難聴を伴なう家族性腎疾患の症例
1972 年 15 巻 2 号 p. 96-101
詳細
1972 年 15 巻 2 号 p. 96-101
The patient was a 15 years old female. Many members of her family are suffering from renal diseases.
At the age of 7, she was attacked by rheumatic fever and her tonsils were removed. But at the age of 9, she developed nephritis.
Two years later, her hearing was progressively impaired and at present she has used the hearing aid.
At the age of 13 she was referred to our department.
Laboratory findings-
Proteinuria was remarkable, but no hematuria. Examination by the contrast medium (Hippuran) showed that the right kidney was smaller than left one, and the left kidney had two pelvises and ureters. Renal biopsy proved “lupus nephritis”.
Audiologic findings-
In the first audiometry at our department, at the age of 15, the average pure tone threshold was 48dB (r), 54dB (l), but speech discrimination score was high (84%). SISI test was 100% (1, 000Hz and 4, 000Hz), TTS was negative, and sensorineural deafness was diagnosed.
Thereafter, photogenic epilepsy (the spasm of limbs) and meningitic signs were repeated several times. She suffered from subacute retrobulbar neuritis and her vision was lowered.
Lately, she was admitted to the Department of Pediatrics because of renal insufficiency. Her hearing loss was deteriorated and became actually deaf without hearing aid.
