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

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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Sequential Bilateral Cochlear Implantation in a Child with Severe External, Middle, and Inner Ear Malformations: Surgical Considerations and Practical Aspects

ORL ◽

10.1159/000515179 ◽

2021 ◽

pp. 1-7

Author(s):

Dejun Zhang ◽

Yongyi Yuan ◽

Yu Su ◽

Guojian Wang ◽

Chang Guo ◽

...

Keyword(s):

Facial Nerve ◽

Inner Ear ◽

Middle Ear ◽

Cochlear Implantation ◽

Intraoperative Imaging ◽

Preoperative Imaging ◽

Anatomical Landmarks ◽

Inner Ear Malformations ◽

Facial Nerve Monitoring ◽

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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THE FACIAL NERVE IN CONGENITAL MIDDLE EAR MALFORMATIONS

The Laryngoscope ◽

10.1288/00005537-198108000-00001 ◽

1981 ◽

Vol 91 (8) ◽

pp. 1217???1225 ◽

Cited By ~ 29

Author(s):

ROBERT A. JAHRSDOERFER

Keyword(s):

Facial Nerve ◽

Middle Ear ◽

Ear Malformations

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Salivary gland choristoma of the middle ear: case treated with KTP laser

The Journal of Laryngology & Otology ◽

10.1258/0022215001906066 ◽

2000 ◽

Vol 114 (7) ◽

pp. 528-532 ◽

Cited By ~ 9

Author(s):

Pakpoom Supiyaphun ◽

Kornkiat Snidvongs ◽

Shanob Shuangshoti

Keyword(s):

Salivary Gland ◽

Facial Nerve ◽

Middle Ear ◽

Proximal Part ◽

Ossicular Chain ◽

Branchial Arch ◽

Surgical Removal ◽

Ktp Laser ◽

Surgical Biopsy ◽

Normal Salivary Gland

Salivary gland choristoma of the middle ear is rare. It consists of non-malignant, non-growing, normal salivary gland tissue in the middle ear. It is a developmental abnormality that occurs around the proximal part of the second branchial arch before the fourth month of intrauterine life.The authors found the 25th recorded case in our centre and another 24 reported cases from a review of the literature between 1961 and 1999. Intratympanic salivary gland choristoma frequently occurs during the first and second decades of life and with a female preponderance (56 per cent). Nearly all the patients (96 per cent) in our review presented with a hearing loss, that had begun since birth, in infancy, or during childhood. Tinnitus (28 per cent), and serous otitis media (24 per cent) were also commonly present. One case complained of otorrhoea. Intratympanic and extratympanic anomalies were found in 96.2 per cent and 34.6 per cent of cases respectively. Of these anomalies, ossicular chain (88.5 per cent), facial nerve (65.4 per cent), middle-ear muscles (30.8 per cent) and labyrinthine windows (23 per cent) were the four most common sites. Therefore, salivary gland choristoma may represent a manifestation of a congenital ear anomaly.Diagnosis of salivary gland choristoma is generally not documented pre-operatively, but is based on surgical biopsy and histopathological investigations. Treatment of this rare lesion depends on the size, location and extent of the mass, degree of anatomical abnormality and expertise of the surgeon. In difficult cases where the mass is attached to the dehiscent or inferiorly placed facial nerve, only biopsy is recommended. However, complete surgical removal is advocated for a mass that is easy to remove. KTP laser use via a 200 micron fibreoptic light carrier can facilitate removal especially in cases with ossicular chain involvement.

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Effect of surgical intervention on middle-ear cholesteatoma with associated facial paralysis

The Journal of Laryngology & Otology ◽

10.1017/s0022215116009804 ◽

2016 ◽

Vol 131 (2) ◽

pp. 113-116 ◽

Cited By ~ 2

Author(s):

Y Ozkul ◽

M Songu ◽

K Onal ◽

A Imre ◽

S Arslanoglu ◽

...

Keyword(s):

Facial Nerve ◽

Facial Paralysis ◽

Middle Ear ◽

Limited Area ◽

Presenting Symptoms ◽

Middle Ear Cholesteatoma ◽

Grade Ii ◽

Operative Findings ◽

Grade Iii

AbstractObjectives:To investigate the presenting symptoms, intra-operative findings and long-term facial nerve function in patients treated for cholesteatoma with associated facial paralysis.Methods:Fifteen patients with facial paralysis due to middle-ear cholesteatoma who underwent tympanomastoidectomy surgery from February 2000 to February 2015 were retrospectively reviewed. After removal of the cholesteatoma, a limited area of the fallopian canal, in which facial nerve oedema or redness was evident, was opened. Incision of the epineural sheath for nerve decompression was not performed.Results:Pre-operative House–Brackmann grade was grade II in two patients, grade III in four, grade IV in seven, grade V in one and grade VI in one. Facial nerve perineurium damage was observed in two patients with poor prognoses. All patients treated within the first 15 days after paralysis onset showed normal facial function at long-term follow up. Post-operative House–Brackmann grade was grade I in 11 patients, grade II in 1, grade III in 2 and grade VI in 1.Conclusion:Early surgical treatment is more likely to give good results, and poor outcomes are observed in patients with facial nerve perineurium damage.

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Middle Ear Salivary Choristoma: A Rare Case Report and Update on Congenital Associations, Facial Nerve Involvement, and Treatment Strategies

Case Reports in Otolaryngology ◽

10.1155/2020/8435140 ◽

2020 ◽

Vol 2020 ◽

pp. 1-8

Author(s):

Allen Young ◽

Lauran Evans ◽

Matthew Ng

Keyword(s):

Salivary Gland ◽

Facial Nerve ◽

Middle Ear ◽

Hearing Aids ◽

Treatment Strategies ◽

Branchial Arch ◽

Nerve Involvement ◽

Rare Case Report ◽

Histological Confirmation ◽

Conductive Hearing

Salivary gland choristoma is an extremely rare middle ear mass and is hypothesized to be caused by second branchial arch developmental anomalies. We present a 14-year-old girl with Dandy–Walker syndrome and conductive hearing loss. Middle ear exploration revealed a large middle ear mass with absent incus and stapes and displaced facial nerve. The mass was completely excised with histological confirmation of salivary gland choristoma. Her hearing was improved with bone-anchored hearing aids (BAHA). As facial nerve involvement is common, physicians should consider partial excision to avoid facial nerve palsy. Hearing restoration can be achieved with OCR or BAHA.

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A comparison of high resolution CT scan of temporal bone and operative findings in middle ear cholesteatoma

Journal of the Korean Radiological Society ◽

10.3348/jkrs.1993.29.5.896 ◽

1993 ◽

Vol 29 (5) ◽

pp. 896

Author(s):

Tae Beom Kweon ◽

Hun Seong ◽

Mal Soon Cheon ◽

Hack Jin Kim ◽

Keung Jae Jang ◽

...

Keyword(s):

High Resolution ◽

Ct Scan ◽

Temporal Bone ◽

Middle Ear ◽

High Resolution Ct ◽

Middle Ear Cholesteatoma ◽

Operative Findings

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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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