Case study
Cochlear implantation in incomplete partition type III
Implantación coclear en partición incompleta tipo iii
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[ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Manuel" "apellidos" => "Manrique" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "Ezequiel" "apellidos" => "Barros" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Department of Otolaryngology, Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Departamento de Otorrinolaringología de la Clínica Universidad de Navarra, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Implantación coclear en partición incompleta tipo <span class="elsevierStyleSmallCaps">iii</span>" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 583 "Ancho" => 1005 "Tamanyo" => 96953 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Preoperative axial computed tomography showing the complete absence of the modiolus, the presence of the interscalar septa, the dilated internal auditory canal and the missing bony separation between the cochlear basal turn and IAC, in the right and left ear.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Case report</span><p id="par0005" class="elsevierStylePara elsevierViewall">We describe a case of a boy diagnosed at 5 years of age with bilateral progressive sensorineural hearing loss.</p><p id="par0010" class="elsevierStylePara elsevierViewall">He was fitted with binaural hearing aids when he was 6 years old with initial benefit but with gradually worse speech perception since 8 years of age.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Considering the progression of the hearing loss and poor outcome with hearing aids [four frequencies (0.5–4<span class="elsevierStyleHsp" style=""></span>kHz) pure tone average (PTA) of 90<span class="elsevierStyleHsp" style=""></span>dB and poor speech perception], he was evaluated for cochlear implantation at 12 years of age.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The high-resolution CT showed bilateral cochlear malformations consistent with incomplete partition-type III (IP-III) as: complete absence of the modiolus, enlargement of the fundus of the internal auditory canal (IAC), and communication between the basal turn of the cochlea and the IAC (<a class="elsevierStyleCrossRef" href="#fig0005">fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">At the age of 13, he underwent a right cochlear implantation via the classical transmastoid-facial recess approach. After performing the mastoidectomy and posterior tympanotomy through the facial recess, the surgeon exposed the round window and perforated its membrane with an abocath, and a profuse cerebrospinal fluid (CSF) gusher was documented. We elevated the head of the operating table and waited. Ten minutes later, the gusher flow had reduced without the need of a lumbar puncture. The surgeon performed an anterior and inferior enlargement of the round window to allow the introduction of a cork-type silicon stopper straight electrode with a ring-shaped temporal fascia around it, with good control of the CSF gusher. Then, the cochleostomy was sealed with fibrin glue, and the remaining electrode was fixed in the upper side of the mastoidectomy with fascia and muscle to avoid extrusion.</p><p id="par0030" class="elsevierStylePara elsevierViewall">The fluoroscopic image confirmed the correct intra-cochlear placement of the electrode and the intraoperative neural telemetry indicated the integrity of the electrodes and the good auditory response to electrical stimulation.</p><p id="par0035" class="elsevierStylePara elsevierViewall">No lumbar drainage was used intra or postoperatively.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Five years after implantation, the patient is using his CI daily without any surgical complications. The audiometry thresholds for frequencies of 0.5–4<span class="elsevierStyleHsp" style=""></span>kHz improved to 35–40<span class="elsevierStyleHsp" style=""></span>dB, with a speech recognition threshold (SRT) of 50<span class="elsevierStyleHsp" style=""></span>dB.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Discussion</span><p id="par0045" class="elsevierStylePara elsevierViewall">Inner ear malformations diagnosed radiologically represent approximately 20% of congenital sensorineural hearing loss and can be separated into 8 groups, according to Prof. Sennaroğlu.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">1</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">The IP-III is responsible for 2% of these malformations and is the type of cochlea abnormality observed and reported in X-linked deafness.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">1</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The IP-III is characterized by a thin otic capsule, complete absence of the modiolus but with the presence of the interscalar septa, cochlea located at the lateral end of the IAC, enlargement of the fundus of the IAC, communication between the basal turn of the cochlea and the IAC, fixed stapes footplate, and a normal cochlear nerve but with the labyrinthine segment located above the cochlea. These changes appear to result from an abnormal vascular supply from the middle ear mucosa as a result of a genetic abnormality in the <span class="elsevierStyleItalic">POU</span><span class="elsevierStyleInf"><span class="elsevierStyleItalic">3</span></span><span class="elsevierStyleItalic">F</span><span class="elsevierStyleInf"><span class="elsevierStyleItalic">4</span></span> gene<span class="elsevierStyleItalic">.</span><a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">1</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Audiometrically, this type of abnormality is associated with a mixed type hearing loss with a progressive sensorineural component where:<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">-</span><p id="par0065" class="elsevierStylePara elsevierViewall">The conductive component is attributed to a fixed footplate;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">-</span><p id="par0070" class="elsevierStylePara elsevierViewall">The bone conduction levels can be explained by: the thin otic capsule that probably makes the cochlea more sensitive to bone conduction; the third window phenomenon due to the defective cochlear base; and the increased perilymphatic pressure leading to cochlear nerve dysfunction.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">2,3</span></a></p></li></ul></p><p id="par0075" class="elsevierStylePara elsevierViewall">The treatment options of this condition include the use of hearing aids until they reach severe to profound hearing levels or poor speech perception, when they are considered and evaluated for cochlear implantation.</p><p id="par0080" class="elsevierStylePara elsevierViewall">During surgery of IP-III patients, two major problems can occur: CSF gusher during cochleostomy; or electrode misplacement into the IAC.</p><p id="par0085" class="elsevierStylePara elsevierViewall">Due to the absence of a bony partition between the basal turn of the cochlea and the IAC, the CSF will flow out into the middle ear cavity during CI surgery.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">3–6</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">Some authors recommend creating a large cochleostomy to provide better access for packing and sealing the expected gusher. Others recommend cochlear implantation with subtotal petrosectomy with blind sac closure of the external auditory canal technique.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">6,7</span></a></p><p id="par0095" class="elsevierStylePara elsevierViewall">Because of the absent modiolus and enlarged fundus of the IAC, the implantation may result in electrode insertion into the IAC.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">3–6</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">Electrodes with complete rings or contact surface on both sides may provide better stimulation. Also, the probability of longer electrodes entering the IAC is bigger, therefore shorter electrodes are recommended to achieve full insertion and full stimulation. Modiolar hugging electrodes may have a tendency going towards the center of the cochlea, which may result in misplacement and injury of the nerves inside the IAC in IP-III patients.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">3</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">Enlargement of the cochleostomy also allows for visualization of the basal turn, the outer anti-modiolar wall, and the fundus of the IAC, facilitating the electrode array placement.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">4,5</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall">Position monitoring with intraoperative imaging is advised to assist in accurate placement of the electrode within the cochlea.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">6,8</span></a></p><p id="par0115" class="elsevierStylePara elsevierViewall">In our case, the profuse gusher started when the round window was opened and widened, as expected. After the CSF flux reduced, we inserted the electrode and sealed the round window with pieces of fascia and fibrin glue. The gusher was controlled and no post-operative leakage was documented.</p><p id="par0120" class="elsevierStylePara elsevierViewall">We used the straight 25<span class="elsevierStyleHsp" style=""></span>mm electrode with a “cork” type stopper developed by <span class="elsevierStyleItalic">Senarroğlu</span>, ideal for properly sealing the cochleostomy and also making one full turn around the cochlea. However, selection of the electrode is controversial. While <span class="elsevierStyleItalic">Senarroğlu</span> recommends the use of electrodes with contact surface on both sides to provide better stimulation, <span class="elsevierStyleItalic">Incesulu</span> recommends the use of half-banded electrodes to prevent the risk of facial nerve stimulation.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">2,5</span></a></p><p id="par0125" class="elsevierStylePara elsevierViewall">Meningitis is also another possible complication, and the risk is higher in children with inner ear abnormalities. Covering the patient with vaccination and firm sealing of the cochleostomy to stop the CSF leak can prevent the risk.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">5,8</span></a></p><p id="par0130" class="elsevierStylePara elsevierViewall">To conclude, although we cannot entirely predict the results of surgery in cochlear malformations, patients with IP-III abnormalities can successfully undergo cochlear implantation with great auditory and speech outcomes.</p><p id="par0135" class="elsevierStylePara elsevierViewall">The surgeon should take special considerations, particularly with the CSF gusher, to avoid complications such as meningitis, and electrode position.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Case report" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Discussion" ] 2 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2021-12-09" "fechaAceptado" => "2022-03-24" "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 583 "Ancho" => 1005 "Tamanyo" => 96953 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Preoperative axial computed tomography showing the complete absence of the modiolus, the presence of the interscalar septa, the dilated internal auditory canal and the missing bony separation between the cochlear basal turn and IAC, in the right and left ear.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:8 [ 0 => array:3 [ "identificador" => "bib0045" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Cochlear implantation in inner ear malformations-a review article" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "L. 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| 2023 August | 8 | 2 | 10 |