Children with a bilateral sensorineural hearing impairment averaging 30 dBHL or or more were investigated for possible congenital or intrauterine infection as causes at the Centre for Audiology, Manchester University, UK. Of a total of 339 cases studied, 23% had a positive family history of deafness in parents or siblings (genetic group); 13% had suffered from birth asphyxia, respiratory distress syndrome, intraventricular hemorrhage, infections, or hyperbilirubinemia (perinatal group); 8% had congenital rubella or cytomegalovirus infection; 7% had a history of bacterial meningitis; 5% chromosomal anomalies; 5% were diagnosed with Waardenburg, Hurler, Hunter, Klippel Feil, or other syndromes; and 34% were of unknown cause. Autosomal recessive inheritance was probably responsible for most cases of unknown etiology. [1]
COMMENT. The authors are critical of the present screening arrangements for hearing impaired children. They advocate early identification by screening, not only in the at risk children but universally. Testing only ‘at risk’ children leads to identification of less than half those affected. Regular school screening should help in diagnosis, and genetic counselling might lead to a reduction of numbers in the largest etiological group of cases. Hearing tests are important in the neurologic evaluation of children presenting with learning or attention deficit disorders, especially in those with a positive family history of deafness or a prior risk factor illness.