Aberrant internal carotid artery causing objective pulsatile tinnitus and conductive hearing loss

Abstract Aberrant internal carotid artery (ICA) in the middle ear is a rare, dangerous vascular anomaly and conservative follow-up was usually adopted in most reported cases. Here we report the case of an 8-year-old girl with symptoms of objective pulsatile tinnitus and conductive hearing loss in the right ear. Otoscopic examination, computed tomography, and conventional angiography were performed. An aberrant ICA combined with a 'third mobile window' was suspected preoperatively and confirmed at exploratory surgery of the middle ear. The aberrant ICA was treated, and the pulsatile tinnitus disappeared and hearing recovered after the surgery. This case suggests that surgery is practical to relieve troublesome tinnitus and hearing loss in appropriate cases with aberrant ICA.     

Aberrant internal carotid artery as a cause of objective pulsatile tinnitus

An aberrant internal carotid artery (ICA) is a rare vascular anomaly and causes to objective pulsatile tinnitus and retrotympanic mass. In the past, it was often diagnosed during ear surgery or biopsy, which may lead to massive bleeding. We present a case of 37-year-old woman complaining of pulsatile tinnitus. The tinnitus was objectively audible at the right ear through an otoscope. Previously, MRA plays a pivotal role in the evaluation of the aberrant ICA. But in this case, the resolution of magnetic resonance angiography (MRA) was not enough to portray the course of the ICA, because dephasing of the spins due to turbulence may cause loss of the signal intensity on MRA. Computed tomography (CT) of the temporal bone revealed aberrant internal carotid artery passing through the middle ear. It is important to diagnose approximately this anomaly using with combination of high-resolution CT, MRI, and MRA.     

Objective tinnitus caused by an aberrant internal carotid artery

We report the case of a 29 year-old woman who complained of pulsatile tinnitus and impaired hearing. On otoscopic examination, her right tympanic membrane was observed to be in contact with a mass in the middle ear cavity, with the formation of a meniscus at the point of contact. Using a high-sensitivity microphone inserted into the external auditory canal, we recorded pulsatile tinnitus that was synchronous with the electrocardiogram. Magnetic resonance angiography revealed that the middle ear mass was an aberrant internal carotid artery coursing through the hypotympanum.     

Objective tinnitus and serous otitis media as symptoms of aberrant carotid artery (author's transl)

Following a review of the literature a case of a middle ear aberrant carotid artery is reported. To avoid dangerous haemorrhage with surgery auscultation of the external auditory canal is necessary in every case of serous otitis media with a pulsating tympanic membrane. The main symptom in this condition is objective tinnitus.     

Aberrant internal carotid artery in the temporal bone: imaging findings and management

OBJECTIVES: To describe the clinical and radiological features of the vascular anomaly aberrant internal carotid artery (ICA) in the temporal bone and to discuss management strategies. DESIGN: Retrospective study. PATIENTS: Sixteen cases of aberrant ICA were diagnosed between 1982 and 2003. RESULTS: Of 16 cases, 11 were recognized by imaging assessment, 4 were recognized during middle ear surgery, and 1 was recognized clinically. Among the 11 cases, 8 malformations were diagnosed because of otologic symptoms related to the abnormal ICA or chronic otitis, while the other 3 were identified incidentally because of an underlying accompanying disease. In 4 cases, the diagnosis was made during surgery related to chronic otitis media (n = 2) or conductive hearing loss (n = 2). In these 4 cases, massive bleeding resulted from surgical injury to the vessel. Packing the external auditory canal and the middle ear first controlled the bleeding. Endovascular procedure was required in 2 cases to exclude an aneurysm or to control bleeding but was followed by anterior cerebral stroke in 1 case. The aberrant ICA could be identified on computed tomographic scan by the following features: intratympanic mass, enlarged inferior tympanic canaliculus, absence of the vertical segment of the ICA canal, and absence of bone covering the tympanic portion of the ICA. Conventional angiography was mandatory when intervention was planned to control bleeding or aneurysm. CONCLUSIONS: This study highlights that aberrant ICA has to be identified before any middle ear surgery because misdiagnosis may lead to dramatic surgical complications, whereas diagnosis with computed tomographic scan is easy. Bleeding is a minor complication compared with the putative neurologic deficit due to endovascular occlusion.     

Tympanic paraganglioma with extension into the Eustachian tube and nasopharynx: a case report

Tympanic paragangliomas usually present as a vascular middle ear mass, with the most common presenting symptoms being pulsatile tinnitus and hearing loss. We report an unusual case of a recurrent tympanic paraganglioma extending along the Eustachian tube and nasopharynx, presenting with recurrent epistaxis.     

Cavernous hemangioma of the tympanic membrane

Cavernous hemangioma seems to most frequently arise in the posterior portion of the external auditory canal. However, they rarely occur in the tympanic membrane. A 49-year-old male patient was referred for evaluation of right-sided pulsatile tinnitus that he'd experienced for the previous 2 years. Temporal bone computerized tomography showed an isolated soft tissue mass just lateral to the tympanic membrane. There was no evidence of bony erosion or middle ear invasion. The patient underwent excision of the mass using a postauricular approach. The mass was removed en bloc and the defect of the tympanic membrane was repaired by tympanoplasty type I. There was no recurrence after 1 year of follow-up.     

Persistent stapedial artery with stapes ankylosis

The persistent stapedial artery (PSA) is a very rare, congenital, vascular anomaly. It presents as a pulsatile middle ear mass and sometimes causes conductive hearing loss. The diagnosis of the presence of a PSA is always accidental, because it is so rare and difficult to predict. CT findings include the absence of the foramen spinosum and a soft-tissue prominence in the region of the tympanic segment of the facial nerve. The risks of surgery include facial palsy, hemiplegia caused by coagulation of the PSA, and bleeding due to injury of the carotid artery during surgery in cases of aberrant internal carotid. In this article we report a case of PSA with stapes ankylosis for which we performed malleus-stapedotomy using a Teflon wire piston. We did not coagulate the PSA. Nevertheless the PSA attached to the prosthesis, the patient presented significant improvement in hearing level and had no complaint of pulsating tinnitus. Thus, we have shown that attachment of the prosthesis to the PSA does not necessarily disturb improvement of hearing level after malleus-stapedotomy for otosclerosis with PSA. Based on our experience, many cases can be treated by stapedotomy using a prosthesis and without coagulating the PSA.     

Aberrant internal carotid artery in the middle ear with a deficiency in the origin of the anterior cerebral artery: A case report

An aberrant internal carotid artery (ICA) in the middle ear is rare and difficult to diagnose, and may lead to severe complications. We present here a case of aberrant ICA with a deficiency in the origin of the anterior cerebral artery. The only symptom was aural fullness, and a nonpulsatile and white tympanic mass in the anteroinferior area was noted. Computed tomography (CT) and magnetic resonance angiography (MRA) are useful tools that provide excellent visualization of the temporal bone for the diagnosis of aberrant ICA by the following features: intratympanic mass, enlarged inferior tympanic canaliculus, absence of the vertical segment of the ICA canal, and absence of bone covering the tympanic portion of the ICA. In addition, in this case, a deficiency in the origin of the anterior cerebral artery on the same side was identified by MRA, and cerebral arteriography and a carotid occlusion test were performed. Because of the deficiency in the origin of the anterior cerebral artery, the ICA compression revealed that there was almost no cross flow from the other ICA. Our experience illustrates that after confirmation of the diagnosis of aberrant ICA, localized treatment and/or surgical procedures should be considered carefully.     

Tympanometry in the evaluation of vascular lesions of the middle ear and tinnitus of vascular origin.

The occurrence of vascular perturbations in the tympanogram in association with glomus tumors has previously been noted in the literature. We have broadened the scope of this method of diagnosis in the study of 3 glomus tympanicum and 3 glomus jugulare tumors, 1 dehiscent high jugular bulb, 1 aberrant carotid artery in the middle ear and 2 cases of pulsatile tinnitus of vascular origin. Analysis of illustrative tympanograms at standard and at increased sensitivity (x 20) obtained in these cases are presented. The following factors as they relate to the mechanism of recording the vascular perturbations are presented: air pressure and/or presence of fluid in the middle ear; compliance of the tympanic membrane and ossicular chain as affected by the mass, size and degree of vascularity of the mass and presence of extraneous sounds. A new method of recording the vascular perturbations utilizing a time-base generator affixed to an X-Y plotter is described. It is the purpose of this study to demonstrate the utility of obtaining tympanograms at standard and at increased sensitivity in the evaluation of vascular middle ear lesions and tinnitus of vascular origin.     

Cavernous hemangioma of the tympanic membrane and external ear canal

OBJECTIVES: The aims of this study are to document the occurrence of a cavernous hemangioma of the tympanic membrane (TM) and external auditory canal (EAC) that invaded the middle ear spaces and to review the relevant literature. METHODS: The clinical presentation, imaging studies, operative report, and histologic findings of this new case of cavernous hemangioma are reviewed. RESULTS: A cavernous hemangioma of the TM and EAC involving the middle ear spaces was surgically excised without complications. The pulsatile tinnitus, which affected our patient at the same ear where the lesion was situated, disappeared after surgery. Our case represents the first documented cavernous hemangioma of the TM and EAC that invaded the middle ear spaces and the eighth case of cavernous hemangioma of the EAC/TM. Computed tomography is the method of choice in evaluating this lesion. CONCLUSIONS: Hemangiomas of the EAC and/or TM are extremely rare entities amenable to surgical excision. With magnetic resonance imaging, there is difficulty in defining the exact location of the tumor and degree of soft tissue involvement.     

颈静脉球瘤10例诊疗分析

目的分析探讨颈静脉球瘤临床特点，总结手术疗效，提高该病诊治经验。方法回顾性分析10例经数字减影血管造影和/或术后病理证实的颈静脉球瘤患者的临床资料，总结该病的临床特征、影像学特点、围手术期处理、手术方式及随访效果。结果所有患者均为单耳发病，其中男2例，女8例。表现为单侧搏动性耳鸣6例，听力下降8例，耳痛及眩晕3例，耳流脓性或脓血性分泌物4例，因体检发现耳道肿物就诊1例。6例因肿物突入外耳道，鼓膜不能窥及，4例可透过鼓膜看见鼓室内红色搏动，鼓膜稍向外膨隆。纯音测听示传导性聋4例，混合性聋5例，感音神经性聋1例。所有患者均行颞骨CT及MRI检查，鼓室球瘤可见鼓室、外耳道内充填不规则软组织密度影；颈静脉球体瘤可见颈静脉孔区软组织密度影，周围呈“蚕蚀样”骨质破坏，肿物突向鼓室及外耳道，增强扫描可见明显强化。3例颈静脉球体瘤行DSA检查可见颈静脉孔区浓密肿瘤染色，2例由咽升动脉供血，1例由咽升动脉和枕动脉供血。6例鼓室球瘤患者均行耳道乳突径路鼓室球瘤切除术，3例颈静脉球体瘤患者行颞下窝A型径路颈静脉球体瘤切除术，1例颈静脉球体瘤患者行伽玛刀治疗。所有病例随访10个月至5.5年，均无复发，未出现影响生活的严重并发症。结论颈静脉球瘤有特征性的临床表现，以单侧搏动性耳鸣和不同程度的听力下降最为多见，结合仔细的专科查体和完善的影响学检查不难诊断。鼓室球瘤患者通过中耳显微手术能达到良好疗效，颈静脉球体瘤患者因解剖位置特殊，对术者及围手术期的规范化处理要求较高，并且要求诊疗团队具有侧颅底外科手术的知识及经验，否则容易引起严重并发症。     

Otoacoustic emissions in an aberrant internal carotid artery: a case report

A 52-year-old Korean woman who complained of impaired hearing and tinnitus of the left ear was diagnosed with an aberrant internal carotid artery (ICA) in the middle ear using a temporal bone high resolution CT (HRCT) scan. Upon otoscopic examination, her left eardrum was found to be in contact with a mass in the middle ear cavity. Upon audiometric evaluation, a mild conductive hearing impairment was documented, and a discrepancy in the results of otoacoustic emissions (OAEs) was found for the left ear. This case report studies the aberrant ICA in the middle ear that revealed abnormal OAE findings.     

Aberrant internal carotid artery in the middle-ear space

The incidence of an aberrant internal carotid artery in the middle ear is approximately one per cent and most patients are asymptomatic. We present two patients with an aberrant internal carotid artery who presented with pulsatile tinnitus and an intra-tympanic mass. Here we discuss the clinical presentation, relevant radiographic investigations and further management of these patients.     

Superior canal dehiscence with tegmen defect revealed by otoscopy: Video clip demonstration of pulsatile tympanic membrane

Superior canal dehiscence is a pathologic condition of the otic capsule acting as aberrant window of the inner ear. It results in reduction of inner ear impedance and in abnormal exposure of the labyrinthine neuroepithelium to the action of the surrounding structures. The sum of these phenomena leads to the onset of typical cochleo-vestibular symptoms and signs. Among them, pulsatile tinnitus has been attributed to a direct transmission of intracranial vascular activities to labyrinthine fluids. We present the first video-otoscopic documentation of spontaneous pulse-synchronous movements of the tympanic membrane in two patients with superior canal dehiscence. Pulsating eardrum may represent an additional sign of third-mobile window lesion.     

Stapedius muscle myoclonus

Myoclonus of the middle ear is a very rare condition. We present the case of a 20-year-old soldier who was exposed to intense artillery noise during a fierce battle and immediately afterward complained of an incapacitating tinnitus in both ears. Microscopic examination of the ears demonstrated rapid rhythmic movements of the tympanic membrane coinciding with the tinnitus, which was also easily heard by the examiner. There was no evidence of palatal myoclonus, and thus the diagnosis of middle ear myoclonus was made. Exploratory tympanotomy confirmed the diagnosis of stapedial muscle myoclonus. Bilateral sectioning of the stapedial tendons brought immediate relief of the tinnitus to the patient. We review the literature of middle ear myoclonus and discuss the symptoms, evaluation, and management of this rare condition.     

Aberrant ectatic internal carotid artery in the middle ear

We report the case of a 34-year-old man with pulsatile tinnitus and a reddish mass in the anteroinferior quadrant of the middle ear. Physical examination and imaging were unable to establish a diagnosis, so an exploratory tympanotomy was performed. Exploration revealed the presence of an ectatic aberrant internal carotid artery in the middle ear. Aberrations of the internal carotid artery in the middle ear are rare. Even so, our case is unusual in that all initial investigations had failed to establish the diagnosis. This case highlights the limitations of modern imaging techniques in certain situations.     

Intratympanic aberrant and hypoplastic carotid artery

Intratympanically aberrant internal carotid artery(ICA) is a rarely seen vascular abnormality. We present here the combination of aberration and prominent hypoplasia of the ICA in a case. Intratympanic aberrant ICA, which is rarely cited as a cause of tinnitus and hearing loss, should be known as a reason to be kept in mind as it may lead to life-threatening complications. Generally, it has been defined upon massive bleeding during myringotomy, ear surgery or biopsy procedure. In this article, the audiological and radiological studies confirmed with CT and MR angiography conducted on an aberrant and hypoplastic internal carotid artery that was identified under the manubrium mallei in a 28-year-old, young male patient who presented with complaints about hearing loss and fullness in the left ear were presented along with a literature review.     

A case of a coexisting aberrant internal carotid artery and persistent stapedial artery: the role of MR angiography in the diagnosis

We describe the case of a 37-year-old woman who presented with a complaint of recurrent headaches since childhood. Clinical examination revealed the presence of a reddish-bluish mass in the inferior half of the tympanic membrane in her right ear. Source and three-dimensional time-of-flight magnetic resonance angiography (MRA) detected a protruded right internal carotid artery (ICA) in the hypotympanum. The vertical segment of the ICA was absent, and the artery was narrowed and lateralized and had a "reverse 7" shape. Meanwhile, a persistent stapedial artery (PSA) was seen originating in the petrous portion of the ICA to form a middle meningeal artery. The A1 segment of the right anterior cerebral artery was hypoplastic. Based on these findings and on follow-up findings on computed tomography, the patient was diagnosed with a concomitant aberrant ICA and PSA. No intervention was undertaken. We review the management of this rare finding, and we discuss the role of MRA in its diagnosis.     

The effect of iontophoresis of lignocaine in the treatment of tinnitus

Lignocaine is the only drug available that suppresses tinnitus when given intravenously, but the mechanism and the site of its action are not well understood. Iontophoresis, a method by which chemical agents may be transported by electrical current from the ear canal throughout the tympanic membrane into the middle ear, could be expected to have a suppressive effect on tinnitus because the drug is brought so close to the cochlea. In the present study none of the 46 patients reported the suppression of tinnitus to a non-annoying level. Neither lignocaine nor any of its metabolites was found in blood sampled during the period of this treatment.