Delayed recognition of profound hearing loss in a 7-year-old girl with a neurological condition

Kelly is a 7-year-old girl with a complex medical history including asthma, mild spastic diplegia, and seizure disorder that is controlled with carbamazepine. She had a significant receptive and expressive language impairment and milder delays in gross and fine motor skills. Kelly is currently repeating first grade in a self-contained classroom; she receives speech, occupational, and physical therapy. At the 7-year-old well child visit, her mother is worried about Kelly's poor progress in school, and she expresses concern about her daughter's hearing. Her pediatrician observes that Kelly is withdrawn, uses minimal language, and is fearful of the examination. Kelly was born full-term by Cesarean section because of placental abruption. She was in the neonatal intensive care nursery for 2 weeks with metabolic acidosis because of acute tubular necrosis. One day after arriving home, she had a cardiopulmonary arrest followed by emergency open-heart surgery for critical pulmonary hypertension. Her postoperative course was significant for renal failure, extracorporal membrane oxygenation, ventilator dependency, tracheostomy, and gastrostomy. By 3 years of age her medical condition stabilized, and the tracheostomy and gastrostomy tubes were removed. A review of Kelly's previous audiological tests revealed a failed otoacoustic emission test at 5 months. An auditory brain stem response test at 8 months recorded normal hearing in the right ear. At 4 years, behavioral audiometry was attempted but not completed because Kelly cried throughout the session. At 5 years, testing with ear inserts showed normal hearing bilaterally. Because of the concerns raised by Kelly's mother at the pediatric visit, she was referred to audiology for a reevaluation. Testing at this time revealed moderate to profound sensorineural hearing loss in both the ears, which was confirmed on subsequent examinations. Kelly was promptly fitted for hearing aids. Her individual education plan was changed to reflect the diagnosis of hearing impairment, and hearing services were implemented in the classroom. On a recent follow-up visit, Kelly was talkative, engaging, and cheerful.