I have autosomal dominant polycystic kidney disease (ADPKD) and notice my ears are unusually large but I have normal hearing and no ear symptoms—does this require any specific evaluation or treatment?

Large Ears in ADPKD: No Specific Evaluation or Treatment Required

Large ears are not a recognized extrarenal manifestation of ADPKD and do not require any specific evaluation or treatment in the absence of hearing loss or other ear symptoms. 1

Why This Is Not a Concern

The most recent and comprehensive KDIGO 2025 ADPKD guidelines provide an exhaustive catalog of extrarenal manifestations across multiple organ systems, and ear abnormalities (beyond rare hearing loss) are notably absent from this list. 1

The documented extrarenal manifestations of ADPKD include:

Vascular Manifestations

• Intracranial aneurysms (12.9% prevalence) 1
• Thoracic aortic aneurysm (~1.5%) 1
• Arterial dissections (rare) 1

Cardiac Manifestations

• Mitral valve prolapse (3-26%, though recent studies suggest only 3.4% using current definitions) 1
• Pericardial effusion (~20%) 1
• Cardiomyopathy (rare) 1

Hepatobiliary and Abdominal

• Polycystic liver disease (>80% by age 30) 1, 2
• Pancreatic cysts (~10%) 1
• Abdominal wall hernias (common) 1

Neurological

• Arachnoid cysts (8-15%) 1, 3
• Meningeal cysts (rare) 1

Other Rare Manifestations

• Situs inversus and cardiac transposition (rare, mostly PKD2) 1
• Congenital heart malformations (rare) 1

The Hearing Loss Connection

While one isolated 1999 case report described a family with ADPKD associated with bilateral sensorineural hearing loss across four generations, this represents an extremely rare and likely coincidental association rather than a true ADPKD manifestation. 4 Importantly, you have normal hearing, which makes this even less relevant to your situation. 4

What You Should Actually Monitor

Instead of focusing on ear size, your ADPKD management should prioritize:

Blood Pressure Control

• Target systolic <120 mm Hg for most patients 5, 6
• Even lower targets (110/75 mm Hg) for high-risk patients with rapid progression 6

Intracranial Aneurysm Screening (If Indicated)

• Consider screening if you have a family history of intracranial aneurysms or subarachnoid hemorrhage 1
• Prevalence is 12.9% in ADPKD population 1

Cardiac Evaluation (If Indicated)

• Echocardiography should be considered if you have severe/uncontrolled hypertension, heart murmur, cardiac symptoms, or family history of thoracic aortic aneurysm 1

Liver Cyst Monitoring

• Liver cysts are present in >80% of ADPKD patients by age 30 2
• Symptomatic polycystic liver disease affects <5%, predominantly females 1

Bottom Line

Your large ears are almost certainly unrelated to your ADPKD diagnosis and require no specific action. 1 Focus your clinical attention on the well-established extrarenal manifestations that actually impact morbidity and mortality in ADPKD, particularly cardiovascular complications and disease progression monitoring. 1, 6