HEARING IMPAIRMENT IN ASSOCIATION WITH DISTAL RENAL TUBULAR-ACIDOSIS AMONG SAUDI CHILDREN

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