Cases reported "Hearing Loss"

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1/60. A case of endolymphatic sac tumor with long-term survival.

    A 72-year-old man developed left facial palsy at age 14 and left-sided hearing loss at age 20. At the age of 59, he presented with gait disturbance, and a large left cerebellopontine angle tumor was detected, which had markedly destroyed the pyramidal bone. The tumor was subtotally resected, but he required two more operations at the ages of 64 and 69 because of tumor regrowth. At the present time, recurrent tumor has destroyed the occipital bone and is invading the scalp. However, even though he has several cranial nerve palsies and cerebellar ataxia, he remains in stable condition and demonstrates long-term survival. The patient's surgical specimens revealed a papillary adenoma, which was recently thought to be of endolymphatic sac origin, although the origin of this kind of tumor, whether arising from the middle ear or from the endolymphatic sac, has not been established with certainty so far. In this paper, we provide further evidence that this tumor originates from the endolymphatic sac, based on anatomical, histopathological, and embryological evidence.
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2/60. Sudden unilateral hearing loss with simultaneous ipsilateral posterior semicircular canal benign paroxysmal positional vertigo: a variant of vestibulo-cochlear neurolabyrinthitis?

    We describe 4 patients who all simultaneously developed a sudden total or partial unilateral sensorineural hearing loss and an unusual acute peripheral vestibulopathy in the same ear characterized by posterior semicircular canal benign paroxysmal positional vertigo with intact lateral semicircular canal function. Two patients also had ipsilateral loss of otolith function. The vertigo resolved in all 4 patients after particle-repositioning maneuvers. The findings of audiometry and vestibular tests indicated that the lesion responsible for this syndrome was probably located within the labyrinth itself rather than within the vestibulocochlear nerve and that it was more likely a viral vestibulocochlear neurolabyrinthitis than a labyrinthine infarction.
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3/60. Orofacial paraesthesia: an unusual presentation of acoustic neuroma.

    patients with acoustic neuromas commonly present with unilateral hearing loss, tinnitus and unsteadiness. An uncommon initial feature is involvement of the trigeminal nerve. The unusual feature in the case reported here was orofacial paraesthesia, which preceded hearing loss by 3-4 years. diagnosis was made following a referral by the patient's dentist.
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4/60. Vestibular schwannoma with contralateral facial pain - case report.

    BACKGROUND: Vestibular schwannoma (acoustic neuroma) most commonly presents with ipsilateral disturbances of acoustic, vestibular, trigeminal and facial nerves. Presentation of vestibular schwannoma with contralateral facial pain is quite uncommon. CASE PRESENTATION: Among 156 cases of operated vestibular schwannoma, we found one case with unusual presentation of contralateral hemifacial pain. CONCLUSION: The presentation of contralateral facial pain in the vestibular schwannoma is rare. It seems that displacement and distortion of the brainstem and compression of the contralateral trigeminal nerve in Meckel's cave by the large mass lesion may lead to this atypical presentation. The best practice in these patients is removal of the tumour, although persistent contralateral pain after operation has been reported.
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5/60. A small vestibular schwannoma arising from the inferior vestibular nerve.

    The investigation of a complaint of unilateral right sensorineural hearing loss led to the identification of a right 3 mm vestibular schwannoma arising from the inferior division of the right vestibular nerve. On investigation the patient was found to have normal caloric function, this being mediated by the superior vestibular nerve. Both transient and distortion product otoacoustic emissions were absent in the right ear, and ABR was abnormal on this side. These findings are of interest as they indicate that this small lesion produced a hearing loss that was both cochlear and retrocochlear. The anatomical finding that the medial auditory efferents run within the inferior vestibular nerve is considered. This patient did not experience tinnitus or hyperacusis despite the site of the lesion arising from the inferior vestibular nerve.
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6/60. Subtonsillar placement of auditory brainstem implant.

    A 26-year-old female with NF2 underwent removal of an acoustic schwannoma via extended retrosigmoid approach with subtonsillar placement of the auditory brainstem implant. The patient had already shown palsy of the lower cranial nerves on the opposite side due to previous surgery. Differing from conventional routes such as the translabyrinthine and the retrosigmoid, the subtonsillar approach enabled us to observe the entire cochlear nucleus and avoid injury to the 9th and 10th cranial nerves when applying the electrode. Hearing in pure tone average was maintained almost at the preoperative level. We present a new use of this approach in placing the electrode and discuss its advantages.
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7/60. radiation-induced schwannomas of the nervous system. Report of five cases and review of the literature.

    radiation therapy has important delayed effects on the central nervous system. Prominent among these effects is radiation necrosis of nervous tissue, but an oncogenic effect is also recognized. Both benign and malignant intracranial tumors can develop in irradiated fields, particularly in children. Most of these tumors are sarcomas, meningiomas or gliomas and only occasionally schwannomas. We report 5 cases of postirradiation acoustic nerve schwannoma observed in our Department.
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8/60. Auditory disturbance as a prodrome of anterior inferior cerebellar artery infarction.

    OBJECTIVES: To investigate the clinical and radiological features of patients presenting with an acute auditory syndrome as a prodromal symptom of anterior inferior cerebellar artery (AICA) infarction. methods: 16 consecutive cases of AICA infarction diagnosed by brain magnetic resonance imaging completed a standardised audiovestibular questionnaire and underwent a neuro-otological evaluation by an experienced neuro-otologist. RESULTS: Five patients (31%) had an acute auditory syndrome as a prodrome of AICA infarction one to 10 days before onset of other brain stem or cerebellar symptoms. Two types of acute auditory syndrome were found: recurrent transient hearing loss with or without tinnitus (n = 3), and a single episode of prolonged hearing loss with or without tinnitus (n = 2). The episodic symptoms were brief, lasting only minutes. The tinnitus preceding the infarction was identical to the tinnitus experienced at the time of infarction. At the time of infarction, all patients developed hearing loss, tinnitus, vertigo, and ipsilateral hemiataxia. The most commonly affected site was the middle cerebellar peduncle (n = 5). Four of the five patients had incomplete hearing loss and all had absence of vestibular function to caloric stimulation on the affected side. CONCLUSIONS: Acute auditory syndrome may be a warning sign of impending pontocerebellar infarction in the distribution of the AICA. The acute auditory syndrome preceding an AICA infarct may result from ischaemia of the inner ear or the vestibulocochlear nerve.
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9/60. facial nerve anomalies in association with congenital hearing loss.

    OBJECTIVE: facial nerve (FN) anomalies are exceedingly rare. The association between a hearing loss and an anomalous facial nerve has never been touched deeply in the literature. The aim of this study is to report facial nerve anomalies in patients presenting with congenital hearing loss (COHL) and/or facial nerve palsy. methods: A retrospective chart review over 10 years from two academic tertiary referral centers. Eight patients were diagnosed with facial nerve anomalies in association with congenital hearing loss. The age ranged from 7 months to 13 years. RESULTS: Five patients had conductive congenital hearing loss (CCHL). Two had congenital sensorineural hearing loss (CSNHL). The eighth patient had a mixed hearing loss (MHL). Surgical findings for patients with conductive congenital hearing loss included facial nerve passing inferior to oval window (OW) (two patients), facial nerve covering head of stapes (three patients), deformed stapes (two patient), deformed incus (two patient), and absent incus (one patient). Radiological findings for patients with congenital sensorineural hearing loss and mixed hearing loss include, single cavity anomaly (one patient), dilated vestibule and superior semicircular canal (one patient), and absent internal auditory canal (IAC) (two patients). For patients with conductive congenital hearing loss, the radiological findings consistency demonstrates a soft tissue opacification in the middle ear. CONCLUSIONS: Congenital hearing loss may be associated with facial nerve anomalies. Pediatric otolaryngologists should be cautious when exploring patients with conductive congenital hearing loss.
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10/60. Isolated monolateral neurosensory hearing loss as a rare sign of neuroborreliosis.

    lyme disease, or borreliosis, is a zoonosis transmitted by borrelia burgdorferi which also involves the central nervous system (CNS), in 15% of affected individuals, with the occurrence of aseptic meningitis, fluctuating meningoencephalitis, or neuropathy of cranial and peripheral nerves. Encephalopathy with white matter lesions revealed by magnetic resonance imaging (MRI) scans in late, persistent stages of lyme disease has been described. In this report, we describe a patient with few clinical manifestations involving exclusively the eighth cranial nerve, monolaterally and diffuse bilateral alterations of the white matter, particularly in the subcortical periventricular regions at cerebral MRI. This single patient study shows that the search for antibodies against Borrelia burgdoferi should always be performed when we face a leukoencephalopathy of unknown origin. An isolated lesion of the eighth cranial nerve can be the only neurologic sign in patients with leukoencephalopathy complicating lyme disease.
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