CHARGE syndrome is associated with a variety of temporal bone anomalies and deafness. The lack of surgical landmarks and facial nerve irregularities make cochlear implantation in this population a challenging endeavor. This video describes a safe and efficacious transcanal approach for cochlear implantation that obviates the need to perform a mastoidectomy and facial recess.
- The ear is prepped and draped in a standard fashion that includes utilization of facial nerve monitoring and pre-incision antibiotics. 1. Modified Rambo meatoplasty for closure of the external auditory canal. 2. Postauricular incision for access to the external auditory canal. Remove all canal skin and tympanic membrane to prevent future cholesteatoma formation. 3. Canaloplasty to ensure canal skin removal and increase exposure to the middle ear. 4. Identify the facial nerve course in the middle ear. The nerve stimulating probe can help to map it out. 5. Cochleostomy is preferably at the round window if it can be identified. In the case of round window atresia, its expected location or a safe location away from the mapped course of the facial nerve is selected as the cochleostomy site. 6. The internal device is secured in a standard fashion. The electrode is implanted and the electrode array can be positioned in a shallow suprameatal trough. Any excess electrode is coiled and secured within the expanded external auditory canal.
Standard age and device appropriate cochlear implant candidacy applies. This technique is consider in cases of severe middle or inner ear malformation, such as those associated with CHARGE syndrome.
Absolute: Absence of a cochlear nerve. This is particularly relevant in patients with CHARGE syndrome as they have a higher rate of cranial nerve eight aplasia. Relative: This is not the preferred implantation technique if normal mastoid, middle ear, and inner ear anatomy is present.
Standard operative room setup for cochlear implantation including facial nerve monitoring, betadine skin prep, pre-incision antibiotics, and antibiotic irrigation.
Preoperative evaluation consisted of computed tomography (CT), magnetic resonance imaging (MRI), audiometry, cochlear implant evaluation, and cognitive assessment. All patients underwent unilateral implantation after MRI confirmed an intact cochlear nerve on that side.
The typical anatomical landmarks for ear surgery are often absent or severely distorted. For more information on CHARGE syndrome and its anatomical variations, we suggest reviewing the references listed below.
Advantages: The transcanal approach eliminates the risk of performing a mastoidectomy and facial recess in this vulnerable patient population that often lacks standard anatomical landmarks like the lateral semicircular canal. Direct access to the middle ear allows early identification of the facial nerve and ability to map its course through the middle ear. Avoiding a mastoidectomy eliminates encountering any anomalous vasculature that may be running through a hypoplastic mastoid cavity. Disadvantages: Additional steps are necessary to close off the ear canal. Diligent canal skin and tympanic membrane removal must be performed to prevent future cholesteatoma formation. The facial nerve is still at risk of injury, particularly during the posterior aspect of the canaloplasty.
- Bleeding, infection, facial nerve injury, CSF leak/gusher, meningitis, improper electrode placement, electrode extrusion. - Cochlear implant outcomes are still multifactorial and vary widely in this patient population based on their other medical co-morbidities, developmental delay, supportive environment, and other factors known to influence cochlear implant performance.
- Bleeding, infection, facial nerve injury, CSF leak/gusher, meningitis, improper electrode placement, electrode extrusion. - Cochlear implant outcomes are still multifactorial and vary widely in this patient population based on their other medical co-morbidities, developmental delay, supportive environment, and other factors known to influence cochlear implant performance.
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1. Vesseur AC, Verbist BM, Westerlaan HE, et al. CT findings of the temporal bone in CHARGE syndrome: aspects of importance in cochlear implant surgery. Eur Arch Otorhinolaryngol 2016;273:4225-4240.
2. Morimoto AK, Wiggins RH 3rd, Hudgins PA, et al. Absent semicircular canals in CHARGE syndrome: radiologic spectrum of findings. ANJR Am J Neuroradiol 2006;27:1663-1671.
3. Zentner GE, Layman WS, Martin DM, Scacheri PC. Molecular and phenotypic aspects of CHD7 mutation in CHARGE syndrome. Am J Med Genet A 2010;152A:674-686.
4. Buchman CA, Copeland BJ, Yu KK, Brown CJ, Carrasco VN, Pillsbury HC. Cochlear implantation in children with congenital inner ear malformations. Laryngoscope 2004;114:309-316.
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This video shows a transotic approach for a cochlear schwannoma in a 59-year-old female who presented with sudden sensorineural hearing loss in her left three years prior. She was found to have subsequent growth of her tumor on imaging and elected to undergo surgery. The transotic approach is a valuable approach within the armamentarium of a skull base team and differs from the transcochlear approach in the handling of the facial nerve. Techniques for ear canal overclosure, eustachian tube packing and mastoid obliteration are also highlighted.
Here, we have a 39 yrs old female with complaints of noisy breathing for last two years post thyroidectomy. Flexible laryngoscopy confirmed bilateral vocal cord paralysis. She was planned for coblation assisted cordectomy.
Patient was taken up for procedure under general anaesthesia. She also started having stridor after induction. Nasopharyngeal intubation with spontaneous breathing technique was used. Entropy leads were placed over forehead to monitor the depth of anaesthesia. Tube position was confirmed with endoscopic view and Benjamin lindohlm laryngoscope was suspended. As the patient was spontaneously breathing, the stridor became more prominent, with stable vitals and the procedure was continued. The vocal cord retractor was fixed and coblation wand was then used with 7:3 settings for ablation and coagulation respectively. The surgical limits were-anteriorly the junction between ant 2/3 and post 1/3 of the vocal cord, posteriorly just anterior to the vocal process of arytenoid to prevent cartilage exposure and post operative granulations. Superely till the ventricle and inferioly till the medial most surface of the subglottis. Laterally approx. 5 mm depth was defined to prevent injury to the superior laryngeal artery branch and further bleeding. Once the final airway was achieved , the topical lignocaine was used to prevent laryngeal spasm post extubation.
The patient was shifted to the ward without oxygen, the voice was assessed on post op day 2.
Patient was called for follow up on post op day 14th and good voice outcomes were achieved.
So lets have a look on some tips & tricks for the safe procedure—–
Nasopharyngeal insufflation technique with entropy monitor will give adequate and safe surgical field
2. Appropriate exposure will help you to delineate the surgical margins
3. Topical anaesthesia before and after the procedure will prevent sudden laryngeal spasm
4. Firm holding of coblation device will help to prevent injury to surrounding structures like anterior 2/3 vocal cord, opposite side vocal cord, medial surface of vocal cord or aryteroid posteriorly
5. Do not ablate more laterally to prevent bleeding, if at all it happens, use patties or coagulation switch for hemostasis.
6. And at the end of the procedure ,use catheter suction to suck out blood clots or saline from the airway if any….
To Conclude-Coblation Assisted Cordectomy( CAC) can be performed safely with good outcomes in case of bilateral vocal cord paralysis using tubeless anesthesia technique without tracheostomy !
We present the harvest of a osteocutaneous fibula free flap for head and neck reconstruction performed at the University of Cincinnati. This reconstructive technique has a wide variety of implications but has found greatest utility in the reconstruction of mandibular defects.
Review Modified Rambo Transcanal Approach for Cochlear Implantation in CHARGE Syndrome.