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Human Nonsyndromic Sensorineural Deafness
Annual Review of Genomics and Human Genetics, 2003Given the unique biological requirements of sound transduction and the selective advantage conferred upon a species capable of sensitive sound detection, it is not surprising that up to 1% of the approximately 30,000 or more human genes are necessary for hearing. There are hundreds of monogenic disorders for which hearing loss is one manifestation of a
Thomas B, Friedman, Andrew J, Griffith
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Genetic Sensorineural Deafness in Adults
Annals of Otology, Rhinology & Laryngology, 1975Progressive genetic sensorineural hearing loss is a common problem in adults which is frequently incorrectly diagnosed or escapes diagnosis as to etiology altogether. If the hearing problem becomes manifest in the twilight of the patient's life it is often identified with the nondescriptive term “presbycusis.” In all patients with sensorineural ...
M M, Paparella +3 more
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Unilateral Sensorineural Deafness in Children
Otolaryngology–Head and Neck Surgery, 1988Heredity, viral infection, and head or acoustic trauma are considered the common etiologies for a unilateral sensorineural deafness in children. The incidence of perilymphatic fistula in a unilateral hearing loss is still unknown. Inner ear‐related symptoms in children are scarce, and little diagnostic laboratory testing is available.
I H, Kielmovitch, W H, Friedman
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Ocular albinism with sensorineural deafness
International Journal of Dermatology, 2004Ocular albinism (OA) is an inherited disorder characterized by partial or complete absence of pigment in the eyes. Ocular albinism is inherited in two patterns, X‐linked and autosomal pattern. X‐linked OA includes type I OA (Nettership‐Falls type), type II OA (Forsius‐Eriksson type), and OA with late‐onset sensorineural deafness.
Woo-Jung, Tak +5 more
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[Hereditary sensorineural deafness].
La Revue du praticien, 2000Deafness is the most common sensory defect. The investigation of the cause of deafness is critical for genetic counselling, and sometimes for appropriate management of associated pathologies. About two thirds of cases of congenital deafness are genetic forms, and the proportion is probably similar concerning the forms of deafness that appears during ...
Denoyelle, Françoise +3 more
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Electrocochleography (Ecog) In Sensorineural Deafness
Acta Oto-Laryngologica, 1975We examined 340 normal ears and cases of sensorineural deafness with electrocochleography using click stimuli (duration: 0.5 standard deviation of a population; repetition rate: plus or minus 10/sec; N=1 000, alternately positive and negative; analysis time: 31 standard deviation of a population).
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Sensorineural Deafness Due to Osteitis Fibrosa
Archives of Otolaryngology - Head and Neck Surgery, 1976A 91-year-old woman with deafness died from renal failure. Autopsy revealed osteitis fibrosa cystica generalisata, chronic myeloid leukemia, renal atrophy, and hyperplastic parathyroid glands. The temporal bones showed senile osteoporosis, osteitis fibrosa, and chronic myeloid leukemia.
J R, Lindsay, F, Suga
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Autosomal dominant ectrodactyly with sensorineural deafness
Clinical Dysmorphology, 2000Ectrodactyly or split hand/split foot malformation as it is now known, is a rare developmental disorder sometimes associated with other systemic malformations. Very few cases of congenital hearing loss coexisting with ectrodactyly have been reported world-wide.
P, Mishra, M, Muranjan, B A, Bharucha
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Sensorineural deafness associated with IgA nephropathy
The Journal of Laryngology & Otology, 1989AbstractIgA nephropathy is an immune complex glomerulonephritis in which examination of the renal biopsy by immu-nofluorescence reveals IgA as the predominant antibody deposited in the glomerular mesangium (Clarkson et al., 1984). It was first described by Berger in 1969.
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Contemporary review of the causes and differential diagnosis of sudden sensorineural hearing loss
International Journal of Audiology, 2019Objectives: This article provides an overview of the causes and differential diagnosis of sudden deafness (SD) and sudden sensorineural hearing loss (SSHL). Design: Contemporary review.
Y. Young
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