09-10, 07:00–07:30 (Europe/Istanbul), Hearing Implant 1
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.
Objective: To present the cases of cochlear implantation in patients with CHARGE-syndrome; evaluate the audiological outcomes following cochlear implantation
Prof. Khassan Diab, born in 1973. With extensive training and expertise, he has become a leading authority in hearing improvement surgical interventions, including cochlear implantation. His surgical prowess is evidenced by an impressive record of over 5000 cochlear implantations in Russia and other countries and numerous complex surgeries for various ear conditions.
Education
Ph.D., Medical Care Faculty Rostov State Medical University, 1999
Residency, ENT Department Rostov State Medical University & St. Petersburg Research Institute of Ear, Nose, Throat and Speech, 1999-2003
Fellowship Rostov State Medical University, 2003-2005 Thesis: "Unsatisfactory results of stapedeoplasty and ways of their correction"
Professional Experience
Research Associate Ear Pathophysiology Department, St. Petersburg Research Institute of Ear, Nose, Throat and Speech November 2006 - 2010
Senior Research Associate St. Petersburg Research Institute of Ear, Nose, Throat and Speech 2011 - 2014
Head of Cadaveric Temporal Bone Courses St. Petersburg Research Institute of Ear, Nose, Throat and Speech 2006 - 2014
Chief Research Associate Department of Ear Diseases, Federal Research Clinical Center of Otorhinolaryngology, Moscow December 2014 - March 2016
Head of the Department of Ear Diseases Federal Research Clinical Center of Otorhinolaryngology, Moscow April 2016 - January 2020
Professor of Otolaryngology Faculty of Postgraduate Education, Pirogov Russian National Research Medical University Since November 2016
Deputy Director for International Affairs Curator of the Scientific and Clinical Department of Ear Diseases National Medical Research Center for Otorhinolaryngology, Federal Medico-Biological Agency of Russia January 2020 - Present
Achievements and Affiliations
· Honor Member Black Sea Otology and Neurotology Association
Member of the Board of the Society of Paediatric Otorhinolaryngology and Head and Neck Surgery Specialists
Member of the National Association of Otorhinolaryngologists of Russia.
· Chairman of the Expert Council of the Eurasian Academy of Otorhinolaryngology
· Official representative of Russia in The International Federation of Otorhinolaryngological Societies (IFOS).
· Annually conducts advanced dissection courses with international participation "Oto-surgery and surgery of the lateral skull base".
· Publications Author of more than 300 articles and 20 patents in the field of ear pathology
· Lecturer Engaged in various International scientific conferences and forums, including international events
Medical Centres Affiliations:
· Medical Centre Hospital of the President’s Affairs Administration of the Republic of Kazakhstan
· Municipal Childrens No.2 Hospital Astana, Republic of Kazakhstan
· City Clinical Hospitals N.5, Almaty Republic of Kazakhstan
· University Medical Centre Corporate Foundation Astana Republic of Kazakhstan
· Republican Specialized Scientific Practice Medical Centre of Paediatrics Tashkent, Uzbekistan
· ShifoNur Clinic Tashkent, Uzbekistan
· Erebuni Medical ENT (Ear, Nose, & Throat) & Cochlear Implants Centre, Yervan, Armenia
· Al Qassimi Hospital – Ministry of Health and Prevention UAE, Sharjah, United Arab Emirates
Surgical Expertise
Conducted over 5000 cochlear implantations
Successfully performed 100 implantations of bone conduction hearing aids
Completed approximately 100 surgical treatments for Meniere's disease
Executed more than 15,000 different types of tympanoplasties, including cases of petrous bone cholesteatoma
Proficient in surgical treatment of temporal bone tumors, including glomus tumors, facial nerve schwannoma, and neuroma of the internal auditory canal (500 cases)
Actively operating on patients (50 per year) with congenital external auditory canal malformations
Pioneering the simultaneous method of formation of the external auditory canal and external ear reconstruction using Medpor