Chapter 23 Otoneurologic Manifestations of Otologic and Systemic

Chapter 23 Otoneurologic Manifestations of Otologic and Systemic Disease Joseph M. Furman and Andrew A. Mccall Copyright © 2014 Elsevier Inc. All rights reserved. 1

FIGURE 23 -1 Computed tomography (CT) scan showing right petrous apicitis. Upper scan: Soft tissue windowing demonstrating enhancement at the tip of the petrous apex affecting Dorello canal (arrow head). Lower scan: Bone windowing demonstrating a lytic cell in the petrous apex with loss of septation (arrow). (Courtesy of Hugh D. Curtin, MD, Massachusetts Eye and Ear Infirmary, Boston. ) Copyright © 2014 Elsevier Inc. All rights reserved. 2

Figure 23 -2 Sigmoid sinus thrombosis. CT venography performed after retromastoid craniectomy reveals a filling defect in the right transverse (dashed arrow) and sigmoid (solid arrow) sinuses, representing subocclusive thrombus. (Courtesy of Barton F. Branstetter, MD, University of Pittsburgh. ) Copyright © 2014 Elsevier Inc. All rights reserved. 3

Figure 23 -3 Dix–Hallpike Maneuver. The Dix–Hallpike test of a patient with benign paroxysmal positional vertigo affecting the right ear. A, The examiner stands at the patient’s right side and rotates the patient’s head 45 degrees to the right to align the right posterior semicircular canal with the sagittal plane of the body. B, The examiner moves the patient, whose eyes are open, from the seated to the supine right-ear-down position and then extends the patient’s neck so that the chin is pointed slightly upward. The latency, duration, and direction of nystagmus, if present, and the latency and duration of vertigo, if present, should be noted. The arrows in the inset depict the direction of nystagmus in patients with typical benign paroxysmal positional vertigo. The presumed location in the labyrinth of the free-floating debris thought to cause the disorders is also shown. (From Furman JM, Cass SPC: Benign paroxysmal positional vertigo. N Engl J Med 341: 1590, 1999, with permission. ) Copyright © 2014 Elsevier Inc. All rights reserved. 4

Figure 23 -4 Particle repositioning maneuver for the bedside treatment of a patient with benign paroxysmal positional vertigo affecting the right ear. The presumed position of the debris within the labyrinth during the maneuver is shown in each panel. The maneuver is a three-step procedure. First, a Dix–Hallpike test is performed with the patient’s head rotated 45 degrees toward the right ear and the neck slightly extended with the chin pointed slightly upward. This position results in the patient’s head hanging to the right (A). Once the vertigo and nystagmus provoked by the Dix–Hallpike test cease, the patient’s head is rotated about the rostral-caudal body axis until the left ear is down (B). Then the head and body are further rotated until the head is face down (C). The vertex of the head is kept tilted downward throughout the rotation. The maneuver usually provokes brief vertigo. The patient should be kept in the final, face-down position for about 10 to 15 seconds. With the head kept turned toward the left shoulder, the patient is brought into the seated position (D). Once the patient is upright, the head is tilted so that the chin is pointed slightly downward. (From Furman JM, Cass SPC: Benign paroxysmal positional vertigo. N Engl J Med 341: 1590, 1999, with permission. ) Copyright © 2014 Elsevier Inc. All rights reserved. 5

Figure 23 -5 Magnetic resonance imaging (MRI) of a large acoustic neuroma with brainstem compression. Axial contrast-enhanced T 1 -weighted MRI reveals a large, lobulated, enhancing mass (arrows) filling the left cerebellopontine angle, with compression of the pons and thinning of the brachium pontis. The patient was known to have neurofibromatosis type 2, and this lesion is most likely a schwannoma of the eighth cranial nerve. (Courtesy of Barton F. Branstetter, MD, University of Pittsburgh. ) Copyright © 2014 Elsevier Inc. All rights reserved. 6

Figure 23 -6 Coronal CT scan through the level of the temporal bone demonstrating superior canal dehiscence. Arrow points to the left superior semicircular canal dehiscence. The membranous superior semicircular canal (seen in cross section) is in contact with the overlying contents of the middle cranial fossa. Compare this with the right side, where a very thin plate of bone is separating the superior semicircular canal from the middle cranial fossa. (Courtesy of Andrew A. Mc. Call, MD, University of Pittsburgh. ) Copyright © 2014 Elsevier Inc. All rights reserved. 7

Figure 23 -7 Chiari malformation. Sagittal T 2 -weighted MRI through the cranio-cervical junction shows the cerebellar tonsils (solid arrow) extending far below the foramen magnum (dashed line). The medulla is compressed. (Courtesy of Barton F. Branstetter, MD, University of Pittsburgh. ) Copyright © 2014 Elsevier Inc. All rights reserved. 8

Figure 23 -8 Herpes simplex virus (HSV) in a mouse model of Bell palsy. A, The intratemporal portion of the facial nerve from the paralyzed side showing a neuron in the geniculate ganglion. Replicating HSV-1 viruses in the rough endoplasmic reticulum of a neuron in the geniculate ganglion are indicated by the arrows. B, The geniculate portion of the facial motor nerve. Arrows indicate vacuolar changes in the cytoplasm of a Schwann cell which resulted in demyelination. Scale bars: A, 1 µm; B, 5 µm. (From Takahashi H, Hitsumoto Y, Honda N, et al: Mouse model of Bell’s palsy induced by reactivation of herpes simplex virus type 1. J Neuropathol Exp Neurol 60: 621, 2001, with permission. ) Copyright © 2014 Elsevier Inc. All rights reserved. 9

Figure 23 -9 Chronic otitis media in Wegener granulomatosis. Granulomatous swelling of the posterior part of the left eardrum viewed through an otomicroscope (arrow). The large white area inferior and to the left of the eardrum is the anterior wall of the external ear canal. (From Rasmussen N: Management of the ear, nose, and throat manifestations of Wegener granulomatosis: an otorhinolaryngologist’s perspective. Curr Opin Rheumatol 13: 3, 2001, with permission. ) Copyright © 2014 Elsevier Inc. All rights reserved. 10

Figure 23 -10 Osteogenesis imperfecta of the temporal bone. Histologic section of the right temporal bone from a patient with osteogenesis imperfecta, showing an otosclerotic lesion anterior to the oval window. (From Santos F, Mc. Call A, Chein W, et al: Otopathology in osteogenesis imperfecta. Otol Neurotol 33: 1562, 2012, with permission. ) Copyright © 2014 Elsevier Inc. All rights reserved. 11

Figure 23 -11 Paget disease of the temporal bone. Histologic section through the left temporal bone showing pagetic bone surrounding the internal auditory canal. (From Bahmad F Jr, Merchant SN: Paget disease of the temporal bone. Otol Neurotol 28: 1157, 2007, with permission. ) Copyright © 2014 Elsevier Inc. All rights reserved. 12

Figure 23 -12 Histology of fibrous dysplasia. Note the irregular shapes of woven bone. (From Speight PM, Carlos R: Maxillofacial fibro-osseous lesions. Curr Diagn Pathol 12: 1, 2006, with permission. ) Copyright © 2014 Elsevier Inc. All rights reserved. 13

Figure 23 -13 Hair cell loss from gentamicin ototoxicity. A and B demonstrate a normal complement of inner (IHC) and outer hair cells (OHC) of the cochlea. C shows severe loss of outer hair cells and many inner hair cells after exposure to gentamicin (mouse cochlea). (From Matt T, Ng CL, Lang K, et al: Dissociation of antibacterial activity and aminoglycoside ototoxicity in the 4 monosubstituted 2 -deoxystreptamine apramycin. Proc Natl Acad Sci USA 109: 10988, 2012, with permission. ) Copyright © 2014 Elsevier Inc. All rights reserved. 14

Figure 23 -14 MRI of endolymphatic sac tumor. Axial contrast-enhanced T 1 -weighted MRI demonstrates an irregular, enhancing mass along the posterior border of the petrous apex, at the expected location of the endolymphatic sac (dashed arrow). The mass erodes into the underlying temporal bone (solid arrow). (Courtesy of Barton F. Branstetter, MD, University of Pittsburgh. ) Copyright © 2014 Elsevier Inc. All rights reserved. 15

Figure 23 -15 MRI of a patient with microvascular disease seen in disequilibrium of aging. Axial fluid-attenuated inversion recovery (FLAIR) sequence through the bodies of the lateral ventricles shows extensive, confluent areas of abnormal signal in a periventricular and subcortical distribution, consistent with white matter microvascular disease. The ventricles are slightly enlarged from associated central volume loss. (Courtesy of Barton F. Branstetter, MD, University of Pittsburgh. ) Copyright © 2014 Elsevier Inc. All rights reserved. 16

Figure 23 -16 Audiogram of presbycusis. This audiogram shows sensorineural hearing loss in a downsloping pattern, typical of presbycusis. Copyright © 2014 Elsevier Inc. All rights reserved. 17