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IntroductionCase reportConclusionWolfram 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. [ABSTRACT FROM AUTHOR]

Background and Objectives: Various ear, nose, and throat (ENT) conditions can result in vision loss. The purpose of this study is to identify the etiologies, presentations, and radiological findings associated with impaired vision in the context of ENT. Additionally, this article discusses management protocols, including optic nerve decompression and orbital decompression. Methods: In a retrospective study, we examined the period from 2016 to 2022 at a tertiary care hospital in Mumbai, India. The analysis included 11 patients who presented with progressive diminution of vision. All patients received a regimen of broad-spectrum intravenous antibiotics and high-dose intravenous steroids. This was followed by either endoscopic optic nerve decompression or orbital decompression. Subsequent improvements in vision were documented, and any complications were evaluated. Results: A total of 11 patients were treated with medical management followed by successful surgery, with 10 patients demonstrating significant vision improvement. Conclusion: Identifying the etiology of vision loss and managing the condition can present challenges for otorhinolaryngologists. A thorough grasp of the underlying pathophysiology, combined with active surveillance of clinical and radiological indicators, can enable these clinicians to achieve effective and rewarding outcomes. [ABSTRACT FROM AUTHOR]