Audiologic findings in young patients with hypophosphatemic bone disease.

Audiometric tests were conducted on 19 subjects with hypophosphatemic bone disease to investigate whether auditory impairment like that reported in affected adults occurs in young patients. No hearing loss or significant auditory findings were noted among the children or young adult patients. However, sensorineural hearing loss of cochlear origin was identified in the three oldest subjects (40 to 58 years), although a history of noise exposure in two of them could explain the observations. The results indicate that if an association exists between hypophosphatemic bone disease and hearing impairment, the auditory signs will not develop until adulthood in treated patients. Additional studies of large populations of affected adults are needed to identify the incidence and mechanism(s) of the auditory system abnormalities and to assess any effect of medical therapy for the metabolic bone disease.