3p deletion syndrome: implications for cochlear implantation.

OBJECTIVE AND

IMPORTANCE

To describe the role of cochlear implantation in patients with 3p deletion syndrome. Additionally, this paper will review non-mastoid approaches to the cochlea potentially used in cases of mastoid abnormalities.

CLINICAL PRESENTATION: A 23-month-old child with 3p deletion syndrome (3pDS) presented with suspected hearing loss and characteristic craniofacial abnormalities. Bilateral sensorineural hearing loss (SNHL) was confirmed. Temporal bone computed tomography showed poorly developed mastoids bilaterally.

INTERVENTION: Cochlear implantation was successfully undertaken using a retro-auricular transmeatal approach.

CONCLUSION

SNHL is commonly encountered in patients with 3pDS and can be improved by cochlear implantation. In this patient population, particular attention must be given to pre-operative imaging and possible altered mastoid anatomy may exist. In doing so, intra-operative complications can be avoided.