Pediatric Ear Diseases

Pediatric Ear Diseases

Yasushi Naito

Pediatric Ear Diseases

Diagnostic Imaging Atlas and Case Reports

242 figures, 7 in color and 5 tables, 2013

_____________________

Dr. Yasushi Naito

Kobe City Medical Center General Hospital

Kobe City Hospital Organization

Kobe, Japan

Library of Congress Cataloging-in-Publication Data

Naito, Yasushi.

Pediatric ear diseases : diagnostic imaging atlas and case reports / Yasushi Naito. p. ; cm.

Includes bibliographical references and index.

ISBN 978-3-318-02232-2 (hardcover : alk. paper) -- ISBN 978-3-318-02233-9 (e-ISBN)

I. Title.

[DNLM: 1. Ear Diseases--diagnosis--Atlases. 2. Ear Diseases--diagnosis--Case Reports. 3. Adolescent. 4. Child. 5. Ear, Inner--abnormalities--Atlases. 6. Ear, Inner--abnormalities--Case Reports. WV 17]

RF291.5.C45

618.92’0978--dc23

2013002892

Bibliographic Indices. This publication is listed in bibliographic services, including Current Contents® and PubMed/MEDLINE.

Disclaimer. The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publisher and the editor(s). The appearance of advertisements in the book is not a warranty, endorsement, or approval of the products or services advertised or of their eff ectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

Drug Dosage. The authors and the publisher have exerted every eff ort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant fl ow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any change in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.

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© Copyright 2013 by S. Karger AG, P.O. Box, CH-4009 Basel (Switzerland)

www.karger.com

Printed in Germany on acid-free and non-aging paper (ISO 97069) by Kraft Druck, Ettlingen

ISBN 978-3-318-02232-2

eISBN 978-3-318-02233-9

Contents

Preface

Note Concerning Images Used in This Book

Chapter Normal CT Images of the Temporal Bone

Infant

Older Child

Chapter Postnatal Growth of the Temporal Bone

External Auditory Canal

Mastoid Air Cells

Internal Auditory Canal

Vestibular Aqueduct

Chapter Congenital Anomalies

External Auditory Canal

EAC Atresia and Stenosis

Case 1 : Congenital EAC Atresia

Case 2 : Congenital EAC Stenosis with Cholesteatoma

Auditory Ossicles and Middle Ear

Congenital Ossicular Malformation

Stapes Surgery in Children

CT Diagnosis of Ossicular Malformation

Case 1 : Ossicular Disruption with Stapes Fixation

Case 2 : Ossicular Deformities with Stapes Fixation

Case 3 : Ossicular Deformities

Case 4 : Oval Window Atresia

Case 5 : Skull Base Vascular Anomalies (CHARGE Syndrome)

Case 6 : Facial Nerve Anomaly

Inner Ear

Congenital Malformation of the Inner Ear

Genesis of the Inner Ear

Histopathological Classification of Inner Ear Malformation

Classification Based on Clinical Imaging

Role of CT and MRI in Diagnosis of Inner Ear Anomalies

Case 1 : Michel Aplasia (Inner Ear Aplasia)

Case 2 : Cochlear Aplasia

Case 3 : Common Cavity Deformity (1)

Case 4 : Common Cavity Deformity (2)

Case 5 : Aplasia of Cochlear Upper Turns and Semicircular Canals with Cochlear Nerve Canal Stenosis

Case 6 : Incomplete Partition Type I (IP-I): Case of Cochlear Implantation Revision

Case 7 : Incomplete Partition Type II (IP-II): Mondini Dysplasia (Enlarged Vestibular Aqueduct)

Case 8 : Incomplete Partition Type III (IP-III)

Special Article: Incomplete Partition Type III. Levent Sennaroglu, M.D.

Internal Auditory Canal

IAC Stenosis

Case 1 : IAC Stenosis

Case 2 : Stenosis of Cochlear Nerve Canal

Case 3 : IAC Malformation, Arachnoid Cyst of Fallopian Canal

Chapter Inflammatory Diseases of the Middle Ear

Otitis Media and Cholesteatoma

Eustachian Tube Function and Mastoid Air Cell Development

Case 1 : Congenital Cholesteatoma

Case 2 : Recurrent Otitis Media, Otitis Media with Effusion

Case 3 : Adhesive Otitis Media

Case 4 : Acute Otitis Media, Sigmoid Sinus Thrombosis

Case 5 : Cholesteatoma: Pars Flaccida Cholesteatoma (1)

Case 6 : Cholesteatoma: Pars Flaccida Cholesteatoma (2)

Case 7 : Cholesterol Granuloma

Image Findings after Tympanoplasty

Classification of Tympanoplasty

Ossiculoplasty

Evaluation of Postoperative Results

Case 1 : Cholesteatoma, 1 : Good Aeration after Primary Operation

Case 2 : Cholesteatoma, 2 : No Aeration after Primary Operation

Case 3 : Cholesteatoma, 3 : Type III Incus Interposition Ossiculoplasty

Case 4 : Cholesteatoma, 4 : Type III Ossiculoplasty with Long Columella

Case 5 : Cholesteatoma, 5 : Recurrence

Chapter Other Ear Disorders

Case 1 : Pericochlear Hypoattenuating Foci and Stapes Fixation

Case 2 : Traumatic Ossicular Disruption

Case 3 : Cochlear Implant Magnet Trouble after Head Trauma

Case 4 : Cochlear Implantation in an Ear with Extensive Cholesteatoma

Case 5 : Meningitic Labyrinthitis

Index

Author & Acknowledgments

Preface

This book consists of two sections: a pediatric temporal bone imaging atlas, followed by case reports on a variety of typical pediatric ear diseases. As an atlas, this book shows complete contiguous temporal bone CT sections of an infant and of an older child, listing detailed anatomic names of the structures, including very fine ones, that appear in each image. In addition, developmental changes in the size, shape, location and orientation of the primary components of the temporal bone are also shown to demonstrate how the temporal bone grows with age. This book will be of great help to those who are interested in pediatric ear diseases, since accurate assessment of the disorders is very difficult without this sort of atlas, which has not been published so far.

The section following the atlas contains a collection of case reports. In this section, case images are shown alongside normal reference images of a child in the same age range as the patient, allowing readers to identify the key findings for diagnosing the disorder without needing to refer to an atlas of normal images. Images taken before and after treatment are also displayed side by side, to clearly illustrate the point of the post-treatment follow-up. Such layout is unique to this book, and is very effective for learning image diagnosis. To obtain a complete perspective of a disease, it is necessary to know not only the steps leading up to its diagnosis but also the treatment and the results following it. This is why I made the latter half of this book a collection of case reports, not simply a display of the diseases’ key images.

I hope that this book will be of use to those who are involved in the medical care of children suffering from ear diseases.

Yasushi Naito

Kobe, Japan, 2013

Note Concerning Images Used in This Book

Most of the images shown in this book are temporal bone CTs, but in some cases MRIs are also employed to observe structures such as the inner ear, internal auditory canal, and posterior cranial fossa. The temporal bone imaging parameters described below pertain to the majority of the images contained herein. Although different parameters are employed in a portion of the CT and MR images, a detailed explanation of each would be of little clinical significance. As most readers who are not radiologists are likely unfamiliar with the values described below, we recommend that, when asked for direction regarding temporal bone CT or MRI examination procedures by radiologists either at your own facility or at an outsourced imaging lab, you photocopy this page and present it as an example. However, regarding the voxel size values shown below, please be aware that these are the sizes of the minimum units comprising the image and structures smaller than this cannot be isolated and depicted, so represent the maximum resolution of the images shown herein.

As a general rule, the images shown are rectangular with an aspect ratio of 3:4. The axial cross-sections display the area indicated inside the box in figures 1 (CT) and 2 (MRI) below, centered on the inner ear and tympanic cavity. The coronal cross-sections generally display the area from the inferior margin of the mastoid process to the superior margin of the anterior semicircular canal.

Temporal Bone Target CT Imaging Parameters

Fig. 1. Temporal bone CT image

Principal equipment used: GE BrightSpeed (16 MD CT), 120 kV, helical pitch of 0.562, Bone reconstruction algorithm. Axial cross-sections: bilateral simultaneous imaging, FOV: 150 mm, matrix size: 512 × 512, slice thickness: 0.625 mm, no gap (voxel size: 0.29 × 0.29 × 0.63 mm). Coronal cross-sections: unilateral imaging, FOV: 96 mm, matrix size: 512 × 512, slice thickness: 0.625 mm, no gap. Display window width is 3800, window level is 30.

A number of problems arise when attempting to display in print form clinical images normally viewed either as backlit transparencies or on a computer display. It is difficult in actual printed images to fully satisfy the conflicting objectives of losing as little information included in the image as possible while preventing the display of data that should not have been shown in the original image. We have made an effort to fulfill both objectives as much as possible but, in some images, areas that were originally air are sometimes depicted as slightly shaded, or structures such as tympanic membranes or tendons that should be delicately expressed with intermediate gradations become difficult to distinguish. We hope that you will take the above difficulties into consideration when viewing the images presented in this book.

Temporal Bone MR Imaging Parameters

Fig. 2. Temporal bone MRI

Equipment used: Siemens Avanto 1.5T MRI system, SPACE (Sampling Perfection with Application optimized Contrasts using different flip angle Evolution) pulse sequence, Turbo Spin Echo, 3D T2-weighted images. Imaging parameters: FOV: 170 mm, slice thickness: 0.7 mm, matrix size: 256 × 256 (voxel size: 0.66 × 0.66 × 0.7 mm), TR: 1300 ms, TE: 253 ms, flip angle: 160 deg (variable), number of excitations: 2. GRAPPA used for parallel imaging.

Pediatric Ear Diseases

Diagnostic Imaging Atlas and Case Reports

Chapter n1

Normal CT Images of the Temporal Bone

The foundation for temporal bone imaging diagnosis lies in obtaining a thorough understanding of the ear’s normal anatomical structures and their three-dimensional relationship. Through repeated comparison and identification of the details of normal structures and the anatomical terms that describe them, one gradually forms a mental image of the temporal bone’s overall three-dimensional orientation. When one concurrently views clinical case images, one’s eyes are drawn naturally to those forms that differ from the norm, which can then be compared to various known disease findings to arrive at an accurate diagnosis. This process also applies to pediatric cases but, with the exception of minor ailments such as otitis media, encounters with infant diseases in everyday clinical practice are infrequent, and examples of imaging diagnosis even rarer. Consequently, pediatric images are usually interpreted with normal adult anatomy in mind. However, the temporal bone of infants in particular differs from that of an adult’s with respect to the sizes and relative ratios of each component, so caution is required in reading and interpreting findings.

This chapter displays a complete series of serial cross-section and descriptive images, without omission, of temporal bone CT axial sections and coronal sections from both infants and older children. By examining the images from infants and older children, first separately and then in comparison, we will be able to develop a mental image of the anatomy of the temporal bone and its postnatal development.

p1 Infant

p1 Older Child

p1 Infant

Figure 1 shows the basic anatomy of the ear. All the anatomical components shown in this figure exist from birth; however their sizes and locations change with age. The inner ear and ossicles in an infant’s temporal bone are the same size as an adult, but the external auditory canal, internal auditory canal, and mastoid air cells are still small and grow with age. Roughly speaking, the cochlea, vestibule, semicircular canals, and tympanic cavity are at the center and change little, while the periphery expands anteroposteriorly, laterally, and vertically. Horizontal expansion, both laterally and front to back, can be observed through axial sections and vertical expansion through coronal sections. Of the various structures of the temporal bone, normal development of the mastoid air cells is suppressed by otitis media. Consequently when viewing pediatric temporal bone images, along with age, one must also take previous middle ear diseases into consideration.

In order to avoid surgical complications during ear surgery, it is necessary to have an accurate grasp of the positions of major anatomical structures within the temporal bone. However, an infant’s temporal bone is smaller and more delicate than an adult’s and its anatomical orientation during surgery is different. When performing temporal bone surgery under a microscope, even an error of 1 mm may result in injury to the facial nerve, the semicircular canals, or the stapes. Common preoperative checkpoints for most otological surgical procedures include: 1) degree of mastoid air cell development and the height of the base of the middle cranial fossa lateral to the epitympanum (attic); 2) lateromedial width and air cell development of the facial recess region; 3) distance between the sigmoid sinus and the posterior wall of the external auditory canal; 4) pneumatization (=air cell development) in the direction of the mastoid process; and 5) thickness of the cranial wall in the temporoparietal region. If any of these are narrower or smaller than normal, one must plan ahead to determine how to overcome the difficulties presented to secure sufficient surgical field visibility and achieve one’s objective.

The images shown are of a 4-month-old female infant who underwent CT examination for bilateral hearing loss. They are presented here as normal temporal bone CT images as no clear abnormal findings were discovered in them. The coronal section images were reconstructed from data taken in the axial section images, with no direct images taken from a supine hanging-head position. For display purposes, coronal section images have been magnified to approx. 1.7 times the axial section images. Also, the images are arranged from bottom to top (inferior to superior) for the axial sections and from front to back (anterior to posterior) for the coronal sections. Images of the same cross sections are arranged side by side, with the right image annotated to indicate each anatomical structure. (Scale shown in images indicates 1 cm)

The base line for the CT images was set based on the plane that includes bilateral OM lines, the line that passes through the outer canthus of the eye and the center of the external auditory canal.

Fig. 1. The anatomy of the ear. 2

Female, 4 months old: left axial section

1 OM line -2.91 mm

2 OM line -2.31 mm

3 OM line -1.71 mm

4 OM line -1.12 mm

5 OM line -0.52 mm

6 OM line +0.08 mm

7 OM line +0.68 mm

8 OM line +1.28 mm

9 OM line +1.88 mm

10 OM line +2.48 mm

11 OM line +3.08 mm

12 OM line +3.68 mm

13 OM line +4.28 mm

14 OM line +4.88 mm

b=basal turn of cochlea; p=promontory; mm=manubrium of malleus; su=subiculum of promontory; fr=facial recess

15 OM line +5.48 mm

b=basal turn of cochlea; p=promontory;mm=manubrium of malleus; le=lenticular process of incus;h=head of stapes; su=subiculum of promontory;ts=tympanic sinus; fr=facial recess

16 OM line +6.08 mm

b=basal turn of cochlea;o=osseous spiral lamina;p=promontory;n=neck of malleus;le=lenticular process of incus; a1 =incudostapedial joint;h=head of stapes;ts=tympanic sinus;fr=facial recess

17 OM line +6.68 mm

18 OM line +7.28 mm

a=apical turn of cochlea; m=middle turn of cochlea; b=basal turn of cochlea; v=vestibule; s=singlar canal; h=head of malleus; ib=body of incus; il=long process of incus; is=short process of incus; ac=anterior crus of stapes; pc=posterior crus of stapes; ts=tympanic sinus

19 OM line +7.88 mm

a=apical turn of cochlea; m=middle turn of cochlea; b=basal turn of cochlea; v=vestibule; s=singlar canal; h=head of malleus; asr =malleoincudal joint; ib=body of incus; il=long process of incus; is=short process of incus; ac=anterior crus of stapes; pc=posterior crus of stapes; ts=tympanic sinus

20 OM line +8.48 mm

a=apical turn of cochlea; m=middle turn of cochlea; b=basal turn of cochlea; *=modiolus; f=footplate of stapes; v=vestibule; s=singlar canal; h=head of malleus; er =malleoincudal joint; ib=body of incus

21 OM line +9.08 mm

a=apical turn of cochlea; m=middle turn of cochlea; b=basal turn of cochlea; *=modiolus; v=vestibule; sr=spherical recess; c=anterior attic bony plate (cog); h=head of malleus; er =malleoincudal joint; ib=body of incus

22 OM line +9.68 mm

b=basal turn of cochlea; *=modiolus; v=vestibule; c=anterior attic bony plate (cog); h=head of malleus; er =malleoincudal joint; ib=body of incus; ad=aditus ad antrum

23 OM line +10.28 mm

24 OM line +10.88 mm

b=basal turn of cochlea; er =Bill’s bar; sv=superior vestibular nerve; v=vestibule; ad=aditus ad antrum

25 OM line +11.48 mm

26 OM line +12.08 mm

27 OM line +12.68 mm

28 OM line +13.28 mm

29 OM line +13.88 mm

30 OM line +14.48 mm

31 OM line +15.07 mm

32 OM line +15.67 mm

33 OM line +16.27 mm

34 OM line +16.87 mm

35 OM line +17.47 mm

36 OM line +18.07 mm

Female, 4 months old: left coronal section

1 +4.00 mm from center of external auditory canal

2 +3.35 mm from center of external auditory canal

3 +2.70 mm from center of external auditory canal

4 +2.04 mm from center of external auditory canal

5 +1.39 mm from center of external auditory canal

b=basal turn of cochlea; m=middle turn of cochlea; a=apical turn of cochlea; h=head of malleus