Bilateral congenital absence of stapes and oval window in 2 members of a family: etiology and management

Yi, Zixiang; Yang, Jiangsun; Li, Zhichune; Zhou, Aidong; Lin, Yefen

doi:10.1016/s0194-5998(03)00086-x

Cited by 18 publications

(21 citation statements)

References 14 publications

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“…These surgeries are not only technically challenging but also show unreliable postoperative hearing outcomes [2][3][4] . In particular, these operations have risks of causing inner ear damage and subsequent permanent sensorineural hearing loss as well as vestibular dysfunction [6] .…”

Section: Discussionmentioning

confidence: 99%

“…Both hearing aids and surgical approaches have been used for treatment, including vestibulotomy [2] , fenestration surgery of the lateral semicircular canal [3] , and malleostapedotomy [4] . However, these surgical treatments are technically difficult, and the risks of inner ear damage and subsequent sensorineural hearing loss are not insignificant [5] .…”

Section: Introductionmentioning

confidence: 99%

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Bonebridge Implantation for Conductive Hearing Loss in a Patient with Oval Window Atresia

Kim

2015

Int Adv Otol

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The occurrence of oval window atresia is a rare anomaly with conductive hearing loss. Traditional atresia surgeries involve challenging surgical techniques with risks of irreversible inner ear damage. Recent reports on Bonebridge (Medel, Innsbruck, Austria), a novel implantable bone conduction hearing aid system, assert that the device is safe and effective for conductive hearing loss. We present a case of Bonebridge implantation in an eight-year-old girl with bilateral oval window atresia.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Bonebridge Implantation for Conductive Hearing Loss in a Patient with Oval Window Atresia

Kim

2015

Int Adv Otol

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“…The stapes superstructure may be present or absent, but there is no footplate or annular ligament, in contrast to congenital otosclerosis [2,10]. Bilateral absence of the oval window has been reported in several cases [5,11,12]. In bilateral cases, the middle ear anomalies may be similar or different.…”

Section: Discussionmentioning

confidence: 99%

“…Lambert [15] described fenestration of the oval window. Although lateral canal fenestration is an old technique, the modified Lempert fenestration of the horizontal semicircular canal has been proposed a good choice as reported in 2 patients [11]. It was suggested that vestibulotomy above a severely displaced facial nerve represents a new surgical approach to achieve serviceable hearing, and that the lack of facial nerve injury and the potential for hearing restoration make this procedure feasible in patients who would otherwise be marginal or poor surgical candidates [16].…”

Section: Discussionmentioning

confidence: 99%

Labyrinthotomy or Vestibulotomy in Anatomic and Congenital Variations of the Oval Window and Facial Nerve

Al-Mazrou

Bayazıt

2012

ORL

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Objective: To present the results of our experience with labyrinthotomy or vestibulotomy in cases where the oval window is blocked by the facial nerve and in the presence of bilateral congenital agenesis of the oval window, respectively. Study design: Retrospective analysis of the records of the patients operated in two different centers. Methods: Between 2007 and 2012, 5 ears of 4 patients who were operated on in two different clinics with a presumptive diagnosis of otosclerosis were included in the study. There were 3 female patients and 1 male. The ages ranged from 10 to 26 (mean 19 years). All patients had unilateral conductive hearing loss except 1 (10-year-old girl or patient 1). Pure tone averages were calculated at the frequencies 0.5, 1, 2 and 4 kHz both pre- and postoperatively according to the Committee on Hearing and Equilibrium 1995 Guidelines for the Evaluation of Results of Treatment of Conductive Hearing Loss. All patients underwent a middle ear exploration and postoperatively the initial audiological examination was performed after 6 months. Results: Retrospective analysis revealed that vestibulotomy or labyrinthotomy was performed in 5 ears of 4 patients. Postoperative dizziness was encountered in 2 patients who had vestibulotomy due to oval window agenesis, which ceased spontaneously at 1 month postoperatively. The perioperative period was otherwise uneventful. None of the patients had sensorineural hearing loss or deterioration of hearing. There was a significant improvement in hearing after the operation. A 28-dB improvement in the mean air conduction pure tone thresholds was achieved. Conclusion: Vestibulotomy and labyrinthotomy are safe and effective procedures in terms of hearing restoration, which can be applied in cases of congenital agenesis of the oval window or obstruction of the oval window by the facial nerve.

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“…The CAS has been described in association with other head and neck anomalies and syndromes or as an isolated event [1, 3, 6, 7]. CAS may also occur in patients with normal auditory canal and tympanic membrane, due to their different embryological origin, presenting only as a long history of conductive hearing loss and identified only after surgical exploration [1, 2, 6, 8]. The otologic surgeon should be able to identify these ossicular chain malformations and to understand the embryologic origin of the different structures of the middle ear in order to safely and effectively treat this condition.…”

Section: Introductionmentioning

confidence: 99%

Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings

Undabeitia

Cianci

Padilla

et al. 2014

Case Reports in Otolaryngology

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Middle ear ossicle malformations are an uncommon event. Among them, the congenital absence of the stapes is a very rare condition that is seldom described in the literature. We report the cases of two women, aged 19 and 22 , who presented with a long history of conductive deafness. An exploratory tympanotomy was performed and the absence of the stapes superstructure and an abnormal position of the facial nerve could be observed. A bone anchored hearing aid (BAHA) was implanted in both patients with good results. It is believed that stapes agenesis is related to an abnormal development of the facial nerve, which by the 5th to 6th week of gestation would interpose between the otic capsule and the stapes blastema, preventing these structures from contacting. A long history of nonprogressive hearing loss from birth or early childhood is the key to reach a diagnosis. Several treatment options have been described. The authors opted for a hearing aid due to the high risk of facial nerve lesion, with good functional results.

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Bilateral congenital absence of stapes and oval window in 2 members of a family: etiology and management

Cited by 18 publications

References 14 publications

Bonebridge Implantation for Conductive Hearing Loss in a Patient with Oval Window Atresia

Bonebridge Implantation for Conductive Hearing Loss in a Patient with Oval Window Atresia

Labyrinthotomy or Vestibulotomy in Anatomic and Congenital Variations of the Oval Window and Facial Nerve

Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings

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