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Hearing impairment in association with distal renal tubular acidosis among Saudi children

Published online by Cambridge University Press:  29 June 2007

Siraj M. Zakzouk ,
Samia H. Sobki ,
Faizeh Mansour  and
Fatma H. Al Anazy
Siraj M. Zakzouk*
Affiliation:
Departments of Otorhinolaryngology. Riyadh, Saudi Arabia.
Samia H. Sobki
Affiliation:
Departments of Chemical Pathology. Riyadh, Saudi Arabia.
Faizeh Mansour
Affiliation:
Departments of Paediatric Nephrology, Riyadh, Saudi Arabia.
Fatma H. Al Anazy
Affiliation:
Departments of Otorhinolaryngology. Riyadh, Saudi Arabia.
*
Professor Siraj Zakzouk, Department of ORL, King Saud University, PO Box 245, Riyadh 11411, Saudi Arabia.
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Abstract

A follow-up of seven patients with the autosomal recessive inherited syndrome of distal renal tubular acidosis (RTA) and sensorineural hearing loss is described. Five patients were diagnosed as having primary distal renal tubular acidosis and rickets, four were found to have severe sensorineural hearing loss of over 80 dB: two of which are brothers. Two patients were diagnosed as having secondary distal renal acidosis due to a genetic disorder called osteopetrosis; they are brothers and their audiograms showed a mild conductive hearing loss of an average 35 dB bilaterally. All patients had growth retardation with improvement due to alkaline therapy but their hearing loss was not affected by the medication. The pedigrees of two families with half sibs showed the familial incidence for consanguineous marriage. Consanguinity was found to be positive in five out of the seven patients. The tribal tradition in Saudi Arabia fosters consanguineous marriages for cultural and social reasons and pre-arranged marriages are still seen.

Keywords

Acidosisrenal tubularHearing losssensorineuralConsanguinity

Information

Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 109 , Issue 10 , October 1995 , pp. 930 - 934
Copyright
Copyright © JLO (1984) Limited 1995

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