Rare case of bilateral aural atresia and cochlear dysplasia: when cochlear implantation is not the answer.

OBJECTIVE AND

IMPORTANCE

Reports of patients with concurrent middle and inner ear anomalies are rare. These patients present a surgical challenge for cochlear implantation. The surgical risk must be weighed against the predicted benefit of the patient's hearing outcome and subsequent development of speech and language as well as their quality of life.

CLINICAL PRESENTATION: Thirteen-year-old boy presented to the Otology clinic for auditory rehabilitation options. He has mild developmental delay, is non-verbal and communicates via American Sign Language. He was born with bilateral aural atresia and never wore amplification. On exam he has grade 1 microtia and complete ear canal atresia bilaterally. His behavioural hearing test shows profound sensorineural hearing loss of both ears. The computed tomography scan shows bilateral underdeveloped and completely opacified mastoid and middle ear, complete bony atresia of the ear canals, and an under-partitioned cochlea with poorly defined modiolus, among other abnormalities. The patient and his family were counselled on the available options as well as the need for any further studies.

INTERVENTION: Counselling of patient and family.

CONCLUSION

While there have been reports in the literature of performing cochlear implantations in patients with a concurrent atresia and cochlear dysplasia, these were patients whose degree of inner ear anomalies was relatively minor and their prognosis of a good audiological outcome was favourable. The presented case is that of a patient for whom the surgical approach to the cochlea alone would be difficult. More importantly, his quality of life would not significantly improve in light of the predicted limited hearing and language development outcomes, given the severity of his inner ear abnormalities, limited communication abilities, prolonged period of deafness and developmental delays.