THE FACIAL NERVE IN CONGENITAL MIDDLE EAR MALFORMATIONS

Cochlear implantation (CI) is a safe and beneficial surgery for children with congenital inner ear malformations, with the exception of cochlear nerve aplasia. The combination of microtia with middle and inner ear abnormalities is extremely uncommon and sufficiently severe to make a surgical approach to the cochlea difficult. We report herein the case of a 2-year-old girl who presented with profound bilateral sensorineural hearing loss, congenital aural atresia, microtia, and inner ear malformations. High-resolution computed tomography revealed poor development of the bilateral middle ear spaces, absence of the incus and stapes, aberrant courses of facial nerves, aplastic lateral semicircular canals, and covered round windows. With intraoperative imaging assistance, sequential bilateral CI was performed using a transmastoid approach with no complication. We propose that CI is feasible in patients with severe external and middle ear malformations. However, major malformations increase the risk of complications. As the facial nerve and cochlea are difficult to locate due to the lack of important anatomical landmarks, detailed planning and adequate preparation, including review of the preoperative imaging data, and the use of facial nerve monitoring and intraoperative imaging are very important. In addition, experienced surgeons should perform CI to ensure the success of the operation.

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Vitamin A-Induced Ear Malformations in Rats as a Model for Analysis of Atresia Auris Congenita a Microsurgical and Histological Study

Annals of Otology Rhinology & Laryngology ◽

10.1177/00034894810900s201 ◽

1981 ◽

Vol 90 (2_suppl) ◽

pp. 3-12 ◽

Cited By ~ 5

Author(s):

Manfred Weidenbecher

Keyword(s):

Vitamin A ◽

Facial Nerve ◽

Middle Ear ◽

Histological Study ◽

Branchial Arch ◽

Functional Disturbance ◽

Ear Malformations ◽

Operative Findings ◽

Atresia Auris Congenita ◽

Atresia Auris

Teratogenic damage was induced in pregnant Wistar rats with an overdose of vitamin A to clarify the genesis of the atresia plate, the behavior of the facial nerve, and the rotation of the middle ear. Results suggested that contradictory statements in the literature concerning the atresia plate could be explained in the following way: the plate does not arise from a single branchial arch. The os tympanicum, the pars squamosa of the temporal bone, and the hyperplastic labyrinthine capsule share in its formation. Findings similar to the human atresia auris congenita, such as malformation of the auricle, atresia of the external ear canal, and changes in the ossicles and lumen of the middle ear space, were obtained. A cranial and labyrinthine dysmorphia leads to rotation of the middle ear and a walling-in of the facial nerve, which explains its functional disturbance. Extreme variations in the course of the facial nerve were not found. In 62 cases of severe human ear malformation, the operative findings corresponded to the experimentally produced ear malformations.

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Otopathologic Abnormalities in CHARGE Syndrome

Otolaryngology ◽

10.1177/01945998211008911 ◽

2021 ◽

pp. 019459982110089

Author(s):

Rafael da Costa Monsanto ◽

Renata Malimpensa Knoll ◽

Norma de Oliveira Penido ◽

Grace Song ◽

Felipe Santos ◽

...

Keyword(s):

Facial Nerve ◽

Middle Ear ◽

Spiral Ganglion ◽

Internal Auditory Canal ◽

Semicircular Canals ◽

Charge Syndrome ◽

University Of Minnesota ◽

Bony Canal ◽

Ganglion Neurons ◽

Temporal Bones

Objective To perform an otopathologic analysis of temporal bones (TBs) with CHARGE syndrome. Study Design Otopathologic study of human TB specimens. Setting Otopathology laboratories. Methods From the otopathology laboratories at the University of Minnesota and Massachusetts Eye and Ear Infirmary, we selected TBs from donors with CHARGE syndrome. These TBs were serially sectioned at a thickness of 20 µm, and every 10th section was stained with hematoxylin and eosin. We performed otopathologic analyses of the external ear, middle ear (middle ear cleft, mucosal lining, ossicles, mastoid, and facial nerve), and inner ear (cochlea, vestibule, internal auditory canal, and cochlear and vestibular nerves). The gathered data were statistically analyzed. Results Our study included 12 TBs from 6 donors. We found a high prevalence of abnormalities affecting the ears. The most frequent findings were stapes malformation (100%), aberrant course of the facial nerve (100%) with narrow facial recess (50%), sclerotic and hypodeveloped mastoids (50%), cochlear (100%) and vestibular (83.3%) hypoplasia with aplasia of the semicircular canals, hypoplasia and aplasia of the cochlear (66.6%) and vestibular (91.6%) nerves, and narrowing of the bony canal of the cochlear nerve (66.6%). The number of spiral ganglion and Scarpa’s ganglion neurons were decreased in all specimens (versus normative data). Conclusions In our study, CHARGE syndrome was associated with multiple TB abnormalities that may severely affect audiovestibular function and rehabilitation.

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Tomography in Inner and Middle Ear Malformations

Radiology ◽

10.1148/92.1.11 ◽

1969 ◽

Vol 92 (1) ◽

pp. 11-20 ◽

Cited By ~ 15

Author(s):

K. Reisner

Keyword(s):

Middle Ear ◽

Ear Malformations

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The Facial Nerve in Congenital Ear Malformations

The Facial Nerve ◽

10.1007/978-3-642-85090-5_107 ◽

1994 ◽

pp. 299-301

Author(s):

R. A. Jahrsdoerfer ◽

A. M. Gillenwater

Keyword(s):

Facial Nerve ◽

Ear Malformations

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Using Tympanometry to Evaluate Middle Ear Function in Children and Adolescents with Signs of Peripheral Facial Nerve Palsy

Journal of Otology & Rhinology ◽

10.4172/2324-8785.1000318 ◽

2017 ◽

Vol 06 (03) ◽

Author(s):

Kopala W ◽

Kukwa A

Keyword(s):

Facial Nerve ◽

Children And Adolescents ◽

Middle Ear ◽

Nerve Palsy ◽

Facial Nerve Palsy ◽

Peripheral Facial Nerve Palsy

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Hemifacial spasm secondary to middle ear cholesteatoma

Ear Nose & Throat Journal ◽

10.1177/014556131809700602 ◽

2018 ◽

Vol 97 (6) ◽

pp. E31-E32

Author(s):

Maheep Sohal ◽

Nicholas Karter ◽

Marc Eisen

Keyword(s):

Facial Nerve ◽

Middle Ear ◽

Hemifacial Spasm ◽

Final Diagnosis ◽

Computed Tomographic Angiography ◽

Vascular Compression ◽

Computed Tomographic ◽

Middle Ear Cholesteatoma ◽

Facial Spasm ◽

Tomographic Angiography

Hemifacial spasm is a peripheral myoclonus of the VIIth cranial nerve that is characterized by paroxysmal contraction of the muscles of facial expression. It exists in both primary and secondary forms. In rare cases, hemifacial spasm is caused by middle ear pathology. We describe the case of a 90-year-old man with recurrent cholesteatoma and tympanic segment fallopian canal dehiscence manifesting as right-sided hemifacial spasm. His history was significant for a right-sided tympanomastoidectomy for cholesteatoma 6 years earlier. Computed tomographic angiography performed to look for vascular compression of the facial nerve demonstrated a right middle ear opacification. Middle ear exploration revealed a completely dehiscent tympanic segment with cholesteatoma abutting the facial nerve. The overlying keratin debris and matrix were carefully dissected off, and facial nerve function was preserved. The final diagnosis was hemifacial spasm. During 14 months of postoperative follow-up, the patient experienced no further facial spasm.

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High-resolution computed tomography evaluation of congenital aural atresia – how useful is this?

The Journal of Laryngology & Otology ◽

10.1017/s002221512000136x ◽

2020 ◽

Vol 134 (7) ◽

pp. 610-622

Author(s):

R Gautam ◽

J Kumar ◽

G S Pradhan ◽

J C Passey ◽

R Meher ◽

...

Keyword(s):

Computed Tomography ◽

High Resolution ◽

Facial Nerve ◽

External Auditory Canal ◽

Test Results ◽

High Resolution Computed Tomography ◽

Aural Atresia ◽

Ear Malformations ◽

Congenital Aural Atresia ◽

Bone Abnormalities

AbstractObjectiveTo depict various temporal bone abnormalities on high-resolution computed tomography in congenital aural atresia patients, and correlate these findings with auditory function test results and microtia subgroup.MethodsForty patients (56 ears) with congenital malformation of the auricle and/or external auditory canal were evaluated. Auricles were graded according to Marx's classification, divided into subgroups of minor (grades I and II) and major (III and IV) microtia. Other associated anomalies of the external auditory canal, tympanic cavity, ossicular status, oval and round windows, facial nerve, and inner ear were evaluated.ResultsMinor and major microtia were observed in 53.6 and 46.4 per cent of ears respectively. Mean hearing levels were 62.47 and 62.37 dB respectively (p = 0.98). The malleus was the most commonly dysplastic ossicle (73.3 vs 80.8 per cent of ears respectively, p = 0.53). Facial nerve (mastoid segment) abnormalities were associated (p = 0.04) with microtia subgroup (80 vs 100 per cent in minor vs major subgroups).ConclusionMicrotia grade was not significantly associated with mean hearing levels or other ear malformations, except for external auditory canal and facial nerve (mastoid segment) anomalies. High-resolution computed tomography is essential in congenital aural atresia, before management strategy is decided.

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