Hearing rehabilitation in patients with congenital aural atresia: an observational study in a tertiary center

Abstract

Background: Congenital aural atresia (CAA) and stenosis is a rare defect affecting the external auditory canal which causes varying degrees of hearing impairment. This malformation may be unilateral or bilateral. Timely hearing rehabilitation in these patients is paramount to ensure appropriate speech and language development. Thus, early detection and hearing assessment with timely intervention in the form of hearing device is crucial. There is a wide range of hearing amplification devices available for these patients, catering to individual needs and preferences. This study investigated the options and choices of hearing rehabilitation among patients with CAA in our center. This study also compared the hearing gain of different types of hearing devices and patient satisfaction levels. Methods: This was a retrospective analysis of 55 patients with congenital aural atresia or stenosis under follow-up at the Otorhinolaryngology and Audiology Clinic of a tertiary academic institution. Data on the timing of hearing loss diagnosis and intervention, type of hearing rehabilitation, hearing gain, Jahrsdoerfer score from temporal bone computed tomography, and patient satisfaction level was collected and analyzed. Results: Fifty-five patients were recruited with ages between 3.6 months to 58 years old. The majority of patients (63.6%) had moderate hearing loss, followed by severe hearing loss (18.2%), mild hearing loss (9.1%) and profound hearing loss (7.3%). 32.7% of patients had early hearing loss diagnosed by 3 months old, while 67.3% were diagnosed after 3 months of age. Bone anchored hearing implant (BAHA Attract/Connect) gave the highest mean hearing gain of 35.23 dB (SD ± 9.75) compared to air conduction hearing aid, bone conduction hearing aid, and canalplasty. Patients implanted with BAHA Attract/Connect reported the highest average satisfaction scores of 7.74. Conclusion: A significant proportion of patients have delayed diagnosis and intervention. Bone-anchored hearing implants provide excellent hearing improvements in patients with CAA with good patient satisfaction.