The Effect of Bisphosphonate Therapy on Hearing and Middle Ear Dysfunction in Pediatric Osteogenesis Imperfecta

Osteogenesis imperfecta (OI) is a heterogeneous inherited connective tissue disorder characterized by impaired bone matrix formation. Hearing loss is observed in 28 to 65 percent of patients with OI. Most patients with OI experience conductive hearing loss (CHL); however, mixed and sensorineural (SNHL) hearing loss has also been described.

The primary aim of this retrospective study was to characterize the rates of middle ear dysfunction (MED) and hearing loss (HL) with pediatric OI and investigate the efficacy of bisphosphonate therapy in HL.

Retrospective chart review of pediatric patients with OI.

Pediatric patients with OI treated between January 2000 and January 2021 were included. HL was defined according to pure tone audiometry, while MED was determined by tympanometry at 226 Hz. Patients were categorized by the OI type and considered to be using bisphosphonates if they had been initiated within 1 year prior to testing.

No direct intervention was performed. Comparisons between bisphosphate use and no bisphosphonate use were explored.

The chi-square test was utilized for categorical data to investigate whether a significant association with two-by-two variables existed.

This study included 144 patients. Of these patients, 12.5 percent had HL. Tympanometry revealed that 25 percent had MED. Additionally, 82.6 percent were currently using bisphosphonates. Across all OI types, no association between current bisphosphonates and HL or MED was observed. However, a subanalysis of patients with type I OI indicated that the absence of MED was statistically associated with current bisphosphonate therapy (p = 0.025).

Bisphosphonates are associated with the absence of MED in pediatric patients with type I OI. Because of its mild disease course, bisphosphonates are not commonly used for many patients with type I OI. By reducing MED and its associated comorbidities, our results suggest that bisphosphonate use may confer a significant therapeutic benefit.

HL is common in individuals with OI. Our study suggests that initiating bisphosphates at an earlier age may delay middle ear changes that contribute to HL.