The Bones That Speak: Diagnosing Paget's Disease Behind Years of Headache and Hearing Loss

A 68-year-old Bahraini woman presented to our clinic with a two-year history of persistent headaches, tinnitus, and progressive bilateral hearing loss. Initial examination revealed frontal bossing — a subtle but telling physical sign. Investigations pointed toward an unexpected diagnosis: Paget's Disease of Bone.

Laboratory findings were striking — serum alkaline phosphatase was markedly elevated at 854 IU/L, while calcium, phosphorus, and vitamin D levels were within acceptable ranges. CT brain imaging demonstrated expansion of the diploic bone, highly characteristic of Paget's disease. Bone scintigraphy confirmed active lesions in the skull and pelvis. DEXA scan revealed a T-score of -4.0, indicating severe osteoporosis.

Paget's Disease of Bone is a chronic disorder of bone remodelling — characterised by excessive, disorganised bone resorption followed by accelerated but structurally inferior new bone formation. The resulting bone is larger, more vascular, and more vulnerable to fracture. While the condition is most common in individuals of Northern European descent, sporadic cases occur globally. Neurological manifestations — including headache, hearing loss, and cranial nerve compression — arise when pagetic bone encroaches on adjacent neural structures.

The patient was treated with intravenous Zoledronic acid, addressing both the Paget's disease and her underlying age-related osteoporosis. Her headaches resolved significantly, and alkaline phosphatase normalised to 135 U/L on follow-up — a remarkable clinical response reflecting the power of accurate diagnosis and targeted treatment.

Key Learning Points:

• Persistent headache and hearing loss in an elderly patient warrant investigation for skeletal pathology.
• Frontal bossing and markedly elevated alkaline phosphatase are key diagnostic clues for Paget's Disease.
• Zoledronic acid is highly effective for both Paget's Disease and concurrent osteoporosis.
• Untreated Paget's Disease can lead to fractures, high-output cardiac failure, and rarely, bone sarcoma.

 

Credits: Case managed and reported by Dr. Faisal, Consultant, Bahrain Specialist Hospital