Cases reported "Hearing Disorders"

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1/5. hyperacusis: case studies and evaluation of electronic loudness suppression devices as a treatment approach.

    hyperacusis, as defined here, is a relatively rare condition in which the patient, with or without hearing loss, experiences severe loudness discomfort to everyday environmental sound levels. The case studies of 14 patients with severe hyperacusis are described; all wore passive attenuators (earplugs and/or earmuffs) in an attempt to alleviate their discomfort, frequently producing communication difficulties. These subjects were fitted binaurally with experimental electronic loudness suppression devices housed in in-the-ear casings. The devices supplied low-level amplification followed by an extreme form of amplitude compression for moderate or high-level inputs in an attempt to reduce loudness discomfort without reducing audibility. Many of the subjects were found to function with a wider dynamic range with the active devices compared with passive attenuators or the unoccluded ear, and most reported that they benefited from the devices in at least some listening situations.
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ranking = 1
keywords = loudness, hyperacusis
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2/5. Exaggerated acoustic reflex response in a patient with transient facial palsy and hyperacusis.

    An unusual acoustic reflex response was documented by immittance recording in a patient with acute facial palsy. The amplitude of the stapedial response to sustained stimulation was reduced when recorded from the affected side, suggesting the facial nerve was affected proximal to the stapedius muscle. In addition, the impedance change at onset of intense acoustic stimulation of the affected side was unusually large, regardless of which stapedius response was recorded. The large change was coincident with the reported hyperacusis in the stimulated ear. This suggested the cochlear nerve as site of involvement of hyperacusis in this case of facial palsy.
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ranking = 0.52680924481687
keywords = hyperacusis
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3/5. hyperacusis and williams syndrome.

    Abnormal sensitivity to environmental sounds is a short-term feature often observed following insertion of grommets. Here we describe a child with this symptom who was found to have williams syndrome, a condition in which hyperacusis is observed in 95 per cent of patients.
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ranking = 0.087801540802812
keywords = hyperacusis
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4/5. Bekesy comfortable loudness: supportive case studies.

    Two cases of radiographically or surgically confirmed acoustic neurinoma are presented. In each case a mild, unilateral sensorineural hearing loss was identified. Results of threshold Bekesy audiometry were negative while the Bekesy comfortable loudness (BCL) procedure indicated significant auditory adaptation in both patients.
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ranking = 0.65157032799799
keywords = loudness
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5/5. Auditory deficits and hearing loss associated with focal brainstem haemorrhage.

    Four cases of central pontine haemorrhage are described in which auditory dysfunction was documented. Two cases had a hearing loss, in one of which there was recovery of the low frequencies. This case provides support for the tonotopic organization of the auditory pathways in the caudal pontine area, with the lowest frequencies being encoded medially. In all cases, there were abnormalities of the auditory brainstem responses, wave V being consistently involved, while wave III was abnormal in only one patient. In three cases, the masking level differences and crossed acoustic reflex thresholds were abnormal. The ipsilateral reflex thresholds were normal at least on one side in all cases. In the patient with the most significant hearing loss, loudness recruitment, assessed both psychophysically and with the acoustic reflex thresholds, was evident. These data are interpreted in terms of there being damage to the medial superior olivary nuclei and trapezoid body involving both afferent and efferent fibres.
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ranking = 0.1303140655996
keywords = loudness
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