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Cochlear implant in Wolfram syndrome: A case report
Hetal Marfatia1, Anav Rattan1, Anushka Jain1
1Department of ENT and Head & Neck Surgery, Seth GS Medical College & K.E.M. Hospital, Mumbai, India.
Cochlear Implants International
|December 24, 2024
Summary
Cochlear implantation significantly improved hearing in a patient with Wolfram syndrome (DIDMOAD), a rare genetic disorder. This intervention offers a viable solution for hearing loss associated with this condition.
Area of Science:
- Genetics
- Otolaryngology
- Endocrinology
Background:
- Wolfram syndrome (DIDMOAD) is a rare autosomal recessive disorder.
- It is characterized by diabetes insipidus, juvenile diabetes mellitus, optic nerve atrophy, and deafness.
- Progressive hearing loss is a common and debilitating symptom.
Purpose of the Study:
- To report a case of cochlear implantation in a patient with Wolfram syndrome.
- To evaluate the effectiveness of cochlear implantation for hearing loss in this condition.
Main Methods:
- A 21-year-old male with Wolfram syndrome and progressive bilateral hearing loss underwent cochlear implantation.
- Audiometry confirmed severe to profound sensorineural hearing loss.
- A Nucleus Profile™ CI 632 cochlear implant was used.
Main Results:
- The patient experienced significant hearing improvement post-implantation.
- Three months post-implantation, a speech discrimination score of 90% was achieved.
- Hearing aids had previously provided minimal benefit.
Conclusions:
- Cochlear implantation is an effective treatment for high-frequency sensorineural hearing loss in Wolfram syndrome.
- Hearing rehabilitation should be considered to enhance the quality of life for affected individuals.
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