What are the recommended evaluation and treatment options for a patient with Paget's disease affecting the cochlea?

Paget's Disease Affecting the Cochlea: Evaluation and Management

Initial Diagnostic Evaluation

For patients with suspected Paget's disease affecting the cochlea, obtain comprehensive audiometry promptly to document the type and severity of hearing loss, as this is the most common otologic manifestation and occurs in 55% of unselected Paget's patients. 1

Audiometric Assessment

• Obtain a comprehensive audiologic examination including pure tone audiometry (air and bone conduction at 250-8000 Hz), speech audiometry, and tympanometry to characterize the hearing loss pattern 2, 3
• Expect to find sensorineural hearing loss (65% of cases), mixed hearing loss (35%), or rarely conductive loss 4
• Document baseline word recognition scores, as these guide rehabilitation decisions 2
• Use the Hearing Handicap Inventory for the Elderly-Screening (HHIE-S) as an effective screening tool - a score >8 increases the odds of detecting moderate hearing impairment (>40 dBHL) by 5.1-fold in Paget's patients 1

Laboratory Evaluation

• Measure alkaline phosphatase, calcium, 25-hydroxy-vitamin D, phosphate, and parathyroid hormone levels to confirm Paget's disease activity and exclude other metabolic bone diseases 5
• Alkaline phosphatase serves as a marker of disease activity and treatment response 6

Imaging Studies

• Obtain temporal bone CT scan to document the extent and pattern of temporal bone involvement 4
• Temporal bone involvement typically begins in the petrous apex and progresses laterally, with otic capsule demineralization occurring late in the disease process 4
• Consider MRI if there are focal neurological abnormalities, asymmetric hearing loss, or concern for retrocochlear pathology 2, 3
• CT is specifically appropriate in Paget's disease due to known bone involvement, unlike routine hearing loss evaluation where it should be avoided 2, 3

Treatment Algorithm

For Progressive Hearing Loss

The evidence for medical therapy preventing hearing loss progression in Paget's disease is limited and conflicting. 7, 6

• Bisphosphonates (such as risedronate or etidronate) combined with calcitonin may stabilize or even reverse hearing loss in some patients, based on older case reports showing halted progression over 3.5-5 years 6
• However, one recent case demonstrated that potent bisphosphonates (oral risedronate for 6 months) failed to prevent hearing loss progression 7
• Despite uncertain efficacy for hearing preservation, bisphosphonates remain indicated for managing the underlying bone disease 5

Amplification Strategy

For patients with moderate hearing loss (>40 dBHL), refer to audiology for hearing aid evaluation regardless of whether medical therapy is initiated. 3

• Hearing aids improve communication, quality of life, and may delay cognitive decline 3
• Address the high rate of hearing handicap in Paget's patients - 41% report moderate-severe handicap compared to 0% of age-matched controls 1
• Paget's patients are significantly more likely to have difficulties hearing normal speech, watching TV, or hearing over background noise 1

Cochlear Implantation Criteria

For patients with severe-to-profound sensorineural hearing loss who have appropriately fitted amplification but continue to have poor speech understanding, refer for cochlear implant evaluation. 3, 7, 8

• Cochlear implantation is highly effective in Paget's disease, with documented cases achieving 74-100% word recognition scores in quiet conditions 7, 8
• Successful electrode insertion (Nucleus 24 Contour array) has been achieved without surgical complications despite abnormal temporal bone architecture 7, 8, 4
• Do not delay referral until hearing is profound - earlier intervention improves outcomes 3
• Two patients in the largest temporal bone series underwent successful cochlear implantation with open-set speech recognition 4

Monitoring and Follow-Up

Audiometric Surveillance

• Reassess hearing at least every 2-3 years, or more frequently if symptoms progress 2, 3
• Paget's patients demonstrate hearing loss progression at rates greater than expected for normal aging 4
• Long-term audiometric follow-up documents that hearing deterioration continues despite stable bone disease 4

Associated Symptoms to Monitor

• Evaluate for tinnitus (present in 19% at presentation) - if persistent and bothersome, recommend cognitive-behavioral therapy rather than medications or supplements 2, 9
• Screen for dizziness/vestibular symptoms (present in 52% at presentation) 4
• Monitor for cranial neuropathies, including facial nerve involvement and other compressive symptoms 4
• Maintain vigilance for neoplastic transformation, though rare (4% in one series) 4

Common Pitfalls to Avoid

• Do not dismiss hearing complaints in Paget's patients - they significantly under-report hearing problems that can be ameliorated 1
• Do not rely solely on patient self-report - 41% of Paget's patients with moderate hearing loss and 8% with severe-profound loss had HHIE-S scores suggesting no handicap 1
• Do not routinely prescribe antidepressants, anticonvulsants, or dietary supplements (Ginkgo biloba, melatonin, zinc) for associated tinnitus, as evidence does not support their use 2, 9
• Do not delay cochlear implant referral until hearing is profound - candidacy criteria have expanded and earlier intervention improves outcomes 3
• Involve family members in counseling as they are significantly affected by communication challenges 3