In a patient with autosomal dominant polycystic kidney disease who has large ears but no hearing problems, are the ears a manifestation of the disease and do they require any specific work‑up or treatment?

Large Ears in ADPKD: Not a Disease Manifestation

Large ears are not a recognized manifestation of autosomal dominant polycystic kidney disease and require no specific work-up or treatment related to ADPKD. 1

Evidence from Comprehensive ADPKD Guidelines

The 2025 KDIGO guidelines provide an exhaustive catalog of extrarenal manifestations in ADPKD, and large ears are conspicuously absent from this comprehensive list. 1

Documented Extrarenal Manifestations

The established extrarenal features of ADPKD include: 1

Intracranial manifestations:

• Intracranial aneurysms (12.9% of patients) 1
• Arachnoid cysts (8-15%) 1
• Intracranial arterial dolichoectasia (0.7-5%) 1

Cardiovascular manifestations:

• Mitral valve prolapse (3-26%, though recent studies suggest prevalence similar to general population at 3.4% in adults) 1
• Pericardial effusion (~20%) 1
• Thoracic aortic aneurysm (~1.5%) 1
• Cardiomyopathy (rare) 1

Hepatobiliary manifestations:

• Polycystic liver disease (>80% by age 30 years, most common extrarenal manifestation) 1, 2
• Pancreatic cysts (~10%) 1

Other manifestations:

• Abdominal wall hernias (common) 1
• Splenic cysts (~7%) 1

The Single Case of Hearing Loss

Only one isolated case report from 1999 documented familial sensorineural hearing loss in association with ADPKD in a single pedigree. 3 This represents an extremely rare occurrence that has not been validated in subsequent literature or incorporated into clinical guidelines. Importantly, this report described hearing loss, not ear morphology abnormalities. 3

Clinical Approach

For your patient with large ears and no hearing problems:

• No ADPKD-specific evaluation is needed for the ear finding itself 1
• Large ears are a common normal variant in the general population and should be evaluated as they would be in any patient without ADPKD
• Focus clinical attention on the established manifestations that actually affect morbidity and mortality in ADPKD 1

What Actually Requires Surveillance

Blood pressure monitoring: Hypertension affects 70-80% of ADPKD patients and requires aggressive control (target systolic <120 mm Hg, or <110/75 mm Hg for high-risk patients) 2, 4

Kidney function tracking: Use Mayo Imaging Classification with height-adjusted total kidney volume to stratify progression risk 1, 2, 4

Intracranial aneurysm screening: Consider in patients with family history of ICA or subarachnoid hemorrhage, personal history of aneurysm rupture, high-risk profession, or strong patient preference after counseling 1

Liver cyst monitoring: Assess for symptomatic polycystic liver disease, particularly in women where it is more common and severe 1, 2

Key Pitfall to Avoid

Do not attribute every physical finding in an ADPKD patient to their kidney disease. ADPKD has a well-defined spectrum of extrarenal manifestations documented through decades of systematic study. 1 Features outside this spectrum should be evaluated as they would be in the general population, avoiding unnecessary testing and patient anxiety.