Bilateral otoacoustic emissions pass in a baby with Mondini deformity and subsequently confirmed profound bilateral hearing loss.

Evoked otoacoustic emissions are well established as a hearing screening technique and are used extensively in paediatric audiology. They are believed to originate from the outer hair cells and can be detected in almost 100% of normally hearing ears; even a mild hearing loss has been shown to abolish otoacoustic emissions. Results are presented of a baby boy born at 29 weeks' gestation requiring 77 days of neonatal care, but experiencing no complications following discharge from the neonatal unit. This child had clear bilateral evoked otoacoustic emissions at almost four months of age, but was subsequently found to have a profound bilateral hearing impairment and absent otoacoustic emissions. Radiological investigations revealed bilateral Mondini dysplasia, and this child has now been implanted with a multi-channel MXM Digisonic cochlear implant. He is progressing well and shows awareness of sound. Approximately 10 previous cases of otoacoustic emissions occurring in profoundly deaf ears have been reported in the literature. Although it is likely that this child's hearing loss was progressive in nature, the authors believe that this is the first reported case of otoacoustic emissions being recorded in the presence of Mondini dysplasia. This raises concerns about the use of neonatal screening in isolation without adequate mechanisms for later identification of hearing impairment, although it is acknowledged that it represents a rare situation.