Causes of pediatric sensorineural hearing loss: Yesterday and today

Objective: To ascertain the present common causes of sensorineural hearing loss (SNHL) in children and compare them with those of previous reports. Design: A retrospective review of the medical records for all children with a diagnosis of SNHL seen from January 1, 1993, through September 30, 1996, at our institution. Setting: A tertiary care children's hospital. Patients: Three hundred one children, aged 1 week through 18 years, who presented for evaluation of SNHL. Results: Of the 301 children, 68.1% had a definite or probable cause of their SNHL identified; 18.9%, 1 or more possible causes; and 31.9%, no obvious cause. A family history of SNHL or prematurity and/or complicated perinatal course was found in 28.6% of patients. Named syndromes, multiple congenital anomalies, meningitis, or pre- natal maternal factors, including maternal prenatal substance abuse, were present in another 38.5%. However, syndromes commonly reported to be associated with SNHL, such as Waardenburg syndrome, were seen in less than 1% of patients. The average age at diagnosis was 3.02 years for the bilateral moderate or worse SNHL; for unilateral SNHL, the average age was 3.97 years. The most useful diagnostic study was computed tomographic scanning. Conclusions: Sensorineural hearing loss is fairly common in children. Extensive workups, often without clear direction, should be reconsidered based on the children with SNHL who otolaryngologists are now seeing. Infant screening programs, although identifying many children earlier, will also provide the opportunity to fine-tune the evaluation (ie, cytomegalovirus titers and/or cultures at birth), increasing the diagnostic yield.