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Year : 2018  |  Volume : 2  |  Issue : 2  |  Page : 27-29

Mondini deformity implanted with minimal incision

1 Department of ENT, Dr. Rajendra Prasad Government Medical College, Kangra, Himachal Pradesh, India
2 Department of Medicine, Dr. Rajendra Prasad Government Medical College, Kangra, Himachal Pradesh, India

Date of Web Publication24-Apr-2019

Correspondence Address:
Dr. Amit Saini
Department of ENT, Dr. Rajendra Prasad Government Medical College, Tanda, Kangra, Himachal Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/aiao.aiao_1_18

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Mondini deformity accounts for majority of cochlear malformations. These children can be given a near-normal lifestyle by performing cochlear implant (CI) surgery. The results of standard incision cochlear implantation (SICI) in these children are very encouraging. However, there are very few centers in world which offer the minimal incision cochlear implantation (MICI) in these patients. We report a case of 2-year-old child suffering from Mondini deformity who underwent CI with MICI. MICI reduces the impact of surgery drastically in these children as it can be performed without shaving of hairs with a 2-cm incision. The complication rate of MICI is comparable to SICI. Apart from better cosmesis, another advantage is early switch on of the device. We are of the opinion that MICI is safe and can be successfully performed in cases of Mondini deformity without any fear.

Keywords: Cochlear implant, hearing loss, high-resolution computed tomography, magnetic resonance imaging, minimal incision cochlear implantation, standard cochlear implantation

How to cite this article:
Saroch M, Saini A, Gargi G. Mondini deformity implanted with minimal incision. Ann Indian Acad Otorhinolaryngol Head Neck Surg 2018;2:27-9

How to cite this URL:
Saroch M, Saini A, Gargi G. Mondini deformity implanted with minimal incision. Ann Indian Acad Otorhinolaryngol Head Neck Surg [serial online] 2018 [cited 2022 Jan 21];2:27-9. Available from: https://www.aiaohns.in/text.asp?2018/2/2/27/256990

  Introduction Top

Mondini dysplasia was first described in Latin by Carlo Mondini, an Italian anatomist (1729–1803) in 1791.[1] Mondini deformity is an anatomical deformity of the cochlea consisting of a triad of a cochlea with one-and-a-half turns, an enlarged vestibule with normal semicircular canals, and an enlarged vestibular aqueduct. Mondini deformity accounts for 50% of cochlear malformations, resulting invariable degrees of otological dysfunction. Patients may experience profound hearing loss (HL) and may not be responsive to hearing aids and other auditory devices.[2] The evaluation and management of children with profound HL and associated inner ear developmental malformations present a significant challenge to even the most experienced clinician. Different types of incisions have been described. There is an incision in C shape, S shape, and J shape[3],[4] with wide dissection of muscle-periosteum and skin flaps. These incision techniques require wide hair shaving, produce more edema in the surgical area, and take longer surgical time, and the healing process may delay the adjustment and activation of the external device. Minimal incision cochlear implant (CI) surgery has been developed with the aims of reducing the impact of surgery to the patient and improving cosmesis while maintaining the low morbidity of conventional wider access approaches.[5],[6]

  Case Report Top

We report a case of cochlear implantation in a 2-year-old female child suffering from Mondini deformity using minimal incision technique. The subject of this case had a history of congenital profound bilateral HL due to cochlear malformations. The HL was identified at 14 months of age via auditory brainstem response testing that indicated no response to click stimuli presented in either ear at 100 dB nHL. The otologic examination and impedance testing indicated normal middle ear function bilaterally. High-resolution computed tomography temporal bone was normal for both the ears. Magnetic resonance imaging inner ear indicated dilated cochlear aqueduct [Figure 1].
Figure 1: Magnetic resonance imaging showing dilated cochlear aqueduct

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Surgical technique

The minimal incision cochlear implantation (MICI) process is described as follows. The incision is marked 10-mm posterior to postauricular sulcus. The superior end of the incision is marked at a point corresponding to the root of the helix, and this is usually just anterior to the hairline, so no shave is necessary. We currently use 20-mm long incision. Markings are made over the skin [Figure 2].
Figure 2: Postauricular marking for incision

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Incision is deepened temporalis fascia graft harvested. C-shaped flap is elevated up to the periosteum and retracted anteriorly, the surgeon shifts to another side of the patient, and subperiosteal flap is elevated with the assistant retracting the skin [Figure 3].
Figure 3: Subperiosteal flap elevated

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Cortical mastoidectomy is completed and posterior tympanotomy done round window is identified, and cochleostomy is done at the anteroinferior end of the round window niche [Figure 4]. Well is drilled for the receiver-stimulator pedestal [Figure 5]. The device is fixed in the well [Figure 6]. The cochlear electrode array is inserted and the cochleostomy is packed with small pieces of the temporalis fascia. The periosteum is then closed as completely as possible [Figure 7]. Intraoperative neural response telemetry and postoperative radiographs are used to confirm appropriate electrode placement if necessary. The use of intravenous antibiotic prophylaxis is recommended to reduce the incidence of infection in the postoperative period.
Figure 4: Cortical mastoidectomy completed

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Figure 5: Well drilled for receiver-stimulator

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Figure 6: Device fixed in well

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Figure 7: Final closure

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  Discussion Top

The patient with cochlear malformation can benefit from cochlear implantation.[7] However, the various degree of deformities may pose problems during surgery. Jackler et al. have proposed a classification system for the congenitally malformed inner ear based on the theory that a variety of the anomalies result from arrested development at different stages of embryogenesis.[8]

In general, inner ear deformities are no longer an absolute contraindication to cochlear implantation. However, the more severe the deformity, the more challenging the surgery. The postoperative results are also less predictable. Therefore, detailed preoperative evaluation is important in order to get a good surgical result without any postoperative complication.[8]

The concept of minimal incision CI was first introduced by O'Donoghue and Nikolopoulos.[6] Shorter incisions and less hair shave are beneficial in the pediatric population because they improve the esthetic quality of the procedure and reduce the psychological trauma of the intervention. A retrospective study showed no evidence that minimal hair shave adversely affects the rates of wound complications.[9],[10] Another advantage of MICI is early mapping of the CI.

In all surgical disciplines, the trend has been for minimally invasive surgery that reduces the trauma related to surgical access. The positive experience of other centers with minimal access CI surgery encouraged the development of the smaller retroauricular incision and device fixation using tie-down ligature, tight subperiosteal pocket, and closure of the periosteum without drilling of the bony recess for the receiver-stimulator.

MICI is as safe as standard cochlear implantation and affords with other benefits. Eliminating the scalp flap avoids devascularization and minimizes the opportunity of flap infection or necrosis.


Written informed consent was obtained from the patient for publication of this case report.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

This manuscript was presented as poster presentation at 14th International Conference on Cochlear Implants and other Implantable Technologies, May 11–14, 2016, Toronto, Canada.

  References Top

Mondini C. Anatomia surdi nati sectio: De bononiensi scientiarum etArtium institute atque academia commentarii. Bononiae 1791;7:419-28.  Back to cited text no. 1
Niparko JK, editor. Cochlear Implants: Principles and Practices. 2nd ed. New York: Lippincott Williams & Wilkins; 2009.  Back to cited text no. 2
Robert K. Cochlear implantation. In: Jackler R, editor. Atlas of Neurotology and Skull Base Surgery. 1st ed., Ch. 25. St Louis: Mosby; 1996. p. 239-43.  Back to cited text no. 3
Roberson JB Jr., Stidham KR, Scott KM, Tonokawa L. Cochlear implantation: Minimal hair removal technique. Otolaryngol Head Neck Surg 2000;122:625-9.  Back to cited text no. 4
Gibson WP, Harrison HC, Prowse C. A new incision for placement of cochlear implants. J Laryngol Otol 1995;109:821-5.  Back to cited text no. 5
O'Donoghue GM, Nikolopoulos TP. Minimal access surgery for pediatric cochlear implantation. Otol Neurotol 2002;23:891-4.  Back to cited text no. 6
Phelps PD. Cochlear implants for congenital deformities. J Laryngol Otol 1992;106:967-70.  Back to cited text no. 7
Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: A classification based on embryogenesis. Laryngoscope 1987;97:2-14.  Back to cited text no. 8
Howard NS, Antonelli PJ. Complications of cochlear implant placement with minimal hair shave. Am J Otolaryngol 2004;25:84-7.  Back to cited text no. 9
Arnold W, Bredberg G, Gstöttner W, Helms J, Hildmann H, Kiratzidis T, et al. Meningitis following cochlear implantation: Pathomechanisms, clinical symptoms, conservative and surgical treatments. ORL J Otorhinolaryngol Relat Spec 2002;64:382-9.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]


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