European Annals of Otorhinolaryngology, Head and Neck diseases 132 (2015) 239–241
Available online at
ScienceDirect www.sciencedirect.com
What is your diagnosis?
Pulsatile tinnitus and an unusual ossicular anomaly D.-T. Ginat a,∗ , M.-B. Gluth b a b
Department of Radiology, University of Chicago Medical Center, 5841 South Maryland Avenue, Chicago IL 60637, United States Department of Otolaryngology, University of Chicago Medical Center, 5841 South Maryland Avenue, Chicago IL 60637, United States
1. Description A 49-year-old female presented with long-standing right pulsatile tinnitus, hearing loss, hyperacusis, and otic fullness. She reported that tinnitus was worse when lying supine, but it was not affected by head turning, lying in the decubitus position, or Valsalva maneuver. The patient denied otalgia, otorrhea, vertigo, headache, head trauma, or past ear surgery. Otoscopic examination and the pneumatic fistula test were normal. Auscultation of the neck and ear did not reveal a bruit and tinnitus was not subjectively affected by jugular compression. An audiogram
∗ Corresponding author. E-mail address: [email protected] (D.-T. Ginat). http://dx.doi.org/10.1016/j.anorl.2014.12.001 1879-7296/© 2015 Elsevier Masson SAS. All rights reserved.
demonstrated low frequency conductive hearing loss affecting 250, 500, and 1000 Hz with air conduction thresholds in the 30–40 dB range and bone conduction thresholds at 0 dB. The Weber test lateralized to the right with the 256 Hz tuning fork and there was perception of sound in the right ear with placement of the 128 Hz tuning fork on the knees and ankles. Cervical vestibular evoked myogenic potentials were intact with thresholds of 90 dB on the right and 85 dB on the left with symmetric amplitude. A temporal bone computed tomography (CT) scan with 3D reconstruction was performed was performed for further evaluation (Fig. 1a and b).
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D.-T. Ginat, M.-B. Gluth / European Annals of Otorhinolaryngology, Head and Neck diseases 132 (2015) 239–241
Fig. 1. a: coronal CT image shows a right sigmoid sinus diverticulum and dehiscence in the mastoid cortex (arrow); b: 3D surface rendered CT image shows a left-sided thick, straight monopod stapes (arrow).
What is your diagnosis?
D.-T. Ginat, M.-B. Gluth / European Annals of Otorhinolaryngology, Head and Neck diseases 132 (2015) 239–241
The patient had a sigmoid sinus diverticulum with dehiscence on the right side and an incidental columellar-type monopod stapes on the left. A wide variety of conditions can produce pulsatile tinnitus (Table 1). Sigmoid sinus diverticulum and dehiscence is perhaps the most common identifiable cause for pulsatile tinnitus of venous origin, with a prevalence of 23% in symptomatic patients, and is potentially treatable [1,2]. Nevertheless the relationship between the vascular abnormality and pulsatile tinnitus is only presumptive, since it was not treated. Dehiscence of the sigmoid sinus can involve erode into the mastoid air cells or the mastoid cortex, or both, as demonstrated in this case. Thus, temporal bone CT is important for defining the abnormal anatomy prior to surgical intervention and should be included in a comprehensive evaluation of pulsatile tinnitus [1]. In general, vascular imaging can also be useful for evaluating patients with pulsatile tinnitus. The stapes develops from the cranial mesenchyme of the second arch and normally consists of a footplate, anterior and posterior crura, and a capitulum [3]. Although uncommon, there is a wide Table 1 Differential diagnosis for pulsatile tinnitus. Category
Condition
Arterial
Arteriovenous malformation; arteriovenous fistula; arteriosclerosis; dissection; aneurysm; fibromuscular dysplasia; aberrant carotid artery and other arterial anomalies; glomus tumors and other hypervascular neoplasms; otospongiosis High-riding or dehiscent jugular bulb; sigmoid sinus diverticulum and/or dehiscence; anomalous condylar and emissary veins; pseudotumor cerebri and other causes of intracranial hypertension Muscular tinnitus (myoclonus, multiple sclerosis, and amyotrophic lateral sclerosis); superior semicircular canal dehiscence; Paget disease
Venous
Non-vascular and miscellaneous
241
variety of stapes anomalies, which may occur with or without footplate fixation, lateral ossicular chain malformations, or vascular abnormalities such as a perisitant stapedial artery [4]. A columellar stapes consists of one central crus with absence of the normal superstructure and may be related to malformation of Reichert’s cartilage [5]. A columellar monopod stapes differs from a unicruriate stapes in that it is relatively thick, straight, and attaches to the center of the footplate [5], as demonstrated in this case. These findings are readily depicted on high-resolution temporal bone CT and accompanying 3D surface renderings can be particularly useful for providing an overview of the ossicular chain morphology. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. Acknowledgements We thank Dr. Michael Rozenfeld for generating the 3D surfaced rendered image. References [1] Grewal AK, Kim HY, Comstock 3rd RH, Berkowitz F, Kim HJ, Jay AK. Clinical presentation and imaging findings in patients with pulsatile tinnitus and sigmoid sinus diverticulum/dehiscence. Otol Neurotol 2014;35:16–21. [2] Schoeff S, Nicholas B, Mukherjee S, Kesser BW. Imaging prevalence of sigmoid sinus dehiscence among patients with and without pulsatile tinnitus. Otolaryngol Head Neck Surg 2014;150:841–6. [3] Juliano AF, Ginat DT, Moonis G. Imaging review of the temporal bone: part I. Anatomy and inflammatory and neoplastic processes. Radiology 2013;269:17–33. [4] Park HY, Han DH, Lee JB, Han NS, Choung YH, Park K. Congenital stapes anomalies with normal eardrum. Clin Exp Otorhinolaryngol 2009;2:33–8. [5] Kurosaki Y, Kuramoto K, Itai Y. Demonstration of columella-like stapes by CT. J Comput Assist Tomogr 1995;19:506–7.
Available online at
ScienceDirect www.sciencedirect.com
What is your diagnosis?
Pulsatile tinnitus and an unusual ossicular anomaly D.-T. Ginat a,∗ , M.-B. Gluth b a b
Department of Radiology, University of Chicago Medical Center, 5841 South Maryland Avenue, Chicago IL 60637, United States Department of Otolaryngology, University of Chicago Medical Center, 5841 South Maryland Avenue, Chicago IL 60637, United States
1. Description A 49-year-old female presented with long-standing right pulsatile tinnitus, hearing loss, hyperacusis, and otic fullness. She reported that tinnitus was worse when lying supine, but it was not affected by head turning, lying in the decubitus position, or Valsalva maneuver. The patient denied otalgia, otorrhea, vertigo, headache, head trauma, or past ear surgery. Otoscopic examination and the pneumatic fistula test were normal. Auscultation of the neck and ear did not reveal a bruit and tinnitus was not subjectively affected by jugular compression. An audiogram
∗ Corresponding author. E-mail address: [email protected] (D.-T. Ginat). http://dx.doi.org/10.1016/j.anorl.2014.12.001 1879-7296/© 2015 Elsevier Masson SAS. All rights reserved.
demonstrated low frequency conductive hearing loss affecting 250, 500, and 1000 Hz with air conduction thresholds in the 30–40 dB range and bone conduction thresholds at 0 dB. The Weber test lateralized to the right with the 256 Hz tuning fork and there was perception of sound in the right ear with placement of the 128 Hz tuning fork on the knees and ankles. Cervical vestibular evoked myogenic potentials were intact with thresholds of 90 dB on the right and 85 dB on the left with symmetric amplitude. A temporal bone computed tomography (CT) scan with 3D reconstruction was performed was performed for further evaluation (Fig. 1a and b).
240
D.-T. Ginat, M.-B. Gluth / European Annals of Otorhinolaryngology, Head and Neck diseases 132 (2015) 239–241
Fig. 1. a: coronal CT image shows a right sigmoid sinus diverticulum and dehiscence in the mastoid cortex (arrow); b: 3D surface rendered CT image shows a left-sided thick, straight monopod stapes (arrow).
What is your diagnosis?
D.-T. Ginat, M.-B. Gluth / European Annals of Otorhinolaryngology, Head and Neck diseases 132 (2015) 239–241
The patient had a sigmoid sinus diverticulum with dehiscence on the right side and an incidental columellar-type monopod stapes on the left. A wide variety of conditions can produce pulsatile tinnitus (Table 1). Sigmoid sinus diverticulum and dehiscence is perhaps the most common identifiable cause for pulsatile tinnitus of venous origin, with a prevalence of 23% in symptomatic patients, and is potentially treatable [1,2]. Nevertheless the relationship between the vascular abnormality and pulsatile tinnitus is only presumptive, since it was not treated. Dehiscence of the sigmoid sinus can involve erode into the mastoid air cells or the mastoid cortex, or both, as demonstrated in this case. Thus, temporal bone CT is important for defining the abnormal anatomy prior to surgical intervention and should be included in a comprehensive evaluation of pulsatile tinnitus [1]. In general, vascular imaging can also be useful for evaluating patients with pulsatile tinnitus. The stapes develops from the cranial mesenchyme of the second arch and normally consists of a footplate, anterior and posterior crura, and a capitulum [3]. Although uncommon, there is a wide Table 1 Differential diagnosis for pulsatile tinnitus. Category
Condition
Arterial
Arteriovenous malformation; arteriovenous fistula; arteriosclerosis; dissection; aneurysm; fibromuscular dysplasia; aberrant carotid artery and other arterial anomalies; glomus tumors and other hypervascular neoplasms; otospongiosis High-riding or dehiscent jugular bulb; sigmoid sinus diverticulum and/or dehiscence; anomalous condylar and emissary veins; pseudotumor cerebri and other causes of intracranial hypertension Muscular tinnitus (myoclonus, multiple sclerosis, and amyotrophic lateral sclerosis); superior semicircular canal dehiscence; Paget disease
Venous
Non-vascular and miscellaneous
241
variety of stapes anomalies, which may occur with or without footplate fixation, lateral ossicular chain malformations, or vascular abnormalities such as a perisitant stapedial artery [4]. A columellar stapes consists of one central crus with absence of the normal superstructure and may be related to malformation of Reichert’s cartilage [5]. A columellar monopod stapes differs from a unicruriate stapes in that it is relatively thick, straight, and attaches to the center of the footplate [5], as demonstrated in this case. These findings are readily depicted on high-resolution temporal bone CT and accompanying 3D surface renderings can be particularly useful for providing an overview of the ossicular chain morphology. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. Acknowledgements We thank Dr. Michael Rozenfeld for generating the 3D surfaced rendered image. References [1] Grewal AK, Kim HY, Comstock 3rd RH, Berkowitz F, Kim HJ, Jay AK. Clinical presentation and imaging findings in patients with pulsatile tinnitus and sigmoid sinus diverticulum/dehiscence. Otol Neurotol 2014;35:16–21. [2] Schoeff S, Nicholas B, Mukherjee S, Kesser BW. Imaging prevalence of sigmoid sinus dehiscence among patients with and without pulsatile tinnitus. Otolaryngol Head Neck Surg 2014;150:841–6. [3] Juliano AF, Ginat DT, Moonis G. Imaging review of the temporal bone: part I. Anatomy and inflammatory and neoplastic processes. Radiology 2013;269:17–33. [4] Park HY, Han DH, Lee JB, Han NS, Choung YH, Park K. Congenital stapes anomalies with normal eardrum. Clin Exp Otorhinolaryngol 2009;2:33–8. [5] Kurosaki Y, Kuramoto K, Itai Y. Demonstration of columella-like stapes by CT. J Comput Assist Tomogr 1995;19:506–7.
