Résumé
The hearing loss is a problems of public health which can be described as major from the economic and human point of view, since 1 child over 1000 has at the birth a severe or profound deafness. This deafness has a prevalence of 1 per 10 in certain Tunisian geographical isolates. Our work concerned 33 patients with nonsyndromic sensorineural deafness who are regarded as probands and are recruited in service ORL of the Hospital La Rabta of Tunis. For each patient, a family investigation was carried out in residence and complete pedigrees were established. In addition, we selected a sample of 320 unaffected subjects for whom we established pedigrees in order to compare their inbreeding coefficient with that of the patients The samples of this study are orginated from the governorate of Bizerte The segregation analysis by the method of the maximum likelihood applied to the sample of deafs allowed to estimate the segregation frequency by taking account of various modes of selection in the sibships. The results obtained are compatible with autosomic recessive model with complete penetrance of the gene. The recessive hypothesis is strenghthened by the increase in the inbreeding coefficient of the probands compared to that of the controls