THE ASSOCIATION of hearing loss and hereditary chronic renal disease was first noted by Alport in 1927.1 Most of the subsequent work on Alport's syndrome concerns itself with investigation of the renal abnormality and the mode of inheritance. It is the purpose of this paper to present temporal bone and audiometric findings in a case of classic Alport's syndrome.
Report of a Case
A 22-year-old white man was admitted to the University of Minnesota Hospital on January 20, 1962 for investigation of chronic anemia and uremia. The patient was the product of a normal term pregnancy and delivery. At age 5 albuminuria was noted. At age 12, the patient became aware of a gradually progressive bilateral hearing loss. He reported occasional buzzing tinnitus but no vertigo. The family history included a maternal aunt who died age 23, and a maternal uncle who died age 14 both of "Bright's Disease."
Winter LE, Cram BM, Banovetz JD. Hearing Loss in Hereditary Renal Disease. Arch Otolaryngol. 1968;88(3):238–241. doi:10.1001/archotol.1968.00770010240005
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