John Greinwald
Cincinnati Children's Hospital Medical Center, USA.
Title: Diagnostic utility of various tests and treatment option for pediatric patients presenting with a unilateral sensorineural hearing loss (SNHL)
Biography
Biography: John Greinwald
Abstract
Objective: To determine the diagnostic utility of various tests and treatment option for pediatric patients presenting with a unilateral sensorineural hearing loss (SNHL)
Study Design: Retrospective chart review, uncontrolled case series
Setting: Tertiary academic center; Pediatric Hospital
Subjects: Pediatric patients (<18 years old) evaluated for unilateral SNHL
Methods: Upon IRB approval patients with single sided SNHL were identified using an internal database. Audiologic data, genetic testing, imaging results and pertinent clinical information were analyzed to determine the most efficacious method of evaluating these patients.
Results: 407 patients were identified with average age (SD) of 87.4 months (53.9). 233 patients were males (53%). 37 patients had high frequency (HF) SNHL alone, 51 patients with normal HF PTA (abnormal low frequency PTA) and 319 with a flat audiometric configuration. Hearing loss was more common on the left side and in males. More than 50% of patients had mild to moderate SNHL. GJB2 was a rarely seen (0.7%), while mutations in the SLC26A4 gene were seen in 18% of patients. Enlarged vestibular aqueduct (EVA) was found in 17% of patients. In patients with unilateral EVA, 19% had contralateral ear involvement. Overall, 19% of patients developed bilateral hearing loss. Treatments varied from observation, traditional hearing aids, CROS hearing aids and cochlear implantation. Seven patients with single sided deafness were implanted. All patients continue wearing their implants and getting appropriate aided benefit with speech reception thresholds at 30dB or better with a minimum of 2 years of follow up.
Conclusion: USNHL is a relatively common problem affecting children. In our population, progression occurred relatively frequently. Temporal bone anomalies such as EVA, were common. Cochlear nerve deficiencies are also demonstrated in our study and are best demonstrated on MRI. Pendred syndrome and DFNB1 are rare. Based on our data, an algorithm for children with USNHL shows that imaging should be the primary diagnostic study. Magnetic resonance imaging may be the best imaging modality due to its ability to diagnose deficient cochlear nerves in addition to common temporal bone anomalies. Treatment should be individualized based on the medical needs of the child. Cochlear implantation is now an option for patients with single sided deafness.