Cochlear implant in Wolfram syndrome: A case report

Wolfram syndrome, a rare autosomal recessive disorder, is characterised by diabetes insipidus, juvenile diabetes mellitus, optic nerve atrophy and deafness (DIDMOAD).

We present a case of a 21-year-old male diagnosed with Wolfram syndrome who underwent cochlear implantation due to progressive hearing loss. The patient first complained of bilateral hearing loss at the age of 8 years. As the hearing loss progressed hearing aids provided minimal benefit. A multidisciplinary team evaluated his extensive medical history, which included juvenile-onset diabetes mellitus, seizures, vision abnormalities and hypergonadotrophic hypogonadism. Pure tone audiometry was done which showed sloping bilateral severe to profound hearing loss, more at higher frequencies. Hrct and Mri temporal bone showed normal cochlear architecture and cochlear nerve. The patient received a Nucleus Profile™ CI 632 cochlear implant. Postoperative evaluation revealed significant improvement, with a speech discrimination score of 90% at the most comfortable level three months post-implantation.

Wolfram syndrome is a rare genetic disorder with multisystem involvement and debilitating symptoms. High-frequency sensorineural hearing loss is a common association and hearing rehabilitation using hearing aids and cochlear implants must be considered to improve the quality of life.