Background:
CHARGE syndrome is a genetic disorder resulting in the association of multiple congenital anomalies. The diagnosis of CHARGE syndrome is based on a combination of major and minor characteristics. Approximately half of individuals with CHARGE have severe to profound hearing loss.

Materials and Methods:
The authors present a case of a 4 year old child diagnosed with CHARGE syndrome that was referred to our department due to complete absence of expressive language and reaction to sounds. The protocol included: acoustic immittance, observation audiometry and click ABR on air and bone conduction under natural sleep and short duration general anesthesia.

Results:
The investigations revealed mixed profound hearing loss with middle ear effusion on the left side and severe sensorineural hearing loss on the right ear. The first option of treatment was classic bilateral hearing aid, after grommet insertion in the left tympanic membrane and effusion drainage.

Conclusions:
Any infant suspected of having CHARGE syndrome should undergo complete audiological examination since the frequency of sensory-neural hearing loss or vestibular problems are as high as 75-80%. Evaluation of hearing sensitivity during infancy and provision of amplification are important components in the process of auditory habilitation. Negligence in monitoring for auditory disabilities may result in increased developmental delay.