[Late hereditary degenerative sensorineural hearing loss associated with IgA mesangial glomerulonephritis of probable autosomal dominant heredity].
We studied four generations of a Canary Islands family presenting a tardive heredodegenerative hearing loss, associated with IgA mesangial glomerulonephritis, of probable autosomal dominant heredity. With respect to the family, we revised Alport's syndrome, for possible transmission associated with X chromosome, as well as heredodegenerative hearing loss associated with renal pathology of autosomic transmission currently described; we differentiate these hearing losses from our case study, and we discuss the pathogeny of the auditive affection in the said hereditary syndromes. Lastly, we stress the autoimmune hypothesis because of the IgA nephropathy association in the family case, and we list the characteristics of the syndrome described.
de Serdio
J L
JL
Hospital Nuestra Señora de la Candelaria, Tenerife.
Chahin
J
J
Gil-Curbelo
J A
JA
Perera
A
A
Saavedra
J A
JA
D000293:Adolescent; D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D005799:Genes, Dominant; D005922:Glomerulonephritis, IGA / Q000235:genetics*; D006312:Hearing Loss, Bilateral / Q000235:genetics; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009394:Nephritis, Hereditary / Q000175:diagnosis; D010375:Pedigree; D013030:Spain; D013577:Syndrome
Hipoacusia neurosensorial heredodegenerativa tardía, asociada a glomerulonefritis mesangial IgA, de probable herencia autosómica dominante.