Cases reported "Hearing Loss, Bilateral"

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1/5. Useful residual hearing despite radiological findings suggestive of anacusis.

    A severe malformation of the inner ear, often referred to as severe labyrinthine dysplasia or common cavity deformity, consists of an absent or dilated cochlear basal coil, wide communication with the vestibule and a tapered internal acoustic meatus and can be associated with absent hearing. We discuss two children with severe labyrinthine dysplasia as shown by computed tomography (CT) scans and, in the first case, an absent VIIIth nerve bilaterally shown by magnetic resonance imaging (MRI). In 1995, both cases were precluded from cochlear implantation, on the basis of the absent VIIIth nerve (first case) and increased risk of CSF leak during operation (second case). However, audiometric results and vocalization patterns of both children suggested the presence of some residual hearing function, while recently reported specific surgical techniques have been found to be safe and effective in the cochlear implantation of the common cavity deformity. The management of such cases should be decided on the grounds of a full audiological assessment in conjunction with the radiological features, in the light of current surgical trends shown to be safe and effective.
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ranking = 1
keywords = communication
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2/5. Transient musical hallucinosis of central origin: a review and clinical study.

    A 52 year old, right handed, hearing impaired woman was admitted with headache and neck stiffness. The only neuropsychological symptom was transient auditory perceptions in the left ear, which were musical, seemed familiar and were not influenced by verbal communication. CT and MRI showed a right subarachnoid haemorrhage, while brainstem auditory evoked potentials failed to reveal a brainstem lesion. In patients with organic cerebral disease, unilateral auditory hallucinations (AHs) may indicate a lesion in the contralateral hemisphere. However, according to this review the type of AHs (verbal versus musical) is not consistently associated with a cerebral lesion on either side.
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ranking = 1
keywords = communication
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3/5. Surgical and prosthetic restoration of binaural hearing in an 88-year-old man.

    We report the restoration of binaural hearing in an 88-year-old man who was experiencing significant communication difficulties. His preoperative air conduction thresholds had revealed a pure-tone average of 55 dB for the left ear and 107 dB for the right, and he was dissatisfied with the benefits provided by his BICROS hearing aid arrangement that had been fitted to his left ear. The combined effects of surgery and a hearing aid offered a 70 to 80 dB improvement for his right ear and restored the communication advantages of binaural hearing.
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ranking = 2
keywords = communication
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4/5. Immune complex-associated deafness: preliminary communication.

    During the last year patients presenting with progressive bilateral or sudden sensorineural deafness of unknown aetiology have been investigated for possible abnormal immune activity. Twenty-six cases are reported in which significantly raised circulating immune complexes were present, together with 2 cases of clinical autoimmune deafness where the complexes were normal. Thirty-six control subjects were also studied. Following a review of the clinical features, the pathogenesis of this new association with sensorineural deafness is discussed. In some cases the aetiology is probably autoimmune, and in others related to infection. In certain patients the deafness has been partly reversed by medical treatment with systemic steroids or plasma exchange. It is hypothesized that circulating immune complexes may reflect a previously unrecognized final common pathophysiological pathway in a variety of cochleovestibular disorders.
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ranking = 4
keywords = communication
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5/5. Isolated congenital internal auditory canal atresia with normal facial nerve function.

    The internal auditory canal forms as a result of mesoderm enveloping the eighth cranial nerve in the developing embryo. The mesoderm eventually transforms into cartilage and ultimately ossifies around the nerve, forming the internal auditory canal. It is theorized that atresia or stenosis of the internal auditory canal results from altered cochleovestibular nerve development secondary to faulty chemotactic mechanisms or a lack of end organ targets. Unilateral internal auditory canal anomalies are frequently seen in conjunction with other inner ear anomalies and occasionally with middle or external ear anomalies. Infrequently, it will occur as either an isolated or bilateral finding, but rarely simultaneously. The few citations of isolated, unilateral or bilateral internal auditory canal anomalies that are reported in the literature are usually associated with other systemic developmental anomalies, such as, cardiac septal defects, polycystic kidney disease, skeletal deformities and duodenal atresia. We present a case report of a patient with bilateral, congenital, internal auditory canal atresia and cochleovestibular deficits but, normal facial nerve function. A review of the literature is discussed as well as diagnostic considerations and treatment options including audiologic and communication rehabilitation.
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ranking = 1
keywords = communication
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