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P092: Otologic Manifestations of Susac’s Syndrome
Scientific Posters—Wednesday
P092 Otologic Manifestations of Susac’s Syndrome Michelle M Roeser, MD (presenter); Jan L Kasperbauer, MD; Colin L W Driscoll, MD; Michael Brian Gluth, MD OBJECTIVES: Retinocochleocerebral vasculopathy is an unusual clinical syndrome of unknown etiology described in 1979 by Susac, Hardman, and Selhort. Patients manifest sensorineural hearing loss, vestibulocochlear dysfunction, visual defects, and multifocal encephalopathic symptoms due to a microangiopathy of the retina, brain, and ear. This report reviews the otologic manifestations of retinocochleocerebral vasculopathy (Susac Syndrome) and examines the world literature on approximately 60 cases previously reported. METHODS: Retrospective case review of 22 patients with the diagnosis of Susac Syndrome seen at a large tertiary care center from 1979 until present. Demographics, clinical course, and results of audiometric testing were gathered and analyzed. A review of the world’s literature was conducted using MEDLINE searches for “Susac Syndrome” and “retinocochleocerebral vasculopathy,” and attention was paid to the references of each article retrieved. RESULTS: Of 22 patients with Susac Syndrome, 18 (82%) were female. Mean age was 37 years old, ranging from 20 to 69 years old. Ten patients (45.5%) reported a fluctuating hearing loss, 13 (59%) reported tinnitus, 12 (54.6%) noted vertigo. Ten patients (45.5%) presented with bilateral symptoms and 12 (54.6%) were unilateral. In the 32 affected ears, the pure tone average was 37 dB, and the mean percent hearing loss was 21.8%. Fifty percent of affected ears had AAO-HNS hearing classification type A. Only 28% of affected ears had 100% word recognition. CONCLUSIONS: Retinocochleocerebral vasculopathy, Susac Syndrome, is a rare and potentially devastating disease. Knowledge of this illness by otolaryngologists should prompt earlier diagnosis and treatment.
P093 Measurement of Angle and Length of Eustachian Tube Using CT Kenji Takasaki, MD (presenter); Haruo Takahashi, MD; Kaori Enatsu, MD; Hidetaka Kumagami, MD OBJECTIVES: Obtain clues on the anatomical features of the eustachian tube (ET) related to the susceptibility to otitis media with effusion (OME) in children. METHODS: The angle and length of the ET in children with OME (54 ears) and without OME (50 ears, normal children),
as well as those of normal adults were measured on the CT using the multiplanar reconstruction technique. RESULTS: The angles of ET in the OME children’s group, normal children’s group, and normal adult group were 20.4⫾3.5 and 21.2⫾4.8 , 19.9⫾3.4 and 20.0⫾3.6 , and 27.3⫾2.7 and 27.3⫾2.8 in the right and left sides, respectively. The lengths of each group were 37.2⫾3.0 mm and 37.6⫾3.2 mm, 37.5⫾3.3 mm and 38.0⫾3.2 mm, and 42.5⫾2.8 mm and 42.9⫾2.9 mm in the right and the left side, respectively. There was no significant difference between the right and left sides in any groups. Both the angles and lengths were significantly greater in the normal adult group than in either the OME children group or normal children group (p⬍0.05), but there was no significant difference either in the angle or length of the ET between the OME group and normal children’s group (p⬎0.05). CONCLUSIONS: The angle and length of ET are more horizontal and shorter in infants, but there is no statistical difference between infants with and without OME. These results suggest that short and horizontal ET may be one of the predisposing factors related to the high susceptibility of OME in infants and children rather than etiological factors.
P094 Ruptured Carotid Artery Pseudoaneurysm in the Middle Ear Ryan G Porter, Sr, MD (presenter); John P Leonetti, MD; Sam J Marzo, MD; Lotfi Hacein-Bey, MD OBJECTIVES: 1. Describe management of a carotid artery pseudoaneurysm rupture during middle ear surgery. 2. Review the literature with respect to the diagnosis and management of petrous internal carotid artery pseudoaneurysm hemorrhage. METHODS: The following is a case report describing management of a two-centimeter pseudoaneurysm of the petrous portion of the internal carotid artery. This anomaly was discovered in a 47-year-old female undergoing a tympanoplasty and revision mastoidectomy in January 2007 at a tertiary care academic medical center. A review of the literature relating to petrous carotid artery pseudoaneurysms is included. RESULTS: Iatrogenic carotid artery pseudoaneurysms may result from traumatic instrumentation of an aberrant carotid artery during middle ear surgery. Pre-existing pseudoaneurysms are also an important phenomenon to consider when preparing a patient for any otologic procedure. Although injury to a pre-existing pseudoaneurysm is a potentially life-threatening event, the situation can be successfully managed using a multidisciplinary approach involving contemporary interventional radiology techniques coupled with obliteration of the vascular lesion through a subtotal petrosectomy with closure of the external auditory canal. CONCLUSIONS: The patient was taken to the interventional neuroradiology suite from the operating room and underwent
SCIENTIFIC POSTERS
gested that this new surgical technique prevented further deterioration of hearing loss and became a pivotal treatment of PLF.
P243
P092 Otologic Manifestations of Susac’s Syndrome Michelle M Roeser, MD (presenter); Jan L Kasperbauer, MD; Colin L W Driscoll, MD; Michael Brian Gluth, MD OBJECTIVES: Retinocochleocerebral vasculopathy is an unusual clinical syndrome of unknown etiology described in 1979 by Susac, Hardman, and Selhort. Patients manifest sensorineural hearing loss, vestibulocochlear dysfunction, visual defects, and multifocal encephalopathic symptoms due to a microangiopathy of the retina, brain, and ear. This report reviews the otologic manifestations of retinocochleocerebral vasculopathy (Susac Syndrome) and examines the world literature on approximately 60 cases previously reported. METHODS: Retrospective case review of 22 patients with the diagnosis of Susac Syndrome seen at a large tertiary care center from 1979 until present. Demographics, clinical course, and results of audiometric testing were gathered and analyzed. A review of the world’s literature was conducted using MEDLINE searches for “Susac Syndrome” and “retinocochleocerebral vasculopathy,” and attention was paid to the references of each article retrieved. RESULTS: Of 22 patients with Susac Syndrome, 18 (82%) were female. Mean age was 37 years old, ranging from 20 to 69 years old. Ten patients (45.5%) reported a fluctuating hearing loss, 13 (59%) reported tinnitus, 12 (54.6%) noted vertigo. Ten patients (45.5%) presented with bilateral symptoms and 12 (54.6%) were unilateral. In the 32 affected ears, the pure tone average was 37 dB, and the mean percent hearing loss was 21.8%. Fifty percent of affected ears had AAO-HNS hearing classification type A. Only 28% of affected ears had 100% word recognition. CONCLUSIONS: Retinocochleocerebral vasculopathy, Susac Syndrome, is a rare and potentially devastating disease. Knowledge of this illness by otolaryngologists should prompt earlier diagnosis and treatment.
P093 Measurement of Angle and Length of Eustachian Tube Using CT Kenji Takasaki, MD (presenter); Haruo Takahashi, MD; Kaori Enatsu, MD; Hidetaka Kumagami, MD OBJECTIVES: Obtain clues on the anatomical features of the eustachian tube (ET) related to the susceptibility to otitis media with effusion (OME) in children. METHODS: The angle and length of the ET in children with OME (54 ears) and without OME (50 ears, normal children),
as well as those of normal adults were measured on the CT using the multiplanar reconstruction technique. RESULTS: The angles of ET in the OME children’s group, normal children’s group, and normal adult group were 20.4⫾3.5 and 21.2⫾4.8 , 19.9⫾3.4 and 20.0⫾3.6 , and 27.3⫾2.7 and 27.3⫾2.8 in the right and left sides, respectively. The lengths of each group were 37.2⫾3.0 mm and 37.6⫾3.2 mm, 37.5⫾3.3 mm and 38.0⫾3.2 mm, and 42.5⫾2.8 mm and 42.9⫾2.9 mm in the right and the left side, respectively. There was no significant difference between the right and left sides in any groups. Both the angles and lengths were significantly greater in the normal adult group than in either the OME children group or normal children group (p⬍0.05), but there was no significant difference either in the angle or length of the ET between the OME group and normal children’s group (p⬎0.05). CONCLUSIONS: The angle and length of ET are more horizontal and shorter in infants, but there is no statistical difference between infants with and without OME. These results suggest that short and horizontal ET may be one of the predisposing factors related to the high susceptibility of OME in infants and children rather than etiological factors.
P094 Ruptured Carotid Artery Pseudoaneurysm in the Middle Ear Ryan G Porter, Sr, MD (presenter); John P Leonetti, MD; Sam J Marzo, MD; Lotfi Hacein-Bey, MD OBJECTIVES: 1. Describe management of a carotid artery pseudoaneurysm rupture during middle ear surgery. 2. Review the literature with respect to the diagnosis and management of petrous internal carotid artery pseudoaneurysm hemorrhage. METHODS: The following is a case report describing management of a two-centimeter pseudoaneurysm of the petrous portion of the internal carotid artery. This anomaly was discovered in a 47-year-old female undergoing a tympanoplasty and revision mastoidectomy in January 2007 at a tertiary care academic medical center. A review of the literature relating to petrous carotid artery pseudoaneurysms is included. RESULTS: Iatrogenic carotid artery pseudoaneurysms may result from traumatic instrumentation of an aberrant carotid artery during middle ear surgery. Pre-existing pseudoaneurysms are also an important phenomenon to consider when preparing a patient for any otologic procedure. Although injury to a pre-existing pseudoaneurysm is a potentially life-threatening event, the situation can be successfully managed using a multidisciplinary approach involving contemporary interventional radiology techniques coupled with obliteration of the vascular lesion through a subtotal petrosectomy with closure of the external auditory canal. CONCLUSIONS: The patient was taken to the interventional neuroradiology suite from the operating room and underwent
SCIENTIFIC POSTERS
gested that this new surgical technique prevented further deterioration of hearing loss and became a pivotal treatment of PLF.
P243