Comparative Study of the Development of Hearing in Children with Various Genetic Backgrounds of Hearing Loss - OMAI 2018- The 10th International Symposium on Objective Measures in Auditory Implants

Jozsef Geza Kiss ¹ Dora Torok ² Katalin Farkas ² Roland Nagy ¹ Attila Nagy ¹ Janos Andras Jarabin ¹ Balazs Dimak ¹ Viktoria Szuts ⁴ Marta Szell ^2,3 Laszlo Rovo ¹

¹Department of Otorhinolaryngology, Head and Neck Surgery, University of Szeged, Szeged, Hungary
²Department of Medical Genetics, Faculty of Medicine, University of Szeged, Szeged, Hungary
³MTA-SZTE Dermatological Research Group, University of Szeged, Szeged, Hungary
⁴Department of Plant Biology and Biochemistry, Biological Research Centre, Hungarian Academy of Sciences, Szeged, Hungary

Background: Hearing loss is probably one of the most common sensory deficits. The etiology and onset of the hearing impairment influences the progress as well as the overall success of rehabilitation. Some of the hearing losses can be inherited, some are acquired and the possibilities for rehabilitation depend on the cause of the impairment. A mutation in the GJB2 gene, resulting in praelingual sensorineural deafness is the most common cause of congenital hearing impairments.

Objective and Methods: Children of 2-4 years old, suffering either from praelingual hearing loss with confirmed GJB2 mutation, other genetic factors or unknown etiology, were recruited. The primary screening was objectively performed by Auditory Brainstem Response. A comparative study was performed based on their intraoperatively evoked stapedius reflex threshold levels, cochlear implant impedance telemetry data, postoperative threshold- and comfort levels as well as on their results of auditory and speech tests.

Results: Our findings indicate that the speech development of patients with GJB2 deafness shows significantly better outcomes than the speech development of patients with other etiologies of hearing loss.

Conclusion: Based on our findings, we could conclude that the pathological background of hearing loss do play a significant role in hearing development, as well in the postoperatively acquired communication skills in childhood. Based on our findings it may worth considering how and possibly when the rehabilitation of these patients should begin.