Cochlear implantation in a patient with profound hearing loss with the A1555G mitochondrial DNA mutation and no history of aminoglycoside exposure.

The A1555G mitochondrial deoxyribonucleic acid (mtDNA) point mutation has classically been associated with sensorineural hearing loss in patients following aminoglycoside exposure. More recently, the mutation has been implicated in sensorineural hearing loss in patients without previous aminoglycoside use. In addition, cochlear implantation has been shown to be effective in the group of patients with prior aminoglycoside exposure but, to date, no case of cochlear implantation in a patient with the A1555G mutation and no prior exposure to aminoglycosides has been explicitly described in the literature. We report the case of an 80-year-old woman with the A1555G mtDNA mutation, a 35-year history of bilateral progressive hearing loss and no history of aminoglycoside exposure who underwent successful implantation of a Nucleus 24 Contour device at our institution. Post-operatively, the patient exhibited marked improvement in tests of auditory performance. We conclude that cochlear implantation can be an effective method to restore some sense of hearing in patients with the A1555G mtDNA mutation and sensorineural hearing loss.

Rhabdomyosarcoma presenting as an anterior neck mass and possible thyroid malignancy in a seven-month-old.

A seven-month-old male presented with a one month history of an enlarging left neck mass and worsening inspiratory stridor. Upon excision of the mass, pathologic examination was consistent with embryonal rhabdomyosarcoma (RMS). Preoperative imaging and intraoperative exploration were consistent with tumor replacing the left lobe of the thyroid. No cases of either anterior neck rhabdomyosarcoma or thyroid rhabdomyosarcoma have been explicitly described in the literature. The distinction between the two malignancies becomes important when considering prognosis and treatment protocols.