Salivary gland choristoma of the middle ear

Choristoma is a mass of tissue histologically normal for an organ or part of the body other than the site at which it is located. A rare case of ectopic salivary gland choristoma in the middle ear is described in a 14-year-old girl whose only symptom was a 55 dB conductive hearing loss. The left middle ear mass appeared whitish and was located behind the intact tympanic membrane. We first suspected it to be congenital middle ear cholesteatoma. Exploratory tympanotomy, however, revealed a salivary gland choristoma that adhered tightly to the facial nerve. Differential diagnosis and treatment of this rare condition are discussed.     

Salivary gland choristoma in the middle ear: a case report.

Salivary gland choristoma (ectopic or heterotopic salivary gland tissue) is a rare condition that occurs in various locations within the head and neck regions. We present a 13-year-old boy with a salivary gland choristoma in the middle ear associated with congenital alopecia around the auricle in addition to facial nerve and ossicular chain abnormalities. Because the lesions commonly involve the facial nerve, intraoperative facial nerve monitoring is helpful in allowing safe biopsy without facial nerve damage.     

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

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 fibre-optic light carrier can facilitate removal especially in cases with ossicular chain involvement.     

Huge middle ear adenoma with delayed facial nerve paralysis

Middle ear adenoma is a rare disease that arises from the mucosa of the middle ear. Only a few cases of associated facial nerve paralysis have been reported. Facial nerve involvement is most likely related to nerve compression rather than tumor invasion of the nerve. We describe a case of a huge middle ear adenoma in a 63-year-old man. He presented with a 1-month history of right-sided otalgia, otorrhea, and facial palsy; he also had a 10-year history of right-sided hearing loss. A tympanomastoidectomy was performed. Intraoperatively, the tumor was found to fill the middle ear cavity as well as the entire diameter of the external auditory canal. The tumor had eroded the wall of the facial canal at the second genu, and it was tightly adherent to the epineurium. Focal inflammation around the tumor was observed at the exposed facial nerve. The tumor was removed and the facial nerve was decompressed. Immediately after surgery, the patient's aural symptoms resolved. The final pathology evaluation established the diagnosis of a middle ear adenoma. At the 3-year follow-up, the ear cavity was completely healed and facial nerve function was improved.     

A bifid intra-tympanic facial nerve in association with a normal stapes

A rare facial nerve anomaly was incidentally discovered whilst performing a tympanoplasty and ossicular reconstruction on a patient with an acquired unilateral conductive hearing loss. The nerve was seen to bifurcate and straddle a normal stapes superstructure as it ran posteriorly through the middle ear, a unique and as yet unreported combination. This case highlights the importance of vigilance regarding facial nerve anatomical variations encountered during middle-ear surgery thus avoiding inadvertent damage. The purported embryological mechanism responsible for such anomalies of the intra-tympanic facial nerve is discussed.     

Middle ear adenoma diagnosed by recurrent facial paralysis

Middle ear adenoma is a rare disease derived from the middle ear mucosa. It is usually lack specific findings and easily mistaken for other conditions, delaying a correct diagnosis. In particular, few cases with facial nerve paralysis have been reported. We describe a case of middle ear adenoma that caused hearing loss and recurrent facial paralysis in a 29-year-old woman. In an attempt to treat the facial nerve paralysis, we performed tympanomastoidectomy and facial nerve decompression. By pathological examination, we finally diagnosed it middle ear adenoma with neuroendocrine differentiation. Retrospectively, if we meet the antibiotic resistant mass in the middle ear, we should suspect the tumor. One year after surgery, the adenoma has not recurred but long-term observation is required.     

Large middle ear schwannoma of the Jacobson's nerve with intracranial extension

The patient is a 64-year-old woman who developed a sensation of right ear fullness and hearing loss in early November 2010. Physical examination revealed a painless reddish granular lesion filling in the right external auditory canal. Her right ear was deaf, and no facial palsy was noted. Computed tomography, magnetic resonance imaging and positron emission tomography revealed a middle ear mass extending to the external auditory canal with intracranial invasion causing temporal lobe retraction and inferiorly extending just anterior to the jugular bulb as well. A combination of transmastoid and middle cranial fossa approach along with anterior rerouting of the facial nerve was employed for a near-total removal of the tumor. Based upon the operative findings, it was deemed that the tumor could have arisen from the Jacobson's nerve.     

Primary middle ear schwannoma

Schwannomas of the middle ear may originate from the nerves of the middle ear cavity or by extensions from neighboring structures. We present a case of a 51-year-old female patient with primary middle ear schwannoma believed to arise from Jacobson's nerve. The tumor was easily divided from the facial nerve and the chorda tympani nerve. Erosion of the promontory was noted, and the jugular foramen and posterior wall of the ear canal were preserved without destruction. The mass was successfully removed by a canal down mastoidectomy procedure, while preserving the hearing and facial nerve functions of the patient. To our knowledge, this is only the second reported case of a patient with Jacobson's nerve schwannoma.     

Salivary gland choristoma of the middle ear: A case report and review of the literature

Salivary gland choristomata are heterotopic rests which have rarely been reported in the middle ear. A case report of a salivary gland choristoma of the middle ear is presented and the literature reviewed. The frequent association of ossicular chain and facial nerve anomalies is emphasized.     

Salivary gland choristoma (hamartoma) of the middle ear: a case report

OBJECTIVE: Middle ear salivary gland choristoma are extremly rare. We report a case, describe the clinical management and review the literature. CLINICAL CASE: A 12 year old boy presented with unilateral conductive hearing loss associated with a large inferior retraction pocket on otoscopy. CT scan demonstrated a large mass in the left middle ear cavity. The incus was absent and the stapes was partially eroded. Middle ear exploration demonstrated an 8 mm yellow/red mass in the region of the fallopian canal. This mass was comptly removed and histopathology confirmed salivary gland choristoma. CONCLUSION: These lesions result from an abnormal development of the second branchial arch. It is important to consider these lesions as part of the differential diagnosis for any unilateral hearing loss associated with a middle ear mass in children.     

Preservation of conductive hearing in approaches to tumors of the jugular foramen.

OBJECTIVE: Surgical approaches to the jugular foramen, most often used for the resection of glomus jugulare tumors, may include removal of the external auditory canal wall and overclosure of the meatus, resulting in maximal conductive hearing loss. Modifications have been described that maintain hearing by preserving the canal wall at the price of decreased exposure and are, therefore, suitable only for small and favorably located tumors. Our technique for removal and then reconstruction of the canal wall with hydroxyapatite cement allows for complete anterior translocation of the facial nerve as far proximal as the geniculate ganglion, giving uncompromising exposure of even the most extensive tumors, with the potential for preservation of normal hearing. The purpose of this study was to describe and report our experience with this technique. STUDY DESIGN:: Retrospective review. SETTING: Private otology practice. PATIENTS: Between 2000 and 2005, seven patients between the ages of 34 and 77 years were identified who underwent procedures using this technique. INTERVENTION: Surgical management of jugular foramen tumors. MAIN OUTCOME MEASURES: Successful anatomical reconstruction of the external auditory canal and middle ear. Preoperative and postoperative audiograms are compared, and facial nerve function is reported. Complications are discussed. RESULTS: All seven patients had successful reconstruction of the external auditory canal. Complications were minor and did not require additional surgical intervention. Postoperative pure-tone average differed from the preoperative average by a mean of 7.5 dB. Facial nerve function ranged from House-Brackmann Grades I to III when checked at least 7 months after surgery. CONCLUSION: This study reveals that this technique of external auditory canal reconstruction using hydroxyapatite cement allows complete anterior translocation of the facial nerve, while safely and reliably preserving the potential for normal hearing, without any compromise in exposure of the jugular foramen in the setting of an infratemporal fossa approach.     

Acute myelogenous leukemia presenting as atypical mastoiditis with facial paralysis

Symptomatic otologic involvement by leukemic infiltration is unusual, most often occurring in the already-diagnosed leukemic patient as postauricular mass, acute hemorrhagic otitis media, mastoiditis, cranial neuropathy, vertigo, hearing loss, or leptomeningitis. We think ours is the first reported patient whose leukemia presented as atypical mastoiditis and facial paralysis due to granulocytic sarcoma (chloroma). At mastoidectomy, tan lobulated rubbery tumor filled the mastoid antrum and middle ear cleft. Complete remission and full return of facial nerve function was achieved with 2500 R local radiation and systemic chemotherapy. We discuss the role and extent of surgery in atypical acute mastoiditis when unsuspected middle ear and mastoid tumor, with inconclusive intra-operative histopathologic data, is found.     

Salivary gland choristoma of the middle ear in a child with situs inversus totalis

Salivary gland choristoma of the middle ear cavity is an extremely rare entity. It is thought to be a developmental abnormality and may be associated with abnormalities of adjacent structures. We report a case of salivary gland choristoma of the middle ear with prominent Körner's septum in a 7-year-old girl with situs inversus totalis. Situs inversus totalis is the mirror image reversal of the normal position of the internal organs and frequently associated with other congenital anomalies. This patient is the first reported case with situs inversus totalis, having middle ear salivary gland choristoma and prominent bilateral Körner's septae. The management and the differential diagnosis of this condition are discussed with the review of the literature.     

Cryosurgical treatment of glomus juglare tumor

Summary A 35 year-old Japanese female complained of a right-sided pulsation tinnitis, hearing disturbance, and facial weakness. Extensive radiographic studies including angiograms and retrograde juglar venography provided a diagnosis and localization of a tumor. Radical mastoidectomy was performed and a red grape-like glomus juglare tumor along the facial nerve was extirpated as there was a profuse hemorrhage from the tumor mass.Cryosurgery was then performed. Complete surgical removal is possible when the tumor is small, however, when the tumor involves the middle ear and mastoid area, complete extirpation cannot always be done. Radical mastoidectomy plus cryosurgery appears to be the most feasible management in the surgical treatment of glomus juglare tumor.     

Cholesteatoma invasion into the internal auditory canal

Cholesteatoma invasion into the internal auditory canal (IAC) is rare and usually results in irreversible, complete hearing loss and facial paralysis on the affected side. This retrospective study examines the clinical characteristics of seven patients with cholesteatoma invading the IAC, analyzes possible routes of the cholesteatoma’s extension and describes the surgical approaches used and patient outcome. Extension to the IAC was via the supralabyrinthine route in most patients. A subtotal petrosectomy, a translabyrinthine approach or a middle cranial fossa approach combined with radical mastoidectomy were required for the complete removal of the cholesteatoma. All seven patients presented with some preoperative facial nerve palsy. The facial nerve was decompressed in four patients and facial nerve repair was performed in three others, two by hypoglossal-facial anastomosis and one by a greater auricular nerve interposition grafting. All patients ended up with total deafness in the operate ear. At 1 year following surgery, the facial nerve function was House–Brackmann grade III in six cases and grade II in one. In conclusion, cholesteatoma invading the IAC is a separate entity with characteristic clinical presentations, require a unique surgical approach, and result in significant morbidity, such as total deafness in the operated ear and impaired facial movement.     

Cryosurgical treatment of glomus juglare tumor.

A 35 year-old Japanese female complained of a right-sided pulsation tinnitis, hearing disturbance, and facial weakness. Extensive radiographic studies including angiograms and retrograde juglar venography provided a diagnosis and localization of a tumor. Radical mastoidectomy was performed and a red grape-like glomus juglare tumor along the facial nerve was extirpated as there was a profuse hemorrhage from the tumor mass. Cryosurgery was then performed. Complete surgical removal is possible when the tumor is small, however, when the tumor involves the middle ear and mastoid area, complete extirpation cannot always be done. Radical mastoidectomy plus cryosurgery appears to be the most feasible management in the surgical treatment of glomus juglare tumor.     

耳畸形手术的面神经防护(519例手术回顾分析)

为总结耳畸形手术中面神经防护的经验，分析了１９８３年８月～１９９５年７月底５１９例耳各类耳畸形手术的临床资料。单纯中耳畸形１３０例中，面神经畸形率为６０．８％（７９／１３０），鼓索神经畸形率为５．４％（７／１３０）；外耳道狭窄伴中耳畸形７５例中，面神经畸形率为６０．０％（４５／７５），鼓索神经畸形率为１３．３％（１０／７５）；外耳道骨性闭锁伴中耳畸形３１４例中，面神经畸形率为４２．５％（１４２／３１４），鼓索神经畸形率为１００．０％（３１４／３１４）。４２８例（８２．５％）术后听力增进，其中２２１例（４２．６％）获实用听力（３０～４５ｄＢ）。远期随访１９８例，其中１５２例（７６．７％）仍保持近期听力水平。术后近期面瘫２例（０．４％）。耳畸形术中面神经保护的关键是尽早辨认面神经，采用高分辨率ＣＴ颞骨扫描，熟悉颞骨解剖标志，必要时应行面肌电图监测     

Salivary gland choristoma of the middle ear: a case report

A choristoma is a nonneoplastic proliferation of histologically normal tissue that forms at an abnormal site. It is extremely uncommon in the middle ear space. It appears to be a developmental abnormality and may be associated with abnormalities of adjacent structures. It usually occurs with unilateral conductive hearing loss and requires a differential diagnosis from other mass lesions in the middle ear cavity. This article discusses a case of salivary gland choristoma of the middle ear that we believe to be the 24th case reported on this subject.     

耳畸形手术的面神经防护（519例手术回顾分析）

为总结耳畸形手术中面神经防护的经验，分析了１９８３年８月￣１９９５年７月底５１９例耳各类畸形手术的临床资料。单纯中耳畸形１３０例中，面神经畸形率为６０．８％（７９／１３０），鼓索神经畸形率为５．４％（７／１３０）；外耳道狭窄伴中耳畸形７５例中，面神经畸形率为６０．０％（４５／７５），鼓索神经畸形率为１３．３％（１０／７５）；外耳道骨性闭锁伴中耳畸形３１４例，面神经畸形率为４２．５％（１４２／３１４     

Salivary gland choristoma of the middle ear. A review

While head and neck surgeons are accustomed to recognizing malignancies in their practices, developmental lesions are much less often encountered, and as such are not as likely to be included amongst other (more aggressive) differential diagnostic considerations. One such developmental lesion is the choristoma, defined as an architecturally normal arrangement of mature tissues found in a location not normally host to such tissues. Choristomas composed of recognizable salivary gland tissue may be found in a variety of locations including the middle ear. Middle ear salivary choristomas are distinctly unusual lesions and typically manifest as unilateral conductive hearing loss; while both adult and pediatric patients have been diagnosed with middle ear choristomas, most patients have been in the first two decades of life. As these are benign, nonprogressive lesions, treatment has been directed toward complete surgical excision whenever feasible, and biopsy for diagnosis with subsequent observation in the case of those lesions which are difficult to remove without endangering the facial nerve. There have been rare instances in which choristomas have been linked to the subsequent development of neoplasms, but in the main choristomas are regarded as self-limited developmental heterotopias.