Bilateral sensorineural hearing disorders in children: etiology of deafness and evaluation of hearing tests

Objective: The purpose of this study was to determine the etiology of bilat eral sensorineural hearing disorders in children and to evaluate the perfor med hearing tests by comparison of the results of the objective and subject ive tests. Methods: The medical history and the hearing tests (behavioral o bservation audiometry, acoustic evoked potentials and pure tone audiometry) of 106 bilaterally hearing impaired children were analyzed in a retrospect ive follow-up study. Results: The total group included 52 males and 54 fema les. The ages at first diagnosis ranged from 4 months to 11 years with a me an age of 42 months and a median of 33 months. The degree of hearing loss f or the better hearing ear was mild in one child, moderate in 28 children, s evere in 29 children, profound in 32 children and total in 16 children. The delay between the first examination and diagnosis ranged from 0 to 597 day s with a mean of 83 days and a median of 28 days. In 47 children (44%) no c ause of hearing impairment could be determined. Nineteen children (18%) had a history of familial hearing loss, 40 (38%) suffered from acquired hearin g loss (seven children had prenatal causes, 21 perinatal and 12 postnatal). A comparison between behavioral observation audiometry and brainstem evoke d response audiometry revealed a statistically good agreement. Twenty-nine children (32%) showed progressive hearing loss, which was defined as a thre shold shift of +10 dB or more in the pure tone average in at least one ear. Conclusions: In a significant number of children with early hearing impair ments the etiology still remains uncertain. Further research in the field o f genetic disorders will diminish this number. Evaluation of hearing tests showed that behavioral observation audiometry still is an excellent tool in the hands of an experienced examiner. The age at identification of hearing disorders in industrialized countries still is unacceptably high. To obtai n ideal care of hearing impaired children, universal neonatal hearing scree ning programs are mandatory. (C) 2000 Elsevier Science Ireland Ltd. All rig hts reserved.