Altered clinical course of glomus tympanicum — a case report

Glomus tumours of temporal bone are rare and usually present with symptoms of hearing loss and tinnitus. Diagnosis is often delayed due to the slow growth of the tumour. Here we present a case report of a patient diagnosed as glomus tympanicum who presented only with unilateral progressive hearing loss for the past one year and rapidly detoriating hearing loss since two months who was managed successfully.     

An aneurysm of the petrous internal carotid artery.

Internal carotid artery aneurysms are a rare cause of pulsatile tinnitus and conductive hearing loss but should be borne in mind when there is a suspected diagnosis of glomus jugulare or high-riding jugular bulb. Most cases are congenital. We present a case of otorrhagia which was initially thought to be a glomus jugulare, the diagnosis of internal carotid artery aneurysm was made at angiography and treated by balloon embolization.     

Cochlear implantation after surgery for bilateral jugular foramen paragangliomas: a case report

This paper describes a case of cochlear implantation for deafness due to bilateral glomus jugulare tumors (paragangliomas) as well as an unusual complication after cochlear implantation in a 31-year-old male. A 31-year-old male with profound sensorineural hearing loss on the right side and deafness on the left side, caused by bilateral jugular foramen paragangliomas, was implanted with a multi-channel cochlear implant (Combi 40+, Med-El) on the right side during subtotal petrosectomy for removal of the right-sided glomus jugulare tumor. No postoperative medical complications were observed. The patient responded to acoustic stimuli. Postoperative computed tomography (CT) did not show any misplacement of the electrode. Three years after implantation, an acute, rapidly progressive hearing impairment with pain to acoustic stimuli was observed. A CT scan at that time showed cochlear bone resorption. No radiologic evidence of paraganglioma recurrence was observed. The implant was removed and a biopsy of the cochlea was performed which on histologic examination showed fibrosis without any sign of any tumor recurrence. Cochlear implantation can restore hearing in deafness due to bilateral glomus jugulare tumors. Cochlear resorption may occur as a late complication in the implanted side in the patients.     

Reversible sensorineural hearing loss

We present an unusual case of temporary sensorineural hearing loss in a 6-year-old child due to carbon monoxide. This was shown on both the audiograms and confirmed with objective testing using otoacoustic emissions. Carbon monoxide poisoning is one of the few recognised causes of reversible sensorineural hearing loss, though it may also lead to a permanent deficit. This is discussed along with other potential causes of reversible sensorineural hearing loss.     

Glomus jugulare tumour presenting with isolated accessory nerve palsy

Glomus tumours of the skull base are rare, and most frequently present with symptoms of hearing loss and tinnitus. Diagnosis is often delayed due to the slow growth of the tumour. We describe the previously unreported occurrence of a glomus tumour presenting with a unilateral accessory nerve palsy.     

Vestibular Schwanomma with normal hearing

Schwanomma of the vestibular nerves usually present with sensorineural hearing loss of varying degrees. We report here a large (5.5cm x 3.5cm) vestibular schwanomma with normal hearing. Other unusual features of this case were the young age (20 yrs) of the patient and absence of any otological symptoms.     

Hearing loss after general anaesthesia (a case report and review of literature)

Unilateral hearing loss has been reported as an unusual complication in general anaesthesia, and so far no reports on bilateral hearing loss have been published. In this paper we present a case with bilateral sensorineural hearing impairment immediately following a minor abdominal surgical procedure. The literature is reviewed and the mechanisms causing hearing loss during anaesthesia are discussed.     

Fluctuating hearing loss with otorrhoea in Hughes syndrome

We present an unusual case of bilateral aural symptoms in a 34-weeks pregnant woman with Hughes syndrome. This report underscores the unpredictable nature of this syndrome and its ENT complications. No previous case of fluctuating hearing loss and otorrhoea in Hughes syndrome has been described.     

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.     

A case report of fluctuant sensorineural hearing loss after hepatitis B vaccination

Hepatitis B vaccine is known to induce accidents and side-effects. We report a case of unilateral fluctuant sensorineural hearing loss with tinnitus after a series of hepatitis B vaccinations. Tinnitus regressed and hearing thresholds were normalized 6 months later. The authors discuss the potential causality of hepatitis B vaccine in the occurrence of fluctuant sensorineural hearing loss and propose a physiopathological hypothesis to explain this unusual case.     

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。     

Paragangliomas of the Head & Neck: the KMC experience

To determine the clinical features, investigations, intra-operative findings, surgical approaches used and the results of the treatment for paragangliomas of the head and neck. Retrospective study of 14 cases of paragangliomas in head and neck seen over a period of 10 years including five carotid body tumors, seven glomus jugulares and two glomus tympanicums. HRCT scans and bilateral carotid angiography were done in all cases of glomus jugulare. Pre-operative embolization was done in most cases. The trans-cervical approach was used for all cases of carotid body. In three cases of Type B jugulare tumors, a post-aural tympanotomy was used. A Fisch Type A approach was done for three cases of Type D jugulare tumors. Postaural tympanotomy approach was used for both patients with glomus tympanicum. In one case of extratympanic glomus jugulare tumor with hypoglossal palsy, a neck exploration was done to isolate and excise the tumor. Five patients with carotid body tumors presented as unilateral, painless, pulsatile swelling in the upper neck. Intra-operatively, three of the tumors were classified into Shamlin's Grade II and one each into Grade III and Grade I. A carotid blow-out occurred in one of the patients with Grade II disease, which was managed. ECA resection had to be done in one case. Seven patients were diagnosed to have glomus jugulare and two with glomus tympanicum. Six glomus jugulare tumors presented with hearing loss, ear discharge and obvious swelling. Glomus tympanicums presented with hearing loss but no bleeding from the ear. On examination, tumors presented with an aural polyp with no VII nerve deficits. Both tympanicums were classified as Fisch Type A, three of the jugulares classified as Type B, two as Type D2 and one as Type D1. Tumors were found to be supplied predominantly by the ascending pharyngeal artery. In three cases of Type B jugulare tumors, a post-aural tympanotomy was used. A Fisch Type A approach was done for three cases of Type D jugulare. The transcanal approach was used for both patients with glomus tympanicum. Paragangliomas are uncommon tumors that need accurate diagnosis and skilled operative techniques. Though the surgical approaches may appear complicated, the removal provides good cure rates with minimal morbidity and recurrence. Lateral skull base approaches should be the armamentarium of every head and neck surgeon.     

Middle fossa repair of bilateral large congenital tegmental defects with meningoencephaloceles

Spontaneous temporal meningoencephaloceles are unusual. When they do occur, they present with a variety of signs and symptoms, which can make diagnosis and management challenging. We report the interesting case of a 49-year-old woman with bilateral congenital temporal meningoencephaloceles. She presented with a 12-month history of bilateral fluctuating hearing loss, and she more recently developed right-sided acute otitis media with meningitis. The presentation of bilateral extensive tegmental defects and meningoencephaloceles with a fluctuating hearing loss and meningitis associated with acute otitis media affecting one ear and then subsequently the other ear is extremely rare and difficult to diagnose. It requires a very careful clinical and radiologic assessment. Methods of surgical repair differ depending on the size of the defects.     

Epistaxis as a rare presenting feature of glomus tympanicum

Glomus tumours are the most common primary neoplasms of the middle ear, typically benign and slowly progressive. Pulsatile tinnitus and ipsilateral hearing loss are the most common symptoms at presentation by far; otalgia, aural fullness and otorrhoea being less frequent. A case of primary glomus tympanicum presenting with recurrent epistaxis, previously unreported in the literature, is described and associated imaging presented.     

Cochlear implantation in a bilateral endolymphatic sac tumor patient. A case report

At the present time, the optimal management of patients with locally aggressive ELSTs includes: early diagnosis, surgical complete excision and long term follow up to monitor for recurrent disease. The presence of bilateral tumors produces a significant risk for bilateral profound hearing loss. We report the unusual case of bilateral endolymphatic sac tumor in a 14-year-old girl who presented bilateral anacusis and in whom a cochlear implant was performed after removal of the second tumor.     

Simultaneous recurrent multiple chemodectomas

We report a case of concomitant, recurrent carotid body tumor and recurrent bilateral glomus jugulare tumors, with extensive intrajugular-venous extension. The patient's family history confirms the previously reported familial occurrence of chemodectomas. The value of preoperative angiographich evaluation is emphasized in establishing a diagnosis and formulating a therapeutic approach. Recurrence of chemodectomas after appropriate therapy, although unusual, was present in all three previously treated sites in this patient.     

Considerations of isolated destruction of the short process of the incus in a case of cholesteatoma

Cholesteatoma occurs frequently with the destruction of the ossicles. The most commonly eroded ossicle has been reported to be the incus. Many studies report destruction of the long process and body of the incus. However, isolated erosion of the short process of the incus is an unusual finding. We present such a case with a slight conductive hearing loss that remained unchanged at 2 1/2 years postoperatively. The short process of the incus may contribute to hearing up to 10–15 dB. It is also possible that it plays a role in epitympanic aeration by supplying an attachment surface to the incudal folds.     

Unusual electrocochleography cases

The electrocochleographic findings of 3 unusual cases from about 350 performed on patients with various hearing loss are presented; they demonstrate the value of electrocochleography as a clinical tool in otological investigation. The first case was an electrocochleographically detected acute impairment of the first neuron, the second a hereditary hearing loss with endolymphatic hydrops, and the last shows the effect of an endolymphatic shunt on a hydropic hearing loss.     

Steroid-responsive hearing loss in temporal arteritis

An unusual case of temporal arteritis presenting with sensorineural hearing loss is presented. Deafness was improved dramatically by corticosteroid treatment. The diagnosis of temporal arteritis should be considered in elderly patients presenting with sensorineural hearing loss.     

Middle ear hemangiona: a case report

A middle ear hemangioma was detected in a 51-year-old man who suffered from fullness and hearing loss in the left ear. This case is presented with a review of the relevant literature. The mass was surgically resected for diagnostic and therapeutic reasons, and pathologic examination revealed a benign hemangioma. The CT, MRI, angiography, and light microscopy findings of the tumor are documented and compared with those a glomus tumor. The significance of this diagnosis is also discussed.