Page 142 - Demo

Chapter 4140Most adults had received speech therapy (n=47, 78.3%). Ear tube placement was the most reported surgical intervention (n=23, 38.3%). Five adults (8.3%) had hearing aids prescribed prior to their visit (at age 57, 55, 32, 21 and 19 years), of whom 4 (6.7%) bilateral (Table 3). The 21-year-old female, and the excluded 39-year-old female, reported not to use the hearing aids. Another five adults (8.3%) were advised to consider hearing aids after audiologic examination. Treatments that were recommended after routine examination included muscle relaxation therapy for globus (n=4, 6.7%) and dysphagia (n=1, 1.7%), and balance exercises for benign paroxysmal positional vertigo (n=1, 1.7%). Additional examinations were recommended for sleep disorders (n=2, 3.3%; i.e., polysomnography) and vestibular dysfunction (n=1, 1.7%). Five adults were advised to start medication for otitis externa (n=2, 3.3%), rhinitis (n=2, 3.3%), and otitis media (n=1, 1.7%), respectively. Eight adults were advised to start (n=6, 10%) or continue (n=2, 3.3%) aural toileting for ear wax. Two adults (3.3%) qualified for surgical correction of nasal septum deviation.Factors related to severity of hearing lossThe regression model predicting severity of hearing loss in 22q11.2DS was significant (p=0.002, Nagelkerke=0.29). Higher age (OR=1.08, 95% CI=1.03-1.12, Wald=11.30, p=0.001) and a history of chronic otitis media (OR=2.87, 95% CI=1.07-7.67, Wald=4.43, p=0.04), but not male sex (OR=0.65, 95% CI=0.24-1.73, Wald=0.74, p=0.39) or FSIQ (OR=0.99, 95% CI=0.95-1.03, Wald=0.26, p=0.61), were related to the severity of hearing loss.DiscussionThe results of this study involving a relatively young adult sample of 22q11.2DS indicate that a large proportion of adults with 22q11.2DS have hearing loss, mostly limited to the higher frequencies, and often unrecognized. Age and a history of chronic otitis media, but not sex or FSIQ, were positively related to the severity of hearing loss. Lifetime history of otolaryngologic conditions that were frequently reported included chronic otitis media, globus pharyngeus, swallowing difficulties, balance problems and OSA.