A hearing screening program was performed to determine the prevalence of hearing 
loss and abnormal tympanometry in individuals with short-stature skeletal 
dysplasias attending a national meeting. Behavioral audiometry, otoacoustic 
emission testing, and tympanometry were used to assess hearing. Failed hearing 
screen was defined as hearing ≥ 35 dB at one or more frequencies or by "fail" on 
otoacoustic emissions. One hundred ten of 112 subjects completed the screening. 
58 (51.8%) were children. Seventy-three (65.2%) had achondroplasia, 34 (30.4%) 
had one of 11 other diagnoses, and 5(4.4%) were undiagnosed. 25.8% of children 
failed hearing screening in one or both ears, while 46.3% of adults failed in 
one or both ears. 55.1% of adults and 25.0% of children with achondroplasia 
failed screening. Abnormal hearing was also found in the some patients with 
spondyloepiphyseal dysplasia congenital (SEDC; 75%), diastrophic dysplasia 
(66%), and Morquio (66%). Hearing was normal in those with hypochondroplasia, 
pseudoachondroplasia, and microcephalic osteodysplastic primordial dwarfism. 
Tympanometry was abnormal in at least one ear in 53.3% of children and 38.5% of 
adults. Abnormal tympanometry in the absence of functioning tympanostomy tubes 
was associated with 9.5 greater odds of hearing loss in children and 2.8 greater 
odds of hearing loss in the total cohort. Only 3 (2.7%) respondents reported the 
use of hearing aids. Hearing loss and middle ear disease are common in both 
children and adults with skeletal dysplasia. Adults were more likely to fail 
hearing screening than children. Abnormal tympanometry is associated with 
hearing loss. Hearing screening with appropriate intervention is recommended for 
these patients.