Differential Diagnosis

The patient's presentation of elevated renal profile, hematuria, family history of renal failure, and bilateral hearing loss suggests a hereditary condition affecting the kidneys and hearing. Here's a categorized differential diagnosis:

• Single most likely diagnosis

  • C. Alport syndrome: This condition is characterized by hereditary nephritis, hearing loss, and sometimes eye abnormalities. The family history of renal failure and the patient's symptoms align closely with Alport syndrome, making it the most likely diagnosis.

• Other Likely diagnoses

  • D. IgA nephropathy: Although IgA nephropathy is a common cause of hematuria and renal failure, it doesn't typically cause hearing loss. However, it could be considered if the family history of renal failure is not strongly linked to a hereditary condition.
  • A. Granulomatosis with polyangiitis: This is a form of vasculitis that can affect the kidneys and cause renal failure, but it's less likely given the family history and the specific combination of symptoms.

• Do Not Miss

  • B. Eosinophilic granulomatosis with polyangiitis: Although less common, this condition can cause renal failure and should be considered, especially if there are other systemic symptoms. However, the hearing loss is not a typical feature.
  • Other systemic vasculitides or conditions that could lead to renal failure and have a familial component, albeit rare, should be kept in mind to avoid missing a potentially treatable condition.

• Rare diagnoses

  • Other hereditary conditions that affect the kidneys and hearing, such as Branchio-Oto-Renal syndrome, although these are less common than Alport syndrome and might not fit as well with the described family history of renal failure on hemodialysis.
  • Fabry disease, a lysosomal storage disorder that can cause renal failure, hearing loss, and has a hereditary pattern, but it typically includes other systemic symptoms like skin lesions and neuropathic pain.

Each of these diagnoses should be considered in the context of the patient's full clinical picture, including further diagnostic tests such as genetic testing, audiometry, and renal biopsy, to confirm the diagnosis.