Value of systematic aetiological investigation in children with sensorineural hearing loss

SummarySensorineural hearing loss is the most common form of sensory impairment in children. As a precise aetiological diagnosis has major prognostic and management implications, it is useful to evaluate the contents of the aetiological investigation of sensorineural hearing loss in France. This article presents a retrospective review of professional practices by comparing the aetiological investigation of hearing loss in children with a cochlear implant and children without a cochlear implant.Patients and methodsOne hundred and seven children under the age of 18 years with unilateral or bilateral sensorineural hearing loss attending the paediatric ENT department for the first time between January 2007 and January 2009 were included in the study. Data from the clinical interview and all complementary investigations were analysed.ResultsThe various aetiologies of hearing loss were classified as genetic, acquired, or unknown in each of the two populations. Hearing loss was of unknown origin in 52% of the 87 non-implanted children and 15% of the 20 children with a cochlear implant.ConclusionThis study demonstrates the heterogeneous practices in terms of aetiological investigation of sensorineural hearing loss as a function of the target population. A more systematic aetiological investigation was performed in children fitted with a cochlear implant, requiring multidisciplinary management. This study indicates the need to define a standard aetiological investigation for all children with sensorineural hearing loss.