OBJECTIVE: To determine the prevalence of hearing loss and abnormal tympanometry 
in children with skeletal dysplasia.
DESIGN: Clinical screening program.
SETTING: National convention of the Little People of America.
PATIENTS: Convenience sample of volunteers aged 18 years or younger with 
skeletal dysplasias.
INTERVENTIONS: Hearing screening with behavioral testing and/or otoacoustic 
emissions, otoscopy, and tympanometry.
MAIN OUTCOME MEASURES: A failed hearing screen was defined as hearing 35 dB HL 
(hearing level) or greater at 1 or more tested frequencies or by a "fail" 
otoacoustic emissions response. Types B and C tympanograms were considered 
abnormal.
RESULTS: A total of 58 children (aged ≤18 years) with skeletal dysplasia 
enrolled, and 56 completed hearing screening. Forty-one children had normal 
hearing (71%); 9 failed in 1 ear (16%); and 6 failed in both ears (10%). 
Forty-four children had achondroplasia, and 31 had normal hearing in both ears 
(71%); 8 failed hearing screening in 1 ear (18%), and 3 in both ears (7%). 
Tympanometry was performed in 45 children, with normal tympanograms found in 21 
(47%), bilateral abnormal tympanograms in 15 (33%), and unilateral abnormal 
tympanograms in 9 (20%). Fourteen children with achondroplasia had normal 
tympanograms (42%); 11 had bilateral abnormal tympanograms (33%); and 8 had 
unilateral abnormal tympanograms (24%). For those children without functioning 
tympanostomy tubes, there was a 9.5 times greater odds of hearing loss if there 
was abnormal tympanometry (P = .03).
CONCLUSIONS: Hearing loss and middle-ear disease are both highly prevalent in 
children with skeletal dysplasias. Abnormal tympanometry is highly associated 
with the presence of hearing loss, as expected in children with eustachian tube 
dysfunction. Hearing screening with medical intervention is recommended for 
these children.