65, 80;
called Caulacau, ii. 94 n. 3.
See First Man, Caulacau, Hades
Adamas, king of the Twelve Aeons in Pistis Sophia, his rebellion, ii.
48 n. 4, 152 n. 1;
place of, ii. 137 n. 3;
ruler of Zodiac, ii. 152;
delays redemption of souls, ii. 153;
sends demon in shape of flying arrow, ii. 156;
probably Diabolos or Cosmocrator of Valentinus, ii. 163.
See Sabaoth Adamas
Adamas of the Light, in neo-Manichaeism, ii. 325;
slayer of monster, ii. 329
Adonai, epithet of Zeus in Mag. Pap., i. 106;
in Coptic, ii. 46 n. 3;
son of Ophite Sophia, ii. 47;
ruler of planetary sphere in Diagram, ii. 69;
meaning of name of, ii. 71 n. 1;
address of soul to, ii. 72
Adonis, wailed for in Athens, temp. Alcibiades, i. 16;
Dying God of Mediterranean, i. 37;
Asiatic form of Dionysos, i. 47;
identified with Osiris, i. 55;
identified with Dionysos by Orphics, i. 137, 145;
identified with Dionysos at Eleusis, i. 139 n. 1;
androgyne, i. 185;
Ophites attend mysteries of, ii. 21, 54;
identified with Phrygian god, ii. 31;
fiend in hell in Texts of Saviour, ii. 186
Advent, the. See Parusia
Aegean, islands of, birthplace of gods, i. 16, 52;
early worship of Alexandrian gods in, i. 52;
and of Eleusinian, ii. 135
Aeinous or Aionios (Everlasting), member of Valentinian Dodecad, ii.
101
Aelius Aristides, quoted, i. 55 n. 2, 58, 60, 64 n. 3; ii. 66 n. 2
Aeon, Thirteenth, highest place of Left in P.S., ii. 143, 150;
Authades would-be ruler of, ii. 151, 153;
first dwelling-place of Pistis Sophia, ii. 155;
place below it made for Pistis Sophia, ii. 155, 156;
Pistis Sophia restored to, ii. 157
Aeons, the Twelve, described, ii. 143, 152, 153;
souls made from tears of rulers of, ii. 153;
Jesus takes away part of their power, ii. 154;
divided into repentant and unrepentant, ii. 182;
the mystery of, in Bruce Papyrus, ii. 195.
See Zodiac
Aerodios, power mentioned in Bruce Papyrus, ii. 191
Aeschines, son of Glaucothea, i. 22;
Demosthenes’ invective against, quoted, i. 138.
See Sabazius
Aeschylus, quoted, i. 48, 55, 123
Aether, offspring of Time ap. Orphics, i. 123
Afghanistan, included in Persian Empire, i. 1
Africa, political power of priesthoods in, i. 31;
Mithraism in Northern, ii. 230;
christianized Manichaeism of, ii. 339
Agape or Love, supreme God of Diagram, ii. 68, 123 n. 3;
supreme God of Valentinus, ii. 98 n. 1;
feminine member of Valentinian Dodecad, ii. 101;
supreme God of Marcion, ii. 210;
seal of Azrua in neo-Manichaeism, ii. 341.
See Eros
Agdistis, name of androgyne Cybele, ii. 39, 40
Ageratos or Never-ageing, member of Valentinian Decad, ii. 101
Agla, cabalistic word used in mediaeval magic, ii. 139 n. 1
Agra on the Ilissus, mysteries of, i. 41
Agrestius, a clarissimus and high priest of Mithras, ii. 239
Ahnas-el-Medineh or Heracleopolis, mentioned in magic spell, i. 98
Ahriman, Areimanios, or Arimanius, in Bundahish slayer of
Gayômort, i. 126 n. 3;
not entirely evil till Sassanid times, ii. 232, 253;
Magi sacrifice to, ii. 234;
son of Zervan Akerene (Cumont), ii. 236, 252;
altars dedicated to, ii. 239;
Mithras superior to, ii. 240;
in Bundahish slayer of bull Goshurun, ii. 246, 254;
ruler of earth in Mithraism, ii, 255, 256;
modified worship of, in Mithraism, ii. 278;
likeness of representation of, to Manichaean Satan, ii. 291.
See Goshurun
Ahura Mazda, the Omniscient Lord, i. liii;
father of Gayômort, i. lxi;
Supreme Being of Yashts, ii. 231;
his relations to Amshaspands, ii. 232;
in Behistun inscription, ii. 233;
not mentioned in Mithraic monuments, ii. 239;
in Bundahish, ii. 246;
replaced by Jupiter O.M. in Mithraism, ii. 246;
worship of, restored by Ardeshîr, ii. 284
Ailoaios or Eloaeus, ruler of planetary sphere in Diagram, ii. 69, 70 n.
2, 74 n. 3;
address to, ii. 73;
sphere of Venus, 74 n. 1
Akae, cryptographic name in Book of Enoch, i. 169, 170
Akinetos or Immovable, member of Valentinian Decad, ii. 101
Albigenses, successors of Manichaeans, ii. 357
Al-Bîrûnî, quoted, ii. 279, 280, 283, 284, 286 n. 1, 307
Alcibiades, goes to Susa, i. 7;
Adonis wailed for when Sicilian expedition of, sails, i. 16
Alcmaeon of Crotona, calls stars gods, i. 186 n. 2
Aldabeim, name of sun in Mag. Pap., ii. 46 n. 3
Aletheia, member of 1st Valentinian syzygy, ii. 98
Alexander of Abonoteichos, his impostures, i. 24;
comes to Rome under Marcus Aurelius, ii. 203
Alexander, King of Epirus, Asoka’s mission to, i. 20
Alexander the Great, his conquests hellenize Mediterranean Basin, i.
lviii;
the world before and after, i. 1 sqq.;
greatest individual in history, i. 4, 12 sqq.;
his aims and achievements, i. 5-8, 26-27;
his deification explained, i. 18;
religious associations follow conquests of, i. 22-26 sqq.;
his work in Egypt, i. 29, 44;
his probable plans for universal religion, i. 30;
breaks down national barriers, i. 54, 107;
makes world-religions possible, i. 111;
his conduct towards Jews, i. 150;
re-settles Samaria, i. 177;
son of Zeus in serpent form, ii. 49;
his effect on cosmology and ethics, ii. 86;
consoled by Anaxarchus for death of Clitus, ii. 87.
See India
Alexander, bishop of Lycopolis, quoted, ii. 294 n. 2, 295 n. 2.
Alexander Severus, the Emperor, gods in lararium of, i. 82;
his success against Persians, ii. 226
Alexander the Valentinian, leader of Anatolic School, ii. 119
Alexandria, its foundation by Alexander, i. 5;
its importance not at first recognized, i. 28;
Sema of Alexander at, i. 30;
a Greek city, i. 44;
Serapeum of, i. 48, 51, 58 n. 1;
oracle of Serapis at, i. 77;
worship of Serapis at, i. 82 n. 2, 86;
destruction of temples at, by Theodosius, i. 83, 84;
Hadrian’s opinion of, i. 86;
early Gnostics start from, i. 111; ii. 8;
Orphics plentiful at, i. 156;
Simon Magus’ doctrines at, i. 198; ii. 89;
intellectual centre of Roman world, ii. 88;
Basilides teaches at, ii. 90
Alfenius Julianus Kumenius, clarissimus and priest of Mithras, ii. 268
Allat, the goddess, Ereshkigal an epithet of, i. 100
Alleius Craeonius, author on magic, i. 105
Amazons, the story of, suggests bisexual deity, ii. 40
Ambrose of Milan, convert from Valentinianism, i. 112 n. 1; ii. 21 n. 5;
his date, ii. 132 n. 2
Amélineau, E., translates Pistis Sophia, ii. 13;
translates Bruce Papyrus, ii, 190;
his date for Bruce Papyrus, ii. 194;
quoted, ii. 178, 191, 192, 193, 195
Amen of Thebes, the god, father of Alexander, i. 18;
priesthood of, i. 23, 31 sqq.;
Ptolemies raise temples to, i. 52
Amenhotep IV, King of Egypt, failure of monotheistic teaching of, i.
11;
priests of Amen crush heresy of, i. 31
Amens, the Three, powers mentioned in P.S., ii. 142;
and in Bruce Papyrus, ii. 193
Amens, the Seven, powers mentioned in P.S., ii. 141;
and in Bruce Papyrus, ii. 193
Amenti, the god called Lord of, i. 33;
Osiris the bull of, i. 45, 102;
Jesus the conqueror of, i. 102 n. 1;
a hell in Texts of Saviour, ii. 182, 186;
horrors of Egyptian, ii. 196.
See Aberamenthôu, Jesus, Khent-Amentit
Ameretât or Immortality, one of the Amshaspands, i. 181 n. 1; ii. 324
n. 4, 355.
See Appellant and Respondent
Amitrochates, son of Chandragupta, his desire for Greek learning, i.
8 n. 3
Amon. See Amen of Thebes
Amos, the Prophet, inspired by Ialdabaoth ap. Ophites, ii. 81 n. 2
Amshaspands, the Seven, and the Seven Planets, i. 117;
names of, i. 181, n. 1; ii. 103 n. 3;
likeness of, to “roots” of Simon Magus, ii. 103 n. 3;
and to Aeons of Valentinus, ii. 103 n. 3;
in Avesta, ii. 232;
absent from early Manichaeism, ii. 327 n. 4;
mention of, in neo-Manichaeism, ii. 330, 355
Anat, the goddess, assessor of Yahweh, ii. 32 n. 4
Anatolia, its religious peculiarities temp. Christ, ii. 28 sqq., 77;
its worship of double axe, 67 n. 3
Anaxarchus the Atomist philosopher, consoles Alexander after death
of Clitus, ii. 87
Ancient of Days, name of Valentinian Ialdabaoth, ii. 107 n. 2
Andrew the Apostle, Saint, name of, shows predilection of Jews for
Greek names, i. 173 n. 2;
mentioned in Pistis Sophia, ii. 157
Anebo, letter of Porphyry to, for threats of Egyptian magicians to
gods, i. 104 n. 3
Angels, Essenes sworn to preserve the names of, i. 153, 157;
no names of, in O.T., until Daniel, i. 158;
rulers over tribes of demons, ibid.;
sinning, cast into abyss of fire (Baruch), i. 165;
Ennoia produces world-making, ap. Simon M., i. 187;
patterns after which worlds made (Philo), i. 187 n. 3;
world to be freed from rule of, ap. Simon, i. 196;
Simonians say God of Jews one of world-making (Epiphanius), i.
199;
seven heavens are also, ap. Valentinians (Irenaeus), ii. 107 n. 4;
are Logoi sent into soul by Jesus and Sophia, ii. 110;
souls after death, brides of, ibid.;
terror of angels at speech of man (Valentinus), ii. 112 n. 3;
Archons of Adamas in Texts of Saviour beget, ii, 152 n. 1;
Splenditenens and Atlas of Manichaeism, ii. 297, 298.
See Enoch, Gabriel, Great Council, Michael, Tertullian
Annu or On, Egyptian name of Heliopolis and chief seat of worship of
Râ, i. 31
Anthesteria, ceremonies of, show resurrection and marriage of
Dionysos, i. 42
Anthropos, member of 3rd Valentinian syzygy, ii. 98
Antigonus Monophthalmos, King of Syria, his retort when hailed as a
god, i. 19;
Phrygia occupied alternately by him and Lysimachus, ii. 29
Antigonus Gonatas, King of Macedonia, mission of Asoka to, i. 20.
See Tarn
Antinous, death of, fixes date of Hadrian’s letter to Servian, i. 86 n. 5
Antioch, worship of Serapis at, i. 35;
birthplace of Carpocrates, i. 111;
and of Saturninus, ii. 9, 89
Antiochus I Soter, King of Syria, mission of Asoka to, i. 20
Antiochus III the Great, King of Syria, seizes Palestine, i. 151;
transports Jewish families to Anatolia, ii. 28
Antiochus IV Epiphanes, King of Syria, attempts to hellenize Jews, i.
151, 156, 162, 163;
Book of Daniel written temp., i. 158;
caught between Romans and Parthians, i. 160;
his mystic antagonist Taxo, i. 170;
Samaritans accept reforms of, i. 177
Antitheses, the. See Marcion
Antonines, the, Isis-worship at its apogee temp., i. 54, 81
Antoninus Pius, the Emperor, Simonians in Rome temp., i. 199
Anubis, the god, son of Osiris and Nephthys, i. 35;
tribal deity of jackal totem, i. 36;
his seeking for Osiris in Rome, i. 70;
in procession at Cenchreae, i. 72;
mask of, used as disguise, i. 78.
See Marcus Volusius
Apelles, the Marcionite, his tenets, ii. 218
Apep, the serpent, enemy of the sun-god Ra, ii. 78
Aphrodite, the goddess, worshipped under other names by
confraternities, i. 25;
and Adonis, i. 37; ii. 31;
daughter of Zeus, i. 124 n. 3;
identified by Orphics with Isis and others, i. 137 n. 1;
Orphic hymn to, i. 142 n. 2;
called Cytheraea, i. 143;
the Mother of the Gods in Cyprus, ii. 40;
called Mother of All Living in Asia, ii. 135 n. 3;
on Mithraic monuments, ii. 238.
See Venus
Apis, the “life” of Osiris, i. 32, 45, 49
Apocalypse of St John, the, its date, ii. 26 n. 3;
quoted, i. 145 n. 1, 158, 169, 182 n. 4; ii. 4 n. 1, 25
Apocatastasis, return of the worlds to God, an Ophite doctrine, ii. 42,
57
Apollo, the god, his birthplace, i. 16;
identified with Horus, i. 48, 63;
his contempt for mankind, i. 57;
his place in Orphic legend, i. 125, 147;
on Mithraic monuments, ii. 238;
distinct from Helios, i. 240;
worship of, under Julian, i. 269
Apollonius of Tyana, image of, in Alexander Severus’ lararium, i. 82
Apophasis of Simon Magus, the, described, i. 179;
quoted, i. 182, 188, 189, 193, 194; ii. 90 n. 5
Apostles, demand only faith from converts, i. lvii;
do not borrow from earlier creeds, i. 88;
their meeting with Simon Magus, i. 176, 177;
in Clementines, i. 178;
intolerance of, due to Jewish origin (Bouché-Leclercq), ii. 10;
souls of, in P.S. drawn from Treasure-house, ii. 137, 147
Apostolical Constitutions, their date, ii. 7 n. 2;
quoted, i. 87 n. 1; ii. 7 nn. 2, 3, 219 n. 2
Appellant and Respondent gods, the, in neo-Manichaeism, ii. 302 n.
1, 324, 343, 354, 355
Apuat, the god, “opener of the ways,” i. 33
Apuleius of Madaura, quoted, i. 56, 59, 60, 61, 62, 63, 64, 65, 66, 67,
68, 71, 73-74, 75, 77, 86 n. 3, 101 n. 2
Aramati, the Vedic goddess, identified with Spenta Armaiti of the
Avesta, ii. 45 n. 1, 300 n. 2
Ararat or Ararad, Mt, Books of Jeû hidden in, ii. 147 n. 5
Arbela, Greek troops on Persian side at, i. 7;
Alexander’s pursuit after, i. 13
Arcadia, Eleusinian triad worshipped in, i. 135
Arcadius, the Emperor, Church dedicated to, in place of Serapeum, i.
84
Archelai Acta. See Hegemonius
Archimedes, his calculation of places of stars sinful (Hippolytus), i.
112 n. 2
Architect of the Universe. See Demiurge
Archon, the Great, of Basilides, the Demiurge, ii. 91;
likeness of, to Ialdabaoth, ii. 94
Archontics, the, a sect related to the Ophites, ii. 77
Arctinus of Miletus, first Greek author to mention purification, i. 121
n. 4
Arctos, the Great Bear, in Mithraism, ii. 266
Arda viraf namak, the, quoted, ii. 264 n. 5
Ardeshîr, the Shah, restorer of Persian nationality, ii. 226, 282;
his son Peroz converted to Manichaeism, ii. 281;
restores worship of Ahura Mazda, ii. 284
Ares, the god, identified with Roman Mars, i. 17;
Homeric or Orphic hymn to, i. 141 n. 2, 142 n. 2;
on Mithraic monuments, ii. 238
Argolis, the, Eleusinian triad worshipped in, i. 135
Ariel, a fiend in Texts of Saviour, ii. 186
Arimaspi, the, fables concerning, i. 2 n. 1
Aristaeus, pro-Jewish writer, i. 173
Aristides. See Aelius Aristides
Aristides, Christian apologist, ii. 203, 204 n. 1
Aristion, Athenian courtezan member of religious confraternity, i. 22
Aristophanes, quoted, i. 17 n. 1, 40 n. 4, 124, 137;
scholiast on, i. 17 n. 1
Aristotle, his monotheism, i. 10;
says that religion follows form of government, i. 12, 15;
that Orpheus did not exist, i. 121 n. 1
Armageddon, covers name of Rome, i. 170 n. 5
Armenia, Ophites in, ii. 76;
kings of, claim descent from Persian heroes, ii. 225 n. 1;
Marcionites and Bardesanites in, ii. 283;
invasion of, by Mihr Nerses, ii. 285
Arnobius, adv. Gentes, quoted, i. 124 n. 3; ii. 39 nn. 2, 4, 264 n. 5
Arrian, Anabasis, quoted, i. 4 n. 1
Arsaces, founder of Parthian kingdom, ii. 224
Arsinoe, wife of Ptolemy Philadelphus, i. 18
Artapanus, On the Jews, quoted, i. 173
Artemis, the goddess, the Ephesian, i. lvi, 40;
birthplace of Greek, i. 16;
Indian worship of, i. 17;
Orthia, i. 100 n. 2;
priestesses of Ephesian, called bees, i. 143 n. 4;
Phrygian, ii. 67 n. 3;
See Diana
Aryans, their dealings with lower races, i. 3, 92
Asar-hapi, Osiris as Apis, i. 49
Asha Vashishta or Truth, the Amshaspand, i. 181 n. 1
Asia, before Alexander, i. 1;
made Greek by Alexander, i. 5;
rush of Greeks to, i. 7;
Greek spoken throughout, i. 8;
cruelty of Assyrian domination in, i. 12;
returns to Persian ways, ii. 225
Asia Minor, native religions of, i. lviii, 37, 126; ii. 29, 36, 49, 67 n. 3;
gods of, coalesce with Greek, i. 17;
home of Dionysos worship, i. 43 n. 3;
Alexandrian gods worshipped in, i. 53;
Vedic gods worshipped in, i. 122 n. 3;
Eleusinian gods worshipped in, i. 136;
Orphics in, i. 141, 156; ii. 236;
priestesses called bees in, i. 143 n. 4;
Jewish atrocities in, temp. Trajan, i. 173 n. 1;
Ophite heresy probably native to, ii. 26, 76;
Jewish settlements in, ii. 28;
Jewish magicians in, temp. Apostles, ii. 33;
matriarchate in, ii. 40;
Babylonian culture in, ii. 48;
serpent worship in, ii. 49, 77, 78;
reverts to Persian ways, ii. 225;
Mithraism in, ii. 229, 232, 268
Askew, Dr, sells Pistis Sophia to British Museum, ii. 134
Asklepios or Aesculapius, the god, Alexander of Abonoteichos priest
of, i. 24;
Serapis statue that of, i. 48 n. 3, 78 n. 2;
identified with Serapis, i. 78, 87
Aso, the Ethiopian queen, enemy of Osiris, i. 33, 37 n. 1
Asoka, his missions to Greek kings, i. 20
Assur-bani-pal, King of Assyria, his library at Kuyunjik, i. 94, 114
Assyria, penitential psalms of, i. 115;
Jews tributary to, i. 160 n. 4
Assyrians, the, tyranny of, i. 3;
suzerains of Hebrews, i. 150;
name used for Syrians in Christian times, ii. 53 n. 4, 54 n. 6;
worship of Mylitta by (Herodotus), ii. 234
Astaphaios or Astaphaeus, ruler of planetary sphere in Diagram, ii.
47;
name derived from magic (Origen), ii. 47, 48;
once called Astanpheus, ii. 47, 69 n.
lord of third gate, ii. 70 n. 2, 73, 74 n. 3;
address to, ii. 73
Astarte, the goddess, worship of, brought into Greece, i. 17;
worshipped by Greek confraternity, i. 25;
Phoenician form of earth goddess, i. 126;
dove, totem-animal of, ii. 135 n. 3;
Mater Viventium, ibid.
Astrampsuchos, name of Roman writer on magic, i. 107;
name of celestial guard in Bruce Papyrus, i. 107 n. 1;
power worshipped by the Peratae, ibid.
Astrology, origin of, in Chaldaea, i. 113;
fundamental idea of, i. 114;
system of correspondences results from, i. 115, 116;
impulse given to, by Greek mathematics, i. 116, 117;
all religions in Graeco-Roman world take note of, i. 117, 118;
gives new life to Gnosticism, i. 119;
Ophites mix astrological ideas with Orphic teaching, ii. 78;
first prominent in Gnosticism in Excerpta Theodoti, ii. 158 n. 1;
its great vogue in Rome under Severi, ibid.;
reprobated in Pistis Sophia, ii. 185;
part of scheme of punishments and salvation in Texts of Saviour,
ii. 185 n. 2;
its importance in Mithraism, ii. 235, 276.
See Babylonia
Atargatis or Dea Syria, favourite deity of Nero, ii. 31;
her Anatolian name and identification with other goddesses, ii. 31
n. 1;
homonym of Derketo (Garstang), ii. 40 n. 1;
her identity with the Mother of the Gods, ii. 299 n. 1;
Manichaean Mother of Life derived from, ii. 300 n. 2
Athamas the Pythagorean, his doctrine of “roots,” i. 197
Athanasius, Saint, creed of, i. 89
Athena, the goddess, identified with Minerva, i. 17;
her part in Eleusinian Mysteries, i. 39;
the Homeric, i. 57, 95, 124 n. 3;
statue of Helena of Tyre as, i. 198;
See Minerva, Pallas
Athenagoras, quoted, i. lvii n. 1, 63 n. 5, 64 n. 3; ii. 18 n. 2
Athens, foreign worships in, i. 16, 17 n. 1, 137;
accepts deification of Alexander, i. 18;
gathering in, for Eleusinian Mysteries, i. 38-41;
Alexandrian religion in, i. 52, 76;
Orphic myths brought into, by Epimenides, i, 121;
Orphic gold plates in Museum at, i. 132
Athos Mt, Philosophumena discovered at, ii. 11
Atlas. See Corybas, Omophorus
Attis or Atys, the god, his worship brought into Greece, i. 17, 136;
his legend, i. 37; ii. 39;
identified with Sun, i. 118;
and with Dionysos, Adonis and Osiris, i. 137 n. 1, 145; ii. 17;
and with Sabazius, i. 138, 139;
androgyne, i. 185;
Gnostics attend mysteries of, ii. 21;
Phrygia, home of worship of, ii. 28, 67 n. 3;
to Ophites, type of world-soul, ii. 65 n. 3
Augustine of Hippo, Saint, convert from Manichaeism, i. 112 n. 1;
well informed about Manichaeans, ii. 352;
quoted, i. 103 n. 4; ii. 10 n. 1, 12 n. 4, 25, 261, 298 n. 1, 317, 319,
331, 332, 343, 346, 349 n. 4, 350
Augustus, the Emperor, Samaria’s capital named Sebaste in honour
of, i. 177;
Galatians become Roman temp., ii. 28;
Parthians’ terror of (Horace), ii. 225
Aurelian, the Emperor, his worship of sun-god, i. 119 n. 1; ii. 228;
position of Christianity under, ii. 23;
restores Roman arms in the East, ii. 226;
gives up Dacia to Goths, ii. 271
Authades, the Proud God of the Pistis Sophia, last member of Triad
of the Left, ii. 151;
his disobedience, ii. 152;
his envy of Pistis Sophia, ii. 155;
sends demon in shape of flying arrow, ii. 156;
his place given to Pistis Sophia, ii. 162
Autogenes, power mentioned in Bruce Papyrus, ii. 192
Autophyes or Self-produced, member of Valentinian Decad, ii. 101
Avebury, Lord, quoted, i. 91, 99 n. 1
Avesta, the Zend, Seven Amshaspands of, i. 117;
emanation doctrine in, ii. 35;
First Man in, ii. 38 n. 3;
Supreme Being in, ii. 231;
Ormuzd and Ahriman in, ii. 236;
bull Goshurun in, ii. 243;
denounces magic, ii. 275 n. 2;
doubtful about eternity of evil, ii. 289;
quoted, ii. 310, 311.

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ISBN: 9780367753184 (hbk)

List of Abbreviations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vii

Editor Biography. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xiv

Section I: Hearing Loss: Approach to the Patient and Management. . . . . . . . . . . . . . . . . . 1

1. Hearing Loss in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

2. Hearing Loss in Adults . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27

3. Sudden Sensorineural Hearing Loss. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49

4. Single-Sided Deafness: A Long-Neglected Entity in Otology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58

Section II: The Discharging Ear: Differential Diagnosis and Management. . . . . . . . . . . 75

5. The Discharging Ear: Differential Diagnosis and Management. . . . . . . . . . . . . . . . . . . . . . . . . . . 76

Section III: Approach to a Patient with Otalgia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97

6. Approach to a Patient with Otalgia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98

7. Vertigo in Children and Adults . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 110

Section V: Tinnitus Symptomatology: Diagnosis and Management. . . . . . . . . . . . . . . . . 137

9. Tinnitus Symptomatology: Diagnosis and Management. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 138

Section VI: Facial Palsy in Otology: Approach to a Patient. . . . . . . . . . . . . . . . . . . . . . . . . . 153

10. Facial Palsy in Otology: Approach to a Patient . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 154

Section VII: Neoplasms in Otology: Guide to Diagnosis and Management. . . . . . . . . . 175

11. Malignant Neoplasms in Otology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176

12. Benign Neoplasms in Otology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 197

Section VIII: Otology at the Frontiers. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 213

13. Endoscopes in Otology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 214

14. Anaesthesia Techniques in Otology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 221

15. Imaging in Otology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 230

16. Tools and Technology in Otology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 245

Section IX: Record Keeping in Otology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 253

17. Record Keeping in Otology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 254

5 FU 5-Fluorouracil CISS Constructive interference in steady

GVE General visceral efferent MML Minimum masking level

SP Summating potential TORP Total ossicular replacement

Dr Manikandan A, MS (ENT) Dr Abha Kumari, MS

Dr Dhanalakshmi B, MD (Radiodiagnosis) Dr Sabarigirish K, MS

Dr Gunjan Dwivedi, MS Dr Smriti Panda, MS

Dr Mary John, MS, DNB, PhD Dr Roohie Singh, MS

Dr Rohit Singh, MS Dr Garima Upreti, MS

Dr Amit Sood, MS Dr Hitesh Verma, MS

HEARING LOSS: APPROACH

1 Hearing Loss in Children

INTRODUCTION children with HL. The advancement in surgical

Figure 1.2 Flowchart for evaluation of children with hearing loss.

Figure 1.3 Tests of Hearing.

Table 1.2: Examples of Speech Perception Tests Used in Assessment of Children

Table 1.3: Commonly Used

Table 1.4: Classification of Inner Ear Malformations (IEM) and Their

Table 1.5: Aetiology and Classification of Hearing Loss in Children

1 Hearing Loss in Children

Table 1.2: Examples of Speech Perception Tests Used in Assessment of Children

Table 1.3: Commonly Used

Table 1.4: Classification of Inner Ear Malformations (IEM) and Their

Table 1.5: Aetiology and Classification of Hearing Loss in Children

Table 1.7: Summary of the Middle Ear Implants Used in Children

Table 1.8: Common Grading and Description of Microtia and Congenital

Table 1.10: Materials Used for

Table 1.11: Various Classifications of Ossicular Chain Defects and

Table 1.12: Classification of Retraction Pockets in Pars Tensa and