#Gusher Syndrome

[crusherthedoctor]

As someone who has long decided to stay away from the fandom (and even the franchise to an extent) I have to say that what gets me the most is not the fandom's penchant for insulting and even harassing others who hold dissenting opinions, that's been a thing since forever, but rather how... justified it has become.

The Sonic fandom has spent so much time sunk neck deep into negativity, wether it was older fans hating the newer stuff or how the media has treated the series, that it has become utterly obsessed with countering any form of negativity....by stamping it out with really negative behaviour and then proceed to think it's doing God's work or something.

There's something....unsettling about this, not to mention just how childish and downright shallow it is to even end entire friendships over dissenting opinions on a blue Hedgehog.

Then again I don't think it's simply the presence of dissenting opinions, it's the insecurity that comes with coming face to face with them, the idea that you feel dumb for Liking something that others are pointing out is really flawed, and so people aggressively attack others just so they no longer have to feel insecure.

They think it's okay when they're the ones doing it.

This has influenced the current state of the fandom in more ways than one. Sonic fans have become very insecure about being fans of Sonic, to the point where I reckon it's why stuff like the zombot arc is considered Deep and Mature. And they've grown very quick to beat IGN to the punch and become their own Arin Hanson, actng like being a fan and enjoying the franchise is a terrible crime that needs to be atoned for, and that the franchise as a whole is nothing but abominable garbage and if you like it, then you must be suffering from Stockholm Syndrome (unless it's the comics, which are naturally singled out as the one good thing of the series).

We've seen fans act high and mighty, yet they reveal themselves to be flaky, ending long friendships just because the other person felt differently about something. "Too negative", they'll say, but only when it's you and Flynn/IDW; they can rant about their favourite punching bags all they want.

As for the harassment, the excuse of dealing with too much negativity is exactly that: an excuse. I know this because as someone who doesn't like Frontiers, hates IDW, has criticisms with games like SA2 and Unleashed, doesn't like Soft!Eggman, and likes the Pontaff games in spite of their shortcomings, that's given people a lot of motivation to give me endless hassle over the years, which continues to this day. They tell me to kill myself because I don't like Ian Flynn's work and unearned reach. They mockingly compare my status as a "mere" fanfic writer to fans who are said to have more impressive talents, like art or translating. And although I have the keen sense to not have an account on Twitter, I know they've brought me up from time to time in hushed whispers on there, as if I shot Bruce Wayne's parents.

And I've felt it. I've felt the misery that comes with all that, so I know how it feels when you're surrounded by said misery. But I never used it as a reason to start harassing people myself. I've never done that, and I never will. All this time, I've only ever given my honest thoughts about what I like and don't like, made a few memes on the side, and call out fans on their bullshit and double standards. That's not harassment, no matter what people who call you a bigot if you think Shadow crying gushers is OoC would have you believe. That's being real.

[ourhaileydavies]

Juniper Publishers-Open Access Journal of Head Neck & Spine Surgery

Cochlear Endoscopy in Cochlear Implantation of a X-Linked Stapes Gusher Syndrome

Authored by Jennifer Ha

Abstract

A 12-year-old boy with a five-year history of bilateral sensorineural hearing loss and X-linked stapes gusher syndrome developed progressive left-sided hearing loss. The pre-operative computed tomography of the temporal bone showed a bulbous internal auditory canal with a dysplastic cochlea and no apparent modiolus. A 1.3mm salivary endoscope was placed at the cochlear entrance to assess the intracochlear anatomy. This revealed membranous structures of the cochlea without direct communication to the internal auditory canal. We advocate for the use of cochlear endoscopy to better delineate inner ear anatomy, which will influence the implant selection and potentially hearing outcomes in patients.

Keywords: Cochlear endoscopy; Otoendoscopy; X-linked stapes gusher syndrome; Cochlear implant; Perilymphatic gusher

Introduction

X-linked stapes gusher syndrome (otherwise known as X-linked deafness type 3 or Nance deafness) is a rare form of sensorineural hearing loss (SNHL) syndrome. Inherited in a sex-linked recessive manner, it is believed to be the consequence of a loss-of-function mutation on the X-chromosome in the POU3F4 gene at the DFN3 locus [1,2]. Males tend to present greater phenotypic severity than females, who present less frequently [3-5].

Affected patients have an abnormal configuration of the lamina cribrosa and internal auditory canal (IAC) [1,3,4,6,7]. This malformation leads to increased perilymphatic pressure and to stapes’ footplate fixation, giving rise to conductive hearing loss and progressive cochlear nerve incompetence. This is relevant for surgeons, as it results in an increased risk of perilymph gusher with surgical manipulation [1,3,4,6,7]. This may lead to other complications, such as otorrhea, rhinorrhea, and recurrent meningitis [8]. Meningitis complicating cochlear implantation (CI), occurs at a higher rate in patients with inner ear (IE) abnormalities, including X-linked gusher syndrome [3,9].

Traditional approaches to imaging for CI utilize computed tomography of the temporal bone (CT TB) and/or magnetic resonance imaging. We report a case whereby intra-operative otoendoscopic visualization allowed for real time visualization of the IE anatomy, which allowed us to optimize our electrode choice for CI. In this case, such visualization was valuable, as it indicated the presence of the membranous portions of the IE to evaluate if the modiolus was present, which we believe implied that a directional electrode was the most appropriate choice.  

Case Report

A 12-year-old boy with a five year history of bilateral SNHL and X-linked stapes gusher syndrome presented with progressively worsening left-sided hearing loss. He was initially performing well with bilateral traditional hearing aids but developed progressive mixed loss and worsening performance in his left ear. His preoperative audiogram can be seen in Figure 1. He was sent for CI candidacy assessment and was deemed to be suitable.

The pre-operative computed tomography (CT) of the temporal bone was assessed. This showed a bulbous IAC with a dysplastic cochlea and no apparent modiolus (Figure 2). A CI24RE (ST) implant was initially selected by the implant team due to the uncertainty regarding the location of the spiral ganglion cells.

A 1mm cochleostomy was made anteroinferior to the round window (RW). Upon entering the cochlea, a moderate perilymphatic gusher was encountered. A 1.3mm rigid salivary endoscope (Karl Storz, Germany) with a 3-chip full HD camera head (Image1 S H3-Z) was approached trans-tympannically and was placed at the edge of the 1mm cochleostomy to assess the intracochlear anatomy.

To avoid injury to the inner ear, the CSF was not suctioned. The same view was not achieved with the microscope as the endoscope provided a more magnified and higher definition view of the modiolus. There was evidence of membranous structures of the cochlea without direct communication to the IAC. Full insertion was achieved on the first attempt without any difficulty and the gusher was controlled with packing of periosteal tissue at the cochleostomy site. An intra-operative plain X-ray confirmed the electrode placement.

Discussion

When the cochlear modiolus and osseous spiral lamina are deficient, the absence of a bony septum creates a common space seen on the imaging study [10] (Figure 2); there is an abnormal communication between the IAC and the vestibule responsible for the periphymphatic gusher. Patients with IE anomalies may have both atypical positions of their spiral ganglion cells (SGC) and have a higher likelihood of having fewer spiral ganglion cells [11]. This has functional implications because at least 10,000 functional SGNs are necessary for effective speech discrimination 12]. Also this number can be further reduced by surgical trauma [12]. Therefore, choosing the proper electrode device, namely, a directional or a full banded electrode can help reduce the number of lost SGNs. The fully banded CI electrode may be more useful in the absence of a modiolus to allow for full and multi-directional stimulation, whereas the pre-curved directional electrode may be more appropriate if there is a modiolus present for precise stimulation [13].

The proper electrode choice may have important hearing outcome implications for patients with IE anomalies following CI due to the challenges of reaching an optimal level of cochlear stimulation, decreased dynamic range, a wider pulse width, and weakened neural synchrony [7]. The functionality of CI is correlated to the number of SGCs and their distances from the stimulating electrode [14].

In our patient, we assumed that there was no IAC communication due to the presence of modiolus. The moderate CSF gusher was presumed to be secondary to his X-lined stapes gusher syndrome and his enlarged IAC. Perilymph gusher during CI in patients with X-linked gusher syndrome is inevitable, but a thorough examination of the IE is still critical. CT of the temporal bone is important for surgical planning, and it is also useful to assess the likelihood of perilymphatic gusher [4]. Distinguishing features on imaging include an enlarged bulbous IAC, a widened cochlear aperture without the lamina cribrosa, cochlear hypoplasia with modiolar deficiency, and a broadening of the bony canal for the labyrinthine portion of the facial nerve [6]. Enlarged vestibular aqueducts may also appear in conjunction with modiolar deficiency [15].

The rarity of X-linked gusher syndrome may result in radiologists failing to recognize these signs, which may mislead surgeons to perform stapedectomies that are otherwise contraindicated. Thus, Incesulu et al. [3] advocated for high resolution CT of the temperal bone to assess for congenital dysplasia with ¬at least 1-mm thick slices [as opposed to 2mm thick slices], which should ideally be assessed by an experienced neuroradiologist [3]. Quan et al. [16] proposed that CT virtual endoscopy should be done to evaluate CT data through threedimensional reconstruction [16]. Limitations to both modes of assessment assume that the CT images correlate perfectly with the anatomy of the IE and that consistent radiological consultation will occur. Our case, however, demonstrated that intra-operative endoscopic findings do not always correlate with CT findings, indicating that cochlear endoscopy may be a useful tool to better delineate the intracochlear anatomy.

The otoendoscopic intracochlear view gave us accurate and real-time information on the anatomy of the IE, which confirmed the presence of a modiolus and the confidence that the CI would not be in the IAC. This has the potential to allow us to make a better-informed decision regarding the type of electrode to place.

Electrode choice is significant for patients with IE abnormalities, since the location of neural tissue may be abnormal. In patients that have an absent modiolus, a circumferentially stimulating electrode may be preferred over a full-banded electrode, which may risk adverse facial nerve stimulation [3]. One explanation for post-operative facial nerve stimulation in children with IE abnormalities is the close vicinity of the electrode to the nerve [14]. Therefore, to avoid injury, the proper choice in electrode should be made.

Conclusion

While CT temporal bone has served as the conventional approach to assessing the anatomy of the IE, fthe endoscope offers better resolution of the modiolus than the CT temporal bone, as the CT indicated that there was no modiolus. We advocate the use of intracochlear endoscopy in selected cases as it offers a better resolution than even high-resolution CT. With the potential to change electrode choices in CI, the customization of electrode choice based on the presence of membranous IE anatomy may change the hearing outcome of the patients with anomalous IE anatomy and patients with uncertain location of spiral ganglion cells.

For more articles in Open access Journal of Head Neck & Spine Surgery | Juniper Publishers please click on: https://juniperpublishers.com/jhnss/index.php

[darklingduke]

Inspired by @/capri-suns-and-gushers and this post about how sys-meds shouldn’t get to write the narrative about endogenic/parogenic/tulpamancy/spiritual/etc systems on this platform. 

We wanted to use this as our official coming-out-as-a-system (on this blog at least) post, as well as answering some things. 

If anyone has any questions, feel free to ask! Don’t send us syscourse or hate, please. (Long post, so it’s cut)

1. What kind of system are you?

We use several different terms to describe our system type, depending on how we’re feeling that day, who’s fronting, or who we’re talking to. We use endogenic, MaDD/paragenic, araisagenic, soul-bonding, and gateway.

Most of us are in here due to maladaptive daydreaming and escapism from the “core”, as well as projecting onto characters or having comfort characters. 

There are a few subsystems in here, though. One of our Remuses is a traumagenic OSDD system, Zak is a traumagenic DID system, Cadence has unknown origins, and Cleo is a paragenic system. 

2. How many of you are there?

Currently around 80 or so, counting subsystem member. But our numbers are constantly fluctuating when someone decides to leave or come back or walk-in. The highest amount we have ever had was around 120. 

3. How long have you known about your plurality?

Our “core” learned the first time about aer plurality in... I wanna say... 2018? Ae came out to someone as a system and it didn’t go well, so ae decided to forget all about it and procrastinated everything. We’ve been out as a system since December of 2019 now, though. 

4. Had you heard of DID/OSDD/DDNOS before you became plural/discovered your plurality?

Kind of? Someone at work approached us back in 2018 (see answer to 3) and whispered “hey. guess what. i’ve got multiple personality disorder. i’m not kyle.” then walked away. Star (”core”) went home that night and researched it and that’s how ae realized ae were plural. 

5. Do you have a wonderland/innerworld? If so, what do you call it, and what are some things that you and your system members do there?

We do, but it’s very difficult to see, due to the body’s aphantasia. Very few people are actually able to see it. It’s more along the lines of us “asking” the brain what it looks like and guessing things until the brain is like “yeah that’s right.” 

We call it our innerworld, or our headspace. 

A lot of people like hanging out in the living room (our “control room”, playing video games on one of the tv’s in here, or hanging out in the pond outside.

6. What do you call your system members?

Headmates. We’re not against the term “alters” but we don’t tend to use it too much. 

7. If you’re plural and don’t use the word “system” to describe you&, what word do you use?

We use the word “system” but we’ve been considering using different terms, just aren’t sure what terms there are to use lol.

8. What are some of the best things about being plural?

Probably getting to hang out with comfort characters. We are all aware that they aren’t their source, but they have many personality traits in common with their source, so it’s a source of comfort to get to hang out with and be friends with them. 

9. What are some not-so-great things about being plural?

The occasional blurriness we get when we realize we don’t know who we are/who’s fronting, and we have to try to figure out who we are. 

10. Do you have a spiritual or psychological view of your plurality?

We think it’s more of a spiritual view, because a lot of us, if not all of us, view it more as that person’s spirit coming to our headspace from a different dimension and hanging out here. 

11. Do you ever experience “switching” or “possession” or any sort of change in who controls the body? If so, what do you call it, how easy/difficult is it, and what is it like? Were you always able to do this, or did you have to learn how over time?

We do experience switching, but it feels different sometimes than others. Sometimes it feels as though whoever is fronting is more along the lines of Star (”core”) being in front and someone else backseat driving, or vice versa. 

We call it switching. It’s usually pretty easy. Sometimes we do it without even trying to or noticing. We’ll be sitting here playing a game or watching Tv and then all of a sudden realize we aren’t who was fronting before. Sometimes we have to focus on “handing over controls” in innerspace and there’s meatspace blurriness while that’s going on before it snaps into full control and the blurriness/dissociation goes away. it doesn’t normally take very long. A minute at most. 

We have been able to do it since actively being plural in 2019 (see answer 3)

12. How do you and your system mates relate to each other? (Are you friends, family, romantically involved, caretakers, etc)

All of the above. There are some people who were born in the system, and their family is in here. There are some people who are dating, who were related before coming here, who adopted each other in here as family, etc. It’s more of a whole world in here than anything. People have different relationships with each other. 

13. Have you come out to anybody in real life/in a singlet space about your plurality? How did it go?

We came out to our partner system’s dad (well they came out to him for us, but we gave them permission to do so), and he completely ignored the situation. And then we came out to the body’s mom, and she ignored us and told us she didn’t think we were safe with our partner system since they came out publically as a system. 

14. What kinds of forms and appearances do your system members take on?

We have some people in here who are human, we have some cat-people, demons, vampires, sirens, ghosts, and other monsters/creatures. We don’t all look like how people look like in this world, though. We don’t look how most people would say is “life like”. It’s more along the lines of video game animation or a more realistic version of pixar animation. 

15. What are you and your system members interested in?

Current interests are: Minecraft, Sanders Sides, Grey’s Anatomy, drawing, writing. Some people are interested in Don’t Starve Together. Some are interested in knitting and painting. Depends on the person. 

16. What is your life like in the meatworld?

Assuming meatworld is like... “real” world. Currently with quarantine, there’s not much to tell. We switch out every few hours if the body’s anxiety or depression gets too bad for us, we vibe with our partner system, and we pretend to be the core around the partner system’s dad (whom we live with).

17. What are your music tastes? Movies? Favorite Colors? Animals? List any other favorites as well. 

Ummm.... lol.... it really depends on the person. We have a playlist on spotify for each person. We don’t have much variety in favorite movies/shows, though. Most of us like animated movies or comedy or romcoms, or stereotypically straight movies (think Hallmark romance movie, or general romcoms, or raunchy comedy).

18. Does your system have a host/original? If so, what do you call them? Explain what role they play in your system. 

We have Star. Ae are the “original” person in here. We call aem the “core” when need be, but we usually just call aem Star lol. Ae are just another person in here, ae don’t really play too much of a “role”. Ae just decides what name the body goes by on default and ae have the final say on haircuts and tattoos/piercings and stuff. 

19. Do any system members have notable relationships outside of the system?

Yes! We have a partner system, @systemofthelostsouls . 

Star is dating Omen, Dee, Animosity, Logan, Regulus, and Story.  Virgil is dating Dee, Regulus, and Ceto. Remus is dating Omen, Dee, Logan, Emile and Story.  Roman is dating Emile and Logan.  Bee is dating Logan, Omen, Nyx, and Dee.  Charlie is dating Omen and Dee.  Denki is dating Jirou. Shinsou is dating Jirou. Kyle is dating Kay and has sort of a thing with Kenny and Stan.

And I’m decently sure there are more, but I can’t remember. I’m pretty new here, myself.

20. If you haven’t been plural for your entire life/haven’t known about your plurality until later in life: what was life like before plurality compared to life now. 

According to Star, life before plurality was a lot more... stable. Now if we want to finish a writing project, Star either has to backseat write, let someone else write for aem, or it takes weeks. We’ve been working on one chapter of a fanfiction for a week now. Before, Star was able to finish a chapter in a day or two. 

21. If you chose to become plural: why? What has changed since then?

We’re not actually sure if Star chose to become plural or not. Sometimes it feels like ae did (which fucks up with our imposter syndrome lmfao), sometimes it feels like Star chose to be plural and invited people here out of loneliness and wanting to fit in with our friend group at the time, but usually it just feels like people chose to come here, rather than the other way around. 

22. Is there anything you’d like to say to the plural community at large?

Don’t gatekeep, please. Let endogenic systems use the words “system” and “switching” if it’s what works for them. They aren’t hurting anyone. 

And please don’t fakeclaim. You don’t know what’s going on in someone’s head. You aren’t in there. 

23. Is there anything you’d like to say to any singlets reading this post?

Thank you for reading such a long thing. If any of this confuses you, or you’d like to learn more, our inbox is always open. But be warned, if you send us hate or something, we’re more likely to just delete your ask than answer it. 

Thank you all for reading! -Cyra

[ourhaileydavies]

Cochlear Endoscopy in Cochlear Implantation of a X-Linked Stapes Gusher Syndrome-Juniper publishers

Abstract

A 12-year-old boy with a five-year history of bilateral sensorineural hearing loss and X-linked stapes gusher syndrome developed progressive left-sided hearing loss. The pre-operative computed tomography of the temporal bone showed a bulbous internal auditory canal with a dysplastic cochlea and no apparent modiolus. A 1.3mm salivary endoscope was placed at the cochlear entrance to assess  the intracochlear anatomy. This revealed membranous structures of the cochlea without direct communication to the internal auditory canal. We advocate for the use of cochlear endoscopy to better delineate inner ear anatomy, which will influence the implant selection and potentially hearing outcomes in patients.

Keywords:MCochlear endoscopy; Otoendoscopy; X-linked stapes gusher syndrome; Cochlear implant; Perilymphatic gusher.

Introduction

X-linked stapes gusher syndrome (otherwise known as X-linked deafness type 3 or Nance deafness) is a rare form of sensorineural hearing loss (SNHL) syndrome. Inherited in a sex-linked recessive manner, it is believed to be the consequence of a loss-of-function mutation on the X-chromosome in the POU3F4 gene at the DFN3 locus [1,2]. Males tend to present greater phenotypic severity than females, who present less frequently [3-5].

Affected patients have an abnormal configuration of the lamina cribrosa and internal auditory canal (IAC) [1,3,4,6,7]. This malformation leads to increased perilymphatic pressure and to stapes’ footplate fixation, giving rise to conductive hearing loss and progressive cochlear nerve incompetence. This is relevant for surgeons, as it results in an increased risk of perilymph gusher with surgical manipulation [1,3,4,6,7]. This may lead to other complications, such as otorrhea, rhinorrhea, and recurrent meningitis [8]. Meningitis complicating cochlear implantation (CI), occurs at a higher rate in patients with inner ear (IE) abnormalities, including X-linked gusher syndrome [3,9].

Traditional approaches to imaging for CI utilize computed tomography of the temporal bone (CT TB) and/or magnetic resonance imaging. We report a case whereby intra-operative otoendoscopic visualization allowed for real time visualization of the IE anatomy, which allowed us to optimize our electrode choice for CI. In this case, such visualization was valuable, as it indicated the presence of the membranous portions of the IE to evaluate if the modiolus was present, which we believe implied that a directional electrode was the most appropriate choice.

Case Report

A 12-year-old boy with a five year history of bilateral SNHL and X-linked stapes gusher syndrome presented with progressively worsening left-sided hearing loss. He was initially performing well with bilateral traditional hearing aids but developed progressive mixed loss and worsening performance in his left ear. His preoperative audiogram can be seen in Figure 1. He was sent for CI candidacy assessment and was deemed to be suitable.

The pre-operative computed tomography (CT) of the temporal bone was assessed. This showed a bulbous IAC with a dysplastic cochlea and no apparent modiolus (Figure 2). A CI24RE (ST) implant was initially selected by the implant team due to the uncertainty regarding the location of the spiral ganglion cells.

A 1mm cochleostomy was made anteroinferior to the round window (RW). Upon entering the cochlea, a moderate perilymphatic gusher was encountered. A 1.3mm rigid salivary endoscope (Karl Storz, Germany) with a 3-chip full HD camera head (Image1 S H3-Z) was approached trans-tympannically and was placed at the edge of the 1mm cochleostomy to assess the intracochlear anatomy.

To avoid injury to the inner ear, the CSF was not suctioned. The same view was not achieved with the microscope as the endoscope provided a more magnified and higher definition view of the modiolus. There was evidence of membranous structures of the cochlea without direct communication to the IAC. Full insertion was achieved on the first attempt without any difficulty and the gusher was controlled with packing of periosteal tissue at the cochleostomy site. An intra-operative plain X-ray confirmed the electrode placement.

Discussion

When the cochlear modiolus and osseous spiral lamina are deficient, the absence of a bony septum creates a common space seen on the imaging study [10] (Figure 2); there is an abnormal communication between the IAC and the vestibule responsible for the periphymphatic gusher. Patients with IE anomalies may have both atypical positions of their spiral ganglion cells (SGC) and have a higher likelihood of having fewer spiral ganglion cells [11]. This has functional implications because at least 10,000 functional SGNs are necessary for effective speech discrimination 12]. Also this number can be further reduced by surgical trauma [12]. Therefore, choosing the proper electrode device, namely, a directional or a full banded electrode can help reduce the number of lost SGNs. The fully banded CI electrode may be more useful in the absence of a modiolus to allow for full and multi-directional stimulation, whereas the pre-curved directional electrode may be more appropriate if there is a modiolus present for precise stimulation [13].

The proper electrode choice may have important hearing outcome implications for patients with IE anomalies following CI due to the challenges of reaching an optimal level of cochlear stimulation, decreased dynamic range, a wider pulse width, and weakened neural synchrony [7]. The functionality of CI is correlated to the number of SGCs and their distances from the stimulating electrode [14].

In our patient, we assumed that there was no IAC communication due to the presence of modiolus. The moderate CSF gusher was presumed to be secondary to his X-lined stapes gusher syndrome and his enlarged IAC. Perilymph gusher during CI in patients with X-linked gusher syndrome is inevitable, but a thorough examination of the IE is still critical. CT of the temporal bone is important for surgical planning, and it is also useful to assess the likelihood of perilymphatic gusher [4]. Distinguishing features on imaging include an enlarged bulbous IAC, a widened cochlear aperture without the lamina cribrosa, cochlear hypoplasia with modiolar deficiency, and a broadening of the bony canal for the labyrinthine portion of the facial nerve [6]. Enlarged vestibular aqueducts may also appear in conjunction with modiolar deficiency [15].

The rarity of X-linked gusher syndrome may result in radiologists failing to recognize these signs, which may mislead surgeons to perform stapedectomies that are otherwise contraindicated. Thus, Incesulu et al. [3] advocated for high resolution CT of the temperal bone to assess for congenital dysplasia with ¬at least 1-mm thick slices [as opposed to 2mm thick slices], which should ideally be assessed by an experienced neuroradiologist [3]. Quan et al. [16] proposed that CT virtual endoscopy should be done to evaluate CT data through threedimensional reconstruction [16]. Limitations to both modes of assessment assume that the CT images correlate perfectly with the anatomy of the IE and that consistent radiological consultation will occur. Our case, however, demonstrated that intra-operative endoscopic findings do not always correlate with CT findings, indicating that cochlear endoscopy may be a useful tool to better delineate the intracochlear anatomy.

The otoendoscopic intracochlear view gave us accurate and real-time information on the anatomy of the IE, which confirmed the presence of a modiolus and the confidence that the CI would not be in the IAC. This has the potential to allow us to make a better-informed decision regarding the type of electrode to place.

Electrode choice is significant for patients with IE abnormalities, since the location of neural tissue may be abnormal. In patients that have an absent modiolus, a circumferentially stimulating electrode may be preferred over a full-banded electrode, which may risk adverse facial nerve stimulation [3]. One explanation for post-operative facial nerve stimulation in children with IE abnormalities is the close vicinity of the electrode to the nerve [14]. Therefore, to avoid injury, the proper choice in electrode should be made.

Conclusion

While CT temporal bone has served as the conventional approach to assessing the anatomy of the IE, fthe endoscope offers better resolution of the modiolus than the CT temporal bone, as the CT indicated that there was no modiolus. We advocate the use of intracochlear endoscopy in selected cases as it offers a better resolution than even high-resolution CT. With the potential to change electrode choices in CI, the customization of electrode choice based on the presence of membranous IE anatomy may change the hearing outcome of the patients with anomalous IE anatomy and patients with uncertain location of spiral ganglion cells.

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