Clinical and audiological features in XXXX .

@entity1 @entity2167 @entity3465 @entity393 @entity3638 @entity306
OBJECTIVE: To medically and audiologically characterize a population of @entity1 diagnosed as @entity2167 ( @entity2167 ). STUDY DESIGN: Retrospective medical chart review. SETTING/SUBJECTS: We identified 22 @entity1 from a pediatric otology clinic in a tertiary care pediatric hospital setting. RESULTS: A genetic factor in @entity2167 is suggested by our identification of 3 families with 2 affected @entity1 and 2 other @entity1 with family histories that were positive for @entity393 . Clinical features common among our population included a history of @entity3638 (n = 11 [50%]), @entity3465 (n = 10 [45%]), @entity2167 drug exposure (n = 9 [41%]), family history of @entity393 (n = 8 [36%]), neonatal ventilator dependence (n = 8 [36%]), and @entity306 (n = 2 [9%]). Full clinical and audiological data were available for 18 of the 22 @entity1 , including otoacoustic emissions, auditory brainstem responses with cochlear microphonics, and age-appropriate audiometric findings. Significantly, 9 of these 18 @entity1 showed improvement in behavioral thresholds over time, indicating that a subset of @entity1 with @entity2167 may recover useful hearing levels. Also significant was the success of cochlear implantation in 4 @entity1 . CONCLUSIONS: Management of @entity2167 in @entity1 requires serial clinical and audiometric evaluations, with a prominent role for behavioral testing. Prematurity, genetics, and @entity3638 appear to be significant factors in the development of @entity2167 ; @entity3638 can be associated with spontaneous improvement of hearing thresholds. For those @entity1 not benefiting from amplification or FM systems, cochlear implantation remains a potentially successful method of habilitation.
