Сochlear implantation in patients with CHARGE syndrome

Kh.M. Diab, N.A. Daykhes, O.A.Pachinina, T.S. Dmitieva, O.S. Panina, M. Hariri

Objective: To present the cases of cochlear implantation in patients with CHARGE-syndrome; evaluate the audiological outcomes following cochlear implantation

Patients and Methods: CHARGE syndrome is a complex genetic disorder that often includes hearing loss among plenty of symptoms, making cochlear implantation a critical intervention for improving auditory function and overall quality of life. Most patients with CHARGE syndrome have profound hearing loss and severe vision loss (”DeafBlindness”). Hearing loss is often due to abnormalities in the structure of the external, middle and inner ear; inflammatory diseases of the middle ear and pathology of the Eustachian tube, nasopharynx.

The data of 9 patients with deafness and CHARGE syndrome who underwent cochlear implantation on the base of the NRMCO were evaluated. Detailed pre-operative and post-operative evaluations were conducted to monitor auditory performance and developmental progress.

Results: According to CT and MRI data in patients with CHARGE syndrome we revealed cochlear hypoplasia type III in 6 cases, incomplete partition type I in 2 cases; in 1 case cochlea was normal. In all cases FN position was not standard, but in 4 cases it totally closed the area of round window niche. Standard approach with retro-auricular C-shaped incision, mastoidectomy, posterior tympanotomy was performed in most of the cases. In 1 case we performed transmastoid-transcanal approach. In 7 cases the round window membrane was not identified because of the abnormal course of facial nerve on the promontory; the electrode was inserted via cochleostomy. The intraoperative measurements showed normal impedance and normal neuronal response telemetry. A postoperative CT scan showed adequate active electrodes position. No complications were observed. Post-operative audiological results were predominantly moderate, with patients demonstrating improved reactions to sounds. Notably, while the auditory improvements were significant, the overall developmental progress in these patients showed marked intensification, suggesting that cochlear implantation positively influenced their broader developmental trajectories.

Conclusion: This study underscores the potential benefits of cochlear implantation in patients with CHARGE syndrome, not only in terms of auditory improvements but also in enhancing overall developmental outcomes. The findings highlight the importance of individualized surgical approaches and comprehensive post-operative care to maximize the benefits of cochlear implantation in this complex patient population.

Panel Presentation Difficult CI Surgery

ABI IN ADULTS: OUR EXPERIENCE

Kh.M. Diab, N.A. Daykhes, O.A.Pachinina, O.S. Panina

Objective: To present the experience in adult auditory brainstem implantation

Patients and Methods: The data of 2 patients with deafness who underwent auditory brainstem implantation on the base of the NRMCO in 2024 were evaluated. Type of pathology, previous surgeries, intraoperative findings, complacations and audiological outcome were evaluated. The follow-up period was 10 months.

Results: In both cases etiological factor was trauma (in one case with associated meningities), that caused total cochlear ossification. In one case ineffective cochlear implantation was performed before. Both patients underwent successful placement of an auditory brainstem implant using a retrosigmoid craniotomy approach. There were no side reactions from ABI implants in these cases intraoperatively. However, in early postoperative period in one case muscle contruction was revealed from 4 electrodes, also patient faced on with IX bilateral paralysis. The audiological results in this case was moderate. In the 2nd case no significant complications were observed except for enlargement of ventricles on the 20th day postop. Ventriculoperitoneal shunting was performed. The audiological results in the patient were good with speech recognition score 60%. The 2nd patient showed poor results without significant speech recognition.

Conclusions: An ABI has proven to be a safe procedure without serious complications. ABI can provide useful hearing in patients when CI is impossible. The entire ABI process should be handled by a multidisciplinary team with extensive experience in cerebellopontine angle tumour surgery and cochlear implantation in adults and children.